Pathology of roots, spinal cord and brainstem in syringomyelia-like syndrome of Tangier disease
| Autor: | Philippes Convers, Jean Christophe Antoine, Michel Tommasi, Simone Boucheron, Daniel Michel, Bernard Laurent |
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| Rok vydání: | 1991 |
| Předmět: |
Male
Pathology medicine.medical_specialty Nerve root Central nervous system Tangier disease medicine Humans Tangier Disease Neurons business.industry Cranial nerves Cranial Nerves Brain Peroneal Nerve Syndrome Anatomy Middle Aged Motor neuron medicine.disease Spinal cord Syringomyelia Microscopy Electron medicine.anatomical_structure Spinal Cord Neurology Vacuoles Autopsy Neurology (clinical) Neuron Spinal Nerve Roots business Brain Stem |
| Zdroj: | Journal of the Neurological Sciences. 106:179-185 |
| ISSN: | 0022-510X |
| DOI: | 10.1016/0022-510x(91)90255-6 |
| Popis: | We report here a post-mortem examination of a 46-year-old patient who died after a 23-year-long syringomyelia-like syndrome of Tangier disease. The L5 dorsal root and the superficial peroneal nerve showed fiber loss and lipid vacuole accumulation in Schwann cell cytoplasm. The L5 ventral root had moderate fiber loss without lipid vacuoles. In the cervical roots, fiber loss was intense and there were no foamy Schwann cells. Motor neuron loss was severe in the cervical spinal cord and the facial nerve nucleus and slight at the lumbar level. Under electron microscopy, some neurons of the lower spinal cord showed atypical inclusions. These data suggest that an unknown metabolic defect is responsible for a primary neuronopathy. Lipid accumulation in Schwann cells, resulting from fiber degeneration is probably transient, accounting for the absence of foamy cells in regions with longstanding involvement. |
| Databáze: | OpenAIRE |
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