Alexander’s disease can be classified based on the age of onset. Some patients present as an adult.

Age at onset: late adolescence into adulthood


Outcome: highly variable, depending on rate of progression


Clinical features:

(1) bulbar or pseudobulbar findings (speech abnormalities, swallowing difficulties, vomiting)

(2) pyramidal findings (spasticity, hyperreflexia, positive Babinski sign)

(3) cerebellar signs (ataxia, nystagmus, dysmetria)

(4) dysautonomia (incontinence or retention of urine and/or stool, impotence, sweating abnormality, hypothermia, orthostatic hypotension)

(5) sleep apnea

(6) gait disturbance

(7) seizures

(8) diplopia

(9) hemiparesis or hemiplegia vs quadriparesis or quadriplegia

(10) weakness

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