Clinical and electrophysiological characteristics of 103 patients with sensory neuronopathy

Lavin, T. and Al-Bachari, Sarah (2015) Clinical and electrophysiological characteristics of 103 patients with sensory neuronopathy. Journal of Neurology, Neurosurgery and Psychiatry, 86 (11). e4. ISSN 0022-3050

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Abstract

Sensory Neuronopathy (SN) represents a distinct peripheral nervous system disorder associated with degeneration of the Dorsal Root Ganglia. We present our retrospective review of 103 patients with an electro-clinical diagnosis of SN. Average age of onset was 54 yr. Aetiologies included Sjogrens (21%), Probable Inflammatory (16%), Idiopathic (29%), Inherited (20%), Toxic (5%) and paraneoplastic (9%). Of those with inherited SN (n=21); CANVAS syndrome 2/21, mitochrondrial cytopathy 9/21 with 4 confirmed POLG1 mutations, 3/21 presumed HSANIIb, Frederich's Ataxia 1/21 and 6/21 unidentified phenotypes. Clinically, acquired causes commonly presented with pain (62%), asymmetrical/non-length dependent sensory disturbance (91%) as compared to the inherited group who are more likely to present with gait disturbance without prominent sensory symptoms (52%). Of the cohort with presumed inflammatory disease, 29 patients were immunosuppressed with 12 patients responding to a combination of steroids and Mycophenolate. IVIG was not found to be beneficial with no sustained benefit in 7 patients. Paraneoplastic causes (n=9) included Breast (n=2), Neuroendocrine tumours (n=2), Carcinoid (n=2), Bowel (n=1), SCLC (n=1) and unknown primary (n=1) with only 4 Hu positive Conclusions Sensory neuronopathy is clinically and aetiologically pleomorphic. In cases with a suspected inflammatory cause it is worthwhile considering a trial of immunomodulatory treatment.

Item Type:
Journal Article
Journal or Publication Title:
Journal of Neurology, Neurosurgery and Psychiatry
Uncontrolled Keywords:
/dk/atira/pure/subjectarea/asjc/1200/1201
Subjects:
ID Code:
126810
Deposited By:
Deposited On:
10 Aug 2018 09:18
Refereed?:
No
Published?:
Published
Last Modified:
07 Apr 2020 05:32