Partial dysgenesis of the corn body. Causes of the Agnesia of the Core Body: What you need to know before pregnancy

Clinical analysis

Agnesia of the Core Body, associated with hereditary syndromes

O.A. Milovanova12, T.Y. Tarakanova1, Yu.B. Pefaeva1, L.P. Katasonova2, S.Kh. Beach Ool2, T.E. Vorozhiyev2.

FGBOU DPO "Russian Medical Academy of Continuing Vocational Education" Ministry of Health of Russia, Moscow, Russia; 2GBUZ Children's urban clinical hospital. BEHIND. Bashleva Health Department of the Government of Moscow, Moscow, Russia

The Agnesia of the Colebone Body (AMT) is found in cerebral digesions associated with various hereditary syndromes. It is traditionally divided into total (there are no commissioned fibers) and partial (the Agnesia of the Rostral and Caudal Departments of the Core Body). AMT may occur in an isolated or combined with other brain defects. Insulated violations of the corpulent body clinically may not manifest itself, which makes it difficult to make timely diagnosis of this pathology. The presence of AMT can be confirmed by data from various methods of neurovalization, including a prenatal ultrasonic studies of the brain. This article presents two own clinical observations of patients with AMT associated with hereditary syndromes. In one case, there was a relatively favorable course of the disease, in the other, a severe infant form with a fatal outcome is described, with the presentation of detailed data of autopsy and morphological studies of the brain. Particular attention is paid to the analysis of clinical phenotypes, a lifetime and post-relief diagnosis of the disease.

Keywords: Amediesia, corn body, clinical manifestation, hereditary syndromes. DOI: 10.18454 / ACEN.2017.2.9

Agenesis of the Corpus Callosum Associated with Hereditary Syndromes

OL "GA A. Milovanova12, Tat" Yana Yu. Tarakanova1, Yuliya B. Pronicheva1, Lyubov "P. Katasonova2, Salbakay Kh. Biche-Ool2, Tat" Yana E. VoroZHBIEVA2

1Russian Medical Academy Of Continuous Professional Education, Ministry of Healthcare Of The Russian Federation, Moscow, RUSSIA

2Tushino Children "S City Hospital, Moscow, Russia

Agenesis of the Corpus Callosum (ACC) Is Detected in Patients with Cerebral Dysgenesis Associated with Various Hereditary Syndromes. IT IS Conventionally Subdivided ICC The Corpus Callosum (Agenesis of the Corpus Callosum) ACC. The Disorder Can Either BE INDIVIDUAL OR Associated with Other Developmental Brain MalFormations. Isolated Pathologies of the Corpus Callosum Can Be Clinically Occult, Thus Significantly Impeding Diagnosis of this Pathology. Aac Can Be Verified Using Various Neuroimaging Data, Including Fetal Brain Ultrasonography. In This Study, We Report Two Cases Ofpatients with ACC Associated with Hereditary Syndromes from Our Own Clinical Experience. In One Case, The Course of The Disease Was RelativeLy Favorable. The Severe Infantile Form with Fatal Outcome IS Reported in the Second Case. The Detailed AutoPsy Data and Results of Morphological Examination of The Brain Are Presented. Special Attention IS PAID TO THE ISSUES ASSOCIATED WITH ANALYSIS OF CLINICAL PHENOTYPES, AS WELL AS LIFETIME AND POSTMORTEM DIAGNOSIS OF THE DISEASE.

Keywords: Agenesis, Corpus Callosum, Clinical Manifestation, Hereditary Syndromes. DOI: 10.18454 / ACEN.2017.2.9

Introduction

COLOR BODY (MT) is the largest commissophole of the brain. The Agnesia of the Core Body (AMT) is a well-known cerebral development anomaly - it provides an absence of a compound between two brain hemispheres. Currently verifying Total AMT (there are no commissioned fibers) and partial AMT (Agnesia of the Rostral and Caudal Departments of MT). In modern medical literature, partial AMT is often called Disence MT, but it is correct to use the term "partial agentsia MT".

Due to the lack of reliable information on the prevalence of AMT, it is rather difficult to establish the true frequency of occurrence of hereditary syndromes (NA), accompanied by the formation of AMT. Diagnosis is hampered by the nonspecification of clinical symptoms in the early stages of the disease and the presence of atypical forms of the disease. AMT frequency is 0.3-0.7% in the overall population and 2-3% among people with disabilities with mental retardation. AMT may occur with NA with autosomal-pre-obstructive, autosomal-recessive or x-clutch types of inheritance. S. Schell-Apacik et al. AMT has been described in 29% of patients with a genetic pathology installed. There is a wide variety of NA,

Clinical parse

Agnesia of the corpulent body

associated with AMT, including forms with point mutations in rare genes, complex cytogenetic syndromes, mitochondrial diseases. AMT is described in hereditary metabolic diseases, genton disease and other hereditary syndromes.

Most ns associated with AMT are multisystems. Neurological manifestations in patients with AMT are due to predominantly accompanying cerebral pathology, cases of isolated pathology practically asymptomatic. In cases of Combined AMT defeat, motor disorders were found in approximately 35-40%. According to S. Santo, the retreat of psychomotor development in young children with AMT is about 25-30%. Among the parks-censal neurological manifestations in children of the first year of life are dominated by infant cramps. M. Bedeschi et al. Investigated 63 cases of AMT in combination with neurological disorders (mental retardation of varying degrees and epilepsy), among which, 33% of patients were confirmed by the NA.

CT / MRI signs of AMT include: the presence of inter-stroke cysts, shifting up extended ventricular III and specific changes in the form of side ventricular bodies - the so-called symptom of "grasp". Prenatal-Naya MRI most reliably confirms the presence of an amt fetus, postnatal MRI has an advantage in the differentiation of related congenital cerebral developmental anomalies.

Specific treatment of AMT is absent. If there are epileptic attacks in patients, the correction of convulsive activity does not differ from those in patients with epilepsy without AMT.

Forecast of AMT depends on the presence or absence of related cerebral pathology and related malformations. In countries in which legislation allows the abortion of pregnancy after the 20th week of gestation, the forecast of neurological outcome in patients with AMT can play a decisive role in solving the issue of continuing or interrupting pregnancy.

Due to the complexity of the diagnosis of combined MT defeat, it seems appropriate to submit a description of two patients under our observation.

Clinical descriptions

Patient I., 3 years 8 months, is observed with a delay in mental, speech and motor development.

Anamnesis of life and illness. The boy was born from the 3rd pregnancy that occurred with toxicosis in the first trimester, ARVI in the II trimester, the delay of intrauterine development in the third trimester. Mother's birth is 2nd urgent, at the 36th week of the gestation. Score from apgar - 7/7 points, body weight at birth - 2050 g, length - 47 cm, head -34.0 cm circle. In the period of early adaptation, the condition of the child is closer to the heavy degree cerebral ischemia (syndrome oppression of CNS), respiratory failure. As a state of health, the child has been transferred to the intensive care unit and intensive therapy (orit),

where he was during the first week of life, further translated into the department for newborns (1st month of life), received neurometabolic and vascular therapy in the age dosages, was discharged home with an improvement. In the first year of life, the boy developed with the delay in the motor, mental and speech development of the average degree. Received regenerative treatment courses (Pan-Toga - 2 g / day, Gammalon - 2 g / day), a common massage, LFC, therapy for the method of Loop, physiotherapeutic measures (ozocenite applications, etc.).

Objectively: skin cleanings are clean, low powering boy. Skull shape hydrocephalic. Teeth: 8/8. Deeply planted eyes, hoppy ears, short neck, walled folds on the neck. In the light breath of Pu-eryl, there are no wheezes. Clear heart tones, rhythm correct, systolic noise is listening over the entire heart area. The belly is soft, painless. Liver, spleen is not enlarged. Exterior genitals are formed on male type.

Neurological status. In consciousness, eye gaps are equal, the pupils of a rounded form of medium size, equal, alternating cut-in strains, photoreacts are alive, the face is symmetrically, there is no bulbar disorders. Diffuse muscular hypotension. Tendor reflexes are symmetrical, medium stress. Motor skills: sits independently, crawling on all fours, walks independently with support for one hand. Evaluation of global motor functions on the R. Palisano scale (GMFCS): 1st level. Higher Brain Functions: A child understands an understanding of simple instructions and situational issues. Violated perception, interpretation and logical sequence of plot pictures; Violated regular and dynamic components of game activity. In the speech area there is no understanding of the deployed statements, complex grammatical structures. Own speech is represented by separate simple words, the phrase is not formed. Evaluation of psychoretical development on scale I.A. Skvortsova - 70 points, which corresponds to the average degree of cognitive disorders.

Data of instrumental and laboratory survey methods

Neurosonography: In the coronary plane, the widespread front horns of the side ventricles are determined, the outer edge is naughty, the cavity of the transparent partition is not visualized. In the sagittal plane: the corn body is not visualized, the fan-shaped tendering of the furrow is noted. CONCLUSION: Total Age-Nezhenous Colebone Body.

Echocardiography: congenital heart disease (interventricular septum defect).

Ultrasound of the internal organs: form anomaly and an increase in the size of the gallbladder. Rotation of the right kidney.

Consultation of the eyepiece: Myopic astigmatism, the defeat of visual conducting tract on both sides.

EEG in a state of wakefulness in dynamics: (at the age of 10 months and 3 years): against the background of the delay in the formation of cortical rhythms of typical epileptiform activity is not registered.

Radiography of knee joints, brushes (1 year 11 months): osteoporosis. Bone age 12 months

Cytogenetic examination (Medical and Genetic Scientific Center): Kariotype 46XYDUB (8) (P23.1P21.3). Conclusion: chromosomal syndrome, partial trisomy 8p.

