Migraine, Familial Hemiplegic 2; Fhm2

Migraine, Familial Hemiplegic 2; Fhm2



Migraine, Familial Hemiplegic 2; Fhm2

Introduction

Familial hemiplegic migraine (FHM) is an autosomal excellent classical migraine subtype that typically includes hemiparesis (weakness of half of the body) during the aura phase. This may be accompanied by other symptoms such as ataxia, coma and seizures. There is clinical overlap in some of FHM patients with episodic ataxia type 2 and spinocerebellar ataxia type 6, benign family infantile convulsions, paralysis and alternating childhood. There are 3 known loci for FHM. FHM1, which accounts for about 50% of patients, FHM, is caused by mutations in the gene encoding the P / Q-type calcium channel a-subunit, CACNA1A.

In addition FHM1 is affiliated with cerebellar degeneration. FHM2, that the jokes on <25% of situations FHM, is caused by mutations in the Na + / K +-ATPase gene ATP1A2. FHM3 is a rare subtype of FHM and is caused by mutations in the sodium channel a-subunit coding genes, SCNA1. These three subtypes do not account for all situations, FHM, which proposes a reality for a little more locus (FHM4). Many of the non-family situations hemiplegic migraine (sporadic hemiplegic migraine) are also initiated by mutations in these loci.

Discussion

Classification

FHM can be roughly divided into two classes: with and without cerebellar signs. Cerebellar ataxia signal to mention, sometimes episodic, and other times the progressive, which can accompany FHM1 mutations and is caused by degeneration of the cerebellum. These results signal the cerebellum in the phenotypic overlap between FHM and episodic ataxia and a spinocerebellar ataxia. This is not surprising, as the subtypes of these disorders (FHM1, EA2 and SCA6) are allelic, ie, result from mutations in the identical genes. Other types of FHM appear distinguishable only on the cornerstone of their genetic cause.

There are also non-family situations hemiplegic migraine, sporadic hemiplegic migraine called. Such a situation appears to be identical as determinants of family situations and are re-mutations. Sporadic situation also clinically well with a family situation other than the need to attack the family annals.

Signs and symptoms

FHM signals overlap with those of migraine with aura. In short, FHM is characterized by migraine with aura associated with hemiparesis and in FHM1, cerebellar degeneration. This cerebellar degeneration may results in episodic or progressive ataxia. FHM can also present with the same signal as benign infantile convulsions family (BFIC) and alternating hemiplegia of childhood. Other symptoms of changed consciousness (for details, some situations seem to ...