Open AccessAn Autopsy Case of Atypical Motor Neuron Disease with Bunina Bodies in the Lower Motor and Subthalamic Neurons
Author(s) -
Takahashi Hitoshi,
Ohama Eisaku,
Ikuta Fusahiro,
Tokiguchi Susumu
Publication year - 1991
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1991.tb03271.x
Subject(s) - subthalamic nucleus , spinal cord , motor neuron , globus pallidus , medicine , red nucleus , neuroscience , anterior horn cell , lower motor neuron , dentate nucleus , anatomy , pathology , amyotrophic lateral sclerosis , parkinson's disease , biology , central nervous system , nucleus , basal ganglia , cerebellum , deep brain stimulation , disease
We report a 37‐year‐old male without any family history of neurological disease who suffered progressive muscular atrophy and sensory impairment of 4 years' duration. Autopsy revealed neuronal loss in the anterior horns of the spinal cord and in the hypoglossal and facial nuclei of the brain stem. The corticospinal tracts of the spinal cord showed only mild degeneration. In addition, there were obvious degenerative lesions manifested by loss of neurons, myelin and axons in the spinal posterior columns, Clarke's column, spinocerebellar tracts and dorsal root ganglia as well as in the subthalamic nucleus, globus pallidus, substantia nigra and cerebellar dentate nucleus. Furthermore, we frequently encountered Bunina bodies not only in the lower motor neurons but also in the subthalamic neurons. We consider this case to be an atypical example of motor neuron disease with features of multisystem degeneration. The fact that Bunina bodies were observed in both lower motor and subthalamic neurons in this case suggests a common etiology of neuronal degeneration in these two different systems. Acta Pathol Jpn 41: 46–51, 1991.