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  • Articles: DFG German National Licenses  (2)
  • Electron microscopy  (1)
  • Key words Motor neuron disease  (1)
  • 1
    ISSN: 1432-0533
    Keywords: Key words Motor neuron disease ; Amyotrophic lateral sclerosis ; Sensory neuronopathy ; Bunina body ; Skein-like inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a 62-year-old man with sporadic motor neuron disease (MND) of 52 months’ duration with progressive sensory disturbance and high cerebrospinal fluid protein content. Neuropathologically, both the upper and lower motor neuron systems were severely affected, and light and electron microscopy revealed Bunina bodies and skein-like inclusions, which are characteristic of amyotrophic lateral sclerosis, in the remaining anterior horn cells. Moreover, there was severe degeneration without inflammatory infiltrates in the spinal posterior columns, spinal ganglia, and peripheral sensory nerves. These findings suggest that this case may be an unusual variant of sporadic MND with severe somatic sensory system involvement.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: HTLV-I-associated myelopathy ; Spinal cord lesion ; Electron microscopy ; Primary demyelination ; Remyelination by oligodendrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We describe postmortem findings in a patient with human T lymphotropic virus type I (HTLV-I)-as-sociated myelopathy (HAM). The patient developed the disease 8 years after blood transfusion and showed good response to corticosteroid treatment but died of cardiac failure. Histologically, chronic, mild meningoence-phalomyelitis was noted predominantly involving the bilateral lateral and anterior columns of the middle to lower thoracic segments. The spinal cord lesions showed obvious loss of myelinated nerve fibers and fibrillary gliosis with minimal inflammatory cell infiltration. Electron microscopy of the lesion revealed disintegration of the myelin sheaths, regular separation of the minor dense line of the myelin sheaths, and completely demyelinated axons. In addition, remyelinated fibers with thin central myelin sheaths and disproportionately large axons were seen frequently. These findings indicate that primary demyelination and remyelination by oligodendrocytes occur in the spinal cord lesions of HAM.
    Type of Medium: Electronic Resource
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