@entity4943 with ubiquitinated neuronal inclusions presenting with @entity4681 and XXXX : clinicopathological report of an autopsy case.

@entity1 @entity2003 @entity322 @entity304 @entity146 @entity55 @entity303 @entity4681 @entity2845 @entity1174 @entity2915 @entity194 @entity1315 @entity4943 @entity746 @entity2777 @entity2901 @entity204
We report a case displaying upper motor sign, @entity304 , and @entity146 , with marked degeneration of the precentral cortex, neostriatum and frontotemporal lobes, as well as ubiquitinated neuronal inclusions. The @entity1 was a 66-year-old male at the time of @entity204 . At age 57, he noticed progressive difficulties in speaking and swallowing. At age 60, he was severely anarthric and displayed @entity2777 and @entity322 . Neurologically, very poor movement of tongue was observed, but without @entity194 or @entity2901 . Deep tendon reflexes were @entity2915 . Grasp reflex and snout reflex were also positive. Needle electromyography revealed no abnormalities. A diagnosis of @entity4681 and character change was made. At age 62, he developed @entity2845 and @entity2003 of the neck and all extremities. Treatment with carbidopa-levodopa was initiated, but resulted in minimal improvement. At age 65, he was bed-ridden, and had repeated occurrences of @entity1174 ; he died of @entity55 . Neuropathological examination revealed marked @entity1315 with Betz cells completely absent and moderate @entity194 . The spinal cord and nerve roots appeared normal. Immunohistochemically, ubiquitin-positive but tau-negative intraneuronal inclusions were found in the frontal and temporal cortices, including the precentral cortex and the hippocampal dentate gyrus, and the neostriatum. This case could be included with inclusion-associated disorders such as @entity4943 or @entity746 with @entity303 , and furthermore, predominant upper motor sign and @entity304 could represent phenotypes of clinical manifestations with such inclusions.
