1/86. Familial hemiplegic migraine with cerebellar ataxia and paroxysmal psychosis.Familial hemiplegic migraine is a rare autosomal dominant disorder associated with stereotypic neurologic aura phenomena including hemiparesis. So far two chromosomal loci have been identified. Families linked to the chromosome 19 locus display missense mutations within the CACNL1A4 gene. Here we report on a family with familial hemiplegic migraine and cerebellar ataxia with recurrent episodes of acute paranoid psychosis with anxiety and visual hallucinations associated with migraine attacks. Based on the clinical and haplotype evidence indicating linkage to chromosome 19 in this family, we hypothesize that a dysfunction of the mutated calcium channel may be involved not only in the development of hemiplegic migraine but also in the acute psychotic episodes observed in these patients.- - - - - - - - - - ranking = 1keywords = familial hemiplegic migraine, hemiplegic migraine, migraine, aura (Clic here for more details about this article) |
2/86. Coincidence of familial hemiplegic migraine and hemicrania continua? A case report.A case is presented of a 39-year-old woman with a history of simultaneous Familial hemiplegic migraine (FHM) and hemicrania continua (HC). The family history of the patient revealed different types of migraine and cyclic syndromes in childhood in four generations. The possible links between FHM and HC are discussed. The pedigree gives further evidence that cyclic syndromes in childhood belong to the spectrum of migraine.- - - - - - - - - - ranking = 2.2702062042025keywords = familial hemiplegic migraine, hemiplegic migraine, migraine (Clic here for more details about this article) |
3/86. genetic heterogeneity in Italian families with familial hemiplegic migraine.OBJECTIVE: To verify linkage to chromosome 19p13, to detect mutations in the CACNA1A gene, and to correlate genetic results to their clinical phenotypes in Italian families with familial hemiplegic migraine (FHM). BACKGROUND: FHM is an autosomal dominant disease, classified as a subtype of migraine with aura. Only a proportion of FHM patients have been associated with chromosome 19p13. Among these, four missense mutations within the CACNA1A gene in five unrelated families have been described. methods: A linkage study was performed in 19 patients affected by FHM from five families by studying microsatellite markers associated with the 19p13 region. All familial and seven additional sporadic patients with FHM were analyzed to search for mutations within the CACNA1A gene by applying the double gradient-denaturant gradient electrophoresis technique. RESULTS: lod score values did not establish significantly linkage to chromosome 19. However, seven new genetic variants were detected: six were new polymorphisms. The seventh was a missense mutation present in family 1, and it was associated with a hemiplegic migraine phenotype without unconsciousness and cerebellar ataxia. Because this missense mutation is absent in the general population and cosegregates with the disease, it may be a pathologic mutation. CONCLUSIONS: genetic heterogeneity of FHM has been shown in familial and sporadic FHM patients of Italian origin. The new missense mutation-G4644T-is associated with milder clinical features compared with typical FHM.- - - - - - - - - - ranking = 2.7994023595375keywords = familial hemiplegic migraine, hemiplegic migraine, migraine, aura (Clic here for more details about this article) |
4/86. A new CACNA1A gene mutation in acetazolamide-responsive familial hemiplegic migraine and ataxia.OBJECTIVE: To search for mutations in the calcium channel gene CACNA1A and to study the genotype-phenotype correlation in a family with a severe familial hemiplegic migraine (FHM) phenotype and a slowly progressive cerebellar ataxia. BACKGROUND: CACNA1A gene mutations on chromosome 19 are involved in approximately 50% of FHM families. The association of FHM and cerebellar ataxia has been reported in a small number of FHM families, all linked to chromosome 19. methods: The proband, in addition to typical hemiplegic migraine attacks, experienced severe episodes during which hemiplegia was associated with acutely altered consciousness and fever lasting several days. She, as well as her affected sister, developed a permanent, late-onset cerebellar ataxia and cerebellar atrophy evident on MRI. Linkage analysis was performed and the whole CACNA1A gene, 47 exon-intron boundaries, was analyzed by double gradient-denaturing gradient gel electrophoresis (DG-DGGE). RESULTS: Genetic studies suggested linkage to chromosome 19p13, and DG-DGGE analysis detected a heteroduplex fragment in exon 13 of the CACNA1A gene. By direct sequencing, a G-to-A substitution resulting in an arginine to glutamine change at codon 583 in the second putative voltage sensor domain of the channel alpha1A-subunit, was identified, possibly representing the disease-causing mutation. The proband and her affected sister were treated with acetazolamide, reporting freedom from new FHM attacks but no benefit in the progression of ataxia. CONCLUSIONS: The combination of episodic dysfunction and permanent deficit could depend on the variety of functions of calcium channels and their distribution in the nervous system.- - - - - - - - - - ranking = 2.7861392642822keywords = familial hemiplegic migraine, hemiplegic migraine, migraine (Clic here for more details about this article) |
5/86. The effect of intravenous verapamil on cerebral hemodynamics in a migraine patient with hemiplegia.OBJECTIVE: To describe the use of intravenous verapamil in a migraine patient with hemiplegia to reverse the symptomatology and hemodynamics of the middle cerebral artery as determined by transcranial Doppler. CASE SUMMARY: A 31-year-old white woman was admitted with an acute exacerbation of migraine with hemiplegia. A transcranial Doppler showed an increased flow velocity through the middle cerebral artery consistent with a migrainous process. The patient was treated with verapamil 5 mg iv and the hemiplegia gradually resolved. A transcranial Doppler indicated that the flow velocity through the middle cerebral artery was decreased after verapamil administration, indicating reversal of the vasospasm. DISCUSSION: Transcranial Doppler has not been previously used to determine the effect of intravenous verapamil on the migrainous process. Intravenous verapamil reversed the altered hemodynamics of the middle cerebral artery as determined by transcranial Doppler. This finding correlated with the gradual resolution of hemiplegia. Whether both subjective and objective findings in this patient can be attributed to the reversal of the cerebral artery hemodynamics is not known. CONCLUSIONS: Intravenous verapamil appears to reverse the vasospasm that may be associated with a migrainous process. Whether this effect is solely responsible for clinical improvement is not known. verapamil may be a consideration for the treatment of intractable migraine, especially when there is evidence of spasm of the major cerebral arteries.- - - - - - - - - - ranking = 0.092694475352792keywords = migraine (Clic here for more details about this article) |
