[ Monash University ]

Museum of Pathology

ADRENOLEUKODYSTROPHY

ADRENOLEUKODYSTROPHY

Clinical History

3 years before admission this 12 year old boy suffered head injury following which he was unconscious for some time. Before that he was of average intelligence and after that intellect deteriorated, sight and hearing became affected and epilepsy and spasticity commenced. There was deterioration and death.

Pathology

The specimen is a horizontal section of brain. In the occipital lobes the white matter is grey and translucent. The cortex and arcuate fibres appear normal. This change extends forward as far as the basal ganglia. The posterior halves of the lateral ventricles are moderately dilated. There is no clear line of demarcation between normal and abnormal white matter. These specimens are from a patient suffering from adrenoleukodystrophy. Histologically the involved white matter showed gross destruction. There were many giant astrocytes and around the blood vessels many small round cells and compound granular corpuscles. Adrenoleukodystrophy is a familial progressive demyelinating disorder affecting the cerebral white matter.