Abstract Details

Title Paraneoplastic Encephalitis Presenting as Subacute Hemidystonia, Ataxia, and Status Epilepticus in a Man with Neurodevelopmental Disorder and a Scrotal Germ Cell Tumor

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (11:45 AM-12:45 PM)

Poster/Presentation
Number 8-010

Objective To present a case of KLHL11-Ma2/Ta Antibody Encephalitis in a man with adult-onset seizures, ataxia, subacute cognitive decline, and hemidystonia found to be antibody positive with a scrotal mixed germ-cell tumor.

Background NA

Design/Methods NA

Results A 35-year-old man with callosal dysgenesis, ventriculomegaly, cerebellar atrophy and baseline cognitive impairment presented with first-time seizures. Initial workup was significant for noninfectious, noninflammatory CSF, negative CSF HSV PCR, and brain MRI revealing known developmental structural abnormalities plus right mesial temporal, thalamic, and hypothalamic FLAIR hyper-intensities thought to represent postictal reactivity. He was diagnosed with epilepsy and discharged on maintenance levetiracetam. He later developed unilateral dystonia, neurocognitive decline, and rapidly-progressive ataxia. He presented to our facility six months later in status epilepticus which resolved with a short course of IV anesthetics. Neurologic exam was notable for right-sided hemidystonia with upper and lower extremity posturing, ataxia, and impairments in executive function and language. Basic CSF studies were noninfectious, and an autoimmune/paraneoplastic antibody panel was sent. He was empirically treated with high-dose IV methylprednisolone for presumed encephalitis. Gender-specific malignancy screening revealed a diffusely-heterogeneous cystic vascular mass of the right testicle for which he underwent right radical orchiectomy. Pathology revealed mixed germ cell tumor. Antibody testing yielded high-reactive CSF KLHL11 antibody IgG and serum Ma2/Ta antibody IgG. He was further treated for paraneoplastic encephalitis with IVIg 2g/kg, for seizures with levetiracetam, and for dystonia with baclofen and diazepam. He improved and was discharged to acute rehabilitation.

Conclusions Seizures and cognitive changes are known clinical manifestations of paraneoplastic encephalitides. Dystonia is often seen in anti-Ma2 encephalitis, while KLHL11 encephalitis often presents with ataxia. While congenital brain malformations often feature epilepsy, intellectual delay, gait abnormalities, and spasticity, the presentation of new-onset unilateral dystonia, cognitive and behavioral changes, and epilepsy in adolescents or adults should prompt autoimmune/paraneoplastic workup.

Authors/Disclosures
Giovanni R. Malaty, MD PRESENTER	Dr. Malaty has nothing to disclose.
Kevin W. Dang, MD	Dr. Dang has nothing to disclose.
Shafeeq Ladha, MD (Barrow Neurological Institute)	Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Ladha has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi. Dr. Ladha has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Ladha has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Annexon. Dr. Ladha has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amicus. Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Neurosense. Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Rapa Therapeutics. Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Ladha has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Sanofi. Dr. Ladha has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Genentech. The institution of Dr. Ladha has received research support from Biogen. The institution of Dr. Ladha has received research support from Sanofi. Dr. Ladha has received publishing royalties from a publication relating to health care.
Michael V. Robers, MD (Barrow Neurological Institute)	Dr. Robers has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Robers has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG therapeutics. Dr. Robers has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Robers has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Robers has received research support from Bristol Myers Squibb Foundation.
Abigail Tittle, NP	Mr. Tittle has nothing to disclose.
Nassim Matin, MD (Barrow Neurological Institute)	Dr. Matin has nothing to disclose.

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