Clinical features of hereditary spastic paraplegia with thin corpus callosum: report of 5 Chinese cases.
BACKGROUND
Hereditary spastic paraplegia is a clinically and genetically heterogeneous group of neurodegenerative disorders of the motor system, characterized by slowly progressive spasticity and weakness of the lower extremities. This study was conducted to investigate the clinical features of hereditary spastic paraplegia with thin corpus callosum (HSP-TCC).


METHODS
Clinical data from five patients and thirty-five previously published case reports of HSP-TCC were analyzed retrospectively.


RESULTS
Most patients were adolescents at the onset of the disease, presenting with spastic paraparesis of the lower limbs and mental impairment. Some patients also had other clinical features, including spasticity of the upper limbs, cerebellar ataxia, and sensory disturbances. Cranial MRIs of the five patients revealed an extremely thin corpus callosum, sometimes with widened cerebral sulci and ventricles, as well as with cerebellar and cerebral atrophy.


CONCLUSIONS
The main clinical features of HSP-TCC include slowly progressive spastic paraplegia, mental impairment during the second decade of life, and an extremely thin corpus callosum as shown on cranial MRIs.
Tang
Bei-sha
BS
Department of Neurology, Xiangya Hospital, Central Southern University, Changsha 410008, China. bstang7398@yahoo.com.cn
Chen
Xin
X
Zhao
Guo-hua
GH
Shen
Lu
L
Yan
Xin-xiang
XX
Jiang
Hong
H
Luo
Wei
W
D000293:Adolescent; D000328:Adult; D061085:Agenesis of Corpus Callosum; D002884:Chromosomes, Human, Pair 15; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D015419:Spastic Paraplegia, Hereditary / Q000235:genetics / Q000473:pathology*