Clinical diagnosis: chromosomal disease (partial trisomy 8p). Congenital brain definition videos: Total corpulent body agenesia. Child cerebral palsy: an ashistatic form. Motor disorders on the GMFCS 1 level scale. General underdevelopment of speech I-II degree. Defect of the interventricular partition.

Patient E., 35th day of life.

History of life and illness: The girl was born from the first pregnancy that took place in the first trimester against the background of the threat of interrupts, in the third trimester was revealed by the delay of intrauterine development. Birth 1st premature, on the 34-35 weeks of pregnancy in the pelvic premium; Score from apgar 5/5 points, body weight at birth - 1570 g, length - 42 cm, head circle - 33 cm. When birth, the child's condition is regarded as extremely heavy (cerebral ischemia II-III degree, sinders of depression of CNS, respiratory failure II-III degree, primary immunodeficiency). As a state of health on the 3rd day of life, the child is translated into the orort, where it was connected to the device of artificial ventilation of the lungs (IVL).

Objectively: The condition is heavy, the girl is connected to the IVL device in BIPAP mode. Power probe. Fenofo-typical features: full double-sided cleaner of the upper lip and solid sky, the wings of the nose are deformed, the nasal stroke and the cartilaginous plate on the right are not formed, low-ranned ear sinks, from two sides of the goat and antiques are practically not formed. Pale color skin with grayish tint, visible mucous pure, wet, pale pink, tongue is covered with a white bloom. The marble of limbs, torso, distal acricyanosis, the tones of the heart are muted, the rhythm is correct, the frequency of heart rate is 120-140 per minute. In the lungs, breathing is weakened, is carried out in all departments. The belly is moderately increased in size, palpation is hardly available. Liver: enlarged in size, dense consistency, the lower edge protrudes from under the rib arc by 3 cm. The spleen: not increased in size.

Neurological status: The level of consciousness is a drug sedation. No meningeal symptoms, eye-line eyeballs, photoreacts are sluggish, reduced spontaneous motor activity, diffuse muscle hypotension, tendon and periosteal reflexes are caused by difficulty. Unconditional reflexes of the non-greeting period are not caused. Higher brain functions in the severity of the state did not seem to be possible.

The deterioration of the child's condition occurred on the 31st day of life. There was an increase in the signs of respiratory failure (acricyanosis), a decrease in the saturation of hemoglobin oxygen to 81%, the development of edema (ascites) and intoxication (fever, microcirculation violation) of syndromes, bradycardia appeared.

Sowing from the oz on the microflora (13th day of life): Klebsiela Pneumoniae 106, Acinetobacter Aumanii L06 is revealed - poly-resistant. Sowing blood: the yeast mushrooms of the genus Candida are highlighted.

Radiography of the chest organs (in dynamics): signs of natural pneumonia in the upper share of the easiest right.

Neurosonography: Partial AMT, perivativericular swelling.

Cytogenetic study: 46xx, Del (7) (Q32): Terminal deletion of the long shoulder of the 7th chromosome.

Common blood test: the number of leukocytes has decreased from the original 21x109 / l to 7.8x109 / l to the 31st day (norm 6.5-13.8x109 / l), platelets - from 129 ^ 109 / l to 83x109 / l (norm Sh-400x109 / l).

In biochemical analysis of blood: the level of C-reactive protein rose to 20 mg, hypoproteinemia with a decrease in the number of albumin.

General urine analysis: Candida's binding yeast mushrooms are revealed.

Clinical diagnosis: congenital generalized infection of bacterial-fungal etiology. The focal drain bilateral pneumonia of purulent-fungal etiology. Partial agentsia of the corpus body. Congenital bilateral cleft of the upper lip and solid sky. Bronchildren dysplasia. Small heart development anomalies. Open oval window. Hydodipla-Zya Timus. Horseshoe kidney. Prematurity 34-35 weeks.

For the introduction of the patient of drugs, a vascular catheter installed in the right connective vein was used. The patient received: antibiotics (ceftriaxone, meropenem, vancomycin), infusion therapy (glucose solutions, aminovena, intralipide, etc.), corticosteroids (dexamethasone), hemostatic therapy (transfusion of the erythrocyte mass, the introduction of dicinone, heparin), antifungal therapy (Fluek-Nazol ) In the age dosages.

Despite the conduct of intensive therapy, the patient has developed irreversible disorders in the brain and violation of the vital functions that caused death.

Brain macroscopy. The brain poorly retains the shape on the table, without differentiation on the white and gray substance, in the occipital proportion of the left hemisphere there is a large-scale subependemary-parenchymatous hemorrhage of irregular shape, with fuzzy boundaries, dark red, measuring 6.5x5.8x5.6 cm with perifocal softening the brain substance. Melno-food and point hemorrhages in a soft cerebral shell in the dark area of \u200b\u200bthe left hemisphere are noted. Maze body is reduced in an end-in size, 1.5 cm wide, 0.3-0.4 cm thick; The cerebellum of the correct form, the conventional brain of the usual structure, the vascular plexus is full (Fig. 1).

Clinical analysis of the agent of the corp

Fig. 1. Macrobreparation of the brain of the patient E. At the age of 1 month, 3 days of life, with multiple congenital defects, partial atrophy of the corn body (AMT). The arrow shows partial AMT (color version, see the cover)

Fig. 1. A GROSS SPECIMEN OF THE BRAIN FROM FEMALE PATIENT E. AGED 1 MONTH AND 3 DAYS, WITH MULTIPLE CONGENITAL DEVELOPMENTAL MALFORMATIONS AND PARTIAL ATROPHY OF THE CORPUS CALLOSUM (ACC). PARTIAL ACC IS SHOWN WITH AN ARROW (See Color Version On The Cover)

Circulatory organs. Heart: dimensions 4.8 * 3.2 * 2.7 cm; Epicard and pericardium subtle, smooth, shiny; Soft-elastic heart muscle consistency. Myocardium blue-red. In the cavities of the heart, liquid dark blood contains. The mocardine thickness of the right ventricle is 0.3 cm, left - 0.6 cm. The endocard is smooth, shiny, transparent. Endocardium right and left atriality with pearlite sections. Focal subendocar dual hemorrhages in the right and left ventricles. In both stomachs, transversely running abnormal chords, in the right ventricle there is a partially split puffy muscle. Three-rigid and double flaps are smooth, shiny, transparent. The oval window is open, with a diameter of 0.4 cm, the arterial duct is closed. The perimeter of the pulmonary trunk is 2.4 cm, the aortic over the valves - 1.6 cm, in front of the Ple-Chegol barrel - 1.6 cm, in the upstream section of -1.5 cm, at the level of the diaphragm - 1.3 cm, the abdominal department - 1.2 cm. Trunk vessels with pale yellow intimate.

The organs of the urogenital system. There is a single under-cube kid with dimensions of 7.0 * 4.2 * 1.1 cm, with the presence of a coastal connecting the lower pole of the kidney, 2.0 cm wide; The surface is quarrel, the view of the cut with a clear differentiation of the cortical and brainstant, the cortical substance of the gray-pink color, the pyramids of gray-red, draws attention to the bright yellow scaching of the pyramids. The mucous membrane of the pelvis is grayish-pinkish, dim, the lumen contains bright yellow urine. Ureterals in the form of narrow heavyings are formed on both sides, a diameter of 0.2-0.4 cm, the bladder contains a small amount of bright yellow urine, folding is stored.

Pathologist diagnosis. Head brain swelling. The focal drain two-sided pneumonia, double-sided hydrohemotorax, fibrinous pleurisy, ascites. Fibra

nose peritonitis, acute hepatitis. Multiple congenital malformations. Partial agentsia of the corpus body. Congenital bilateral cleft of the upper lip and solid sky. Malformation of brain vessels. Timus hypodicia (mass deficiency - 87.3%). Reducing the follicles of the spleen. Deletion of peripheral lymph nodes. Bronchildren dysplasia (fibrosis of inter-vololar partitions). Small heart development anomalies. Open oval window (0.4 cm diameter). Horseshoe kidney with the presence of a few glomerular and tubular cyst.

Discussion_

AMT associated with monogenic and chromosomal syndromes, complex chromosomal aberrations, is quite rare pathology. The true (primary) AMT is a congenital brain defect and formed before the 12-16th week of gestation. In both of our observations, a true AMT is verified associated with chromosomal aberrations. In the first case, partial trisomy 8p was revealed, combined with AMT, in the second - partial monosomy (terminal deletion) of the long shoulder of the 7th chromosome, combined with partial AMT.

In the described observations, intrauterine infection (cytomegalovirus infection, toxoplasmosis, rubella, etc.) was absent, both pregnancy proceeded against the background of the threat of interruption, delays in the intrauterine development of the fetus. However, to clarify an unfavorable factor contributing to the appearance of AMT, it was not possible that it is often noted in foreign studies. The risk of congenital infection in the etiology of AMT is low. Birth in two observations were premature; Both children were born premature, with intrauterine hypotrophy of the I-II degree and a low estimate on the apgar scale, which led to a further delay in the formation of motor skills and higher cortical functions.

With a neurological examination in the 1st observation, minimal motor disorders on the scale of global motor functions were found, the general underdevelopment of the I-II speech of the degree, which implies a relatively favorable course of the disease. On the contrary, in the 2nd observation in the late neonatal period in a sick girl against the background of the congenital deficit of the immune system, a generalized infection of bacterial-fungal etiology developed. In the future, the unfavorable course of the disease, apparently, was influenced by polyorgan deficiency, there was a breaking of the vascular malformation of the occipital lobe of the left hemisphere of the brain, later the edema of the brain was formed. The severity of cerebral and somatic pathology was incompatible with life.

