Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I |

Acute Descending Paralysis Secondary to Variant GBS FREE TO VIEW

Sara Tepe, BA; Tabitha Ku, MD; Rajasekhar Mulyala, MD
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University of Missouri-Columbia, Columbia, MO

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1080A. doi:10.1016/j.chest.2016.08.1187
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Guillain-Barré Syndrome (GBS) is an acute monophasic immune-mediated polyradiculopathy. Neuropathic or radicular pain and dysautonomia are common features in all forms of GBS. Phenotypic variants are hypothesized to be mediated by molecular mimicry targeting peripheral nerve motor axons, with resulting weakness deviating from the classic symmetric “ascending” pattern. Weakness can range from mild to severe flaccid quadriplegia, and respiratory failure within days of onset. Urinary retention and gastrointestinal dysfunction are seen in approximately 5% of variant cases. A spectrum of clinical features necessitates the use of laboratory testing and diagnostic modalities to exclude mimics and confirm a diagnosis of GBS. A high clinical suspicion must exist as GBS responds to treatment with plasmapheresis or IVIG. Our patient embodied both a diagnostically challenging presentation and clinical response consistent with variant GBS.

CASE PRESENTATION: 63 year old male with history of alcoholic cardiomyopathy, non small cell lung cancer s/p partial lobectomy, chronic cavitary aspergilliosis, MAC s/p treatment, and chronic adrenal insufficiency. He presented to the ED because of chest pain and dyspnea, and was admitted for further management of PE. He denied fevers, chills, rash, cough, edema, or recent diarrhea. His hospital course was complicated by rapid development of dysphagia, descending areflexic quadriparesis, urinary retention, decreased rectal tone, and diaphragmatic weakness requiring transfer to MICU for intubation. Cervical imaging was unremarkable; MRI was negative for ischemic spinal cord injury. LP was not obtained due to concurrent anticoagulation. IVIG was empirically given for suspected variant GBS. Initial EMG suggested axonal loss and segmental demyelination. EMG after treatment was remarkable for normal phrenic nerve function and recovering motor response, confirming the diagnosis. He recovered diaphragmatic function and was eventually discharged to a rehabilitation facility.

DISCUSSION: Determination of GBS variants is based on the types of nerve fibers involved, predominant mode of fiber injury and evidence or absence of alteration in consciousness. The pharyngeal-cervical-brachial motor variant manifests with a descending paralysis, mimicking botulism. Treatment strategies vary depending on the breadth and severity of weakness and often involve general supportive care. Indications for plasmapheresis or IVIG include weakness impairing function, or respiratory involvement.

CONCLUSIONS: In patients who present with rapid progression of descending paralysis, variant GBS should be considered amongst the differential.

Reference #1: Dimachkie MM, Barohn RJ. Guillain-Barré Syndrome and Variants. Neurologic clinics. 2013;31(2):491-510. doi:10.1016/j.ncl.2013.01.005.

Reference #2: Wakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. Journal of Neurology, Neurosurgery and Psychiatry. 2014;85(3):339-44. doi: 10.1136/jnnp-2013-305397.

DISCLOSURE: The following authors have nothing to disclose: Sara Tepe, Tabitha Ku, Rajasekhar Mulyala

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