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Alexander's disease: clues to diagnosis
C L Pridmore1, M Baraitser, B Harding
1Department of Paediatric Neurology, Hospital for Sick Children, London, England.
Journal of Child Neurology
|April 1, 1993
Summary
Alexander's disease in children presents two main forms: infantile with macrocephaly and seizures, and juvenile with predominant bulbar signs. Diagnosis relies on combined findings, necessitating histologic confirmation.
Area of Science:
- Pediatric Neurology
- Neurodegenerative Disorders
- Genetic Diseases
Background:
- Alexander's disease is a rare, progressive, demyelinating disorder of the central nervous system.
- It is characterized by the presence of Rosenthal fibers in white matter.