In the first case, the instrumental examination confirmed the presence of total AMT (data of neuralonography), and the congenital heart defect (the interventricular septum defect) was revealed with echocardiography; In the second observation, partial AMT was verified in full and post-melted. In addition, in the second observation of the post-madrontally additionally, malformation of brain vessels, hypo / dysplaysia of thymus, reduction of spleen follicles, depletion of peripheral lymph nodes, bronchopulating display-zia, small heart development anomalies (open oval

window), the only horseshoe kidney with the presence of a few glomerular and canalic cyst.

Thus, a bright feature of the described observations was a combination of somatic and cerebral pathology included in dyshembogenetic syndromes, including the abnormalities of brain development and many

additional additional defects (polyorgan pathology). The direct relationship between the prevalence of congenital pathology and the severity of the course and forecast of the disease is confirmed.

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13. SANTO S., ANTONIO F., HOMFRAY T. ET AL. Counseling in Fetal Medicine: Agenesis of the Corpus Callosum. Ultrasound Obstet Gynecol. 2012; 40: 513-521. PMID: 23024003 DOI: 10.1002 / Uog.12315.

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17. Rapp B., Perrotin F., Marret H. et al. Value of Fetal Cerebral Magnetic Resonance Imaging For The Preenatal Diagnosis and Prognosis of Corpus Callosum Agenesis. J Gynecol Obstet Biol. Reprod. 2002; 31: 173-182. PMID: 12016416.

18. Milovanova O.A., Konovalov R.N., Illaroshkin S.N. Vices for the development of the corpulent body. Clinical and neurovalization manifestations. Tutorial. M.: Media Sphere, 2015. 104 p.

19. Visentialin A., Pilu G., Falco P. et al. The Transfrontal View: A New Approach to The Visualization of the Fetal Midline Cerebral Structures. J ultrasound med. 2001; 20: 329-333. PMID: 11316310.

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2. VOLPE P., Paladini D., Resta M. et al. Characteristics, Associations and Outcome of Partial Agenesis of the Corpus Callosum in the fetus. Ultrasound Obstet Gynecol. 2006; 27: 509-516. PMID: 16619387 DOI: 10.1002 / UOG.2774.

3. Schell-Apacik C.C., Wagner K., Bihler M. et al. Agenesis and Dysgenesis of the Corpus Callosum: Clinical, Genetic and NeuroImaging Findings in a series of 41 Patients. Am J Med Genet. 2008; 146A: 2501-2511. PMID: 18792984 DOI: 10.1002 / AJMG.A.32476.

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5. Mitchel T.N., Free S.L., Williamson K.A. et al. Polymicrogyria and Absence of Pineal Gland Due to Pax6 Mutation. Ann Neurol. 2003; 53: 658-663. PMID: 12731001 DOI: 10.1002 / Ana.10576.

6. KATO M., DAS S., PETRAS K. ET AL. Mutations Ofarx Are Associated with Striking Pleiotropy and Consistent Genotype-Phenotype Correlation. Hum Mutat. 2004; 23 (2): 147-159. PMID: 14722918 DOI: 10.1002 / HUMU.10310.

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12. Goodyear P.W.A., Bannister C.M., Russel S. et al. Outcome in prenatally Diagnosed Fetal Agenesis of the Corpus Callosum. Fetal Diagn Ther. 2001; 16: 139-145. PMID: 11316928 DOI: 53898.

13. SANTO S., ANTONIO F., HOMFRAY T. ET AL. Counseling in Fetal Medicine: Agenesis of the Corpus Callosum Ultrasound Obstet Gynecol. 2012; 40: 513-521. PMID: 23024003 DOI: 10.1002 / Uog.12315.

14. Lacey D.J. Agenesis of the Corpus Callosum: Clinical Features in 40 Children. Am J Dis Child. 1985; 139: 953-955. PMID: 4036933.

15. Bedeschi M.F., Bonaglia M.C., GRASSO R. ET AL. Agenesis of the Corpus Callosum: Clinical and Genetic Study in 63 Young Patients. Pediatr Neurol. 2006; 34: 186-193. PMID: 16504787 DOI: 10.1016 / J.pediatrneuroL.2005.08.008.

16. Milovanova O.A., Alikhanov A.A., Tambiev I.E. et al. . Zhurnal Nevrologii I Psikhiatrii IM S.S. Korsakova. 2017; 1: 63-66 DOI: 10.17116 / jnevro20171171163-66 (in russ.)

17. Rapp B., Perrotin F., Marret H. et al. Value of Fetal Cerebral Magnetic Resonance Imaging For The Preenatal Diagnosis and Prognosis of Corpus Callosum Agenesis. J Gynecol Obstet Biol Reprod. 2002; 31: 173-182. PMID: 12016416.

18. Milovanova O.A., Konovalov R.N., Illarioshkin S.N. Poroki Razvitiya Mo-Zolistogo Tela. Klinicheskie I Neyrovizualizatsionnye ProYavleniya. . MOSCOW: Media Sfera, 2015. 104p. (in russ.).

19. Visentialin A., Pilu G., Falco P. et al. The Transfrontal View: A New Approach to The Visualization of the Fetal Midline Cerebral Structures. J ultrasound med. 2001; 20 (4): 329-33. PMID: 11316310.

Clinical parse

Agnesia of the corpulent body

Information about the authors: Milovanova Olga Andreevna - D.M., prof. Department of Neurology of Children's Age FGBOU DPO Rhmpo Ministry of Health of Russia. 123995, Russia, Moscow, ul. Barricade, 2/1. E-mail: [Email Protected]; Tarakanova T.Yu. - Neurologist, ASP. cafe. Neurology of children's age FSBEA DPO RMPO Ministry of Health of Russia, Moscow, Russia;

Pierzheva Yu.B. - Neurologist, ASP. cafe. Neurology of children's age FSBEA DPO RMPO Ministry of Health of Russia, Moscow, Russia;

Katasonova L.P. - Ph.D., doctor Higher. Cat., Head. The pathologist department of DCGB them. BEHIND. Bachevaeva, Moscow, Russia;

Beach Ool S.Kh. - Pathologist, DCGB them. BEHIND. Bachevaeva, Moscow, Russia; Vozhbiev TE. - DCGB pathologist. BEHIND. Bacheva, Moscow, Russia.

Information about the authors: ol "GA A. Milovanova, D.SCI. (Med.), Prof., Department of Child Neurology, Russian Medical Academy Of Continuous Professional Education, Ministry of Healthcare of the Russian Federation, Moscow, Russia. 123995 , Russia, Moscow Ul. Barrikadnaya, D.2 / 1, E-mail: [Email Protected];

Tat "Yana Y Tarakanova, Neurologist, Phd Student, Department of Child Neurology, Russian Medical Academy Of Continuous Professional Education, Ministry of Healthcare of the Russian Federation, Moscow, Russia;

Yuliya B. Pronicheva, Neurologist, Phd Student, Department of Child Neurology, Russian Medical Academy Of Continuous Professional Education, Ministry of Healthcare Of The Russian Federation, Moscow, Russia;

Lyubov "P. Katasonova, PhD, Head of the Pathology Department, Tushino Children" S City Hospital, Moscow, Russia; Salbakay KH. Bi ^^ OL, Pathologist, Tushino Children "S City Hospital, Moscow, Russia; Tat" Yana E. Vorozhbieva, Pathologist, Tushino Children "S City Hospital, Moscow, Russia.

For citation: Milovanova O.A., Tarakanova T.Yu., Pierzheva Yu.B. and others. Agnesia of the corpus body associated with hereditary syndromes. Annals of clinical and experimental neurology. 2017; 10 (2): 62-67.

For Citation: Milovanova O.A., Tarakanova T.Yu., Pronicheva Yu.B. et al. . Annals of Clinical and Experimental Neurology. 2017; 10 (2): 62-67. (In russ.)

Atrophy of the brain is a decrease in the size of each cell, a decrease in their number. The process is expressed by the deterioration or complete degradation of the functions of the organ. Regardless of the arrangement of atrophic tissues, patients decrease, but cognitive abilities (cognition of new information) are extremely rarely completely disappeared. In most cases, neurological disorders are observed (somatic diseases of the central nervous system), in a quarter of cases, neurotic (mental) pathologies are expressed, and mixed.

Cerebral atrophy of the brain is characterized by accelerating destruction and slowdown in the development of new cells, so the disease is known for slow, but constantly progressive current.

The reasons

The activity of the nervous system is not fully studied, so not all causes of atrophic processes in the tissues are known. There is a version that degenerative processes in cells under cerebral atrophy are possible under the influence of hereditary factors. In some cases, their start provoke harmful effects.

Causes laid in the period intrauterine development:

1. Anomalous genes transmitted by inheritance.
2. Mutations chromosomes.
3. Infections.

Acquired reasons:

1. Incication of the body that continue for a long time.
2. Strong or prolonged brain infections.
3. Radiation (usually provokes minor dystrophic changes).
4. Smoking.
5. Alcoholism.
6. Addiction.
7. The impact of chemicals (in everyday life or at work).
8. Brain injuries accompanied by edema, hematomas, circulatory disorders.
9. Cysts.
10. New formation.

Congenital predisposition Atrophy is considered the prevailing factor, compared with the acquired reasons. They can activate the development of genetically laid anomalies. Acquired atrophic processes in the brain are no more than 5%, the remaining cases are conjugate with congenital pathologies.

