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It is unknown how often patients with sensory neuronopathy (SNN) present with a distal symmetric polyneuropathy (DSP) phenotype. In these cases, electrodiagnostic testing may discriminate SNN with a DSP phenotype from DSP. We reviewed the records of patients who met SNN diagnostic criteria between January 2000 and February 2021 and identified patients with a DSP phenotype at the time of electrodiagnosis. Sixty-two patients fulfilled SNN diagnostic criteria. At symptom onset, 20 (32.2%) patients presented with distal symmetric sensory symptoms limited to the feet. However, most progressed rapidly over 6 months or developed asymmetric symptoms. At the time of electrodiagnosis, only seven (11.3%) patients had a DSP phenotype. Of these seven patients, four had cerebellar ataxia with neuropathy and vestibular areflexia syndrome, one had vitamin B6 deficiency, one was thought to be alcohol-induced, and one was idiopathic. Patients with SNN rarely present with a DSP phenotype at the time of electrodiagnosis. The finding that one third of cases resemble DSP at onset highlights the importance of clinical monitoring. In patients with a DSP phenotype, the presence of ataxia at onset or significant progression within 6 months may suggest the possibility of SNN and should prompt additional investigations, such as electrodiagnosis. © 2022 Wiley Periodicals LLC.


Long Davalos, Dustin G Nowacek, Zachary N London. Distal symmetric polyneuropathy phenotype in patients with sensory neuronopathy at the time of electrodiagnosis. Muscle & nerve. 2022 Apr;65(4):456-459

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PMID: 34994975

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