Autonomic dysfunction is a major feature of cerebellar ataxia, neuropathy, vestibular areflexia 'CANVAS' syndrome

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognized neurodegenerative ganglionopathy. Prompted by the presence of symptomatic postural hypotension in two patients with CANVAS, we hypothesized that autonomic dysfunction may be an associated feature of the...

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Published in:	Brain (London, England : 1878) Vol. 137; no. Pt 10; pp. 2649 - 2656
Main Authors:	WU, Teddy Y, TAYLOR, Jennifer M, ANDERSON, Neil E, SNOW, Barry J, ANDERSON, Tim J, PAERMENTIER, Laura A. F, CUTFIELD, Nick J, CHANCELLOR, Andrew M, MOSSMAN, Stuart S, ROXBURGH, Richard H, KILFOYLE, Dean H, SMITH, Andrew D, MCGUINNESS, Ben J, SIMPSON, Mark P, WALKER, Elizabeth B, BERGIN, Peter S, CLELAND, James C, HUTCHINSON, David O
Format:	Journal Article
Language:	English
Published:	Oxford Oxford University Press 01-10-2014
Subjects:	Adolescent Adult Age of Onset Aged Aged, 80 and over Autonomic Nervous System Diseases - complications Autonomic Nervous System Diseases - physiopathology Biological and medical sciences Cerebellar Ataxia - complications Cerebellar Ataxia - physiopathology Cranial nerves. Spinal roots. Peripheral nerves. Autonomic nervous system. Gustation. Olfaction Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Dizziness - physiopathology Female Hand Strength - physiology Heart Rate - physiology Humans Image Processing, Computer-Assisted Magnetic Resonance Imaging Male Medical sciences Middle Aged Nervous system (semeiology, syndromes) Nervous system as a whole Neurologic Examination Neurology New Zealand Nystagmus, Pathologic - etiology Nystagmus, Pathologic - physiopathology Peripheral Nervous System Diseases - complications Peripheral Nervous System Diseases - physiopathology Reflex, Vestibulo-Ocular - physiology Syndrome Valsalva Maneuver Vestibular Diseases - etiology Vestibular Diseases - physiopathology Vestibular Function Tests Vitamin E - blood Young Adult New Zealand Nervous system diseases CANVAS syndrome neuronopathy Dysfunction Internal ear disease Cerebellar ataxia Diseases of the autonomic nervous system autonomic failure ENT disease Vestibular syndrome Neuropathy vestibular failure cerebellar ataxia
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Summary:	Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognized neurodegenerative ganglionopathy. Prompted by the presence of symptomatic postural hypotension in two patients with CANVAS, we hypothesized that autonomic dysfunction may be an associated feature of the syndrome. We assessed symptoms of autonomic dysfunction and performed autonomic nervous system testing among 26 patients from New Zealand. After excluding three patients with diabetes mellitus, 83% had evidence of autonomic dysfunction; all patients had at least one autonomic symptom and 91% had more than two symptoms. We also found a higher rate of downbeat nystagmus (65%) than previously described in CANVAS. We confirmed that sensory findings on nerve conduction tests were consistent with a sensory ganglionopathy and describe two patients with loss of trigeminal sensation consistent with previous pathological descriptions of trigeminal sensory ganglionopathy. Our results suggest that autonomic dysfunction is a major feature of CANVAS. This has implications for the management of patients with CANVAS as the autonomic symptoms may be amenable to treatment. The findings also provide an important differential diagnosis from multiple system atrophy for patients who present with ataxia and autonomic failure.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0006-8950 1460-2156
DOI:	10.1093/brain/awu196