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Chronic peripheral neuropathy in childhood: an overview
1Department of Neurology, Children's Hospital, Camperdown, New South Wales, Australia.
Summary
Genetic factors are the primary cause of chronic peripheral polyneuropathy in children, with hereditary motor and sensory neuropathies being common. Early diagnosis is achievable through combined clinical, neurophysiological, and histopathological investigations.
Area of Science:
- Pediatric Neurology
- Genetics
- Neuropathology
Background:
- Chronic peripheral polyneuropathy affects children under 17.
- Genetic origins are frequently implicated in pediatric neuropathies.
Purpose of the Study:
- To review cases of childhood peripheral polyneuropathy.
- To identify the etiological origins and diagnostic approaches.
Main Methods:
- Review of 125 biopsy-proven cases of childhood peripheral polyneuropathy.
- Clinical, neurophysiological, and histopathological investigations.
Main Results:
- 71% of cases were of genetic origin.
- Hereditary motor and sensory neuropathies confirmed in over 40% of cases.
- A specific entity, hereditary motor and sensory neuropathy of neuronal type with early childhood onset, was delineated (7.2% of cases).
Conclusions:
- Genetic factors are predominant in childhood chronic polyneuropathy.
- A combined diagnostic approach yields over 80% etiological diagnostic accuracy.