[An autopsy case of XXXX preceded by a urinary disturbance for over 20 years].

@entity1 @entity2011 @entity2003 @entity427 @entity1494 @entity16 @entity400 @entity194 @entity76 @entity1576 @entity179 @entity551 @entity4820 @entity11460 @entity2055 @entity4653
An autopsy case of @entity11460 preceded by urinary disturbance for over 20 years was reported. A 43-year-old @entity1 was admitted to our hospital because of urinary disturbance and @entity551 . At the age of 19 she developed urinary disturbance with @entity2011 and retention. These symptoms were getting worse with years, and at the age of 33 she was diagnosed to have @entity1494 of uninhibited type. During her hospital course her symptom became worse, and by the age of 42 she showed marked @entity4653 , disturbance of smooth pursuit eye movement, @entity4820 , marked @entity2003 and @entity400 of four extremities, generalized @entity427 , marked limb and @entity2055 , @entity1494 and @entity551 . Serial brain CT scan revealed progressive brain stem and @entity76 with clinical course. Severe @entity16 were also documented. She died of @entity1576 at the age of 43. On autopsy, brain stem and cerebellum showed marked @entity194 macroscopically. Microscopically marked depletion of neuron was seen in the substantia nigra, pontine nuclei, inferior olive, Purkinje cells, the intermediolateral column of spinal cord and Onuf's nucleus of S2. Although numerous cases of @entity11460 have been reported in the past, there is no case which developed this syndrome after urinary disturbance of over 20 year's duration. We should be alert to observe the cases with longstanding urinary disturbances in order to not overlook @entity179 as exemplified in this case.
