Spinocerebellar ataxia-10 with paranoid schizophrenia.
Spino-cerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder that is characterized by cerebellar ataxia, seizures and nystagmus with a fragmented pursuit. Schizophrenia has been reported with SCAs 1 and 2 yet in SCA 10, psychiatric manifestations are uncommon. We report a Hispanic family involving a father and his four children with SCA10 genetic mutation. Two of his children, a 20-year-old female and a 23-year-old male, presented with gradually progressive spino-cerebellar ataxia and paranoid schizophrenia. Neurological examination revealed ocular dysmetria, dysdiadokinesia, impaired finger-to-nose exam, gait ataxia and hyperreflexia in both the cases. Additionally, they had a history of psychosis with destructive behavior, depression and paranoid delusions with auditory hallucinations. Serology and CSF studies were unremarkable and MRI brain revealed cerebellar volume loss. Ultimately, a test for ATAXIN-10 mutation was positive thus confirming the diagnosis of SCA10 in father and his four children. We now endeavor to investigate the association between schizophrenia and SCA10.
Department of Neurology, Olive View Medical Center, Sylmar, USA.
Bhavesh
B
Trikamji
Department of Neurology, Harbor Medical Center, Torrance, USA.
Parampreet
P
Singh
Department of Neurology, Keck School of Medicine, University of Southern California, Los Angeles, USA.
Shrikant
S
Mishra
Ataxia; SCA10; cerebellum; schizophrenia; seizures