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Abstract Details

The Red Herring that Mimicked a Chameleon
General Neurology
General Neurology Posters (7:00 AM-5:00 PM)
067
To highlight that very rare patterns of asymmetric white matter damage seen with adrenoleukodystrophy (ALD) can mimic other brain pathologies.
A journalist in his early fifties was diagnosed as epileptic and started on carbamazepine. Brain imaging revealed an old, presumed silent, left hemisphere infarct with hypertensive cerebral small vessel disease (SVD). CT angiography identified an occluded left middle cerebral artery (MCA). Over the next six years he was admitted with severe hyponatraemia, ascribed to carbamazepine, and difficult-to-control seizures. He then had a posterior circulation TIA due to left vertebral artery stenosis. He reported gait difficulty, subtle cognitive issues and language impairment, symptoms often worse after a seizure, ascribed to decompensation from his old stroke and SVD. On-going deterioration prompted further neurology review. When assessed, a markedly spastic paraparesis, sensory level to the waist, and a right inferior homonymous quadrantanopia were noted
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Spinal MRI was normal. Electromyography, nerve conduction studies and central motor conduction times revealed both peripheral and central demyelination. A diagnosis of X-linked adrenoleukodystrophy / adrenomyeloneuropathy (ALD / AMN) was confirmed based on increased very long chain fatty acids

Our case illustrates that very rare asymmetric patterns of white matter damage can be seen with ALD, the commonest pattern being symmetrical MRI T2/FLAIR hyperintensities in the parieto-occipital regions and splenium of the corpus callosum; it is estimated that such asymmetry, is seen in under 5% of ALD cases and can be mistaken for other pathological processes such as tumours or trauma. Our patient also suffered from large vessel atherosclerotic disease which is commonly seen in the general and stroke populations. His occluded left MCA was the ‘red herring’ that resulted in a misdiagnosis of stroke, with this rare asymmetric ALD-related demyelination representing the radiological ‘chameleon’ mimicking it.


Authors/Disclosures
Faizan Aslam, MBBS (St George’s University Hospital)
PRESENTER
Dr. Aslam has nothing to disclose.
No disclosure on file