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.