Table 4.

Alexander Disease: Comparison of Forms by Select Features

NeonatalInfantileJuvenileAdult
Typical age at presentation 1st mo of lifeInfancy or childhoodChildhood or adolescenceAdolescence or adulthood
Core neurologic manifestations Lack of developmental progression; motor impairment w/o spasticity; seizures; megalencephaly; hydrocephalusDevelopmental delay or developmental regression; seizures; megalencephalyBulbar/pseudobulbar signs w/nasal speech, dysphagia, dysphonia; failure to thrive; intractable vomiting; scoliosis; autonomic dysfunctionPyramidal involvement; bulbar dysfunction; autonomic dysfunction
Other findings Feeding difficulties (failure to thrive)Failure to thriveShort statureCerebellar involvement; palatal myoclonus; normocephaly

From: Alexander Disease

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