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Hemiplegic migraine (HM) can cause significant functional impairment and negatively affect the quality of life of affected individuals. Emerging evidence suggests an association between migraines and congenital patent foramen ovale (PFO), which is a small opening between the atria of the heart that normally closes shortly after birth. This report describes a 34 years-old woman with sporadic hemiplegic migraine (SHM) who was diagnosed with PFO. Following percutaneous PFO closure, her hemiplegic symptoms disappeared, but her headache exacerbated. After 3 years of follow-up, her headache severity gradually reduced, and the frequency remained consistent at 2-3 times per year with no aura symptoms. This case highlights the dissociation between the resolution of hemiplegic symptoms and the persistence of headaches after PFO closure in sporadic HM. Patients with HM may experience changes in aura symptoms and headache severity after PFO closure. Before performing PFO closure in patients with hemiplegic migraine, the indications should be thoroughly understood. [ABSTRACT FROM AUTHOR]

Migraine is a disabling neurological disorder burdening patients globally. Through the increasing development of preclinical and clinical experimental migraine models, advancing appreciation of the extended clinical phenotype, and functional neuroimaging studies, we can further our understanding of the neurobiological basis of this highly disabling condition. Despite increasing understanding of the molecular and chemical architecture of migraine mechanisms, many areas require further investigation. Research over the last three decades has suggested that migraine has a strong genetic basis, based on the positive family history in most patients, and this has steered exploration into possibly implicated genes. In recent times, human genome-wide association studies and rodent genetic migraine models have facilitated our understanding, but most migraine seems polygenic, with the monogenic migraine mutations being considerably rarer, so further large-scale studies are required to elucidate fully the genetic underpinnings of migraine and the translation of these to clinical practice. The monogenic migraine mutations cause severe aura phenotypes, amongst other symptoms, and offer valuable insights into the biology of aura and the relationship between migraine and other conditions, such as vascular disease and sleep disorders. This review will provide an outlook of what is known about some monogenic migraine mutations, including familial hemiplegic migraine, familial advanced sleep-phase syndrome, and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. [ABSTRACT FROM AUTHOR]

Background: Only a few studies have focused on hemiplegic migraine (HM) in children despite its early age of onset. The aim of this review is to describe the peculiar characteristics of pediatric HM. Methods: This is a narrative review based on 14 studies on pediatric HM selected from 262 papers. Results: Different from HM in adults, pediatric HM affects both genders equally. Early transient neurological symptoms (prolonged aphasia during a febrile episode, isolated seizures, transient hemiparesis, and prolonged clumsiness after minor head trauma) can precede HM long before its onset. The prevalence of non-motor auras among children is lower than it is in adults. Pediatric sporadic HM patients have longer and more severe attacks compared to familial cases, especially during the initial years after disease onset, while familial HM cases tend to have the disease for longer. During follow-up, the frequency, intensity, and duration of HM attacks often decrease. The outcome is favorable in most patients; however, neurological conditions and comorbidities can be associated. Conclusion: Further studies are needed to better define the clinical phenotype and the natural history of pediatric HM and to refine genotype–phenotype correlations in order to improve the knowledge on HM physiopathology, diagnosis, and outcome. [ABSTRACT FROM AUTHOR]

Background: Neuronal intranuclear inclusion disease (NIID) is considered a heterogeneous disease because of its highly variable clinical manifestations. To date, there are no reports of NIID patients presenting with hemiplegic migraine (HM)‐like headache, or of HM and NIID co‐occurring as comorbidity, and the connection between these 2 seemingly unrelated clinical conditions has yet to be established. Method: We present a patient with NIID who was previously diagnosed with HM. To determine the pathogenesis of HM in this NIID patient, we systematically reviewed published NIID and HM cases and cataloged them based on their clinical manifestations. Result: The clinical manifestations of NIID is highly various; however, there is no case reported to date that shows HM‐like symptoms or cerebral edema. All documented symptomatic HM cases show vascular dysfunction to various degrees, but none of them has been shown to be correlated with NIID. Conclusion: Our patient is the first documented case in which HM and NIID occur simultaneously. Vascular dysfunctions that cause cerebral hypoperfusion and glucose hypometabolism, two of the dominant causes of symptomatic HM, may be associated with the accumulation of eosinophilic hyaline inclusions that cause NIID. However, the existence of inclusions may also alter neuronal behavior and indirectly cause cerebral hypoperfusion and glucose hypometabolism. Further research and observations are needed to examine the relationship between HM and NIID. [ABSTRACT FROM AUTHOR]