Diagnostics

Main methods:

1. MRI - Creating images of sections of parts of the organ (in this case of the brain). A person is placed on the couch, takes the position recommended by the instructor. The device starts and the scan results are displayed on the scan. Gives information about the chemical structure of tissues. Pathological conditions are visible visually in the image.
2. Kt. - layered body research. It helps determine the physical condition of the substance. For diseases, the doctor defines changes in the density of the object.
3. EFECT - Creating a three-dimensional image of the internal structure of the distribution of radionuclides by emitting photons when placing a patient in the gamma chamber.
4. PAT - The study of the human brain through the registration of a pair of gamma quanta, for the formation of which the radiopharmaceutia (radioactive drug) is pre-injected into the body.
5. MP spectroscopy. - Overlooking the processes of metabolism, analysis of biochemical changes of tissues.

Additional ways of diagnosing brain atrophy:

1. UDG (Ultrasound doppler) - identification of pathologies of vessels and arteries of the brain. Man is located on the couch. A gel is applied on the neck. The pattern of vessels is compiled according to the information obtained by the sensor, which is carried out by the location of the vessels.
2. TKDG (transcranial doppler) - A more advanced method for the study of vessels and arteries responsible for the blood supply to the brain. Particular attention is paid to brachiocephalic arteries.
3. Breast angiography - Diagnostics of the state of the vessels after the introduction of radar-contrast substances. Need to assess the condition of the chest aorta. There are direct and indirect methods. The direct means the introduction of the catheter through the elbow or femoral vein. Indirect way provides for the installation of a catheter through a femoral or subclavian artery.
4. Selective angiography - diagnosis by the catheterization method of all vessels participating in the blood supply to the brain.
5. EEG - Obtaining a graphic image of electrical oscillations by electroencephalograph and their comparison with the indicators of the norm to identify deviations during the metabolism of substances of neurons.
6. The method of caused potentials - Tracking brain bioelectric reactions for the study of brain functions (somatosensory (touch, feeling of temperature, pain, position of body parts in relation to each other), visual, auditing), changing or endangered during atrophy.
7. Analysis of blood plasma.
8. Laboratory studies of the cerebrospinal fluid (liquor).

When diagnosing a brain atrophy in children and adults, a common feature in a group or separately may be observed.

Signs condimizing the presence of dementia:

1. Reducing the temporal fractions of the brain.
2. Reducing the volume of hippocampus responsible for the formation of emotions, maintaining the mechanisms of short-term memory and its transition to the long-term needed to hold attention.
3. Expansion of furrows of the cerebral cortex with invaluing changes viewing on it.
4. On the frontal, dark and temporal fractions, a decrease in the subarachnoid space in the convexital (adjacent) surface is found.

Manifestations of cerebrovascular insufficiency (Cerebral vessel changes):

1. The destruction of the boundaries between the white and gray substance.
2. Postiemic microker (after transferred to microinsults) - in most patients.
3. Macokers are more than 5 mm in diameter in a smaller number of sick brain atrophy.
4. Expansion of the lateral furrow (distinguishes the temporal fraction of the brain with the frontal and dark).
5. Involution of the structure of the cerebral cortex.

Degree of disease

Depending on the volume of the affected brain tissue, several degrees of atrophy are distinguished. They are taken into account during the survey to determine the severity of pathologies caused by degenerative processes, if necessary, draw up the rules for behavior and care for patients for relatives.

Atrophy of 1 degree

First, the disease is invisible or the patient nor others. Anxiety of the patient or his environment may appear due to other pathology which directly or indirectly affects the processes of atrophy of the cerebral cells. Depending on the localization of the process: Cork atrophy or damage to subcortical structures, differing changes will be visible.

During the development of moderate atrophy, dizziness, headaches, the course and frequency of which are gradually aggravated. The development of the disease at this stage can often be braked. Based on the MRI testimony, the doctor may find the necessary methods of therapy.

Atrophy of 2 degrees

Clinical manifestations of thinking, speech and physical activity are constantly growing. Depending on the damage to certain structures, specific processes are braked and degraded.

Due to the atrophy of the brain, irreversible changes in motility can occur, as well as in coordination of movements and gait, for example, with the degeneration of the cerebellum. Thinking, memory and intelligence Also suffer. Outwardly, character, man's manners may change. At the last stages of atrophy, patients lose the possibility of using familiar things, such as a toothbrush, cutlery (require manual feeding).

Symptoms

Scroll general symptoms of brain atrophy:

1. Simplification of thinking, reduced analytical abilities.
2. Change speech. It becomes more measured, poor, vague.
3. Reducing memory up to its full loss.
4. Worsening motility.

Symptoms Depending on the atrophic area:

1. Violations breathing.
2. Cardiovascular pathology.
3. Failures B. digestive tract.
4. Absence protective reflexes.
5. Violations tonus muscles.
6. Deterioration coordination of movements.
7. Violations exchange processes.
8. Wrong thermoregulation.
9. Loss of part or all reflexes.

Natural brain atrophy in old age
Atrophy of the brain is a physiological phenomenon, usually moderately begins between the ages of 40 and 60 years. Signs may appear about 70 years. Brain that has been aging for every 10 years, the average decreases by 1-2%.

American scientists believe that aging begins under the influence of the lateral and third ventricles of the brain every year. Aged 65 years the ventricles grow about 0.95 ml each year.

Also, many people in connection with age-related changes also increase the subarachnoid space (the cavity between the brain shells with the spinal fluid). From 40 years old the volume of liquor (spinal fluid) increases by 1 ml. By 90, it may become more than 40 ml, compared with the primary value.

With increasing age, people decrease in the brain hemisphere. Possible dynamics reduction of their volume by 0.23% per year. Lobal Share loses up to 0.55%. Temporary shares become less 0.28%. Toward and dark Reduce 0.30% per year.

Atrophy of the brain predetermines the development of various forms dementia (dementia). 7% People over 65 are the development of this pathology. People in the age group over 80 years old are very often observed.

Age changes are growing gradually. Start with innocuous signs, but when progressing makes a person defective. First, there are changes in the character of a person. Active become passive but sociable, emotional — sluggish and indifferent, closed.

Man begins to use a poorer speech. Lexicon More reduced. Sometimes cultural patients swear in grain words, which may also testify not to deterioration in nature, but about the progressive atrophy of the brain.

Defects Speech - External manifestations of impaired thinking. Patients are not able to think wide. All reflections are tritely simple, and the actions are primitive. Such people do not appreciate their behavior from the side, do illogical actions. All mental activity comes down to the implementation of the simplest cases. (regardless of their relevance), That in progression of the disease can be changed in a complete lack of activity.

Motor activity in the atrophy of the brain, as well as its cortex, always suffers, sometimes even up to complete immobility. Especially visible motility deterioration, therefore patients with brain atrophy cannot do complex work: Neither mental nor physical. The first signs are often expressed by the worsening of the handwriting.

Alcohol atrophy

Signs of even the initial stages of brain atrophy expressed very brightly Since the first manifestation - Encephalopathy, Expressed in a sharp change in the nature and mood of a person to depressive, sometimes with a suicidal bias. Violations appear due to insufficient blood supply to the brain and its increasing dystrophy.

Under the influence of alcohol, neurons of various departments of the head (and spinal cord) are affected with the subsequent formation of clusters of decay products around the affected vessels. Damage to neurons are expressed by several processes: wrinkling, moving or lysis (dissolution).

Symptoms gradually grow and exacerbate. Pathology begins with encephalopathy and delirium (permanent of consciousness, nonsense), subsequently death is possible.

In the process of atrophy of the brain with constant use of alcohol occurs sclerosis of vessels. Around the brown pigment and hemosiderin containing iron, deposits are formed. Such changes lead to hemorrhagiam (hemorrhages in the brain) and the formation of cyst in vascular plexus.

Separately stands out makiyafaby Biniam Syndrome, The manifestation of which is the central necrosis of the mounted body with the appearance of edems. The disease is accompanied by blood hemorrhages in the brain and demyelinization (the destruction of the myelin layer of nerve fibers of the nervous system).

Cortical atrophy of the brain substance

If the neurons of the knee of the brown body or the front of the rear leg of the cerebellum, develops hemiplegia (paralysis of half of the body). The rear sections of the brain cortex substance are losing the control functions, so symptoms spread on the floor of the body are possible:

1. Gemiagesezia (skin loss).
2. Gemianopsy (Impossibility to see objects at a certain side, loss of right or left fields of vision).
3. Different muscle groups move differently, However, muscular weakness is not observed.
4. Also one of the sides of the body can completely lose sensitivity.

Multisystem atrophy

Degeneration of various neurons is called multisystem atrophy or shea-Drajder syndrome. The disease becomes the cause of violations in various systems of the body.
It begins with S. primary signs:

1. Akineathic-Rigid Syndrome (Movement is rare and inhibited with a slight muscle tension).
2. Jellying ataxia (Violations of gait, sustainability, disturbances are possible in performing arbitrary limbs).
3. Urinary problems.

Progression of the disease brings new symptoms:

1. Parkinsonison (slow motion, small letter with round, uneven letters).
2. Cerebellum dysfunction (Sustainable violation of coordination of movements, the impossibility of constant maintenance of the balance, frequent fall).
3. Ortostatic hypotension (In the vertical position, a person suffers a sharp drop in pressure due to the inability of the vessels to support it, expressed in dizziness and fainting).
4. Disturbance disorders.
5. Urinary incontinence or on the contrary The impossibility of urination at the certain time.
6. Constipation.
7. Impotence In men.
8. Dry skin and mucous membranes.
9. Violations of speech and difficulty when making food (Swallowing) due to paralysis of voice ligaments.
10. Twist in the eyes.
11. Loud breathing during sleep. Possible: shortness of breath, snoring, string (whistle).
12. Sleep disorders in particular apnea (stopping breathing for a few seconds or minutes with subsequent awakening), fast eye movements.
13. Cognitive degradation (Infertility of the ability to know the new).

