The hallmark of hemiplegic migraine is unilateral weakness that accompanies a migraine headache attack. Acute encephalopathy in familial hemiplegic migraine with. Genes for familial hemiplegic migraine fhm and episodic ataxia type2 ea2 have been mapped to chromosome 19p. Familial hemiplegic migraine is an autosomal dominant disorder of unknown pathogenesis in which the migrainous attacks are marked by the occurrence of a transient hemiplegia. Familial hemiplegic migraine fhm falls within the category of migraine with aura. Familial hemiplegic migraine fhm is a rare, dominantly inherited subtype of migraine with aura, where hemiplegia occurs during the aura phase. Advances in genetics of migraine the journal of headache. Neuropsychological profile of hemiplegic migraines. In the initial stages, hemiplegic migraine may mimic cerebral infarction. Hemiplegic migraine hm is a rare variety of migraine with motor aura migraine accompanied by transient motor weakness. This type of migraine can occur as a sporadic or a familial disorder. Familial hemiplegic migraine type 1 fhm1 is a form of migraine with aura caused by heterozygous mutations in 4 genes.
This form of hm occurs in families in which there may. Familial hemiplegic migraine fhm is a rare, monogenic, autosomal dominant subtype of migraine, in which three genes, cacna1a, atp1a2. In addition, the aura of fhm may include visual disturbances, sensory loss, dysphasia, and seizures. Hemiplegic migraine can be easily misdiagnosed at its first presentation with an atypical sev. Individuals with no known family history are labeled with sporadic hemiplegic migraine shm, although absence of a family history.
Familial hemiplegic migraine genetic testing hereditary. Hemiplegic migraine is a rare form of migraine where people experience weakness on one side of their body hemiplegia in addition to the migraine headache attack. Familial hemiplegic migraine fhm is a rare and genetically heterogeneous autosomal dominant subtype of migraine with aura. Sporadic hemiplegic migraine is a rare form of migraine headache. A novel atp1a2 gene mutation in familial hemiplegic migraine and epilepsy. Three genes have been identified, all involved in ion transport. People with hemiplegic migraine typically are treated with the same medicines used for typical migraine with aura for pain, and to stop or prevent migraines. We report an adolescent with sporadic hemiplegic migraine. Many of the 28 million people who suffer from migraine headaches each year 1 need psychiatric care in addition to headache relief. Hemiplegic migraine is a type of migraine with aura that causes motor impairment such as weakness in addition to at least one visual, sensory, or speech disturbance aura that occurs before the migraine headache begins. Pdf objective familial hemiplegic migraine fhm is an autosomal dominantly inherited subtype of migraine with aura, characterized.
In migraine with aura including familial hemiplegic migraine the neurologic symptoms of aura are unequivocally. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Familial hemiplegic migraine fhm is an autosomal dominant headache disorder characterized by migraine with aura and reversible motor weakness that typically resolves. Sporadic hemiplegic migraine with scn1a gene mutationa. Jan 02, 2019 familial hemiplegic migraine fhm is an inherited form of hemiplegic migraine. Familial hemiplegic migraine is a rare autosomal dominant subtype where at least one first or seconddegree relative also has migraine auras including motor weakness. They point out that a threoninetomethionine substitution at position 666 in the polypeptide chain of the. There are no official treatment guidelines for familial hemiplegic migraine fhm, and no randomized, controlled trials regarding treatment for hemiplegic migraine have been conducted. Mutations in the genes cacna1a and scna1a, encoding the poreforming. Familial hemiplegic migraine and episodic ataxia type2 are. Fhm commonly begins during childhood or adolescence. Review of familial hemiplegic migraine, successful outcome. To characterize phenotypes of a novel cacna1a mutation causing familial hemiplegic migraine type 1.
These symptoms may last several weeks before resolving but some may not fully resolve, leading to a migraine. Prolonged hemiplegic migraine associated with unilateral. A hemiplegic migraine can run in the family familial hemiplegic migraine or can occur sporadically in one individual sporadic hemiplegic. This form of migraine with aura may occur either in families or only in one individual sporadic. Triptans may be safe in basilar and hemiplegic migraine. Familial hemiplegic migraine fhm, a rare autosomal dominant subtype of migraine.
The effect of ketamine 25 mg intranasally was studied in 11 patients with severe, disabling auras resulting from familial hemiplegic migraine. Familial hemiplegic migraine and episodic ataxia type2 are caused by mutations in the ca 2. Jul 17, 2017 a familial hemiplegic migraine is a rare type of migraine with an aura that includes muscle weakness. Familial hemiplegic migraine fhm is a rare autosomal dominant. Professor of pediatrics and neurology, childrens hospital of the kings daughters, eastern virginia medical school, norfolk, va after completing this article, readers should be able to. The prevalence of familial hemiplegic migraine with. How long do the symptoms of familial hemiplegic migraines last. Fmh is a rare, autosomal dominant subtype of migraine. A familial hemiplegic migraine, unlike the common migraine, is a very rare disease occurring in around 0. Familial hemiplegic migraine is an autosomal dominant form of migraine with aura, characterized by the occurrence of a motor deficit during the aura. Migraine attacks may be provoked by minor head trauma. Hemiplegic migraine is a type of migraine with aura that causes motor impairment such as weakness in addition to at least one visual, sensory, or speech disturbance aura that occurs before the migraine.