Background and Purpose: To identify the differences in overall occurrence, location, and disease burden of white matter hyperintensities (WMH) in patients with sporadic hemiplegic migraine (SHM) and patients with migraine headaches.Methods: We included patients who met diagnostic criteria proposed by the third International Classification of Headache Disorders (ICHD-3) for SHM and migraine headache. WMHs were identified using T2 fluid-attenuated inversion recovery axial sequence and classified based upon the location. The disease burden was assessed using Scheltens visual rating scale.Results: Fifty patients met the diagnostic criteria for SHM and 100 patients for migraine headache. Patients in the study group were similar to the control group in terms of age (47.7 ± 12.2 years vs. 48.17 ± 9.7 years; P = .814) and gender (M: F; 14:36 vs. M: F 25:75; P = .693). WMH were found in 28 (56%) patients with SHM and 44 (44%) in patients with migraine headache. The proportion of patients with WMH was not different between the two groups (P = .166). On univariate analysis, the proportion of patients with WMH in parietal, occipital, and infratentorial regions was higher in patients with SHM. White matter burden determined by visual rating scale and proportion of patients with lesions ≥5 mm in diameter was also significantly higher in patients with SHM. On multivariate analysis, the WMH occurrence in the parietal lobe (P = .043) was found to be significantly higher in SHM.Conclusions: The WMH occurrence in patients with SHM is significantly more in the parietal lobe when compared to those with migraine headaches. WMH burden was also higher in patients with SHM, and larger white matter lesions occurred more frequently in these patients with SHM (compared to ordinary migraineurs). [ABSTRACT FROM AUTHOR]

We describe the case of one patient with pure sporadic hemiplegic migraine (SHM) with a novel ATP1A2 gene variant and a large patent foramen ovale (PFO) with atrial septal aneurysm. In hemiplegic migraine (HM), the relationship between incomplete penetrance, environmental triggers, and phenotypic expression is underdetermined. A genetic evaluation of the proband was requested for the HM associated genes and extended to the members of his family. Genetic analysis revealed a never described before ATP1A2 gene mutation, inherited by his father, who never experienced motor aura but only typical visual aura. The proband-but not his father-was also affected by a large PFO with atrial septal aneurysm. SHM patient showed a marked reduction in motor aura episodes per year in the 12 months following the PFO percutaneous closure, followed by a complete remission from attacks at least in the following 24 months. We speculated that as well as incomplete penetrance of the novel mutation and natural history of the disease, an additional pathological condition such as the PFO could contribute to the phenotypical expression in this case of HM. [ABSTRACT FROM AUTHOR]

Summary Electroencephalographic (EEG) abnormalities have been reported during migraine attacks but their spatial and temporal distributions are not well known. We report the temporospatial dynamics of EEG during the full duration of a migraine attack with aura in a 19-year-old woman. She experienced episodes of hemiplegic migraine since the age of 2.5 years, with right hemibody paralysis preceded by visual symptoms. She reported severe pain of the right hemibody just before hemiplegia that was enventually suggestive of possible epileptic seizure, justifying diagnostic video-EEG monitoring. Sporadic hemiplegic migraine was diagnosed in the absence of family history. EEG was normal at the beginning of visual aura. After 15 minutes, posterior slow waves appeared over the migrainous hemisphere, spreading progressively towards anterior regions: first the central region (5 minutes after onset of contralateral hemiplegia), then the frontal region and over both hemispheres. A new de novo mutation was identified in the SCN1A gene. [ABSTRACT FROM AUTHOR]

Abstract: My aim was to report for the first time (to my knowledge) the successful treatment of 3 patients with sporadic hemiplegic migraine by surgical cautery of terminal branches of the external carotid artery under conscious sedation. Since the operations (between 1 and 2 years) none of the patients have had further attacks of migraine or hemiplegia. This preliminary report suggests that for patients with sporadic hemiplegic migraine with a confirmed arterial component, surgical cautery of selected terminal branches of the external carotid artery may be effective treatment. [Copyright &y& Elsevier]

The article discusses a case study of a patient with sporadic hemiplegic migraine (SHM), migraine attacks that are associated with motor weakness during the aura phase. Findings of the tests showed a diffusely reduced glucose metabolism of the supratentorial cortex and hypometabolism of the left cerebellum.

