MULTIPLE SYSTEM ATROPHY, CEREBELLAR VARIANT: RARE CLINICAL CASE

Abstract

Introduction: Multisystem atrophy is a rare steadily progressing neurodegenerative disease, manifested by parkinsonism, cerebellar ataxia and autonomic failure. The cerebellar variant of multisystem atrophy is much less common and, when the cerebellar syndrome is dominant, this category of patients is hospitalized under the code for acute cerebrovascular accident in the stroke center. The manifestation of orthostatic syndrome or syncope is explained by the pathology of the main arteries of the head. Methods: general clinical tests, neurological examination with an orthostatic test, brain MRI Results: Patient J. 1951, Kazakh, was admitted to the department of a stroke center with a preliminary diagnosis: vertebrobasilar stroke. At admission, the patient complained of dizziness, unsteadiness, worse in the upright position, the speech, two days later joined pyramidal insufficiency and incontinence. In neurological status: conscious, post-traumatic amaurosis on the right, left pupil of the correct form, photoreaction preserved, bilateral coarse horizontal nystagmus with rotator component noted. Asymmetry of the nasolabial folds, dysarthria. Muscle tone in the limbs is reduced, more in the legs, no paresis, tendon reflexes with an emphasis on the left with inconsistent pathological stop signs on the right. Abdominal reflexes are invoked. Sensitive disorders are not identified. Positive orthoclinostatic test: arterial pressure in a horizontal position 140/100 mm Hg, in a vertical position 80/60 mm Hg. with a tendency to bradycardia (58 per min). In the Romberg position is unstable, deviates in all directions. The coordination tests from 2sides are a rough intentional tremor, mimicking, more pronounced on the left. Pelvic disorders in the form of urinary incontinence. Brain MRI without contrast - foci of brain microangiopathy. Results: In neurological status, pyramidal, cerebellar syndrome, Shay-Draeger syndrome, pelvic disorders of the type of urinary incontinence, the absence of bulbar disorders and focal changes in brain MRI were detected, and neurodegenerative disease of the central nervous system was detected. Conclusion: Neurodegenerative disease of the central nervous system. Multisystem atrophy, cerebellar variant. Cerebellar ataxia. Shay-Drager Syndrome. Moderate pyramidal insufficiency, more pronounced on the left. Pelvic disorders in the form of urinary incontinence.