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Wednesday, October 31, 2007

Alexander Disease

General:
Megalencephaly

Airway:
Copious oral secretions
Large head

Gastrointestinal:
GERD

Neurological:
Seizures
Progressive spasticity & dementia
Hydrocephalus

Considerations:
Aspiration risk
Positioning challenges
Anticonvulsants may affect drug metabolism
Avoid phenothiazines, butyrophenones, metoclopramide, dopaminergic blockers which may affect movement disorders

Links:
Adrenoleukodystrophy
Metachromatic Leukodystrophy
Krabbe disease
Canavan disease
Pelizaeus-Merzbacher disease

References:

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