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]

Brief Summary: The aim of the present study is to explore the importance of migraine genes on the headache/migraine responses after GTN in FHM-patients and healthy volunteers. [clinicaltrials.gov]

• Falling

  Once a treatment plan is established with your physician, it is likely to fall into one of two categories: abortive and pain relief or preventative. Abortive and Pain Relief –Triptans fall into this category of pain management. [migrainetreatmentgroup.com]

  Subtypes of Familial Hemiplegic Migraine: When genetic testing is available and has been performed, the results of that testing may be used to diagnose more specifically hemiplegic migraine falling into these subtypes: 1.2.3.1.1 Familial hemiplegic migraine [healthcentral.com]

  Hormonal migraine is a likely consequence of periodically falling hormone levels causing reduction in protein biosynthesis of metabolic components including intestinal tract serotonin. [pcm.me]

• Cough

  Other migraine symptoms may include Increased sensitivity to light, noise, and odors Nausea and vomiting Worsened pain when you move, cough, or sneeze Postdrome (following the headache). You may feel exhausted, weak, and confused after a migraine. [icdlist.com]

  Cefalea primaria da tosse 4.1.1 Probable primary cough headache 4.2. Cefalea primaria da attività fisica 4.2.1 Probable primary exercise headache 4.3. [curadeldolore.altervista.org]

  […] should also be differentiated from other primary headache disorders, such as stabbing headache, thunderclap headaches, hypnic headaches and hemicrania continua, and primary headache syndromes associated with physical activity (eg, exertional headaches, cough [emedicine.medscape.com]

• Vomiting

  Cyclic vomiting syndrome Cyclic vomiting of childhood is characterized by recurrent attacks of violent or prolonged vomiting without headache, which may last for hours. [48, 49, 50] Cyclic vomiting syndrome should especially be suspected in this setting [emedicine.medscape.com]

  Safe drug treatment options include NSAIDs, antiemetics (for nausea and vomiting) and narcotic analgesics. [diamondheadache.com]

  Slurred speech and other language difficulties Dizziness or vertigo Nausea and vomiting Extreme sensitivity to light, sound, and smell Loss of consciousness or even coma Confusion Symptoms will onset quick and may mimic those of a stroke. [migrainetreatmentgroup.com]

  […] an extremity, such as an arm or a leg speech difficulty headache symptoms similar to meningitis or inflammation associated with it motor weakness clumsiness or lack of coordination confusion drowsiness change in consciousness memory loss nausea and vomiting [medicalnewstoday.com]

  Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and extreme sensitivity to light and sound. [icdlist.com]

• Loss of Appetite

  Often, some of the minor headache phase symptoms may continue, such as loss of appetite, photophobia, and lightheadedness. [pcm.me]

• Photophobia

  Often, some of the minor headache phase symptoms may continue, such as loss of appetite, photophobia, and lightheadedness. [pcm.me]

  Photophobia, phonophobia, nausea and vomiting are also common symptoms. Motor weakness is uncommon in classic migraine (migraine with aura). Nonfamilial groups. [dizziness-and-balance.com]

  […] shimmers, visual field defects), numbness, tingling, trouble speaking Fever Impaired consciousness ranging from confusion to profound coma Ataxia (defective muscle coordination) Nausea and/or vomiting Phonophobia (increased sensitivity to sound) and/or photophobia [americanmigrainefoundation.org]

  (photophobia) Treatments for Hemiplegic Migraines Because hemiplegic migraines are rare many doctors have never treated anybody for the condition and are unsure about the right treatment. [steadyhealth.com]

  Nausea, vomiting, and photophobia can also be had. Two things to remember if you suffer with hemiplegic migraines. Find a very good headache specialist to give the right diagnose. Have medical identification on you at all times. [help-for-migraines.com]

• Diplopia

  Aged 32 she suffered of right hemisomatic sudden-onset paresthesias, hypoesthesia and hyposthenia, confirmed on examination; this episode lasted 45 min and it was followed by headache, sleepness, diplopia. [thejournalofheadacheandpain.biomedcentral.com]