Granular brain atrophy

The disease is very rare. It is characterized by such clinical signs:

1. Stroke . They proceed in acute form. Always accompanied by paralymps (the inability to make arbitrary movements), hemipabs (full or partial loss of power in half of the body).
2. Afaic disorders (aphasia). Violations of speech. There occur with the defeat of the part of the cerebral cortex responsible for speech, as well as the nearest subcortical structures.
3. Dementia. Gradually increases dementia, mental retardation can manifest. A person loses previously acquired knowledge, it does not perceive new ones.

Often diagnosed in old age, but it can begin in any. With granular atrophy appear and progress violation of brain vessels. First of all, the defeat of the arteriole is observed.

When diagnosing (conducting an MRI), the surface of the cortex of the brain changes the external structure on the barride, it becomes wonderful to the branches (granules).

Left hemisphere

Appear speech violations. Develops motor aphasia: It sounds slowly, with the application of great patient's efforts, in some cases all words are made up of individual sounds, sometimes they are illegible.

Logical thinking is noticeably degraded. The patient develops a state of constant depression (one of the primary signs in atrophy of the left hemisphere in the temporal area).

Visible images are not covered by vision completely, And consist of separate fragments. A person cannot read, the handwriting changes, becoming unrecognizable and inaccient. Analytical thinking gradually disappears The incoming information is not analyzed, it is not logically perceived. A person does not remember the dates, not focusing in them, also does not perceive numbers, the ability to account is lost.

Due to improper perception and processing of incoming information multiemic processes are violated (Memory is lost). A person perceives said in his presence of fragments of phrases or even individual words, so the distorted meaning reaches him.

In severe cases of atrophy of the left hemisphere of the brain, it causes partial or complete paralysis of the right side of the body. First, the motor activity is disturbed, and then the loss of sensitivity and a decrease in muscle tone are increasing.

Frontal stories

Worsen mnemic processes (ability to remember), also noticeable degradation (Simplification) thinking. Falls intelligence.

The initial stage is expressed in character change:

1. Man becomes more secretive, but expresses simplified thoughts.
2. Gradually is isolated from others.
3. Making illogical deeds.
4. Sets meaningless purposes.

Phrases and actions are repeated every day. Life passes as in advance written (quite primitive and the same for every day) scenario. All speech comes down to simple proposals. Patients lose most of the vocabulary stock Therefore, they express thoughts and needs one.

If atrophy of the frontal brain shares occurs in Alzheimer's disease, The most suffer from memorization and thinking processes. Personality person, character persists much better than, for example, in peak disease, Therefore, the adequacy of a person can be expected in most cases.

Cerebellum

On the fore changes of muscle tone as well as ataxia (inconsistency of movements). A person loses dexterity, stability when walking and standing, can break the motor skills before manifestations of the inability to perform any work. Possible loss of human ability to self-service.
Disturbance of movements in atrophic changes in the cerebellum have some features:

1. Before the end of the action appears intense jitter (imperceptible alone and manifested in motion, its amplitude is quite low)
2. Hands and legs become more angular Instead of familiar smoothness.
3. All actions (speech and movement) slow down.
4. Chandated speech (Words are pronounced by syllables, and the pronunciation is slow).

In addition to motor disorders, cerebellum atrophy is characterized non-specific symptoms: Head pains, frequent attacks of nausea and vomiting, a person can be drowned, a worsening of hearing is also noted.

Progression of atrophy adds new symptoms:

1. Intracranial hypertension (high blood pressure).
2. Ophthalmoplegia (eye muscle paralysis). In atrophy, the cerebellum occurs due to the paralysis of the cranial-brain (glacial) nerves.
3. Areflexia (The disappearance of reflexes).
4. Enuresis (urine incontinence during sleep).
5. Nistagm (High frequency eye movements are not controlled by the patient).

Brain atrophy in newborns

At the appearance of brain atrophy in babies often affect hydrocephalus, In the people of Lodka. With this pathology the amount of spinal fluid increases, serving protective shell of the brain, but in case of increasing squeezing it.

During internal development, such pathology is detected by the method Ultrasound. Hydrocephalus can develop due to disorders of the formation of the nervous system. The process is influenced intrauterine infections Such as herpes, cytomegaly (salivary glasses, adults are manifested at least).

Lead to hydrocephalus can congenital vices of development. Matter generic injuries Medium or high gravity, in which the infant occurs in the brain hemorrhage, followed by the development of meningitis.

Children with brain atrophy the first Months of life are carried out in intensive care. In some cases, the stay of the house in the pauses between courses of treatment, round-the-clock observation by neurologists. Further children need a long rehabilitation.

Therapeutic methods, developing classes, positive emotions can be made possible or accelerated processes. the assumption of certain functions with healthy areas of the brain for replacing the work of atrophied. The forecast for complete recovery is usually unfavorable.

Treatment

There are no methods to restore atrophied brain cells. One can only relatively slow down atrophy. All treatment methods are directed to relief or relief symptoms appearing in the process of degradation of various parts of the brain.

Patients need a calm atmosphere around. Extremely It is desirable to find the house. The room in the hospital is made in extreme cases. All of them are determined by the lack of ability to ensure proper care for the patient.

Hospitalization or accommodation in the boarding school for disabled people is practiced for patients with obvious strong psyche disorders, inadequacy, mental retardation. It is also possible to move the patient to a specialized institution with the impossibility of permanent care.

It is desirable to create conditions for a person with a brain atrophy activity, healthy lifestyle. Sleep or long stay in daytime (overwhelming) time of day is not required. If possible ill attracts to daily studies with household chores, other work or active activities, entertainment.

Psychotropic drugs in the treatment of brain atrophy are undesirable, However, their relative need is revealed during the manifestations of an excited state, an increased irritability, or vice versa of apathy to varying degrees.

The only treatment of brain atrophy in modern medicine is slowing down the destruction of neurons, cells. Scientists revealed effectiveness in such groups of drugs:

1. Vascular drugs (Cavinton).
2. Nootrops - Brain functions stimulants (cerakson).
3. Metabolic preparations - Means for improving metabolic processes.
4. Vitamin B6. It helps maintain the correct structure of the fibers of the nervous tissue.

Symptomatic treatment will be bought signs of atrophy for a certain time, efficiency have:

1. Antidepressants - stop the depressed state and some violations of the brain functions.
2. Soothing - Deposition of signs of disorders of the nervous system.
3. Tranquilizers - Psychotropic drugs, help on time to remove the anxious state, reassure the person, remove the tension of the muscles, stop convulsions. Have sleeping pills or on the contrary activating effect.

Prevention of brain atrophy

The exact methods of preventing the appearance of this pathology have not been detected. It is possible only to remove this process, do not create additional conditions for the appearance of destructive processes in the brain.

Required compliance with Rules:

1. Timely treatment of any diseases in the body, directly or indirectly affect the brain.
2. Passing of surveys to identify pathologies.
3. Alternation of monotonous work And life with active recreation and sports.
4. Balanced diet With a minimum percentage of harmful substances.
5. Not disdainless rest.
6. Prestressing the development of atherosclerosis cerebral vessels. This requires: control over body weight, refusal of oily food, treatment of pathologies of the endocrine system (highlighting hormones) and metabolism, all obstacle obesity.

It is necessary to exclude risk factors, Which when they are re-published lead to cerebral atrophy. Requires:

1. Throw smoking.
2. Refuse alcohol and drugs.
3. Remove (if possible) all factors depressing the immune system.
4. To not allow psycho-emotional overvoltage, Moderately react to stress.

Practice shows that people with active, cheerful attribute most often live to a deep old age without signs of common pathologies associated with brain atrophy.

Maze body is an important anatomical structure connecting the hemisphere of the brain. It is represented by dense plexus, which consists of two hundred and fifty million nerve cells. Normally, the appearance of first neurons ensuring the relationship between large hemispheres, occurs already on 11-12 weeks of intrauterine development. It is extremely rare (approximately in 1 case for 2000 newborns) diagnose anatomical absence of communication between hemispheres. This state got the name of the agent of the corpus body.

Faced with the diagnosis of the "Agnesia of the Core Body", each parent wonders: "What is it?". The disease has a connection with a hereditary factor. It may occur isolated or combined with many other malformations. Even if the pathology was not detected during the ultrasound examination of the fetus, it is usually diagnosed in the first two years of the child's life.

Corn body functions

Before proceeding with the description of the symptoms of the disease, let's focus on what functions the corn body in the body performs. Two brain hemispheres can work isolated, performing each of their own tasks. It is believed that the right half of the brain is responsible for analytical thinking and the ability to accurate sciences, and the left is for creative thinking and fantasy. The corpus body provides coordination and friendly work of the whole nervous system:

• allows you to proceed correctly and perceive information coming from the senses (visual, auditory analyzer);
• provides community of thought processes.

In the second half of the twentieth century, scientists conducted a number of experiments, during which several subjects were cut with a callous body. The results were amazing: patients began to think and act absolutely separately and illogical. For example, a man with his right hand hug his spouse, and left left. Thus, the corn body coordinates the effect of two isolated, but at the same time completely conscious regions of the brain.

