title: Autonomic dysfunction is a major feature of cerebellar ataxia, neuropathy, vestibular areflexia 'CANVAS' syndrome.
abstract: 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.
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Teddy Y
authors: TY
authors: Wu
authors: 2 Department of Neurology, Wellington Hospital, Riddiford Street, Wellington 6021, New Zealand.
authors: Jennifer M
authors: JM
authors: Taylor
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Dean H
authors: DH
authors: Kilfoyle
authors: 4 Department of Radiology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Andrew D
authors: AD
authors: Smith
authors: 4 Department of Radiology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Ben J
authors: BJ
authors: McGuinness
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Mark P
authors: MP
authors: Simpson
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Elizabeth B
authors: EB
authors: Walker
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Peter S
authors: PS
authors: Bergin
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: James C
authors: JC
authors: Cleland
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: David O
authors: DO
authors: Hutchinson
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Neil E
authors: NE
authors: Anderson
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
authors: Barry J
authors: BJ
authors: Snow
authors: 5 Department of Medicine, University of Otago, Christchurch, 2 Riccarton Avenue, Christchurch 8140, New Zealand 6 New Zealand Brain Research Institute, 66 Stewart Street, Christchurch 8011, New Zealand.
authors: Tim J
authors: TJ
authors: Anderson
authors: 6 New Zealand Brain Research Institute, 66 Stewart Street, Christchurch 8011, New Zealand.
authors: Laura A F
authors: LA
authors: Paermentier
authors: 7 Department of Medicine and Brain Health Research Centre, University of Otago, Dunedin, PO Box 56, Dunedin 9054, New Zealand.
authors: Nick J
authors: NJ
authors: Cutfield
authors: 8 Department of Neurology, Tauranga Hospital, 829 Cameron Road, Tauranga 3112, New Zealand.
authors: Andrew M
authors: AM
authors: Chancellor
authors: 2 Department of Neurology, Wellington Hospital, Riddiford Street, Wellington 6021, New Zealand.
authors: Stuart S
authors: SS
authors: Mossman
authors: 1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand 3 Centre for Brain Research, University of Auckland, 85 Park Road, Auckland 1142, New Zealand richardr@adhb.govt.nz.
authors: Richard H
authors: RH
authors: Roxburgh
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001342:Autonomic Nervous System Diseases / Q000150:complications / Q000503:physiopathology*; D002524:Cerebellar Ataxia / Q000150:complications / Q000503:physiopathology*; D004244:Dizziness / Q000503:physiopathology; D005260:Female; D018737:Hand Strength / Q000502:physiology; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D009520:New Zealand; D009759:Nystagmus, Pathologic / Q000209:etiology / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000503:physiopathology*; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D013577:Syndrome; D014636:Valsalva Maneuver; D015837:Vestibular Diseases / Q000209:etiology / Q000503:physiopathology*; D014724:Vestibular Function Tests; D014810:Vitamin E / Q000097:blood; D055815:Young Adult
chemical_list: D014810:Vitamin E
keywords: CANVAS syndrome; autonomic failure; cerebellar ataxia; neuronopathy; vestibular failure