Adrenoleukodystrophy: adult type

Case contributed by Dr Mustafa Hammad

Presentation

Known case of Addison's disease. Presented with spastic gait and hyperreflexia.

Patient Data

Age: 45 years old
Gender: Male
Modality: MRI

Brain MRI shows symmetrical confluent hyperintnse signal in T2WI, FLAIR involving the posterior (occipitoparietal) periventricular white matter, sparing the subcortical U fibres.

Whole spine MRI shows thinning/atrophic changes of thoracic spinal cord in comparison with the cervical and lumbar regions.

Case Discussion

Adrenoleukodystrophy (ALD) is an X-linked disorder which involves mainly the nervous system white matter and the adrenal cortex. The adult neurologic variant of ALD, adrenomyeloneuropathy (AMN) is the most common form, which is characterised by spinal cord atrophy and white matter changes.

Adrenoleukodystrophy is frequently associated with adrenal insufficiency (Addison's disease). 

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Case Information

rID: 47101
Case created: 31st Jul 2016
Last edited: 11th Dec 2016
Inclusion in quiz mode: Included

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