Hemiplegic migraine (HM) is a rare subtype of migraine with aura, characterized by transient hemiparesis during attacks. Diagnosis is based on the International Classification of Headache Disorders criteria (ICHD-II). Two types of HM are recognized: familial (FHM) and sporadic hemiplegic migraine (SHM). HM is genetically heterogeneous. Three genes have been identified ( CACNA1A, ATP1A2, and SCN1A) but more, so far unknown genes, are involved. Clinically, attacks of the 3 subtypes cannot be distinguished. The diagnosis can be confirmed but not ruled out by genetic testing, because in some HM patients other, not yet identified, genes are involved. The presence of additional symptoms (such as chronic ataxia or epilepsy) may increase the likelihood of identifying a mutation. Additional diagnostics like imaging, CSF analysis, or an EEG are mainly performed to exclude other causes of focal neurological symptoms associated with headache. Conventional cerebral angiography is contraindicated in HM because this may provoke an attack. Because HM is a rare condition, no clinical treatment trials are available in this specific subgroup of migraine patients. Thus, the treatment of HM is based on empirical data, personal experience of the treating neurologist, and involves a trial-and-error strategy. Acetaminophen and NSAIDs often are the first choice in acute treatment. Although controversial in HM, triptans can be prescribed when headaches are not relieved sufficiently with common analgesics. An effective treatment for the severe and often prolonged aura symptoms is more warranted, but currently no such acute treatment is available. Prophylactic treatment can be considered when attack frequency exceeds 2 attacks per month, or when severe attacks pose a great burden that requires reduction of severity and frequency. In no strictly preferred order, flunarizine, sodium valproate, lamotrigine, verapamil, and acetazolamide can be tried. While less evidence is available for prophylactic treatment with topiramate, candesartan, and pizotifen, these drugs can also be considered. The use of propranolol in HM is more controversial, but evidence of adverse effects is insufficient to contraindicate beta-blockers. [ABSTRACT FROM AUTHOR]

Objective: To summarize and evaluate proton (1H) and phosphorus (31P) magnetic resonance spectroscopy (MRS) findings in migraine.Methods: A thorough review of 1H and/or 31P-MRS studies in any form of migraine published up to September 2011.Results: Some findings were consistent in all studies, such as a lack of ictal/interictal brain pH change and a disturbed energy metabolism, the latter of which is reflected in a drop in phosphocreatine content, both in the resting brain and in muscle following exercise. In a recent interictal study ATP was found to be significantly decreased in the occipital lobe of migraine with aura patients, reinforcing the concept of a mitochondrial component to the migraine threshold, at least in a subgroup of patients. In several studies a correlation between the extent of the energy disturbance and the clinical phenotype severity was apparent. Less consistent but still congruent with a disturbed energy metabolism is an observed lactate increase in the occipital cortex of several migraine subtypes (MwA, migraine with prolonged aura). No increases in brain glutamate levels were found.Conclusion: The combined abnormalities found in MRS studies imply a mitochondrial component in migraine neurobiology. This could be due to a primary mitochondrial dysfunction or be secondary to, for example, alterations in brain excitability. The extent of variation in the data can be attributed to both the variable clinical inclusion criteria used and the variation in applied methodology. Therefore it is necessary to continue to optimize MRS methodology to gain further insights, especially concerning lactate and glutamate. [ABSTRACT FROM PUBLISHER]

Hemiplegic migraine is a rare form of migraine characterized by periodic attacks of migraine with neurologic aura and transient hemiplegia. There are familial and sporadic cases, both on a genetic basis; we describe the case of a 6-year-old boy affected by sporadic hemiplegic migraine, showing a novel ATP1A2 gene missense mutation (p.Gly715Arg) in exon 16. Long-term treatment with flunarizine resulted in good clinical response and prevention of further attacks. [ABSTRACT FROM AUTHOR]

Objective: Our objective was to study the long-term prognosis of sporadic hemiplegic migraine (SHM).Methods: We performed a longitudinal follow-up study in 18 patients who were diagnosed with SHM between 1993 and 1996. Follow-up time between the first and second survey ranged from nine to 14 years. These patients were included as part of a genetic study in which we systematically analysed the role of the three known familial hemiplegic migraine (FHM) genes.Results: In 12 out of 18 patients the clinical diagnosis was unchanged. In two of the six remaining patients the attacks were no longer associated with hemiplegia; one of them had an ATP1A2 gene mutation (E120A). In the four other patients, the diagnosis changed into FHM, because a family member had developed hemiplegic migraine since the initial diagnosis was made. In two of the four patients a mutation was demonstrated (CACNA1A [R583Q] and ATP1A2 [R834X]). Conclusion: This study shows that the diagnosis of SHM changes into FHM in a considerable percentage of patients (22% [4 of 18]), almost a decade after the initial diagnosis. This indicates that a careful follow-up of SHM patients and their families is advisable for optimal care and counseling. Diagnostic screening of FHM genes in SHM patients can be of value. Our genetic and clinical follow-up studies reinforce the evidence that FHM and SHM are part of the same spectrum of migraine. [ABSTRACT FROM PUBLISHER]

