pyramidal sign

Clinical features include bilateral pigmentary degeneration of the retina, optic atrophy and progressive dementia, and lay in bed after several years with convulsive seizure, opisthotonus, limb rigidity, hyperreflexia, pyramidal sign positive;

主要表现为视网膜色素变性、视神经萎缩、进行性痴呆，数年后完全卧床，伴有抽搐发作，角弓反张、肢体强直、反射亢进，和双侧病理征阳性；

Progressive cerebellar ataxia was the main clinical symptom, other manifestations included extrapyramidal system and pyramidal tract sign and orthostatic hypotension. MRI showed atrophy olf cerebellum and brainstem.

临床表现为进行性小脑性共济失调，可伴有锥体外系症状，锥体束征及直立性低血压。

The clinical presentation included weakness of lower limbs in 17 cases, ambulation dysfunction in 23 cases, paraparesis in 13, pyramidal sign in 13 and urinary incontinence in 5 cases.

临床表现：双下肢无力17例，行走困难23例，损伤平面以下感觉减退13例，锥体束受损表现13例，括约肌功能障碍5例。