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Alexander disease
Background
Alexander disease is a rare genetic, degenerative disorder of the nervous system with effects on the mid brain and cerebellum; in the most common infantile form of the disease, the frontal white matter is involved. It is one of a group of genetic disorders called the leukodystrophies. The term leukodystrophy comes from the Greek words, 'leuko' meaning white and referring to the 'white matter' of the nervous system and 'dystrophy' meaning imperfect growth or development. In Alexander disease, the growth of the white matter of the brain, or myelin sheath, is affected. The brain stem may be particularly involved; in rare adult forms of the condition resulting in unusual signs such as flapping movement of the palate (palatal myoclonus), limb paralysis and clumsy movements. Cerebellar disease also occurs.
In addition to the infantile form of Alexander disease, very rare juvenile and adult forms have also been described. These forms occur less frequently and have a longer course of progression. Onset of the juvenile form of Alexander disease occurs between seven to fourteen years of age and sadly, death usually occurs within ten years after the onset of symptoms. It is, however, not certain whether the disorders described in older children and adults are the same disorder and should be described as Alexander disease.
There is also a condition attributed to, or resembling Alexander disease called Parkinsonism-dementia complex of Guam. This neurodegenerative disease is characterised by amyotrophy (failure of development, or wasting, of muscles), parkinsonian features including rigidity, bradykinesia (slowed ability to start and continue movements) and resting tremor and dementia.
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