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Abstract Details

A Rare Case of Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) with Hyperreflexia in North America
General Neurology
P1 - Poster Session 1 (5:30 PM-6:30 PM)
4-057
To present a rare case of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) with hyperreflexia in North America.

Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy, classically defined by ascending paralysis, sensory disturbances, and hypo- or areflexia. However, there have been reported cases of GBS with hyperreflexia in Asian and European countries, particularly in association with the axonal variant and/or positive anti-ganglioside antibodies. Magnetic stimulation studies have previously shown evidence of corticospinal tract involvement with significantly delayed central motor conduction time in these patients. In contrast, AIDP, the most prevalent form of GBS in North America, is less known to be associated with hyperreflexia; only a few adult cases have been reported in the literature thus far. 

 

We report a case of a 54-year-old Caucasian male who presented with progressive ataxia and symmetrical, ascending generalized weakness over a 14-day period before reaching a plateau.  His symptoms were preceded by a viral-like illness one week prior.


Case report.
Physical exam was remarkable for diffuse hyperreflexia. MRI spine revealed diffuse nerve root enhancement throughout the cervical and lumbar central canals without radiographic evidence of myelopathy. CSF studies showed albuminocytologic dissociation. EMG/NCS of median and ulnar motor studies showed demyelinating pattern with significantly prolonged distal latencies, conduction slowing, and prolonged F-wave minimal latencies. Classic sural-sparing pattern was also seen. Anti-ganglioside antibodies were negative. The clinic picture was consistent with AIDP, and the patient was treated with IVIG 2g/kg divided into 3 doses. After the third day of treatment, his weakness improved significantly.

Although the majority of the reported cases of GBS with hyperreflexia are associated with an axonal variant and/or positive anti-ganglioside antibodies, an antibody-negative demyelinating variant can also present similarly. It is, therefore, important to raise awareness of such an atypical clinical feature in GBS patients to avoid delays in treatment.


Authors/Disclosures
Xiao Weng, MD (Trinity Health of New England)
PRESENTER
Dr. Weng has nothing to disclose.
Uzma Usman, MD, FAAN (Yale University, Department of Neurology) Dr. Usman has nothing to disclose.
Alison Kostandy, MD (Indiana University, Neuroscience Institute) Dr. Quan has nothing to disclose.
Yazan Al-Hasan, MD, PhD (St. Joseph's Hospital and Medical Center) Dr. Al-Hasan has nothing to disclose.
Justin Hoskin, MD (Barrow Neurological Institute) Dr. Hoskin has nothing to disclose.
Suraj Muley, MD, FAAN (Barrow Neurological Institute) Dr. Muley has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Muley has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Muley has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for CSL Behring. Dr. Muley has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Takeda. Dr. Muley has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Catalyst. Dr. Muley has received publishing royalties from a publication relating to health care.