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Abstract Details

CASPR2-IgG associated Autoimmune Seizures and Epilepsy
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
025

To report clinical presentations and outcomes of CASPR2-IgG associated epilepsy.

CASPR2-IgG has been described in association with varied central and/or peripheral nervous system disorders, but clinical characterization of CASPR2-IgG associated epilepsy is limited. 

 Neuroimmunology laboratory database was utilized to identify and review CASPR2-IgG seropositive (CASPR2-IgG+) patients evaluated at our institution (2009-2019).
Of 66 CASPR2-IgG+ patients, 20 had seizures. All patients were male with median age at onset 68 years (range 19-81).  Only 1 patient was found to have malignancy (colon adenocarcinoma).  Two patients had coexisting LGI1-IgG. 18 patients (90%) had seizures at initial presentation. Focal onset seizures (n=17, 85%) with altered awareness (n=13) were common. Focal motor and/or sensory seizures with preserved awareness were reported in 9 patients.  The majority of patients (n=11, 65%) developed secondarily generalized tonic clonic seizures (GTCs) during their disease course three patients (15%) had GTCS with unknown onset. Seizure clusters occurred in 12 patients. Subacute/chronic cognitive dysfunction (n=16, 80%), episodic emotional lability (n=13, 65%), paroxysmal dizziness (n=9, 45%) and episodic gait instability/ataxia (n=6, 30%) were common. Only three patients (15%) had coexisting peripheral nervous system involvement.  Frontotemporal or temporal ictal and/or inter-ictal EEG abnormalities were present among nine patients and three had multifocal epileptiform abnormalities. MRIs were unremarkable except for 3 patients (15%) with limbic involvement. Elevated CSF protein and/or lymphocytic pleocytosis were present in the majority of the cases (13/14, 93%). Fifteen patients received immunotherapy, of which 8 (53%) reached seizure freedom.  Ten patients experienced seizure relapse. Median duration of follow up was 25 months (range 2-136 months).
CASPR2-IgG evaluation should be considered among older male patients with new focal-onset seizures with/without secondary generalization and coexisting episodic emotional lability, paroxysmal dizziness or episodic ataxia. Early diagnosis and immunotherapy initiation may lead to favorable seizure outcomes among these cases.
Authors/Disclosures
Emilio R. Garrido Sanabria, MD, PhD (Physicians Regional Medical Center)
PRESENTER 		Dr. Garrido Sanabria has nothing to disclose.
Anza Zahid, MD, MBBS (Houston Methodist Hospital) Dr. Zahid has nothing to disclose.
Jeffrey W. Britton, MD, FAAN (Mayo Graduate School of Medicine) Dr. Britton has received personal compensation in the range of $0-$499 for serving as a Online course with American Clinical Neurophysiology Society.
Shailee S. Shah, MD Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Shah has received publishing royalties from a publication relating to health care.
Alfonso S. Lopez, MD (Mayo Clinic) Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech .
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
John R. Mills, MD, PhD (Mayo Clinic) The institution of Dr. Mills has received research support from Werfen Diagnostics. Dr. Mills has received intellectual property interests from a discovery or technology relating to health care.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.