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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report two cases of a particular type of migraine with aura, known as familial hemiplegic migraine (FHM). According to the International Headache Society (IHS) diagnostic criteria, the FHM can be diagnosed with the exception of organic causes, in a patient with migraine with aura including emiparesis of anything severity and with an end occurring a member of the family with similarity in the attach pattern. The two clinical cases reported clearly show these features and they can be considered exemplary for this type of pathology. This rare type of migraine has an unknown etiology, it seems to depend on a decreases of cerebral blood flow originative on the occipital lobe, over the subsequentially spreading anteriory region temporal and parietal lobe. The hypoperfusion with the next following neural ischemia is related to the variation of blood flow and/or "the spreading depression" supported by Leao and Olesen recently. We wanted to show these two cases so that the psychiatrist, the pediatrician, and the neurologist can be able to refer parents to the right approach, considering possibility of a pathology rare but benign; this is the FHM.
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PMID:Familial hemiplegic migraine in developmental age: report of two cases. 917 12

A gene for familial hemiplegic migraine, a subtype of migraine with aura, was assigned to chromosome 19p13. In this region, we identified a brain-specific P/Q-type calcium-channel alpha 1A-subunit gene, CACNA 1A, with 47 exons covering 300 kb. Sequencing of all exons and their flanking surroundings revealed polymorphic variations, including a (CA)n-repeat and a (CAG)n-repeat in the 3' untranslated region. In patients with familial hemiplegic migraine, we found four different missense mutations in conserved functional domains. One of the mutations has occurred on two different haplotypes in unrelated familial hemiplegic migraine families. Moreover, in episodic ataxia type 2, we found two mutations disrupting the reading frame. Thus, familial hemiplegic migraine and episodic ataxia type 2 can be considered as allelic channelopathies. Involvement of this familial hemiplegic migraine locus in migraine with and without aura was demonstrated by sib-pair analysis. We showed an increase of shared marker alleles of locus D19S394, which is tightly linked to the gene. The association between the alpha 1A calcium channel and familial hemiplegic migraine, and the increase of shared alleles in migraine-affected sib-pairs, have uncovered a new pathway for the pathophysiology of migraine. This finding may provide a rationale for the development of specific prophylactic therapy for migraine and other (paroxysmal) cerebral disorders.
Headache 1997 Sep
PMID:Wolff Award 1997. Involvement of a Ca2+ channel gene in familial hemiplegic migraine and migraine with and without aura. Dutch Migraine Genetics Research Group. 932 29

A single familial hemiplegic migraine locus has been previously mapped to 19p13.1 and associated with mutations in a calcium channel gene (CACNL1A4). We describe a new 39-member four-generation family from Wyoming of German-Native American descent with autosomal dominant familial hemiplegic migraine that is not linked to the chromosome 19p locus. Affected individuals showed a stereotypic pattern of migrainous headache associated with hemisensory and hemiparetic attacks, without other headache types. Eighty-three percent reported minor head trauma as a trigger for individual attacks. Seventy-two percent reported other typical migraine triggers for the attacks. Attack frequency decreased with age and the overall course was benign. Genetic linkage studies of this family found strong evidence for the disease gene in this family being located at chromosome 1q31. Multipoint analysis showed lod scores > 3 in a 44-cm region flanked by D1S158 and D1S2781, using 80% penetrance and a phenocopy rate of 1/50. Haplotype and multipoint analysis, including flanking markers, suggested incomplete penetrance and variable expressivity of the disease. A single affected patient who reports atypical symptoms including daily headaches likely represents a phenocopy. This new locus for hemiplegic migraine suggests that mutations of additional calcium channels in the region may cause the disease.
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PMID:A new locus for hemiplegic migraine maps to chromosome 1q31. 937 90

A 6-year-old boy with a family history of hemiplegic migraine had a hemiplegic migraine lasting for 6 days complicated by prolonged fever, lethargy, and two brief focal seizures. An acute single photon emission computerized tomogram (SPECT) demonstrated decreased blood flow in the symptomatic cerebral hemisphere as well as crossed cerebellar diaschisis not previously documented in migraine. Another unique finding was the MRI with enhancement of the meninges and pial vessels over the symptomatic cerebral hemisphere. These findings suggest cerebellar and extra-axial involvement as components of hemiplegic migraine.
Headache 1997 Oct
PMID:Familial hemiplegic migraine with crossed cerebellar diaschisis and unilateral meningeal enhancement. 938 60

