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Current concepts of neurocutaneous disorders
1Department of Neurology, University of California Medical Center, San Francisco 94143.
Brain & Development
|January 1, 1991
Summary
Neurocutaneous diseases involve the nervous system and skin, with some affecting other organs. This review examines major neurocutaneous syndromes, including those without skin abnormalities.
Area of Science:
- Neurology
- Dermatology
- Genetics
Background:
- Neurocutaneous diseases are a complex group of disorders.
- Initially defined by nervous system and skin abnormalities, the category has expanded.
- Other organ systems are often affected.
Purpose of the Study:
- To review the major neurocutaneous syndromes.
- To discuss the evolving definition of these diseases.
- To highlight conditions included despite lacking cutaneous manifestations.
Main Methods:
- Literature review of neurocutaneous diseases.
- Analysis of diagnostic criteria and clinical features.
- Synthesis of current understanding of major syndromes.
Main Results:
- Neurocutaneous syndromes encompass a wide spectrum of conditions.
- The definition has broadened beyond primary neurological and skin involvement.
- Several syndromes are now recognized without apparent skin lesions.
Conclusions:
- The classification of neurocutaneous diseases is dynamic.
- Understanding the expanding scope is crucial for diagnosis and management.
- Further research is needed to elucidate the pathophysiology of diverse presentations.