The digiti quinti sign (DQS), described originally as a clinical indication of subtle motor deficit, consists of a relatively greater abduction of the fifth finger on the affected side when both arms are extended forwards. This sign was previously observed interictally in three consecutive hemiplegic migraine (HM) patients.Patients and methods: To verify whether the DQS specifically discriminates HM from non-hemiplegic migraine (nHM), the angle between the fourth and fifth fingers (ANG) was measured interictally in 10 HM patients, 44 migraine with aura and migraine without aura patients, and 45 healthy controls.Results: The ANG was significantly wider at the symptomatic side in HM as compared with nHM and controls. The differences between the symptomatic and non-symptomatic (for HM) or between the right and left sides (absolute values for nHM and controls) were, respectively, 10.10° ± 9.58°, 4.15° ± 3.95° and 5.37° ± 4.74° (p = .007). The optimal cutoff point for ANG was 15° at the symptomatic side (sensitivity and specificity of 80.0% and 72.2%, respectively), 10.5° at the non-symptomatic side (sensitivity and specificity of 60.0% and 52.3%), and 3° for the difference between sides (sensitivity and specificity of 90.0% and 79.5%).Conclusion: Data show that the DQS discriminates HM from nHM and controls. [ABSTRACT FROM PUBLISHER]

The aim of this study was to investigate the involvement of the CACNA1A and ATP1A2 gene in a population-based sample of sporadic hemiplegic migraine (SHM). Patients with SHM ( n = 105) were identified in a nationwide search in the Danish population. We sequenced all exons and promoter regions of the CACNA1A and ATP1A2 genes in 100 patients with SHM to search for possible SHM mutations. Novel DNA variants were discovered in eight SHM patients, four in exons of the CACNA1A gene and four in exons of the ATP1A2 gene. Six of the variants were considered non-pathogenic. The causal role of the two remaining DNA variants is unknown until functional studies have been made or independent genetic evidence is discovered. Only very few DNA variants were identified in 100 SHM patients, and regardless of whether the identified variants are causal the CACNA1A and ATP1A2 genes are not major genes in SHM. [ABSTRACT FROM AUTHOR]

The aim of this study was to delineate any dysfunction of neuromuscular transmission (NMT) by single-fibre electromyography (SFEMG) in some rare types of migraine. Recent studies have shown subclinical dysfunction of NMT in migraine with aura and cluster headache by using SFEMG, whereas another recent study has shown NMT to be normal in familial hemiplegic migraine (FHM) with CACNA1A mutations. Thirty patients with rare primary headache syndromes [18 with sporadic hemiplegic migraine (SHM), six with FHM and six with basilar-type migraine (BM)] and 15 healthy control subjects without any headache complaints underwent nerve conduction studies, EMG and SFEMG during voluntary contraction of the extensor digitorum communis muscle. Ten to 20 different potential pairs were recorded and individual jitter values calculated. The results obtained from patient groups were compared with those from the normal subjects. Of 600 individual jitter values of the patients, 27 (4.5%) were abnormally high, whereas only 3/205 (1.5%) jitter values from normal subjects were abnormal. Abnormal NMT was found in 4/30 (13.3%) patients (three SHM and one BM), but in none of the control subjects. Only in SHM patients was the number of individual abnormal jitter values slightly but significantly different from normal controls. The present study demonstrates that subclinical NMT abnormality is slightly present in only SHM and BM patients, but not in FHM patients. [ABSTRACT FROM AUTHOR]

Hemiplegic migraine (HM) is a rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. The HM has two main forms according to the familial history: cases where at least one first- or second-degree relative has aura including motor weakness have familial hemiplegic migraine (FHM), and cases without such familial history have sporadic hemiplegic migraine (SHM). FHM is the only variety of autosomal dominant migraine and all three known genes encode ion-transporters. Typical HM attacks include a motor weakness that is always associated to other aura symptoms, the most frequent being sensory, visual, and speech disturbances. In addition, basilar-type symptoms occur in up to 70% of the patients. Severe attacks may occur in FHM as well as in SHM with prolonged hemiplegia, confusion, coma, fever, and seizures. The clinical spectrum also includes permanent cerebellar signs (nystagmus, ataxia, dysarthria) and less frequently various types of seizures and mental retardation. A genetic diagnosis is now possible by screening the two major genes involved in FHM ( and ). Treatment of FHM and SHM is based on what is done in other varieties of migraine with aura. According to the new physiopathologic insights, preventive treatments by various antiepileptic agents seem promising. [ABSTRACT FROM AUTHOR]