The information about these types of migraine is still very new, and these types of migraine. In this issue of the archives, kors et al 1 describe the clinical features of patients with familial hemiplegic migraine fhm in 5 unrelated families. Familial diplegic migraine description of a family. How to control migraines in patients with psychiatric. Cortical spreading depression and familial hemiplegic. Oct 19, 2007 familial hemiplegic migraine is a rare autosomal dominant subtype of migraine with aura. Familial hemiplegic migraine headache jama neurology. Current research is giving us new understanding of familial hemiplegic migraine fhm and how various subtypes may have different symptoms and require different treatment. Hemiplegic migraine presenting with prolonged somnolence. Familial hemiplegic migraine genetics home reference nih. Sporadic and familial hemiplegic migraine the basics about hemiplegic migraines.
Pdf familial hemiplegic migraine fhm is a rare, dominantly inherited subtype of migraine with aura, where hemiplegia occurs. The information about these types of migraine is still very new, and these types of migraine are very rare. Familial hemiplegic migraine genetic and rare diseases. This type of migraine can occur as a sporadic or a familial. Recurrent atp1a2 mutations in portuguese families with. The weakness is a manifestation of motor aura and occurs with other forms of aura that impair vision, speech, or sensation. Pdf a novel atp1a2 gene mutation in familial hemiplegic. In addition, distinct mutations within cacna1a were shown to cause two other human autosomal. The aim of this study is to report a minimal prevalence estimate for familial hemiplegic migraine with cerebellar ataxia and spinocerebellar ataxia type 6 in portugal. Familial hemiplegic migraine type 3 conditions gtr ncbi. Stroke and familial hemiplegic migraine the lancet neurology.
Familial hemiplegic migraine is a rare type of migraine typically characterized by weakness or even paralysis on one side of the body. To explore the neuropsychological correlates and implications of familial hemiplegic migraines fhms. Pdf familial hemiplegic migraine and spreading depression. Mutations in three different genes, two ionchannel genes and one. Familial hemiplegic migraine fhm is an autosomal dominant headache disorder characterized by migraine with aura and reversible motor weakness that typically resolves within minutes to hours. It can be accompanied by other symptoms, such as ataxia, coma, and paralysis. Familial hemiplegic migraine type 1 fhm1 is a dominantly inherited subtype of migraine with aura and transient hemiplegia associated with mutations in the cacna1a gene.
Familial hemiplegic migraine diagnosis and treatment. Genetics of familial hemiplegic migraine the journal of. Review of familial hemiplegic migraine, successful outcome in. Mutations in the genes cacna1a and scna1a, encoding the.
Jan 02, 2019 there are no official treatment guidelines for familial hemiplegic migraine fhm, and no randomized, controlled trials regarding treatment for hemiplegic migraine have been conducted. The family history will allow a distinction to be made between cases of familial hemiplegic migraine. Hemiplegic migraine is a rare type of migraine that may present in children and adolescents. In 20% of families, permanent cerebellar signs ataxia or nystagmus are observed. Familial hemiplegic migraine an overview sciencedirect topics. Familial hemiplegic migraine fhm is an autosomal dominant disorder comprised of migraine with aura and associated neurologic deficit, classically motor ie, hemiparesis, with at least one first. Sep 22, 2004 we report the occurrence of unilateral cerebral hemisphere edema with subsequent cortical laminar necrosis in the setting of familial hemiplegic migraine fhm and permanent neurologic sequelae after resolution of an attack in 1 patient. Familial hemiplegic migraine and episodic ataxia type2. Familial hemiplegic migraine fhm is an autosomal dominant disorder comprised of migraine with aura and associated neurologic deficit, classically motor i. Familial hemiplegic migraine is a rare monogenic form of migraine. The two distinct types of hemiplegic migraine have slightly different causes, symptoms, and risk factors.
