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

Shigeto Nagao, Osamu Yokota, Reiko Nanba, Hiroshi Takata, Takashi Haraguchi, Hideki Ishizu, Chikako Ikeda, Naoya Takeda, Etsuko Oshima, Katsuaki Sakane, Seishi Terada, Yuetsu Ihara, Yosuke Uchitomi

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Abstract

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.

Original languageEnglish
Pages (from-to)147-153
Number of pages7
JournalJournal of the Neurological Sciences
Volume323
Issue number1-2
DOIs
Publication statusPublished - Dec 15 2012

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Progressive Supranuclear Palsy
Motor Neuron Disease
Aphasia
Parkinsonian Disorders
Paralysis
Dementia
Motor Cortex
Motor Neurons
Basal Ganglia
Gait
Brain Stem
Coiled Bodies
Pseudobulbar Palsy
Babinski's Reflex
Neurologic Gait Disorders
Pathology
Lewy Bodies
Apraxias
Dysarthria
Abnormal Reflexes

Keywords

  • Motor neuron disease
  • Primary lateral sclerosis
  • Progressive supranuclear palsy
  • Pseudobulbar palsy
  • Pyramidal signs

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia. / Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda, Naoya; Oshima, Etsuko; Sakane, Katsuaki; Terada, Seishi; Ihara, Yuetsu; Uchitomi, Yosuke.

In: Journal of the Neurological Sciences, Vol. 323, No. 1-2, 15.12.2012, p. 147-153.

Research output: Contribution to journalArticle

Nagao, S, Yokota, O, Nanba, R, Takata, H, Haraguchi, T, Ishizu, H, Ikeda, C, Takeda, N, Oshima, E, Sakane, K, Terada, S, Ihara, Y & Uchitomi, Y 2012, 'Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia', Journal of the Neurological Sciences, vol. 323, no. 1-2, pp. 147-153. https://doi.org/10.1016/j.jns.2012.09.005
Nagao, Shigeto ; Yokota, Osamu ; Nanba, Reiko ; Takata, Hiroshi ; Haraguchi, Takashi ; Ishizu, Hideki ; Ikeda, Chikako ; Takeda, Naoya ; Oshima, Etsuko ; Sakane, Katsuaki ; Terada, Seishi ; Ihara, Yuetsu ; Uchitomi, Yosuke. / Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia. In: Journal of the Neurological Sciences. 2012 ; Vol. 323, No. 1-2. pp. 147-153.
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AU - Haraguchi, Takashi

AU - Ishizu, Hideki

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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.

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