As the disease is manifested

Since the agent of the corrosion body is often combined with other congenital vices of the nervous system, the clinical picture of the disease looks different from each child in different ways. The most common symptoms of pathology are:

• the presence of a large and medium size in the hemisphere of the brain;
• atrophy of the visual (second pair) and the auditory (VIII pair) of the nerves - a severe malfunction, in which a child cannot see or hear;
• schisensephalia is a rough violation of the formation of brain tissues, in which in the cortex hemispheres there is a deep cleft, which is continuing from ventricles to the subarachnoid space. Most often, the fruit with such a defect of development is born dead;
• neoplasms in the area of \u200b\u200ba non-refined corporest body;
• spinal column splitting;
• - a significant reduction in the size of the head and brain;
• lag in mental and psychomotor development;
• epileptic seizures;
• combined congenital malformations of the tract, tumor;
• characteristic facial dyshmephism (changes in the structure of the bones of the facial skull);
• early sexual development.

Diagnosis and treatment

Most often, the agent of the corpus body is diagnosed during ultrasonic screening and fruit surveys. The doctor determines the complete or partial absence of a dense portion of the nervous tissue connecting the large hemispheres of the brain. After the birth of a child to confirm the diagnosis and exclusion of other congenital pathology, additional research methods are carried out:, computer or magnetic resonance tomography. Neurologist is engaged in the treatment of a child with the agentsia of the corn body. It may be necessary to consult genetics, neurosurgeon.

The status therapy is currently not developed. The treatment is reduced to eliminating such hazardous symptoms as convulsions, hydrocephalus, nervous excitement. As a rule, the correction of neurological disorders is very difficult to achieve, potent drugs are used in maximum dosage:

• (benzodiazepines, phenobarbital);
• glucocorticosteroids (prednisone, dexamethasone);
• neuroleptics for the correction of behavioral disorders;
• nootrops (, piracetam) to improve the nutrition of brain tissues.

Forecast

In rare cases, when the vice developed isolated, the forecast is favorable. If the child is diagnosed only by the agent of the corpus body, the effects of health are minimal. Children grow and develop as usual, minor neurological problems and some features in thinking are possible. With congenital congenital defects, it is rare to talk about a good forecast. The consequences of the disease and tactics of the actions of doctors directly depend on the degree of damage to the nervous system.

Agnesia and dysgenesis of the corpus body can be complete or partial. In the last cases, the back part is usually lost, due to the fact that the corpuslike body develops in the front-hand direction, nevertheless, front agnesia is possible (Aicardi et al., 1987; Barkovich and Norman, 1988b; Sztriha, 2005). There are atypical forms, it is difficult to distinguish between Holoprozencephalia (Barkovich, 1990). The agent of the corrosion body is relatively common. The prevalence among the population is generally estimated as 3-7 / 1000 (Bedeschi et al., 2006).

The observed frequency increased with the introduction into the CT and MPT practice. Jeret et al. (1987) revealed 33 cases in a series of 1447 CT shots.

Even now the diagnosis is mainly placed only by patients with neurological symptoms, so the true frequency is not known.

The absence of cornstone spikes, as a rule, is replaced by two longitudinal ligaments, known as the longitudinal corn body or trigger bundles passing along the inside of the hemispheres. Frequently appear furrows on the inside with a radial location and the expansion of the occipital horns, which preserve their fetal morphology, the so-called Callpacephalia (Noorani et al., 1988). Other related abnormalities often include the formation of a whale of the third ventricle, reporting or unlocking (Yokota et al., 1984, Griebel et al., 1995, Barkovich et al., 2001b) Cerezeremki's worm anomalies and brain stem, as well as mixed malformations CNS, such as heterotopia, anomalies of formals of the formal or cefalozele (Jeret et al., 1987; Barkovich and Norman, 1988b; Serur et al., 1988).

Giant cysts may have a favorable outcome, despite the impressive sizes (Lena et al., 1995; Haverkamp et al, 2002). CNS developmental defects were found in 33% of patients with full and 42% of them with partial agnesia (Bedeschi et al., 2006).

It is these combined anomalies that are responsible for clinical manifestations. The molding body lipom almost invariably accompanies the aghensia of this structure (Zee et al., 1981; Vade and Horowitz, 1992). Characterized peripheral malformations (Parrish et al., 1979). Especially often encountered eye anomalies (Aicardi et al., 1987). Maze's hypoplasia (Bodensteiner et al., 1994) may be a minimal form of callosis digesia, but much more often by a consequence of cortical neuronal losses.

Etiology is diverse. At least 46 syndromic defects of development or metabolic disorders and 30 mutant genes are identified (Kamnasaran, 2005). With non-industrial forms, genetic transmission rarely occurs, although there are reports of family cases with autosomal-recessive (Finlay et al., 2000), X-clutch recessive (Menkes et al., 1964; Kaplan, 1983) and autosomal dominant type of inheritance (Aicardi et al. 1987). Many chromosomal defects were revealed, including trisomy 8, 13, 16 and 18, as well as a mixture of less common chromosomal defects.

AGENESIA COLORAL BODY, (Left) MRI (inversion-recovery):
longitudinal cornistic body (trigger beams) near the inner surface of brain ventricular bodies.
(Right) Sagittal projection: complete absence of a corrosion body and a radial distribution of furrows on the inside of the hemispheres.

Serur et al. (1988) viewed 81 cases from literary sources, of which in 21 there was a trisomy 8, in 14 there was a trisomy 13-15 and in 18 touched by chromosome 17 or 18. Of the 34 karyotypes, trisomy was found 8. Subtiterome abberats were detected in 5% of cases of Bedeschi et al. (2006). Among the environmental factors, the fruit alcohol syndrome is isolated. Some metabolic diseases, especially hyperglycemia (Dobyns, 1989), the insufficiency of pyruvate dehydrogenase (Bamforth et al., 1988, Raoul et al., 2003) and other metabolic disorders together account for about 2% of the agensia of the corpus body. In most cases, their origin is unknown.

A description of the clinical manifestations of the Callose Age-Nezness can be divided into two parts: non-indemnome and syndromic forms (Davila-Guttierez, 2002).

Non-embedrome forms are most common (Jeret et al., 1987; SERUR ET AL., 1988). An unknown percentage of cases remains asymptomatic or accidentally detected only due to large sizes. Most patients have a mental delay, convulsions and / or large sizes of the head (Aicardi et al., 1987). Hypelitism is often found. In the study of Jeret et al. (1987) 82% of patients had mental retardation or developmental delay, 43% suffered from convulsions and increased cerebral paralysis in 31%.

However, normal cognitive development was observed in 9 out of 63 children (Bedeschi et al., 2006), and more often, since asymptomatic cases were probably not diagnosed. A seizures of any type are possible, including infantile spasms, but more often focal. Although it is characterized by an increase in the sizes of the head, sometimes more than 5-7 copies of the average, the testimony for shunting is quite strict, since many cases of "hydrocephalius" are spontaneously stabilized, not causing any problems. Macrocephaliya can be partly related to the presence of a giant cyst, located for the third ventricle (Barkovich et al., 2001b).

Specific disorders of the inter imparal transmission are either absent or only minimal (Jeeves and Temple, 1987).

Nevertheless, there are reports of subtle violations of inter impass and topographic memory. In rare cases, endocrinological pathology may be observed (Paul et al., 2003).

Mullet body lipom at an 8-year-old girl with a partial complex of convulsion, but without neurological disorders.
(left) CT: large fatty weights, separated by the front horns of side ventricles, with peripheral calcification and two small lateral areas of the propagation of fat mass.
(centrally) MRI (sagittal projection): the replacement of the corn body with a cloth of lipoma.
(Right) MRI (T 2-weighted sequence): Full replacement of the Kallese body with a fatty tissue.
Mattering body hypoplasia, (left) MRI (axial projection): type of ventricles in accordance with the agent of the corn body.
(in the center) Sagittal projection: a complete corn with knee and roller, but shortened and thinned. Pay attention to the radial location of the medial winding.
(Right) Frontal projection: a wide separation of ventricular bodies with a rolling limbic winding.

Syndromic forms are listed in the table below.

(Chevrie and Aicardi, 1986, Aicardi, 2005) has a ratio of approximately 1% of cases with infantile spasms, probably due to X-clutch dominant mutations. It is found almost exclusively in girls, although there is a message about two cases in boys with the XXY set of chromosomes. It is known only about one family case at two sisters (Molina et al., 1989). Characteristic features of the syndrome include infantile spasms and specific choroidal lacuna, often in combination with a spoolmate of the visual disk. The spine-ripe anomalies are in half cases. Exodus is usually unfavorable, with persistent cramps and deep mental retardation. The spectrum of gravity was wider than previously assumed (Menezes et al, 1994). In rare cases, a corn body may be present (Aicardi, 1994, 1996).

The diagnosis is determined by choroidal lacuna and related anomalies identified with MRI (perivativericular heterotopy, dysplastic cortex, ependimal cysts). In a pathological examination, numerous sections of heterotopia and polyamicrhydrate are found in the brain not divided into layers of type (Billette de villemeur et al., 1992), while the so-called lacquers are thinning the pigment epithelial and vascular layer with the loss of pigment granules. Ependimal cysts often detect around the third ventricle. Cysts or vascular plexus tumors can reach large sizes (Aicardi, 2005). When identifying with the agent of the corn body, a prenatal diagnosis is possible.

Other syndromic forms are rarely found or largely limited to certain ethnic groups.

Askardi syndrome at a three-month girl.
Pay attention to the asymmetry of the hemispheres, bilateral cysts of vascular plexus, cysts around the third ventricle with different signals from and perivativericular heterotopia.
The ependymal origin of the vascular plexus cyst was confirmed with histological examination.
The Agnesia of the TPAP. Ultrasonic Antenatal Research, Sagittal Projection.
Pay attention to the normal fourth ventricle, the lack of echoes from the corpulent body and the expanded side ventricle.
On the left of the photograph - the population population.

