Abstract
Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a slowly progressive ataxic disorder characterized by the triad of cerebellar impairment, bilateral vestibular hypofunction, and sensory polyneuropathy. The paper describes a clinical case of this syndrome in a 40-year-old patient who has been followed up by hematologists for polycythemia vera for 10 years.
Highlights
A clinical case of cerebellar ataxia with neuropathy and vestibular areflexia syndrome in the presence of polycythemia vera
Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a slowly progressive ataxic disorder characterized by the triad of cerebellar impairment, bilateral vestibular hypofunction, and sensory polyneuropathy
The first description of a syndrome characterized by late onset axial and limb ataxia in patients with bilateral vestibular deficit with impaired visually enhanced visual vestibular ocular reflex (VVOR), and cerebellar dysfunction did not include peripheral neuropathy[10]
Summary
Safin Sh.M., Bakirov B.A., Derevyanko Kh.P., Baikov D.E., Khafizov M.M. Bashkir State Medical University, Ministry of Health of Russia, Ufa, Russia 3, Lenin St., Ufa 450008. Intraocular pressure is 14/15 mm Hg. Cerebellar ataxia with neuropathy and vestibular areflexia (CANVAS syndrome) was diagnosed taking into account the course of the disease with polyneuropathic syndrome (currently represented by mild distal paraparesis of lower limbs, hypoesthesia in the distal parts of all limbs, autonomic dysfunctions of the peripheral type in the distal parts of all limbs confirmed twice according to ENMG), the development of moderately pronounced static ataxia syndrome with cerebellar degeneration detected by MRI of the brain and clinically manifested as a distinct impaired gait (combination of cerebellar and sensitive ataxia), the main patient's complaint of inability to walk outside and to cross the road due to the development of lightheadedness with blurred vision (the patient with a slight distal paraparesis of legs moves with a cane for support and sure steps) , decreased vestibulo-ocular reflex. According to laboratory data positive dynamics was revealed represented by a decrease in the level of red blood cells – up to 4.03⋅1012/l, hemoglobin – up to 132 g/l, hematocrit – up to 41.1%, a decrease in platelet count to 394⋅109/l
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