Sporadic Hemiplegic Migraine

Cephalalgia ◽  
2004 ◽  
Vol 24 (12) ◽  
pp. 1016-1023 ◽  
Author(s):  
LL Thomsen ◽  
J Olesen

Sporadic 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.

Cephalalgia ◽  
2003 ◽  
Vol 23 (9) ◽  
pp. 921-928 ◽  
Author(s):  
LL Thomsen ◽  
E Ostergaard ◽  
SF Romer ◽  
I Andersen ◽  
MK Eriksen ◽  
...  

In order to better understand sporadic hemiplegic migraine (SHM) and particularly its relation to familial hemiplegic migraine (FHM), migraine without aura (MO) and typical migraine with aura (typical MA), we investigated the occurrence of MO and typical MA among probands with SHM and their first-degree relatives. The pattern of familial aggregation of MO and typical MA was assessed by population relative risk calculations. A total of 105 SHM probands and 483 first-degree relatives were identified in the Danish population. Compared with the general population, SHM probands had no increased risk of MO, but a highly increased risk of typical MA. First-degree relatives of all SHM probands had an increased risk of both MO and typical MA, whereas first-degree relatives of probands with exclusively SHM had no increased risk of MO but an increased risk of typical MA. Our data suggest that SHM is a genetically heterogeneous disorder.


Neurology ◽  
2003 ◽  
Vol 60 (4) ◽  
pp. 595-601 ◽  
Author(s):  
Lise L. Thomsen ◽  
Elsebet Ostergaard ◽  
Jes Olesen ◽  
Michael B. Russell

Objective: To compare clinical characteristics of patients with sporadic hemiplegic migraine (SHM) with those of patients with migraine with typical aura (MA) and patients with familial hemiplegic migraine (FHM).Methods: The authors used a computer search of Denmark’s National Patient Register to screen the population for patients with migraine with aura with motor weakness, and also examined case records from headache clinics and private practicing neurologists and placed advertisements. The authors screened patients and their relatives with a semi-structured validated telephone interview. All recruited patients were then interviewed by a physician and given a neurologic examination.Results: A total of 105 patients with SHM were identified. Seventy-two percent had four typical aura symptoms: visual, sensory, aphasic, and motor. All had at least two symptoms present during SHM attacks. A gradual progression and sequential appearance of aura symptoms was typical; compared with MA, the duration of each aura symptom was usually prolonged and bilateral motor symptoms were more frequent. Of the patients with SHM, 72% fulfilled the criteria for basilar migraine during SHM attacks. The aura was usually followed by headache, as is common in FHM but not MA.Conclusions: Patients with sporadic hemiplegic migraine had clinical symptoms identical to familial hemiplegic migraine and significantly different from migraine with typical aura. Sporadic hemiplegic migraine is a separate entity, and should be classified with familial hemiplegic migraine.


Cephalalgia ◽  
2015 ◽  
Vol 35 (14) ◽  
pp. 1261-1268 ◽  
Author(s):  
Kjersti Grøtta Vetvik ◽  
Jūratė Šaltytė Benth ◽  
E Anne MacGregor ◽  
Christofer Lundqvist ◽  
Michael Bjørn Russell

Objective The objective of this article is to compare clinical characteristics of menstrual and non-menstrual attacks of migraine without aura (MO), prospectively recorded in a headache diary, by women with and without a diagnosis of menstrual migraine without aura (MM) according to the International Classification of Headache Disorders (ICHD). Material and methods A total of 237 women from the general population with self-reported migraine in ≥50% of their menstrual periods were interviewed and classified by a physician according to the criteria of the ICHD II. Subsequently, all participants were instructed to complete a prospective headache diary for at least three menstrual cycles. Clinical characteristics of menstrual and non-menstrual attacks of MO were compared by a regression model for repeated measurements. Results In total, 123 (52%) women completed the diary. In the 56 women who were prospectively diagnosed with MM by diary, the menstrual MO-attacks were longer (on average 10.65 hours, 99% CI 3.17–18.12) and more frequently accompanied by severe nausea (OR 2.14, 99% CI 1.20–3.84) than non-menstrual MO-attacks. No significant differences between menstrual and non-menstrual MO-attacks were found among women with MO, but no MM. Conclusion In women from the general population, menstrual MO-attacks differ from non-menstrual attacks only in women who fulfil the ICHD criteria for MM.


2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Harsha Bhatia ◽  
Fawzi Babtain

Hemiplegic migraines are characterised by attacks of migraine with aura accompanied by transient motor weakness. There are both familial and sporadic subtypes, which are now recognised as separate entities by the International Classification of Headache Disorders, edition II (ICHD-II). Sporadic hemiplegic migraine is a rare variant of migraine, We report a case of sporadic hemiplegic migraine and seizures with MRI features suggestive of cortical hyper intensity and edema on T2 and FLAIR images with no restriction pattern on diffusion and these changes completely resolving over time, suggesting that these changes are due prolonged neuronal depolarization and not of ischemic origin.


