Brain atrophy following hemiplegic migraine attacks

Abstract

Background Patients with hemiplegic migraine (HM) may sometimes develop progressive neurological deterioration of which the pathophysiology is unknown. Patient We report a 16-year clinical and neuroradiological follow-up of a patient carrying a de novo p.Ser218Leu CACNA1A HM mutation who had nine severe HM attacks associated with seizures and decreased consciousness between the ages of 3 and 12 years. Results Repeated ictal and postictal neuroimaging revealed cytotoxic oedema during severe HM attacks in the symptomatic hemisphere, which later showed atrophic changes. In addition, progressive cerebellar atrophy was observed. Brain atrophy halted after cessation of severe attacks, possibly due to prophylactic treatment with flunarizine and sodium valproate. Conclusion Severe HM attacks may result in brain atrophy and prophylactic treatment of these attacks might be needed in an early stage of disease to prevent permanent brain damage.

Keywords: DWI; Hemiplegic migraine; MRI; migraine prophylaxis.

Figure 1.

At the age of three years, during a hemiplegic migraine attack, FLAIR (a) and T2-weighted (T2W) images (b) showed right hemispheric diffuse cortical swelling. Diffusion-weighted imaging (DWI) (c) showed diffusion restriction, most notable in the right parieto-occipital region (arrows). Mild cerebellar atrophy was present, as illustrated on the sagittal T2W image (d). At the age of ten years, interictal axial T1-weighted (T1W) (e) and T2W images (f) showed marked cortical atrophy in the right parieto-occipital regions, corresponding with the previous area of diffusion restriction (c). Progressive cerebellar atrophy was observed on sagittal T1W images (g, h). At the age of 12 years, during a hemiplegic migraine attack, coronal FLAIR (i) and axial T2W images (j) showed diffuse cortical swelling of the whole left hemisphere (arrow) with slight diffusion restriction on DWI and apparent diffusion coefficient images (k, l). Follow-up MRI at 13 years showed progressive cerebral and cerebellar atrophy on the coronal FLAIR (m), axial T2W (n) and sagittal T1W images (o, p). After the start of prophylactic medication and subsequent ceasing of severe hemiplegic attacks, follow-up MRI at 19 years revealed no evident progression of cerebellar and cerebral atrophy as visible on coronal T1W (q), axial T2W (r) and sagittal T1W images (s, t).