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Neuropediatrics 2022; 53(02): 136-139
DOI: 10.1055/s-0041-1739134
Short Communication

Stiff Person Syndrome and Encephalitis with GAD Antibodies with Severe Anterograde Amnesia in an Adolescent: A Case Study and Literature Review

Léa Herbulot 
1   Neuropaediatric Unit, Hôpital des Enfants, CHU Toulouse [Children's Hospital, Toulouse University Hospital], Toulouse, France
,
Chloé Bost
2   Immunology Laboratory, IFB [Federal Institute of Biology], CHU Toulouse, Toulouse, France
,
Agnès Viguier
1   Neuropaediatric Unit, Hôpital des Enfants, CHU Toulouse [Children's Hospital, Toulouse University Hospital], Toulouse, France
,
Nathalie Faure-Marie
3   Referral Center for Language and Learning Disorders, Hôpital des Enfants, CHU Toulouse, Toulouse, France
,
Eloïse Baudou
1   Neuropaediatric Unit, Hôpital des Enfants, CHU Toulouse [Children's Hospital, Toulouse University Hospital], Toulouse, France
,
Emmanuel Cheuret
1   Neuropaediatric Unit, Hôpital des Enfants, CHU Toulouse [Children's Hospital, Toulouse University Hospital], Toulouse, France
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Abstract

Antiglutamic acid decarboxylase (GAD65) encephalitis is rare and few pediatric cases have been reported, with variable clinical presentations. A 14-year-old female adolescent was managed in our department. She had been treated for several months for drug-resistant temporal lobe epilepsy and gradually presented major anterograde amnesia with confusion. Upon her arrival at the University Hospital Centre, she showed a classical form of stiff person syndrome. The brain magnetic resonance imaging showed bitemporal hyperintensities and hypertrophy of the amygdala. The blood and cerebrospinal fluid were positive for GAD65 antibodies. At 2 years of immunosuppressive treatment and rehabilitation, the course showed partial improvement of the memory and neuropsychiatric impairment, and epilepsy that continued to be active. GAD65 antibodies are associated with various neurological syndromes, and this presentation combining limbic encephalitis and stiff person syndrome is the first pediatric form published to date; there are also few cases described in adults.

Keywords

antiglutamic acid decarboxylase antibodies - autoimmune encephalitis - stiff person syndrome - anterograde amnesia - child

Supplementary Material



Publication History

Received: 06 July 2021

Accepted: 22 September 2021

Article published online:
14 December 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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