Format

Send to

Choose Destination
Brain. 2014 Oct;137(Pt 10):2649-56. doi: 10.1093/brain/awu196. Epub 2014 Jul 28.

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

Author information

1
1 Department of Neurology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
2
2 Department of Neurology, Wellington Hospital, Riddiford Street, Wellington 6021, New Zealand.
3
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.
4
4 Department of Radiology, Auckland City Hospital, 2 Park Road, Auckland 1023, New Zealand.
5
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.
6
6 New Zealand Brain Research Institute, 66 Stewart Street, Christchurch 8011, New Zealand.
7
7 Department of Medicine and Brain Health Research Centre, University of Otago, Dunedin, PO Box 56, Dunedin 9054, New Zealand.
8
8 Department of Neurology, Tauranga Hospital, 829 Cameron Road, Tauranga 3112, New Zealand.
9
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.

Erratum in

  • Brain. 2015 Feb;138(Pt 2):e329.

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.

KEYWORDS:

CANVAS syndrome; autonomic failure; cerebellar ataxia; neuronopathy; vestibular failure

PMID:
25070514
DOI:
10.1093/brain/awu196
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Silverchair Information Systems
Loading ...
Support Center