Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia

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• 作者：Shigeto Nagao^a ; ^b ; Osamu Yokota^b ; ^{oyokota1@yahoo.co.jp} ; Reiko Nanba^c ; Hiroshi Takata^d ; Takashi Haraguchi^a ; Hideki Ishizu^e ; Chikako Ikeda^b ; Naoya Takeda^b ; Etsuko Oshima^b ; Katsuaki Sakane^b ; Seishi Terada^b ; Yuetsu Ihara^a ; Yosuke Uchitomi^b
• 关键词：Motor neuron disease ; Primary lateral sclerosis ; Progressive supranuclear palsy ; Pseudobulbar palsy ; Pyramidal signs
• 刊名：Journal of the Neurological Sciences
• 出版年：2012
• 出版时间：15 December, 2012
• 年：2012
• 卷：323
• 期：1-2
• 页码：147-153
• 全文大小：1789 K

文摘

We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.