Upper motor neuron predominant degeneration with frontal and temporal lobe atrophy

The autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. He showed slowly progressive spasticity, pseudobulbar palsy and character change, and died 32 months after the onset of symptoms. Autopsy revealed severe atrophy of the frontal and temporal lobes, remar...

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Bibliographic Details
Published in:Acta neuropathologica 1998-11, Vol.96 (5), p.532-536
Main Authors: KONAGAYA, M, SAKAI, M, MATSUOKA, Y, KONAGAYA, Y, HASHIZUME, Y
Format: Article
Language:English
Subjects:
Aged
Amygdala
Amyotrophic lateral sclerosis
Atrophy
Autopsy
Biological and medical sciences
Cortex (motor)
Degeneration
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Frontal Lobe - pathology
Gliosis
Humans
Male
Medical sciences
Mimicry
Motor Neuron Disease - pathology
Motor neuron diseases
Motor Neurons - pathology
Nerve Degeneration - pathology
Neurology
Paralysis
Precentral gyrus
Pyramidal tracts
Spasticity
Temporal cortex
Temporal lobe
Temporal Lobe - pathology
Ubiquitin
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Summary:The autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. He showed slowly progressive spasticity, pseudobulbar palsy and character change, and died 32 months after the onset of symptoms. Autopsy revealed severe atrophy of the frontal and temporal lobes, remarkable neuronal loss and gliosis in the precentral gyrus, left temporal lobe pole and amygdala, mild degeneration of the Ammon's horn, degeneration of the corticospinal tract, and very mild involvement of the lower motor neurons. The anterior horn cells only occasionally demonstrated Bunina body by cystatin-C staining, and skein-like inclusions by ubiquitin staining. This is a peculiar case with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease predominantly affecting the upper motor neuron.
ISSN:0001-6322
1432-0533
DOI:10.1007/s004010050930