Serial magnetic resonance imaging findings during severe attacks of familial hemiplegic migraine type 2: a case report

Abstract

Background Hemiplegic migraines represent a heterogeneous disorder with various presentations. Hemiplegic migraines are classified as sporadic or familial based on the presence of family history, but both subtypes have an underlying genetic etiology. Mutations in the ATP1A2 gene are responsible for Familial Hemiplegic type 2 (FHM2) or the sporadic hemiplegic migraine (SHM) counterpart if there is no family history of the disorder. Manifestations include migraine with aura and hemiparesis along with a variety of other symptoms likely dependent upon the specific mutation(s) present. Case presentation We report the case of an adult man who presented with headache, aphasia, and right-sided weakness. Workup for stroke and various infectious agents was unremarkable during the patient’s extended hospital stay. We emphasize the changes in the Magnetic Resonance Imaging (MRI) over time and the delay from onset of symptoms to MRI changes in Isotropic Diffusion Map (commonly referred to as Diffusion Weighted Imaging (DWI)) as well as Apparent Diffusion Coefficient (ADC). Conclusions We provide a brief review of imaging findings correlated with signs/symptoms and specific mutations in the ATP1A2 gene reported in the literature. Description of the various mutations and consequential presentations may assist neurologists in identifying cases of Hemiplegic Migraine, which may include transient changes in ADC and DWI imaging throughout the course of an attack.

Prolonged neurologic deficits with brain MRI changes following ECT in an adolescent with a CACNA1a-related disorder; a case report

“…While ECT is generally a well-tolerated treatment based on published data, there is limited data on its use in children and in patients with specific genetic conditions. In the patient presented here, given their CACNA1a pathogenic variant, the episode of ECT potentially triggered a hemiplegic migraine and cortical spreading depression (CSD), which would be consistent with their MRI changes, cerebral edema and prolonged hemiplegia [ 35 , 36 ]. It is not clear why ECT on the right hemisphere caused left sided symptoms, but during unilateral ECT, spread of seizures to contralateral side has been observed [ 31 ].…”