Confirmation of Virtual Unmyelinated Fiber Absence in Hereditary Sensory Neuropathy Type IV
| Autor: | H. H. Goebel, Stefan Veit, Peter J. Dyck |
|---|---|
| Rok vydání: | 1980 |
| Předmět: |
Male
Marfan syndrome Pathology medicine.medical_specialty Sural nerve Degeneration (medical) Pathology and Forensic Medicine 03 medical and health sciences Cellular and Molecular Neuroscience Nerve Fibers 0302 clinical medicine Congenital insensitivity to pain with anhidrosis Humans Medicine Peripheral Nerves 030304 developmental biology 0303 health sciences business.industry Infant General Medicine Anatomy medicine.disease Tissue Degeneration Nociception medicine.anatomical_structure Neurology Refsum Disease Neurology (clinical) Neuron Abnormality business 030217 neurology & neurosurgery |
| Zdroj: | Journal of Neuropathology and Experimental Neurology. 39:670-675 |
| ISSN: | 0022-3069 |
| DOI: | 10.1097/00005072-198011000-00005 |
| Popis: | Sural nerves from two unrelated young boys were virtually without unmyelinated fibers (UFs). Small myelinated fibers (MFs) may also have been slightly reduced in number. Since no degeneration or regeneration is observed, UF absence is assumed to be congenital, due to either lack of neuron formation or to premature degeneration. The main clinical features of this inherited sensory neuropathy (previously identified by us as type IV) are the inherited nature and abnormality of nociception, of sweating, and of thermal regulation associated with mild mental retardation. Our findings confirm the congenital absence of UFs of cutaneous nerves in cases such as these and provide further evidence that this disorder has a different natural history and pathology than do the other three types of hereditary sensory neuropathy. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|