6/86. A family with hemiplegic migraine and focal seizures.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.- - - - - - - - - - ranking = 0.55809919738088keywords = hemiplegic migraine, migraine (Clic here for more details about this article) |
7/86. epilepsy and paroxysmal movement disorders in families: evidence for shared mechanisms.The epilepsies have been regarded as clinically distinct from the paroxysmal movement disorders. Recently, a variety of ion channel defects have been identified as the biological basis of certain familial epilepsies and paroxysmal movement disorders. We studied two families with the co-occurrence of epilepsy, movement disorders and migraine. Information was obtained on 147 individuals in the two families. In family WF, there was a co-occurrence of epilepsy (benign infantile convulsions, idiopathic generalized epilepsy), episodic ataxia (with cerebellar atrophy and without myokymia) and common migraine. In family CL, epilepsy (febrile seizures, febrile seizures plus), kinesigenic paroxysmal dyskinesia and migraine (including hemiplegic migraine) were observed in various combinations over 3 generations. The observations in these two families, together with review of the literature, suggest that the co-occurrence of epilepsy (particularly benign infantile convulsions), paroxysmal movement disorders and migraine is not due to chance. Thus, these distinct clinical phenomena could have a shared biological basis and ion channel defects are an attractive possibility.- - - - - - - - - - ranking = 0.12702155643849keywords = hemiplegic migraine, migraine (Clic here for more details about this article) |
8/86. Hemiplegic migraine induced by exertion.BACKGROUND: It is known that exertion can aggravate migraine headache. However, the relationship between exertion and migraine aura is unknown. OBJECTIVE: To study the relationship between exertion and migraine aura. DESIGN: Case report. SETTING: Tertiary care hospital. PATIENT: A 67-year-old man presented with recurrent attacks of exertion-induced hemiplegic migraine. Since the hemiparetic attacks were exertion induced, they were initially ascribed to recurrent transient ischemic attacks. However, the clinical picture, normal findings on cerebral angiography and neuroimaging (during the period of hemiparesis), lack of response to treatment with antiplatelets and anticoagulants, and successful treatment with verapamil suggested that the hemiparesis was not due to ischemia, but was indeed a migraine aura. We suggest that exertion induced the aura of hemiparesis by lowering the threshold for the development of cortical spreading depression. Even though our patient had no family history of hemiplegic migraine, a mutation in an ion channel gene (eg, the CACNA1A gene on chromosome 19) might account for his episodic attacks. CONCLUSION: Migraine aura should be included in the differential diagnosis of exertion-induced focal neurologic deficit.- - - - - - - - - - ranking = 0.25414824961576keywords = hemiplegic migraine, migraine, aura (Clic here for more details about this article) |
9/86. Alternating hemiplegia of childhood: no mutations in the familial hemiplegic migraine CACNA1A gene.INTRODUCTION: Alternating hemiplegia of childhood (AHC) is a rare disorder mainly characterized by attacks of hemiplegia and mental retardation. It has been often associated with migraine. The CACNA1A gene on chromosome 19 is involved in familial hemiplegic migraine and other episodic cerebral disorders, but also with progressive neuronal damage. methods: We performed mutation analysis in this gene in four AHC patients, using single strand conformation polymorphism analysis. RESULTS: We found nine polymorphisms, but no mutations in any of the 47 exons. CONCLUSIONS: Other cerebral ion channel genes remain candidate genes for AHC.- - - - - - - - - - ranking = 2.7253280473814keywords = familial hemiplegic migraine, hemiplegic migraine, migraine (Clic here for more details about this article) |
10/86. Dissociated long lasting improvements of straight-ahead pointing and line bisection tasks in two hemineglect patients.In this experiment, we evaluated over a longer time period the previously demonstrated effects of a short prism adaptation on hemispatial neglect. We followed two patients (PE and SA), during a period of 5 days (1 day before and 4 days after the prism adaptation procedure), repeatedly measuring their performances on a straight-ahead pointing task and a line bisection task. We also assessed the comparative temporal evolution of the rightward biased egocentric reference frame (as measured by the straight-ahead demonstration) and a classical neuropsychological symptom of neglect, namely the rightward bias observed on line bisection. Firstly, the results showed that prismatic effect could be maintained for 4 days, on the two tasks (separately straight-ahead for PE, line bisection for SA). This long-term effect implies a very profound action of prism adaptation based on active processes and opens large possibilities for clinical applications. Secondly, no correlation was found between the evolution of the performances on the two tasks, neither for patient PE, nor for patient SA (within-subject double-dissociation). Moreover, a double-dissociation between subjects was demonstrated on long-term effects. A new conception has thus to be found to explain the various symptoms manifested in neglect and more investigations have to be performed in order to establish to what extent they can be considered independent. It can be concluded that elucidating the mechanism through which prism adaptation affects neglect could lead to a better understanding of the neglect syndrome.- - - - - - - - - - ranking = 3.9145515442659E-7keywords = classic (Clic here for more details about this article) |
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