An autopsied case of unclassifiable sporadic four-repeat @entity13859 presenting with XXXX and @entity132 .

@entity1 @entity2003 @entity132 @entity304 @entity146 @entity66 @entity73 @entity5219 @entity575 @entity13859 @entity60977 @entity2845 @entity194 @entity1129 @entity2901 @entity7463 @entity204 @entity528 @entity3668
UNASSIGNED: A 48-year-old Japanese @entity1 experienced slow-onset @entity304 and @entity132 . Neurological examinations revealed @entity2003 in the trunk and extremities, @entity2845 and postural instability, although @entity73 and @entity146 symptoms were not apparent in the @entity66 stage. Neuroimaging revealed progressive bilateral frontotemporal lobe @entity194 with cerebral blood flow hypoperfusion. No apparent signs of lower motor neuron involvement were observed, such as @entity2901 or electromyogram findings. She eventually reached the @entity2845 mutism state, and gastrostomy and tracheotomy were performed at 4   years after onset. A clinical diagnosis of @entity7463 was made prior to her @entity204 , which occurred 6   years after onset. Post mortem examinations revealed that the brain weighed 1200   g and showed @entity194 . Severe @entity528 and @entity5219 were observed in the frontotemporal lobe. The superior and middle frontal gyri were the most severely affected and showed spongiform changes in the superficial layer. The globus pallidus, subthalamic nucleus, cerebellar dentate nucleus, substantia nigra and inferior olivary nucleus also showed @entity528 with @entity5219 . Using hyperphosphorylated tau ( @entity1129 ) immunostaining, pretangle-like neurons, numerous short threads and glial tau pathology were extensively observed. Using Gallyas-Braak @entity575 staining, thin and short threads were also extensively observed, but considerably fewer than those observed by @entity1129 immunostaining. Neither astrocytic plaques nor tuft-shaped astrocytes were observed. Examination by immunoelectron microscopy showed straight fibrils approximately 15   nm in diameter in the neuronal cytoplasmic inclusions in the cerebral cortex and in the fibrillary structures in the cerebral white matter. Western blot analysis of @entity60977 -insoluble tau revealed predominantly four-repeat tau and a banding pattern similar to that seen in @entity7463 . No pathogenic mutations were found during the gene analysis of @entity3668 . After completing our comprehensive investigation, we diagnosed this @entity1 with unclassifiable four-repeat @entity13859 .
