TY - JOUR
T1 - Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies
AU - Rakocevic, Goran
AU - Raju, Raghavan
AU - Semino-Mora, Cristina
AU - Dalakas, Marinos C.
N1 - Funding Information:
Supported by the Intramural Research Program of the NIH, National Institute of Neurological Disorders and Stroke.
Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2006/9
Y1 - 2006/9
N2 - We report five of 38 patients with stiff person syndrome (SPS), who also had cerebellar disease, gait ataxia, dysarthria, and oculomotor dysfunction (SPS-Cer). Cerebellar manifestations either preceded SPS or occurred concurrently. Brain MRI was normal. The intrathecal production of glutamic acid decarboxylase antibodies was elevated. γ-Aminobutyric acid-enhancing drugs and immunotherapies improved only the stiffness. SPS-Cer is a distinct subset of SPS causing a more severe and complex clinical phenotype.
AB - We report five of 38 patients with stiff person syndrome (SPS), who also had cerebellar disease, gait ataxia, dysarthria, and oculomotor dysfunction (SPS-Cer). Cerebellar manifestations either preceded SPS or occurred concurrently. Brain MRI was normal. The intrathecal production of glutamic acid decarboxylase antibodies was elevated. γ-Aminobutyric acid-enhancing drugs and immunotherapies improved only the stiffness. SPS-Cer is a distinct subset of SPS causing a more severe and complex clinical phenotype.
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U2 - 10.1212/01.wnl.0000237558.83349.d0
DO - 10.1212/01.wnl.0000237558.83349.d0
M3 - Article
C2 - 17000981
AN - SCOPUS:33748984274
SN - 0028-3878
VL - 67
SP - 1068
EP - 1070
JO - Neurology
JF - Neurology
IS - 6
ER -