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Serial MRI in a case of familial hemiplegic migraine
D J A Butteriss1, V Ramesh, D Birchall
1Department of Neuroradiology, Regional Neurosciences Centre Newcastle General Hospital, Westgate Road, Newcastle upon Tyne, NE4 6BE, UK.
Abstract:
We report MRI findings in a patient with familial hemiplegic migraine (FHM) with repeated episodes of hemiparesis. FHM is caused by a penetrant autosomal dominant genetic mutation; several mutations have been genotyped, involving brain-expressed ion channels. We found cerebral oedema, dilatation of intracerebral vessels and decreased water diffusion contralateral to the hemiparesis, not respecting vascular territories, with subsequent complete resolution of both clinical and imaging abnormalities. These results are thought to be consistent with an underlying primary neuronal pathology with secondary vascular effects, as opposed to the traditional, primarily vascular, model of migraine aetiology.
Insights
Familial hemiplegic migraine (FHM) involves genetic mutations affecting ion channels. MRI revealed brain swelling and vascular changes, suggesting neuronal issues precede vascular events in FHM.
Area of Science:
- Neurology
- Neuroimaging
- Genetics
Background:
- Familial hemiplegic migraine (FHM) is a rare subtype of migraine with aura, characterized by hemiplegia.
- FHM is linked to autosomal dominant mutations in genes encoding ion channels crucial for neuronal excitability.
Observation:
- This study presents MRI findings in a patient experiencing recurrent hemiparesis, a key symptom of FHM.
- The patient had a confirmed genetic mutation associated with FHM.
Findings:
- MRI revealed transient cerebral edema, intracranial vessel dilation, and restricted water diffusion contralateral to the hemiparesis.
- These imaging abnormalities did not conform to typical vascular territories.
- All clinical and imaging abnormalities resolved completely after the episodes.
Implications:
- The observed findings suggest that primary neuronal dysfunction, rather than a vascular event, may underlie FHM episodes.
- This challenges the traditional vascular theory of migraine pathogenesis.
- Further research into neuronal-vascular interactions in FHM is warranted.