Family Agnesia Casual Body Syndrome With pathology of genitals, which can also manifest itself with microcephalus and other ZNS anomalies, is part of a more extensive spectrum of disorders associated with mutations in the ARX gene on chromosome XP22.3 (Hartmann et al, 2004).

Andermann syndrome It was described by French Canadians in the area of \u200b\u200bLake St. Jones (Andermann, 1981), but several cases were stated outside Canada. This syndrome affects the peripheral nervous system in addition to the agent of the corn body or hypotrophy. The agent of the corrosion body is often part of the fined-finger-type I type.

Periodic hypothermia and sweating syndrome (Shapiro et al., 1969) can be effectively treated with the help of a clonidine, which was tested due to the opening of changes in the metabolism of norepinephrine at the same time syndrome. True, half of the cases are not accompanied by Callose Agnesia (Sheth et al., 1994). There are reports of the agent of the corpulatory body with a periodic hyperthermia ("Return Sypiro Syndrome") (Hrayama et al., 1994).

The diagnosis of the Agnesia of the Callose Body is based on neurovalization data. The diagnosis of complete Agensia is uncomplicated at ultrasonography, CT and MPT (Aicardi et al., 1987; Jeret et al., 1987; Serur et al., 1988). MPT is more effective in the diagnosis of partial agenesia. The diagnosis of neurovalization methods does not represent difficulties, with CT or MRI, the rise of the third ventricle and the wide separation of the front horns with the classical picture of the "bull rographs" on the frontal sections is detected. The diffusion-tensor MR (Lee et al., 2005) allows you to identify the deviation of the paths, especially the trigger bundles, which are sent by the stop near the ventricular wall and do not intersect the opposite direction.

Prenatal diagnostics is possible from 22 weeks (Bennett et al., 1996; Simon et al., 2000a). The decision to interrupt pregnancy is difficult to take unconditionally, there is no data on the prevalence of asymptomatic cases. Blum et al. (1990) reported that in 6 out of 12 newborns, in which the agent of the corrosion body was diagnosed with antenatal, had normal development aged 2-8 years. Moutard et al. (2003) 17 cases were observed with prenatally diagnosed insulated anesthesia with repeated measurements of IQ. At the age of 6, all children had a mental development coefficient at a normal level with a trend towards the lower boundary of the norm. Nine children in research Bedeschi et al. (2006) had normal development.

Nevertheless, 2 out of 9 children, prenatally diagnosed with MRI without combined anomalies, there was a delay in development (VOLPE et al., 2006). Cases associated with other malformations or chromosomal anomalies have always had an adverse outcome. Therefore, fetal karyotyping and full examination for the presence of combined malformations of development is extremely important.

Original research

© Dzhaparalieva N.T., Lorin L.V., 2015 UDC 616.832-004.2: 616.831.39

Atrophic changes of the corn body with multiple sclerosis

N.T. Dzhaparalieva, L.V. Lorina Ryazan State Medical University. Acad. I.P. Pavlova, Ryazan

Analyzed changes in the parameters of the corpus body depending on the type of flow, the period of the disease and the degree of disability during multiple sclerosis. The most sensitive in relation to the test indicators was the trunk of a corpulent body, to a lesser degree of knee. Atrophic changes in the knee and the corolla body trunk are progressing with the weighting type of the course of the disease, an increase in the death rate and an increase in the degree of disability. With the same period of the disease of the atrophy of the corrosion body in patients with the primary-progressive course of multiple sclerosis, much stronger is expressed than with other types of flow. A decrease in the size of the corpus body with progressive types of flow indicates the continuing secondary degeneration of fibers.

Keywords: corn body, MRI Morphometry, atrophy, scarm sclerosis.

Scattered sclerosis (PC) is a disease with autoimmune mechanisms, characterized by the appearance of limited zones of inflammation, demyelinization and axonal damage in the central nervous system, which can be detected morphologically and using magnetic resonance imaging (MRI). It is generally recognized by the diffuse damage of the white and gray substance of the central nervous system, leading to the development of atrophy of the head and spinal cord.

The severity of neurological symptoms under RS \u200b\u200bis largely related to the general atrophy of the brain. Currently, the brain atrophy is considered as the most specific marker of the severity of the disease. The morphological and MRI studies showed a relatively early and rapid increasing atrophy of the corn body (MT). In later stages of the disease, marked

reducing the volume of the corpulent body, developing as the disease progressing. The volume of the corpus body correlated with the severity of the disease, while there were no correlations between the severity of atrophy and the floor, the age of patients, durability and age of the beginning of the disease, the type of the flow of multiple sclerosis. The lack of information about the speed and time interval of the development of callosal atrophy since the destruction of the brain substance reduces the accuracy of such studies. In addition, there is an inconsistency of data on the connection of spike atrophy with clinical symptoms with multiple sclerosis. Studies that demonstrated an important role of atrophy in the development of disability of patients with PCs are subject to the need to accurately measure the severity of atrophic changes in the corpulent body. Modern methods

special approaches require special equipment, computer programs and trained personnel, which is possible only in some specialized centers. In this regard, simple linear methods of atrophy evaluation are of great interest.

Objective: With the help of MRT-morphometry techniques, identify changes in the corn body, observed on MRI in patients with multiple sclerosis, and establish the relationship of these changes with the type of flow, the period of the disease and the degree of disability of patients.

Materials and methods

120 patients were examined, of which 46 men (38.3%) and 74 women (61.7%) aged 19 to 65 years old, an average age of 39.74 ± 11.96. At the time of the examination in the age group, up to 30 years were 31 people (25.8%), in the group 31-40 years - 30 patients (25%), aged 41-50 years - 35 patients (29.2%), older 51 years - 24 people (20%). The diagnosis was made to all patients with reliable sclerosis according to McDonald criteria (2005). The debut of multiple sclerosis (DRS) is diagnosed in 12 people (10%), a remitting PC (RRS) -U 53 patients (44.2%), a secondary-progressional PC (PRS) - in 43 patients (35.8%), primary -Rogredient PC (PPRS) - in 12 people (10%). The term of the disease was from 1 to 20 years, an average of 7.89 ± 5.22, with 2/3 of patients (66.7%) had less than 10 years.

The assessment of the neurological status of patients was carried out on the scale of the functional systems of Kurttsque and the analysis of the disabled of EDSS. The average score of the surveyed group of patients on the EDSS scale was 4.11 ± 1.48 points (from 2.0 to 8.0). Based on the degree of disability, 3 groups of patients were allocated: EDSS< 3 баллов (легкая инвали-дизация) - 40 больных (33,3%); EDSS от 3,5 до 5 баллов включительно (умеренная инва-лидизация) - 53 пациента (44,2%); EDSS более 5,5 баллов (выраженная инвалидиза-ция) - 27 человек (22,5%).

MRI in order to confirm the diagnosis was carried out by all 120 patients. MRI

the study was carried out according to the generally accepted method in three projections in the T1, T2 and proton density modes on the Magnetom Symphony apparatus of the company Siemens with a magnetic field voltage of 1.5 T.. The routine description of the structure of the brain and its changes was supplemented with a special study of the sizes of the corpulent body under the protocol developed at the Department of Neurology, Neurosurgery and Medical Genetics of the GBOU VPO Ryazmma of the Ministry of Health of Russia. For a quantitative assessment of the corpus body, a medium-sensitive slice in T1 was used. With the help of a computer programming program for graphic information during morphomet-rii, a calculation of the following circular body sizes (in mm) was calculated: the knee (the distance between the front and rear points of the MT knee), the trunk (the distance between the upper and lower points of the middle third of the barrel MT), roller (Distance between the front and rear points of the MT roller).

Statistical analysis of the results obtained using the SPSS for Windows 13.0 statistical program. For analysis, non-parametric methods were used, since in most samples the distribution of signs did not correspond to the normal distribution. To describe quantitative signs, median (ME), lower and upper quartiles (LQ-UQ) were calculated. Nominal signs are presented as absolute and relative frequencies, mean values \u200b\u200b- in the form M ± m. The accuracy of the differences in two independent samples was determined using the Mann-Whitney criterion. Under R.<0,05 различие считалось значимым. Статистический анализ связи признаков проводился с помощью непараметрического метода корреляции Спирмена.

Results and its discussion

The values \u200b\u200bof the test parameters of the corrosion body as a whole amounted to: knee - 10.0 (8.0-11.0) mm, trunk - 5.0 (4.06.0) mm, roller - 10.0 (9.0-11 0) mm. Statistically significant difference between group

pairies, depending on the floor and age, was not detected.

When analyzing indicators, depending on the type of flow, a uniform decrease in all parameters in relation to the debut was observed, and the decrease was progressed with the weighting type of flow. When analyzing indicators, depending on the term

Morphometric indicators depending on the type of flow, SRO,

diseases and disability of disability also identified a progressive decrease in the test parameters of the corolla body with an increase in the period of the disease and the severity of the patient's condition. The average parameter values \u200b\u200bdepending on the type of flow, the term of the disease and the EDSS score is presented in Table 1.

