scholarly journals Respective implications of glutamate decarboxylase antibodies in stiff person syndrome and cerebellar ataxia

2011 ◽  
Vol 6 (1) ◽  
pp. 3 ◽  
Author(s):  
Mario U Manto ◽  
Christiane S Hampe ◽  
Veronique Rogemond ◽  
Jerome Honnorat
PM&R ◽  
2016 ◽  
Vol 8 (9) ◽  
pp. S278-S279
Author(s):  
Nicole A. Strong ◽  
Nicholas F. Love ◽  
Kristen M. Brusky ◽  
Sara Salim

2011 ◽  
Vol 54 (4) ◽  
pp. 170-174 ◽  
Author(s):  
Edvard Ehler ◽  
Jan Latta ◽  
Petra Mandysová ◽  
Jana Havlasová ◽  
Milan Mrklovský

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.


2001 ◽  
Vol 40 (9) ◽  
pp. 968-971 ◽  
Author(s):  
Satoshi KONO ◽  
Hiroaki MIYAJIMA ◽  
Masahiro SUGIMOTO ◽  
Yoji SUZUKI ◽  
Yoshitomo TAKAHASHI ◽  
...  

2012 ◽  
Vol 70 (9) ◽  
pp. 657-661 ◽  
Author(s):  
Maurício Fernandes ◽  
Renato P. Munhoz ◽  
Paulo Eduardo Mestrinelli Carrilho ◽  
Walter O. Arruda ◽  
Paulo J. Lorenzoni ◽  
...  

Neurological disorders associated with glutamic acid decarboxylase (GAD) antibodies are rare pleomorphic diseases of uncertain cause, of which stiff-person syndrome (SPS) is the best-known. Here, we described nine consecutive cases of neurological disorders associated with anti-GAD, including nine patients with SPS and three cases with cerebellar ataxia. Additionally, four had hypothyroidism, three epilepsy, two diabetes mellitus and two axial myoclonus.


2019 ◽  
Vol 9 (0) ◽  
Author(s):  
Lliwen A. Jones ◽  
Waqaar Baber ◽  
Mark Wardle ◽  
Neil P. Robertson ◽  
Huw R Morris ◽  
...  

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sinali O. Seneviratne ◽  
Katherine A. Buzzard ◽  
Belinda Cruse ◽  
Mastura Monif

Anti-GAD antibody syndrome is a result of the production of antibodies against glutamic acid decarboxylase (GAD), the main enzyme responsible for the production of gamma-aminobutyric acid (GABA). Several neurological manifestations including cerebellar ataxia and stiff person syndrome have been reported in association with anti-GAD antibodies. In this paper, we present a case of a young woman with anti-GAD antibodies who initially presented with cerebellar ataxia followed by stiff person syndrome three and a half years later. Having both cerebellar ataxia and stiff person syndrome is a rare occurrence in anti-GAD antibody syndrome. We emphasise the importance of long-term follow-up of patients with anti-GAD antibody syndrome, as delayed neurological manifestations can occur.


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