Severe Acute Axonal Polyneuropathy in the Setting of Nutritional Deficiency (P3.152)

Abstract

OBJECTIVE: Retrospective analysis of patients presenting with acute axonal neuropathy and nutritional deficiency resembling Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP). BACKGROUND: An AIDP-like syndrome has been described in patients presenting with acute axonal neuropathy and normal CSF associated with protracted emesis and weight loss after bariatric surgery or alcohol exposure. The nutritional mechanism is unclear. METHODS: Retrospective description and analysis of 6 patients presenting with acute axonal neuropathy, vomiting and nutritional deficiency. RESULTS: 6 patients (mean age 35 years) presented with a severe, progressive, symmetric, often painful, sensory or sensorimotor polyneuropathy over 2-4 weeks with sensory ataxia, areflexia, variable muscle weakness but no ophthalmoparesis. All patients had poor nutritional status and prolonged vomiting with weight loss. 3 patients were consuming alcohol extensively; 1 had undergone bariatric surgery. Electrodiagnostic testing revealed a severe sensory or sensorimotor axonal neuropathy. CSF obtained in 3 patients was normal. Laboratory work-up revealed decreased prealbumin (n=5), low vitamin B6 levels (n=4), elevated MCV (n=4), low-normal copper (n=1). Vitamin B1 and B12 were normal in all patients. Other metabolic derangements included hyponatremia, hypochloremia, hypokalemia, elevated liver function tests, and metabolic acidosis, due to dehydration and vomiting. Nerve biopsy in 1 patient confirmed axonal loss without demyelination or vasculitis. Most patients were treated with thiamine (n=5), multi-vitamin tablets (n=4), copper supplementation (n=1), vitamin B6 (n=1) and immunoglobulin (n=1). Four months later 4 of the 6 patients available for follow up were improved, but all had residual disability.CONCLUSIONS: This case series describes an AIDP-like, acute, severe, axonal sensory or sensorimotor polyneuropathy in association with prolonged vomiting, weight loss, and poor nutritional status. Vitamin B6 was low in the majority of cases but is unlikely the only deficiency. It is hoped that greater recognition and systematic analysis of this neuropathy will identify the critical nutritional causes to guide optimal treatment.

Disclosure: Dr. Hamel has nothing to disclose. Dr. Logigian has received personal compensation for activities with Genzyme Corporation as an consultant.