Neurotologic symptoms are common with migraine, yet relatively little is known about the pathophysiology of such symptoms. Motion sensitivity with bouts of motion sickness occurs in about two thirds of patients with migraine. Episodes of vertigo occur in about one fourth of patients and, in some, vertigo is the only symptom (so-called "migraine equivalent"). Phonophobia is the most common auditory symptom, but fluctuating hearing loss and acute permanent hearing loss occur in a small percentage. Migraine can mimic Meniere's disease and so-called "vestibular Meniere's disease" is usually associated with migraine. The recent discovery of a mutation in a brain calcium-channel gene in families with hemiplegic migraine and in families with episodic vertigo and ataxia suggests a possible mechanism for neurotologic symptoms in patients with more common varieties of migraine. A defective calcium channel, primarily expressed in the brain and inner ear, could lead to reversible hair cell depolarization and auditory and vestibular symptoms. This hypothesis is currently being investigated in other families with migraine headaches and neurotologic symptoms. Hopefully, such studies will lead to improved diagnosis and better treatments in the future.
Headache
PMID:Neurotology of migraine. 943 80

We report the case of a 33-year-old woman who developed a dense hemiplegia immediately after an uncomplicated general anaesthetic for diagnostic laparoscopy. She had a history of recurrent hemiplegic migraine with a strong family history. Her migraine was normally associated with visual disturbances and a unilateral headache followed by a left-sided weakness such that she was unable to walk. Symptoms would last up to 24 h. Her post-operative state was atypical of her normal migraine, in that she had no headache or visual disturbance and initially all four limbs were affected.
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PMID:Hemiplegia following general anaesthesia: an unusual presentation of migraine. 978 80

It has been suggested that mitochondrial mutations cause migraine(-like) symptoms. The presence of mtDNA mutations (3243, 3271, 11084, and deletions) was investigated in three migraine subgroups (maternally transmitted migraine with and without aura, migrainous infarction, and nonfamilial hemiplegic migraine). No mutations were found. These mutations and deletions probably are not involved in the migraine subgroups studied, although an investigation of other material (e.g., muscle tissue) would have shown this with more certainty.
Cephalalgia 1999 Jan
PMID:Search for mitochondrial DNA mutations in migraine subgroups. 1009 55

Migraine has become an important topic in the field of complex genetic disorders. The identification of a gene on chromosome 19p encoding for an alpha 1A calcium channel subunit causing familial hemiplegic migraine has led to the classification of migraine as a channelopathy. More recently, efforts have been made to clarify the genetics of other primary headaches.
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PMID:Genetics of primary headaches. 1049 69

Over a period of ten years, a boy had several episodes of coma, lasting three to five days. Each episode was preceded by hemiparesis or paresthesias, aphasia, headaches and behavioural changes, with subsequent loss of consciousness. Partial seizures occurred during the first episode. A history of migraine or hemiplegic migraine was found in several members of the family. Linkage to chromosome 1q21-23, where a gene for familial hemiplegic migraine has been mapped, was shown in this family.
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PMID:Recurrent episodes of coma: an unusual phenotype of familial hemiplegic migraine with linkage to chromosome 1. 1056 14

Familial hemiplegic migraine is a distinctive form of migraine with autosomal dominant inheritance. The patients undergo attacks of migraine complicated by hemiplegia. Seizures have not been reported as comprising a part of this syndrome. We describe three generations of a family with hemiplegic migraine and focal seizures occurring concurrently with the migrainous attacks. There were five affected family members whose clinical features included unilateral headache and transient hemiplegia. Two family members also had focal seizures during the migrainous attacks. One of the patients was treated with carbamazepine with good results. The only associated neurological finding was ataxia which was found in the oldest patient. The presence of focal seizures during an episode of hemiplegic migraine suggests that the two phenomena of migraine and focal seizures may share the same underlying pathophysiology.
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PMID:A family with hemiplegic migraine and focal seizures. 1072 90


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