Presentation
SPS Nevrološka klinika Klinični center Ljubljana PowerPoint Presentation Download Presentation Prof. dr. Tomaž Pogačnik, dr. med. [slideserve.com]
Case Presentation This 63-year-old woman is a member of a family with FHM1 and cerebellar ataxia. [karger.com]
After assumption of a tablet of triptane, she referred a severe crisis and between the crisis of migraine with motor aurea muscle pain and fatigue were present. [thejournalofheadacheandpain.biomedcentral.com]
It has been shown that FHM1 very often has basilar-type symptoms in addition to the typical aura symptoms and that headache is virtually always present. [ihs-klassifikation.de]
Furthermore, migraines – including hemiplegic migraines – often present during early childhood (usually between the ages of 5 and 8). Fortunately, many cases of hemiplegic migraines subside in adulthood. [migrainerelief.com]
Neurologic
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Nystagmus
The most frequent sign was nystagmus, which was observed in 47 subjects. Gait ataxia was the second most frequent sign (in 36 subjects) and was associated with limb ataxia in 27 subjects, nystagmus in 19, and dysarthria in 9 ( Figure 2 ). [nejm.org]
Some affected individuals develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and rapid, involuntary eye movements called nystagmus. [icdlist.com]
Genetic testing may be of highest yield in people with early onset hemiplegic migraine associated with eye movement abnormalities (nystagmus), seizures or other persistent neurologic symptoms (ataxia). [americanmigrainefoundation.org]
Mutations in this gene also produce episodic ataxia type 2 (EA-2), another autosomal dominant paroxysmal cerebral disorder characterized by acetazolamide-responsive attacks of cerebellar ataxia and migraine-like symptoms, interictal nystagmus, and cerebellar [medlink.com]
About 20 percent of people with this condition develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and rapid, involuntary eye movements called nystagmus. [ghr.nlm.nih.gov]
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Cerebellar Sign
[…] hemiplegic migraine and cerebellar signs. [nejm.org]
You can help by adding to it. ( August 2017 ) Diagnosis [ edit ] Diagnostic criteria require motor symptoms and at least one visual, sensory, or speech symptom, resembling basilar migraine. [4] They may also be associated with cerebellar signs. [5] Investigations [en.wikipedia.org]
It is well described that FHM1 can present with cerebellar signs, but parkinsonism has not previously been reported in FHM1 or EA2 even though parkinsonism has been described in SCA6. [karger.com]
A G301R Na /K -ATPase mutation causes familial hemiplegic migraine type 2 with cerebellar signs. Neurogenetics. 2004;5:177–85. PubMed CrossRef Google Scholar 27. Dichgans M, Freilinger T, Eckstein G, et al. [link.springer.com]
Workup
The workup should include the elimination of these and other disorders with similar symptoms. [pediatricneurologybriefs.com]
A complete exam, including family history and a full neurological workup, is required. What makes the diagnosis of hemiplegic migraine extremely challenging is that its symptoms are so similar to those of vascular disease, stroke or epilepsy. [diamondheadache.com]
Gastroenterologic evaluation and workup typically yield unremarkable results. [48] Abdominal migraine symptoms are usually relieved with sleep. Antiemetics may help in aborting an acute attack. [emedicine.medscape.com]
Treatment
An effective treatment for the severe and often prolonged aura symptoms is more warranted, but currently no such acute treatment is available. [ncbi.nlm.nih.gov]
As with other types of Migraine, a treatment plan for HM includes both acute treatment and preventative treatment. [migraineagain.com]
[…] especially important in the treatment of hemiplegic migraine. [americanmigrainefoundation.org]
The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]
Prognosis
The prognosis is generally good with complete recovery after the attacks. The frequency of the attacks tends to decrease with age, and the pattern becomes similar to migraine with aura. [thejournalofheadacheandpain.springeropen.com]
Etiology
The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]
Ophthalmoplegic migraine: diagnostic criteria, incidence of hospitalization and possible etiology. Acta Neurol. Scand. 1990; 81: 54-60. 33. [yadda.icm.edu.pl]
Additionally, gastroparesis, which occurs during migraine, has been implicated as an etiologic factor for cyclic vomiting and abdominal migraine. [51, 52, 53] A serum lactate level is helpful in these children to exclude mitochondrial disorders. [emedicine.medscape.com]
Epidemiology
An epidemiological survey of hemiplegic migraine. Cephalalgia 2002; 22: 361-375. 40. [yadda.icm.edu.pl]
Comments: Epidemiological studies have shown that sporadic cases occur with approximately the same prevalence as familial cases. [ichd-3.org]
You can help by adding to it. ( August 2017 ) Epidemiology [ edit ] Prevalence is estimated to be 0.005%. [7] The age of onset has been found to be under 15 years in 40% of cases while it is between 10 and 14 years in one third of the cases. [en.wikipedia.org]
Epidemiology of headache in Europe. European journal of neurology : the official journal of the European Federation of Neurological Societies, 13 (4), 333-45 PMID: 16643310 Image via Evgeny Atamanenko / Shutterstock. [brainblogger.com]
Comments : Epidemiological studies have shown that sporadic cases occur with approximately the same prevalence as familial cases. [healthcentral.com]
Pathophysiology
More studies are needed to better understand the pathophysiologic role of these genes in comorbid depression, they added. "All hemiplegic migraine patients should be screened for depression," Terwindt said in an email. [medpagetoday.com]
The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]
@article{Russell2011SporadicAF, title {Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management}, author {Michael Bj{\o}rn Russell and A. H. [semanticscholar.org]
Internal and environmental factors such as hormonal, emotional, nutritional, physiological or weather changes may act as triggers, leading to the activation of several pathophysiological mechanisms. [brainblogger.com]
(FHM3) SCN1A [HSA: 6323 ] [KO: K04833 ] Other DBs ICD-11: 8A80.10 ICD-10: G43.1 MeSH: D020325 OMIM: 141500 602481 609634 Reference PMID: 21458376 (description, gene, drug) Authors Russell MB, Ducros A Title Sporadic and familial hemiplegic migraine: pathophysiological [genome.jp]
Prevention
Preventative Treatment : Prevention is especially important for those with Hemiplegic Migraine, because the symptoms are so severe and acute treatment options are limited. [migraineagain.com]
At this time it is suggested that the best method of treatment is in prevention methods such as the calcium channel blockers. Prevention also begins with your family’s medical history to help in the process of diagnosis. [steadyhealth.com]
Preventive treatment: Given the severity of the symptoms and the contraindication of certain acute medications (triptans and ergotamines), preventive regimens (medications taken daily to prevent the attacks whether you have a headache or not) are considered [americanmigrainefoundation.org]
Preventative – The most common form of treatment for hemiplegic migraines, given the risk of using the abortive triptan medication. [migrainetreatmentgroup.com]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]