Childhood Juvenile and Adult Adrenoleukodystrophies

Childhood, juvenile, and adult adrenoleukodystrophies are characterized by mental regression progressing to dementia, motor disorders, visual and sensorineuronal hearing loss, and seizures culminating in a terminal vegetative state. Death occurs 2 to 3 years after onset in childhood cases. The clinical course is longer in juvenile and adult cases.

The cerebral pathology is characterized by a widespread, often symmetric, degeneration of myelin in the

figure 9.10

Adrenoleukodystrophy. Loose perivascular lymphocytic infiltrations and reactive astrocytes are present in the hemispheric white matter (HE).

cerebrum and cerebellum, with sudanophilic breakdown products within macrophages. These may contain lamellar inclusions of VLCFA esters. The demyelination usually proceeds from the parieto-occipital regions to the frontal areas. Perivascular lymphocytic infiltrations in the demyelinated areas are diagnostic hallmarks. With time, a dense astrocytic fibrosis replaces the myelin (Fig. 9.10).

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