2019 ◽  
pp. 418-434
Author(s):  
Maha Alattar

This chapter covers the relationship between sleep-related headaches and sleep disorders such as obstructive sleep apnea (OSA). Sleep apnea headache (SAH), a type of sleep-related headache that is classified in the International Classification of Headache Disorders, is a distinct subset of headache that is caused by OSA and occurs distinctly on awakening. Once recognized, treatment of OSA is associated with significant improvement in, and often resolution of, SAH. Given the high prevalence of headaches in the general population, sleep disorders must be considered in the evaluation of patients with headaches. A comprehensive sleep evaluation should be an integral part of the assessment of headache disorders. Sleep apnea headache and other types of headaches associated with sleep are reviewed in this chapter.


Cephalalgia ◽  
2009 ◽  
Vol 29 (12) ◽  
pp. 1337-1339 ◽  
Author(s):  
S Debiais ◽  
C Hommet ◽  
I Bonnaud ◽  
MA Barthez ◽  
S Rimbaux ◽  
...  

Familial hemiplegic migraine (FHM) is a rare autosomal dominant subtype of migraine with aura that is characterized by motor weakness during attacks. FHM1 is associated with mutations in the CACNA1A gene located on chromosome 19. We report a severe, prolonged HM attack in a young pregnant patient who had the S218L FHM1. This CACNA1A mutation has been associated with HM, delayed cerebral oedema and coma following minor head trauma. The case history we report suggests a specific, severe phenotype and the co-occurrence of HM and epilepsy related to the S218L FHM1 mutation.


Cephalalgia ◽  
2019 ◽  
Vol 40 (4) ◽  
pp. 337-346 ◽  
Author(s):  
Alberto Terrin ◽  
Federico Mainardi ◽  
Carlo Lisotto ◽  
Edoardo Mampreso ◽  
Matteo Fuccaro ◽  
...  

Background In literature, osmophobia is reported as a specific migrainous symptom with a prevalence of up to 95%. Despite the International Classification of Headache Disorders 2nd edition proposal of including osmophobia among accompanying symptoms, it was no longer mentioned in the ICHD 3rd edition. Methods We conducted a prospective study on 193 patients suffering from migraine without aura, migraine with aura, episodic tension-type headache or a combination of these. After a retrospective interview, each patient was asked to describe in detail osmophobia, when present, in the following four headache attacks. Results In all, 45.7% of migraine without aura attacks were associated with osmophobia, 67.2% of migraineurs reported osmophobia in at least a quarter of the attacks. No episodic tension-type headache attack was associated with osmophobia. It was associated with photophobia or phonophobia in 4.3% of migraine without aura attacks, and it was the only accompanying symptom in 4.7% of migraine without aura attacks. The inclusion of osmophobia in the ICHD-3 diagnostic criteria would enable a 9.0% increased diagnostic sensitivity. Conclusion Osmophobia is a specific clinical marker of migraine, easy to ascertain and able to disentangle the sometimes challenging differential diagnosis between migraine without aura and episodic tension-type headache. We recommend its inclusion among the diagnostic criteria for migraine as it increases sensitivity, showing absolute specificity.


2020 ◽  
Vol 10 (6) ◽  
pp. 372
Author(s):  
Giangennaro Coppola ◽  
Grazia Maria Giovanna Pastorino ◽  
Luigi Vetri ◽  
Floriana D’Onofrio ◽  
Francesca Felicia Operto

An Italian family with familial hemiplegic migraine (FHM) with the absence of mutations in the known genes associated with this disorder, namely ATP1A2, ATP1A3, CACNA1A, and SCN1A, has recently been reported. Soon afterward, whole exome sequencing allowed the identification of the carrier status of a heterozygous ATP1A4 mutation c.1798 C >T, in four affected members of this family. Here we compare the clinical symptoms of the affected family members with those from the other FHM families linked to mutations in the known genes associated with this disorder. A further two-year follow-up, including clinical response to carbamazepine administered to the proband and the maternal grandmother due to a worsening of the migraine symptoms, is reported. The clinical condition of the proband’s brother, carrying the same mutation and suffering from congenital ventricular and supraventricular extrasystoles, isdiscussed as well.


Cephalalgia ◽  
2013 ◽  
Vol 34 (1) ◽  
pp. 68-72 ◽  
Author(s):  
Cinzia Costa ◽  
Paolo Prontera ◽  
Paola Sarchielli ◽  
Alessandra Tonelli ◽  
Maria Teresa Bassi ◽  
...  

Background Familial hemiplegic migraine (FHM) is a rare autosomal dominant migraine subtype, characterized by fully reversible motor weakness as a specific symptom of aura. Mutations in the ion transportation coding genes CACNA1A, ATP1A2 and SCN1A are responsible for the FHM phenotype. Moreover, some mutations in ATP1A2 or SCN1A also may lead to epilepsy. Case Here we report on a three-generation family with five patients having a novel ATP1A2 mutation on exon 19, causing guanine-to-adenine substitution (c.2620G>A, p.Gly874Ser) that co-segregated in the five living relatives with migraine, four of whom had hemiplegic migraine. Moreover, three patients presented with epilepsy, one of whom had generalized epilepsy with febrile seizures plus (GEFS+). Conclusions The present study provides further evidence on the involvement of ATP1A2 mutations in both migraine and epilepsy, underlying the relevance of genetic analysis in families with a comorbidity of both disorders.


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