Acute autonomic and sensory neuropathy: a case series (P5.303)

Objective: To describe three cases with acute autonomic and sensory neuropathy (AASN).

Background: AASN is considered a very rare variant of immune-mediated acute peripheral neuropathy or ganglionopathy. Only a few cases have been documented in the literature so far.

Design/Methods: Case series.

Results: Three previously healthy subjects (11-year-old male; 11-year-old female; 37-year-old female), all of Asian ancestry, presented with acute and severe sensory and autonomic deficits shortly after a brief gastrointestinal or respiratory febrile illness. Autonomic disturbances included vomiting, diarrhea, anhidrosis, abdominal cramps, neurogenic pain, dry mouth and eyes, non-reactive mid-sized pupils, and dizziness upon standing and syncope. Sensory disorders included decreased perception for all sensory modalities with widespread patchy distribution and significant sensory ataxia. Impaired motor control with dysphagia was interpreted as reduced muscle power and prompted an early diagnosis of Guillain-Barre syndrome. Symptoms progressed for a few days and later stabilized. Neurological examination revealed very depressed or absent deep tendon, corneal, and gag reflexes with preserved muscle power. Corneal exam showed paracentral and inferior corneal opacities due to exposure keratopathy and dry eye. Nerve conduction studies showed extremely reduced or absent sensory nerve action potentials with normal motor nerve conduction and F waves. Cardiovascular autonomic evaluation showed decreased heart rate variability, orthostatic hypotension without compensatory tachycardia and very low or absent plasma norepinephrine levels. Spinal cord MRI showed extensive T2 hyperintensities of the posterior cords. Patient #1 had positive anti-sulfatide antibodies. One year after onset, recovery of sensory impairment was poor, and autonomic dysfunction was ameliorated with symptomatic treatment.

Conclusions: This is the second largest case series ever reported of patients with AASN, an immune-mediated disorder mostly affecting Asian subjects. Patients are usually misdiagnosed, which delays the start of appropriate therapy.

Study Supported by: Dysautonomia Foundation, Inc.

Disclosure: Dr. Gutierrez has nothing to disclose. Dr. Palma has received personal compensation for activities with Lunbeck as a speaker and advisor. Dr. Mendoza-Santiesteban has nothing to disclose. Dr. Kaufmann received personal compensation from Lundbeck and Astra Zeneca for his membership on Scientific Advisory Board.. Dr. Kaufmann received personal compensation from Springer for serving as editor in Chief of Clinical Autonomic Research.. Dr. Kaufmann received support for conducting clinical trials of Droxidopa for Chelsea Therapeutics..