Table 1

parameters of the corpulent body of the disease and the degree of disability

Me ^ 0-h) me ^ 0-щ) me ^ 0

DRS 13.0 (11.0-13.0) 6.5 (6.0-7.0) 10.0 (10.0-12.0)

The type of RRS 10.0 (9.0-11.0) 5.0 (4.5-6.0) 10.0 (9.0-11.5)

UPRS 9.0 (7.0-11.0) 4.0 (3.0-5.0) 10.0 (9.0-11.0)

PPRS 8.0 (6.0-9.0) 5.0 (4.0-5.9) 9.0 (8.0-11.0)

1 13,0 (11,0-13,0) 6,5 (6,0-7,0) 10,0 (10,0-12,0)

The term of the disease - 2-5 11.0 (10.0-12.0) 6.0 (5.0-6.0) 10.5 (9.0-12.5)

nor 6-10 10.0 (8.7-11.0) 5.0 (4.0-5.0) 11.0 (9.0-11.0)

11-20 8,0 (6,0-9,0) 4,0 (3,0-5,0) 10,0 (7,3-11,0)

EDSS (points) 1-3 8.0 (6.0-9.0) 5.0 (4.0-5.9) 9.0 (8.0-11.0)

3,5-5 10,0 (9,0-11,0) 5,0 (4,0-6,0) 1,10 (9,0-12,0)

5,5-8 8,0 (6,0-10,0) 4,0 (3,0-5,0) 9,0 (8,0-11,0)

When analyzing the parameters of the corpulent body between groups, depending on the type of flow, the following results were obtained. In the group of patients with the debut of the RS, reliable differences were identified (p<0,01) с остальными группами по толщине колена и ствола мозолистого тела, кроме того, имеется достоверное различие (р<0,05) с

Comparative analysis of morphometry depending on the type

a group of patients with PPRS on the thickness of the core body roller. In the group of patients with a remituration RS detected reliable differences (p<0,01) с группами ВПРС и ППРС по толщине колена и ствола мозолистого тела. Достоверных различий между группами пациентов с ВПРС и ППРС не получено (табл. 2).

Table 2 of the Molden Body Parameters of the Disease

Performance of the knee of MT trunk MT roller MT

and r and r and r

Type of flow DRC - RSP 146.0 0.003 125.0 0,001 248.0 0,230

DRS - VDPs 57.5 0,000 40.5 0.000 200,5 0,235

DRS - PPRS 11.5 0.000 16.5 0.001 30.0 0,013

RRS - PRS 736,0 0,003 618.5 0.000 1081.0 0,663

RRS - PPRS 138.0 0,002 256,0 0,291 213,5 0,072

VPRS-PPRS 2015 0.246 176.0 0.085 194.5 0.190

Analysis of the parameters of the corpulent body, depending on the period of the disease, was carried out between the groups of patients with the debut, the remitting and secondary-struggling the course of the PC. The following results are obtained. In the group of patients with the term of the disease

1 year revealed statistically reliable differences with the rest of the knee thickness and the core body thickness, and with an increase in the period of the disease, the reliability of the gap (p<0,05; р<0,001). В группах больных со сроком заболевания от 2 до 5

from 6 to 10 years, the same results were obtained from 6 to 10 years, while there was a direct dependence: with an increase in the difference in terms of the disease between groups, the accuracy of differences increased.

The results of a comparative analysis of indicators between groups, depending on the period of the disease, are presented in Table 3.

Table 3.

Comparative analysis of the morphometric parameters of the corn body depending on the term of the disease

Performance of the knee of MT trunk MT roller MT

Disease term 1 - 2-5 103.0 0.028 83.0 0.004 152.0 0.429

1 - 6-10 61,0 0,001 42,5 0,000 151,5 0,418

1 - 11-20 39,5 0,000 40,0 0,000 145,0 0,086

2-5 - 6-10 297,5 0,021 242,0 0,001 448,0 0,976

2-5 - 11-20 207,5 0,000 205,5 0,000 437,0 0,181

6-10 - 11-20 359,0 0,019 367,5 0,022 434,5 0,170

In connection with the peculiarities of the flow and speed of progression, a group of patients with primary progressive RS was analyzed separately. The average period of the disease in patients with primary -probred flow was 7.25 ± 4.33 years, i.e. Practically coincided with a general average period of the disease. At the same time, a statistically reliable difference (p<0,001; р<0,05) по толщине

the knee, trunk and roller of the corn body between groups of patients with a period of less than 10 years and patients with primary progressional type of flow. Between patients with a period of more than 10 years and patients with primary progressional PC, a significant difference in the test parameters was not detected. The results of the comparative analysis are presented in Table 4.

Table 4.

Comparative analysis of the morphometric parameters of the corn body at different terms of the disease and in the primary progress

Performance of the knee of MT trunk MT roller MT

Disease term 1 - PPRS 11.5 0,000 16.5 0.001 30.0 0,013

2-5 - PPRS 49.5 0.000 109.5 0.039 113.0 0.057

6-10 - PPRS 96,0 0,018 166,0 0,683 105.5 0.033

11-20 - PPRS 194.0 0,598 139.5 0.061 189.5 0,524

When analyzing the parameters of the corpus body between groups, depending on the disability (EDSS score), the following results were obtained. Revealed a statistically reliable difference (p<0,01) между группами пациентов с лёгкой инва-лидизацией и умеренной инвалидизацией по толщине ствола мозолистого тела. Между группами пациентов с инвалидизацией до 5 баллов и выраженной инвалидизацией (свыше 5,5 баллов) отмечены достоверные различия (р<0,001; р<0,05) по всем параметрам. Результаты сравнительного анализа

indicators between groups, depending on the disability, are presented in Table 5.

Correlation analysis of patient groups and morphometric indicators of the corn body. The positive correlations of the weak force between age and the term of illness, age and the degree of persons with disabilities are revealed; The average forces between the term of the disease and the degree of disability. Received negative correlations of weak power between the term of illness

Table 5.

Comparative analysis of the morphometric parameters of the corn body depending on the degree of disability (BBB score)

Performance of the knee of MT trunk MT roller MT

and r and r and r

(points) 1-3 - 3.5-5 921,0 0,275,683,5 0.003 930,5 0,309

1-3 - 5,5-8 228,0 0,000 188,5 0,000 390,5 0,052

3,5-5 - 5,5-8 382,0 0,001 473,5 0,011 460,5 0,009

and roller thickness body; The average force between the cause of the disease, and the thickness of the knee and the bore of the corpulent body, as well as between the degree of disability and the thickness of the knee and the bore of the corpulent body. With correlation analysis of indicators

the corn body among themselves is revealed by strong positive connections between the knee and the corolla body bar and the connection of the lower force between these parameters and the roller of the corn body. The results of correlation analysis are presented in Table 6.

Table 6.

Correlation analysis of groups of patients and morphometric parameters

morale body

Indicators age term Disease knee MT trunk MT roller MT

Age -, 373 (**), 449 (**), 001 -, 095, 123

Term of the disease, 373 (**) -, 586 (**) -, 504 (**) -, 562 (**) - 196 (*)

449 (**) ,586 (**) - -,371 (**) -455 (**) -,150

Knee MT, 001 -, 504 (**) -, 371 (**) -, 656 (**), 588 (**)

Svat MT -, 095 -, 562 (**) -455 (**), 656 (**) -, 562 (**)

Valik MT, 123 -, 196 (*) -, 150, 588 (**), 562 (**) -

Note. Statistical significance of correlations: * - p<0,05, ** - р<0,01

Analyzed changes in the parameters of the corpus body with multiple sclerosis, depending on the type of flow, the period of the disease and the degree of disabilities. The most sensitive in relation to the test indicators was the trunk of a corpulent body, to a lesser degree of knee. A corrosion body roller changed under the action of the specified parameters is minimal. Atrophic changes of the knee and the corrosion bodies are progressing with the weighting of the course of the disease, an increase in the term of the disease and the increase in the degree of disabled disability. The roller of the corrosion body is subjected to significant atrophy only with the primary-progressive flow of multiple sclerosis and, accordingly, a pronounced disability. With the same

nom the term of the disease of the atrophy of the corrosion body in patients with the primary-progressive course of multiple sclerosis is much stronger than with other types of flow. Under the period of the disease more than 10 years and progressional types of the essential difference between the test parameters were not detected. Thus, callosal atrophy is a marker of neurodego-non-defective processes in the white substance of large hemispheres of the brain. The decrease in the size of the corpus body in progressive types of flow indicates the continuing secondary degeneration of the fibers, while in patients with the primary-progressive type of the flow of multiple sclerosis prevails primary progressive diffuse loss of axons. Obtained

the results reflect the processes of primary and secondary degeneration of the corrosion body with multiple sclerosis.

The use of Callozal Morphomet-Rii allows you to objectively assess the degree of severity of atrophic changes in the corpulent body and the rate of development of it-related processes. A quantitative assessment of the parameters of the corpus body can be used to clarify the type of flow and prognostic assessment of the progression of the disease.

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ATROPHIC CHANGES OF THE CORPUS CALLOSUM IN MULTIPLE SCLEROSIS

N.T. DZHAPARALIEVA, L. V. LORINA

The Corpus Callosum, Depending On The Type of Flow, Duration of Disease and Degree Disability in Multiple Saclerosis. Most Sensitive with ReSpect Treunk of the Corpus Callosum, to a leser Extent The Knee. For the Corpus Callosum in Patients of Multiple Progressive Course of Multiple Sclerosis Is Much More Pronounced Thank Flow. Reducing The Size of the Corpus Callosum in Progressive Types of Flow Suggests Ongooing Secondary Degeleration of Fibers.

Keywords: The Corpus Callosum, Mri Morphometric, Atrophy, Multiple SClerosis.

Lorin L.V. - Ph.D., Doc. Departments of neurology, neurosurgery and medical genetics of the GBOU VPO Ryazhma of the Ministry of Health of Russia.

Dzhaparalieva N.T. - Full-time graduate student of the Department of Neurology, Neurosurgery and Medical Genetics GBOU VPO Ryazhm's Ministry of Health of Russia.

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