Adrenoleukodystrophy: adult type
Known case of Addison's disease. Presented with spastic gait and hyperreflexia.
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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.
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).
- Osborn's Brain, Imaging, Pathology and Anatomy by Anne G. Osborn Diagnostic Imaging: Brain 2nd Edition by Anne G. Osborn
- Laureti S, Casucci G, Santeusanio F, Angeletti G, Aubourg P, Brunetti P. X-linked adrenoleukodystrophy is a frequent cause of idiopathic Addison's disease in young adult male patients. The Journal of clinical endocrinology and metabolism. 81 (2): 470-4. doi:10.1210/jcem.81.2.8636252 - Pubmed