Migraine is a frequent primary paroxysmal headache disorder. Within the migraine spectrum, rare variants can be recognized, such as hemiplegic migraine and basilar-type migraine. Hemiplegic migraine can occur sporadically or run in families with an autosomal dominant inheritance pattern. Genetic research in familial hemiplegic migraine has led to the identification of three genes so far. The CACNA1A gene is associated with the FHM1 phenotype, the ATP1A2 gene with FHM2, and the SCN1A gene with FHM3. A large phenotypic variety is seen, without a clear genotype-phenotype relation. While additional cerebellar ataxia predominantly is associated with the FHM1 phenotype, all three are associated with epileptic phenomena. Mutated genes in all three types of FHM are important in ion transport, defining FHM—and probably migraine in general—as an “ionopathy.” Functional studies of FHM1, FHM2, and FHM3 link the effects to the initiation of cortical spreading depression. With the identification of the three genetic subtypes of FHM, confirmation of a migraine diagnosis by molecular tests has become available. [ABSTRACT FROM AUTHOR]

The aim of this study is to report a possible implication of augmented vasogenic leakage in the mechanism of prolonged aura in sporadic hemiplegic migraine. A 35-year-old woman with sporadic hemiplegic migraine presented with headache followed by right arm weakness, right visual field defect, aphasia and confusion that persisted for 1 week. During the acute stage, focal hyperaemia was seen in the left cerebral hemisphere corresponding to persistent aura symptoms. Augmented vasogenic leakage was demonstrated on delayed enhanced fluid-attenuated inversion recovery image. Magnetic resonance angiography showed dilation of the left middle cerebral artery. During the convalescent stage, such abnormal findings were not seen. Based on these results, we speculate that augmented vasogenic leakage from the leptomeningeal vessels, probably associated with activation of the trigeminovascular system, may delay the recovery of hemiplegic migraine aura. [ABSTRACT FROM AUTHOR]

Thomsen LL&Olesen J. Sporadic hemiplegic migraine. Cephalalgia 2004. London. ISSN 0333-1024Sporadic hemiplegic migraine (SHM) is defined as migraine attacks associated with some degree of motor weakness/hemiparesis during the aura phase and where no first degree relative (parent, sibling or child) has identical attacks. The present review deals with recent scientific studies according to which: The SHM prevalence is estimated to be 0.005%; SHM patients have clinical symptoms identical to patients with familial hemiplegic migraine (FHM) and significantly different from patients with migraine with typical aura (typical MA); SHM affected had no increased risk of migraine without aura (MO), but a highly increased risk of typical MA compared to the general population; SHM patients only rarely have mutations in the FHM gene CACNA1A; SHM attacks in some cases can be treated with Verapamil. The reviewed data underlie the change in the International Classification of Headache Disorders 2nd edition where SHM became separated from migraine with typical aura or migraine with prolonged aura. All cases with motor weakness should be classified as either FHM or SHM. [ABSTRACT FROM AUTHOR]

Abstract: Familial hemiplegic migraine (FHM) is a clinically and genetically heterogeneous disease most commonly linked to CACNA1A gene mutation. Epilepsy rarely occurs in FHM and is seen predominantly with specific CACNA1A gene mutations. Here we report a sporadic case of FHM1 linked to S218L CACNA1A gene mutation with the triad of prolonged hemiplegic migraine, cerebellar symptoms, and epileptic seizures. Epilepsy in this syndrome follows the pattern of isolated unprovoked seizures occurring only during childhood and hemiplegic migraine-provoked seizures occurring during adulthood. Clinical and electrographic status epilepticus can occur during prolonged migraine attacks. We suggest that patients with seizures, ataxia, and hemiplegic migraine be genetically tested for FHM. Patients with prolonged hemiplegic migraine attacks and confusion should be tested with continuous EEG recording to ascertain whether electrographic status is occurring, as intensive antiepileptic treatment not only resolves status but immediately stops hemiplegic migraine and improves associated neurological deficits. [Copyright &y& Elsevier]