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[An autopsy case of chronic inflammatory demyelinating polyradiculoneuropathy with sever degeneration in the
M Sakai1, H Yamamoto, Y Hashizume
1Division of Neurology, Suzuka Byoin National Sanatorium.
Abstract:
An autopsy case of chronic inflammatory demyelinating polyradiculoneuropathy was reported. It took a progressive course and terminated fatally in eight years. A 41-year-old man noticed motor disturbances when he tried to lift a bath pail and to write on July, 1978. Neurological examination revealed proximal dominant muscle atrophy, weakness of all extremities, and moderately diminished tendon reflex. Sensation was normal. The CSF showed albumin cytologic dissociations. Electromyogram showed neurogenic changes. Histological examination of biopsy specimen obtained from the anterior tibial muscle revealed severe neurogenic changes and showed axonal degeneration on the ventral tibial nerve. The treatment by corticosteroids was not effective, and the disease gradually progressed with repeated improvements and exacerbations. Three years after the onset, he showed vesicorectal dysfunctions. He died of respiratory failure on May, 1986. Neuropathological examination showed severe degeneration of middle root zones in the posterior columns, loss of myelinated fibers in Clarke's columns, demyelination and mild loss of axons accompanied by lymphocytic infiltration in the spinal roots, especially in the anterior roots. The histogram of cervical ventral root, ventral and dorsal roots of thoracic and lumbar regions revealed a decreased number of large myelinated fibers. A characteristic finding of this case was the dissociation of clinical features and neuropathological findings; the clinical features showed a typical motor neuropathy, but neuropathological examination showed severe degeneration on posterior columns of spinal cord like a sensory-ataxic neuropathy. Our observation suggest that the pathway which originates from posterior ganglion cells and runs into Clarke's columns passes through the middle root zones, since severe demyelination in Clarke's columns was observed.
Insights
This autopsy case details chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a man whose motor symptoms contrasted with severe spinal cord degeneration. The findings suggest a unique pathway involvement in this fatal neurological disease.
Area of Science:
- Neurology
- Pathology
- Neuroscience
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a progressive autoimmune disorder affecting peripheral nerves.
- CIDP typically presents with symmetrical motor and sensory deficits, but atypical presentations occur.
Observation:
- An autopsy case of a 41-year-old man with a fatal, progressive CIDP over eight years is presented.
- Clinical presentation included proximal muscle atrophy, limb weakness, and normal sensation, with CSF showing albumin cytologic dissociation.
- Neuropathological examination revealed degeneration in posterior spinal cord columns and demyelination in spinal roots, contrasting with the predominantly motor clinical features.
Findings:
- The case demonstrated a dissociation between clinical presentation (motor neuropathy) and neuropathological findings (sensory-ataxic neuropathy pattern).
- Severe demyelination was observed in Clarke's columns and middle root zones of the spinal cord.
- A decreased number of large myelinated fibers was noted in spinal roots.
Implications:
- This case suggests a potential pathway involving posterior ganglion cells, middle root zones, and Clarke's columns in CIDP pathogenesis.
- Understanding this pathway may offer new insights into the diverse clinical manifestations of CIDP.
- Further research is warranted to elucidate the mechanisms underlying the dissociation between clinical and pathological findings in CIDP.