Â鶹´«Ã½Ó³»­

Â鶹´«Ã½Ó³»­

Explore the latest content from across our publications
Join The Â鶹´«Ã½Ó³»­

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Immune mediated radiculoneuropathy associated with serum anti-Lgi-1 autoantibodies
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
062
To describe two cases highlighting a novel association of transient anti-leucine-rich glioma inactivated-1 (Lgi-1) seropositivity with immune mediated radiculoneuropathy of acute-to-subacute onset. 
Anti-Lgi-1 autoantibodies are associated with broad spectrum of central and peripheral nervous system disorders, including small fiber and autonomic neuropathy, autoimmune dementia and limbic encephalitis with a well-established prodrome of faciobrachial dystonic seizures.  While antibodies against the voltage-gated potassium channel (VGKC) complex may lack specificity for neurologic disease, experts in the field consider autoantibodies specific to Lgi-1 protein within VGKC complex, highly specific for neurologic autoimmunity.  
Case 1 is 62-year-old man with rapidly progressive ascending weakness who was noted to have fever, meningismus, diffuse lymphadenopathy and maculopapular rash.  He received broad spectrum antibiotics for possible meningitis. Laboratory workup was notable for eosinophilia, elevated CSF protein with neutrophilic pleocytosis. Imaging revealed diffuse lymphadenopathy.  His quadriparesis progressed and was subsequently treated with IVIG. Electrodiagnostics showed severe, subacute sensory motor axonal neuropathy.  Serum autoantibody testing returned positive for  Lgi-1 antibodies.  Following a course of  intensive rehabilitation he had improvement of strength and spontaneous resolution of rash, leukocytosis, eosinophilia, and lymphadenopathy. Repeat serum testing for anti-Lgi-1 antibodies returned negative. At no point, the patient developed any features of autoimmune encephalopathy/encephalitis.
Case 2 is 73-year-old man with subacute gait instability and ascending pattern of sensory loss. CSF analysis showed albumin-cytological gradient. Electrodiagnostics showed mixed axonal and demyelinating polyneuropathy. Serum autoantibody testing showed elevated striational and Lgi-1 autoantibodies.  He received plasma exchange with improvement of symptoms.  He gradually developed worsening weakness with profound motor and sensory deficits and generalized areflexia. Repeat CSF was comparable to prior. MRI showed diffuse enhancement of nerve roots throughout. Repeat serum testing for Lgi-1 antibodies returned negative. He received corticosteroid and rituximab with resolution of weakness.
Neurologists need to be aware of this unique association.
Authors/Disclosures
Bhavesh Trikamji, MD (University of California Los Angeles)
PRESENTER 		Dr. Trikamji has nothing to disclose.
Leah D. Brancheck, MD (St. Luke's) Dr. Brancheck has nothing to disclose.
Gregory S. Day, MD, MSc, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has or had stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Amgen Pharmaceuticals. The institution of Dr. Day has received research support from AVID Radiopharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with Â鶹´«Ã½Ó³»­. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing Â鶹´«Ã½Ó³»­, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Ionis Pharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Â鶹´«Ã½Ó³»­al Case Development + Presentation (video) with PeerDirect (P\S\L Group). Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development / Presentation (non-CME) with MJH Life Sciences (NeurologyLive). Dr. Day has a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
Bob Bucelli, MD, PhD (Washington University) Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen . Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Bucelli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Regeneron. Dr. Bucelli has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Hamilton Weber. Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for O'Bryan Brown and Toner. Dr. Bucelli has stock in Neuroquestions.com. An immediate family member of Dr. Bucelli has stock in Neuroquestions.com. The institution of Dr. Bucelli has received research support from Biogen. The institution of Dr. Bucelli has received research support from Ionis.