Hemiplegic migraine induced by exertion headache jama. Diagnostic criteria attacks fulfilling criteria for 1. Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and extreme sensitivity to light and sound. This type of migraine can occur randomly or it can run in families. Oct 30, 20 cacna1a gene disorders present a variable familial phenotype of ataxia, migraine with aura, andor hemiplegic migraine. Familial hemiplegic migraine fhm is an inherited form of hemiplegic migraine. Familial hemiplegic migraine is a rare subtype of migraine with aura which includes motor weakness. In five patients ketamine reproducibly reduced the severity and duration of the. Sporadic hemiplegic migraine shm is a subtype of hemiplegic migraine, characterized by episodes of migraine with a reversible motor aura, without a positive family history, and is a mimicker of an atypical severe form of migraine. Jul 17, 2001 familial hemiplegic migraine fhm falls within the category of migraine with aura. These recurrent headaches typically begin in childhood or adolescence and can be triggered by certain foods. Confirmation of a diagnosis of familial hemiplegic migraine through genetic testing may guide treatment and management. If you have fhm, each of your children will have a 50 percent chance of inheriting the condition. Advances in genetics of migraine the journal of headache and.
Familial hemiplegic migraine is a form of migraine headache that runs in families. Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and. Migraine headaches often coexist with depression, 2 anxietypanic. Calcitonin generelated peptide in familial hemiplegic. We believe that our patient fulfilled the criteria for hemiplegic migraine, which, according to the international headache society, is classified under migraine with prolonged aura of hemiplegia as opposed to familial hemiplegic migraine. Fhm1 shares many phenotypical similarities with common types of migraine, indicating common neurobiological pathways. Cacna1a, atp1a2, snc1a and prrt2, but further heterogeneity is expected. It has been established through research that women are more likely to be affected by this disease when compared to men. Familial hemiplegic migraine, neuropsychiatric symptoms, and. Hemiplegia is often associated with reversible visual, sensory, or speech disturbances as well.
Hemiplegic migraine is accompanied by a paralysis of one side of the body. When it occurs randomly, without a family link, it is called sporadic hemiplegic migraine. Common pathophysiologic mechanisms in migraine and epilepsy. Confirmation of a diagnosis of familial hemiplegic migraine through genetic testing. A 32yearold woman with known familial hemiplegic migraine point mutation in exon 22 of the atp1a2 gene presented with an acute confusional state, after an initially typical migraine. Familial hemiplegic migraine fhm is a rare subtype of migraine. Jul 12, 2000 article abstract migraine aura is probably caused by corticalspreading depression. Sporadic hemiplegic migraine with atp1a2 and prothrombin. Common pathophysiologic mechanisms in migraine and. When more than one member of the family has this disorder it is called familial hemiplegic migraine. Hemiplegic and basilar migraine are rare types of migraine. Hemiplegic migraine occurs in roughly one in 10,000 people. Aura in some patients with familial hemiplegic migraine can. Hemiplegic migraine hm is an unusual subset of migraine with aura, in which headache is associated with unilateral motor deficits, thought to be attributable to an underlying calcium channelopathy.
Both familial and sporadic hemiplegic migraines have similar prevalence and clinical characteristics. In migraine with aura including familial hemiplegic migraine the neurologic symptoms of aura are unequivocally localizable to the cerebral cortex or brain stem and include visual disturbance most common, sensory loss e. A gene for familial hemiplegic migraine maps to chromosome 19. Jul 17, 2017 the episodes of muscle weakness and movement abnormalities associated with familial hemiplegic migraine can last for hours or days. A gene for familial hemiplegic migraine maps to chromosome. Familial hemiplegic migraine fhm affects at least two close relatives in the same family.
Hemiplegic migraine hm is a rare, severe subtype of ma, in which migraine symptoms are accompanied by motor symptoms such as temporary numbness or weakness, affecting one side of the body hemiparesis. Familial hemiplegic migraine an overview sciencedirect. Familial hemiplegic migraine is an autosomal dominant disorder of unknown pathogenesis in which the migrainous attacks are marked by the occurrence of a transient hemiplegia during the aura. Sep 28, 2015 the molecular and cellular mechanisms of the primary brain dysfunction leading to the onset of a migraine attack and to susceptibility to cortical spreading depression csd, the neurophysiological correlate of migraine aura and a likely trigger of the headache mechanisms, remain largely unknown and major open issues in the neurobiology of migraine. No treatment for acute and severe migraine aura has been described previously. A first gene, cacna1a, located on chromosome 19 and encoding the main subunit of pq type neuronal calcium channel, is.
Familial hemiplegic migraine fhm is an autosomal dominant type of hemiplegic migraine that typically includes weakness of half the body which can last for hours, days, or weeks. Here have been described clinical and molecular features in patients suffering from migraine with aura ma, without mo and hemiplegic migraine. Familial hemiplegic migraine fhm is a familial form of hm where it is usually inherited in an autosomal dominant manner. Jun 02, 2008 calcitonin generelated peptide in familial hemiplegic migraine fhm and migraine with aura ma cgrp2008 the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Recognize the diagnostic criteria for pediatric migraine. Familial hemiplegic migraine is the form of hm in which this family link can be established, whereas hm that occurs without evidence of this genetic connection is called sporadic hemiplegic migraine. Familial hemiplegic migraine type 1 shows no hypersensitivity.
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