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Abstract Details

Case Report of Autologous Hematopoietic Stem Cell Transplant in a Patient with Anti-IgLON5 Associated Encephalitis
Autoimmune Neurology
P12 - Poster Session 12 (12:00 PM-1:00 PM)
15-003

NA
Antibodies directed toward the IgLON5 adhesion molecule have been shown to accompany a syndrome of a sleep disturbance including a typically obstructive sleep disordered breathing pattern and a movement disorder that can mimic progressive supranuclear palsy. Other phenomena such as dysautonomia, hyperexcitability and neuropsychiatric disturbance appear prevalent in the affected population.
NA
A 47-year-old gentleman with progressive nocturnal dyspnea, dysphagia, fasciculation, cramping and mild parkinsonian features presented for assessment and treatment at the Ottawa Hospital in Ottawa, Ontario Canada. He was diagnosed with anti-IgLON5 associated encephalitis with antibodies found in serum (Weislab, Sweden). Testing for GAD 65, VGKC, CASPR2, LGI1, NMDAR, and glycine antibodies was negative as was genetic testing for Kennedy disease. The patient’s clinical status deteriorated despite treatment with steroids, IVIg, plasmapheresis, azathioprine and Rituximab and ultimately he underwent autologous hematopoietic stem cell transplant (AHSCT). He tolerated the procedure with few complications which included mild transaminitis, two episodes of pneumonia not requiring hospitalization, and mucositis causing transiently reduced oral intake. Following the procedure, he appeared to clinically stabilize with general subjective improvements including those regarding his sleep. Several symptoms persisted requiring ongoing palliating therapies particularly with regards to gastrointestinal symptoms and muscle cramping.
Knowledge of the spectrum of anti-IgLON5 associated disease is currently evolving. Although some patients have been shown to stabilize with conventional therapy, a tendency toward treatment resistance and associated brain tau accumulation driving an inexorable neurodegenerative process suggest that potent and early therapy may be paramount to patient survival. The presented case exemplifies that bone marrow ablation with AHSCT may be a viable option to halt and even reverse the progression of life-threatening symptoms in IgLON5 associated encephalitis. To our knowledge this is the first patient with IgLON5 associated disease for whom AHSCT was provided.
Authors/Disclosures
John A. Brooks, MD (The Ottawa Hospital)
PRESENTER 		No disclosure on file
Pierre R. Bourque, MD (Ottawa Hospital, Civic Campus) Dr. Bourque has nothing to disclose.
No disclosure on file
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.
Carolina A. Rush, MD Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pendopharm . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi .