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Abstract Details

Nonsystemic Vasculitic Neuropathy Masquerading as Guillain-Barre Syndrome
Neuromuscular and Clinical Neurophysiology (EMG)
P12 - Poster Session 12 (12:00 PM-1:00 PM)
1-008

To differentiate between vasculitic neuropathies and demyelinating neuropathies. 

ANCA-associated vasculitis is usually a systemic process that affects multiple organs. Our patient presented with an ANCA-associated vasculitis without any evidence of other organ involvement, including the skin and kidneys. He was initially misdiagnosed as having Guillain-Barre Syndrome based on his initial nerve conduction studies and EMG. Additional testing, including repeat NCS/EMG, helped to clinch the diagnosis.

Our patient presented with five days of ascending symmetric weakness following a prodromal illness. On further history, he also had four weeks of sharp pains in his upper extremities. Exam was significant for weakness in bilateral ankle dorsiflexion and plantarflexion with preserved reflexes. NCS/EMG was consistent with multiple patchy areas of conduction block and he was diagnosed with an atypical variant of Guillain Barre Syndrome. He completed a course of IVIG without improvement.

Repeat NCS/EMG was completed which showed a multifocal neuropathy with axonal features. C-ANCA returned markedly positive. Peroneal nerve biopsy and peroneus brevis muscle biopsy showed vasculitic changes. He was treated with high dose steroids and rituximab.

Nonsystemic vasculitidies are a rare but potentially treatable cause of neuropathy. Our patient was initially misdiagnosed with Guillain Barre Syndrome and was treated with IVIG. However, the atypical presentation prompted further workup. His initial NCS/EMG actually showed pseudo-conduction block, which can be seen with early nerve infarction. Without a strong clinical suspicion, vasculitic neuropathies can be easily missed.

Vasculitic neuropathies should be on the differential for demyelinating polyneuropathies, especially with an atypical presentation. 
Authors/Disclosures
Joshua A. Budhu, MD (Memorial Sloan Kettering Cancer Center)
PRESENTER
Dr. Budhu has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Âé¶¹´«Ã½Ó³»­. The institution of Dr. Budhu has received research support from BMSF. The institution of Dr. Budhu has received research support from Commonwealth Fund. The institution of Dr. Budhu has received research support from National Cancer Institute . The institution of Dr. Budhu has received research support from Memorial Sloan Kettering Cancer Center. The institution of Dr. Budhu has received research support from United Hospital Fund. Dr. Budhu has received personal compensation in the range of $500-$4,999 for serving as a Peer Reviewer with Medscape.