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
