Abstract Details

Title A Young Man with a Twitching Arm

Topic Movement Disorders

Presentation(s) P02 - (-)

Poster/Presentation
Number 073

Objective

Background BACKGROUND: Recently, there has been an increasing number of reports in the literature of CNS disorders associated with auto-antibodies.

Design/Methods DESIGN/METHODS: Case report.

Results RESULTS: A 30-year-old man was admitted to the hospital for a generalized tonic-clonic seizure. After the admission he started to develop abnormal movements of the right arm, with tightening of the right shoulder and elbow and involuntary twitching of the right hand, in addition to slowed speech, word-finding difficulties and twitching of the lips. These episodes recurred every 15-20 minutes, lasting about 10 seconds. Electroencephalogram was normal. Cerebrospinal fluid analysis was unremarkable. A magnetic resonance imaging (MRI) of the brain revealed a gadolinium-enhancing lesion involving the left caudate and globus pallidus. The peculiar semeiology of the crisis and the poor response to anti-epileptic drugs prompted us to test for Leucine-rich glioma inactivated protein 1 (LGI-1) antibodies that were detected in patient's serum. The patient underwent to five cycles of plasmapheresis with complete resolution of seizures. After plasmapheresis, oral prednisone (1 mg/Kg/day) was begun. A new brain MRI showed reduction of contrast enhancement of the lesion previously described.

Conclusions CONCLUSIONS: Clinical features of patient's crisis characterized by facial twitching, dysarthria and hand posturing resembled "faciobrachial dystonic seizures", a recently described entity that occurs almost exclusively in association with LGI-1 antibodies1. The recognition of this clinical phenotype is critical because the response to anti-epileptic drugs is unsatisfactory while response to immunotherapy is excellent. Moreover early initiation of immunosuppressant therapy offers the chance to modify the course of this neurological disorder avoiding the development of full-blown limbic encephalitis. Here we document also that contrast-enhancing lesions involving basal ganglia can be associated with FBDS and LGI-1 antibodies.

Authors/Disclosures
Domenico Plantone PRESENTER	No disclosure on file
	No disclosure on file
Mitchell S. Elkind, MD, MS, FAAN	Dr. Elkind has received personal compensation for serving as an employee of American Heart Association. Dr. Elkind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Atria Academy.
	No disclosure on file
	No disclosure on file
Raffaele Iorio, MD (Catholic University)	Dr. Iorio 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. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dianthus Therapeutics. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Johnson&Johnson.

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