TY - JOUR
T1 - Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia
AU - Nagao, Shigeto
AU - Yokota, Osamu
AU - Nanba, Reiko
AU - Takata, Hiroshi
AU - Haraguchi, Takashi
AU - Ishizu, Hideki
AU - Ikeda, Chikako
AU - Takeda, Naoya
AU - Oshima, Etsuko
AU - Sakane, Katsuaki
AU - Terada, Seishi
AU - Ihara, Yuetsu
AU - Uchitomi, Yosuke
N1 - Funding Information:
We would like to thank Ms. Onbe (Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences) for her excellent technical assistance. Brain tissues were obtained from the Research Resource Network, which is supported by a research grant from the Neurological and Mental Disorders from the Ministry of Health, Labour and Welfare, Japan. This work was supported in part by grants from the Japanese Ministry of Education, Culture, Sports, Science and Technology (No. 21591517 , No. 23591708 ), and the Zikei Institute of Psychiatry .
PY - 2012/12/15
Y1 - 2012/12/15
N2 - 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.
AB - 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.
KW - Motor neuron disease
KW - Primary lateral sclerosis
KW - Progressive supranuclear palsy
KW - Pseudobulbar palsy
KW - Pyramidal signs
UR - http://www.scopus.com/inward/record.url?scp=84867874064&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84867874064&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2012.09.005
DO - 10.1016/j.jns.2012.09.005
M3 - Article
C2 - 23026537
AN - SCOPUS:84867874064
VL - 323
SP - 147
EP - 153
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
IS - 1-2
ER -