title: Stiff-person syndrome following tick-borne meningoencephalitis.
abstract: Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS.
authors.affiliation: Department of Neurology, Pardubice Regional Hospital, Pardubice, Czech Republic. edvard.ehler@nemocnice-pardubice.cz
authors.forename: Edvard
authors.initials: E
authors.lastname: Ehler
authors.forename: Jan
authors.initials: J
authors.lastname: Latta
authors.forename: Petra
authors.initials: P
authors.lastname: Mandysová
authors.forename: Jana
authors.initials: J
authors.lastname: Havlasová
authors.forename: Milan
authors.initials: M
authors.lastname: Mrklovský
mesh_terms: D004576:Electromyography; D004675:Encephalitis, Tick-Borne / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008590:Meningoencephalitis / Q000150:complications*; D008875:Middle Aged; D016750:Stiff-Person Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*