A Rarity Among the Rare: Psychiatric Manifestations in a Young Woman With Stiff-Person Syndrome.

ABSTRACT

Stiff-person syndrome (SPS) is a rare progressive neurologic disease associated with autoantibodies against glutamic acid decarboxylase, an intracellular enzyme involved in the production of gamma-aminobutyric acid.  We present a case involving a 35-year-old Haitian female who was placed under the Baker Act in the emergency department for suicidal behavior and acute psychosis. She has a history of SPS with a positive glutamic acid decarboxylase (GAD) antibody, a condition most commonly found in females between 20 and 50 years of age. The condition was managed by an outpatient neurologist using diazepam, baclofen, and monthly intravenous immunoglobulin treatments. She also has an extensive history of organic neurological conditions, including traumatic brain injury at 18 years old and COVID-19-related anoxic encephalitis that occurred in December 2020. Both psychiatric and neurological physical exams were completed. They revealed a cerebellar tremor, bilateral ptosis, poor eye contact, decreased concentration, poor insight, depressed mood, flat affect, poor judgement, delusional thoughts and a disorganized thought process with tangential speech. CT and MRI imaging of the brain showed no acute intracranial abnormalities. A quantitative titer of the GAD antibody was completed and shown to be elevated >250IU/mL. Depakote 500mg twice daily and risperidone 3mg twice daily were prescribed. The patient had progressive improvement of psychosis including delusional thoughts over the following five days and was able to be discharged with instructions to follow-up with outpatient neurology.