  However, leaving aside the debatable issues in diagnostic criteria, on clinical grounds, the absence of visual symptoms and other brain stem features like vertigo, dysarthria (the girl was dysphasic), diplopia and ataxia would clinch the diagnosis of [neurologyindia.com]

  whereas SCA6 is associated with a CAG repeat expansion in the CACNA1A gene and is clinically characterized predominantly by adult-onset cerebellar ataxia, dysarthria and oculomotor disturbances consisting of gaze-evoked nystagmus, gaze paresis and diplopia [karger.com]

  The most common symptoms are dizziness and vertigo, but symptoms may also include headache accompanied by ataxia, tinnitus, decreased hearing, nausea and vomiting, dysarthria, diplopia, loss of balance, bilateral paresthesias or paresis, altered consciousness [emedicine.medscape.com]

• Facial Pain

  Green has been active in the practice of headache and facial pain since 1978. He has lectured throughout the world on this subject and has written numerous articles on various subjects concerning headache and facial pain. [books.google.com]

  […] neuralgias and central causes of facial pain Other headache, cranial neuralgia, central or primary facial pain Continuum “Glavobol”T. [slideserve.com]

  […] ophthalmoplegic neuropathy 13.10 Burning mouth syndrome (BMS) 13.11 Persistent idiopathic facial pain (PIFP) 13.12 Central neuropathic pain 13.12.1 Central neuropathic pain attributed to multiple sclerosis (MS) 13.12.2 Central post-stroke pain (CPSP) [curadeldolore.altervista.org]

  Al-Din A.S., Mir R., Davey R i wsp.: Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction. Cephalalgia 2005; 25: 605-611. 24. [yadda.icm.edu.pl]

  William Binder, an otolaryngologist and facial plastic surgeon, observed that botulinum toxin type A provided relief to migraine sufferers whom he was treating for cosmetic facial lines (5). [priory.com]

• Aura

  Er migrene med aura arvelig?. [duo.uio.no]

  In addition to the aura symptoms, the duration of a hemiplegic migraine aura is different from that of a typical migraine with aura. [verywell.com]

  Headache that meets criteria B to D for migraine without aura, and begins during the aura or follows aura within 60 minutes. D. [help-for-migraines.com]

  […] the aura may be prolonged and can be benign [ 2 ]. [pediatricneurologybriefs.com]

  […] symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes each aura symptom lasts ≥5 minutes and < 24 hours headache fulfilling criteria B-D for 1.1 Migraine without aura begins during the aura or follows [ihs-klassifikation.de]

• Ataxia

  Abstract Familial hemiplegic migraine type 1 (FHM1), episodic ataxia type 2 (EA2) and spinocerebellar ataxia type 6 (SCA6) are allelic disorders caused by mutations in the CACNA1A gene on chromosome 19p13. [karger.com]

  Episodic ataxia type 2, which is characterized by attacks of paroxysmal ataxia, is mostly associated with truncations of the gene, 14,29,30 and spinocerebellar ataxia type 6, a late-onset progressive ataxia, is associated with CAG-repeat expansions. 31,32 [nejm.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]

  87 364 POLG POLG-related ataxia neuropathy spectrum disorders, Sensory ataxia, dysarthria, and ophthalmoparesis, Alpers syndrome, Progressive external ophthalmoplegia with mitochondrial DNA deletions, Mitochondrial DNA depletion syndrome AD/AR 89 290 [blueprintgenetics.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]

• Seizure

  @inproceedings{Bhatia2011SporadicHM, title={Sporadic Hemiplegic Migraine with Seizures and Transient MRI Abnormalities}, author={Harsha S Bhatia and Fawzi A. [semanticscholar.org]

  The section offers management reviews in headache, seizures, epilepsy, neurobehavioral disorders, school readiness, developmental delay and a range of other conditions. [books.google.com]

  Acephalalgic migraine should be differentiated from transient ischemic attacks, occipital lobe seizures, and temporal lobe seizures. [emedicine.medscape.com]

  […] epileptic encephalopathy 6, Generalized epilepsy with febrile seizures plus, type 2, Febrile seizures, familial 3A AD 718 1585 SLC1A3 Episodic ataxia AD 2 17 SLC2A1 Stomatin-deficient cryohydrocytosis with neurologic defects, Epilepsy, idiopathic generalized [blueprintgenetics.com]

  Diagnosing hemiplegic migraine can be difficult, as the symptoms can mimic stroke, seizures or other conditions. [americanmigrainefoundation.org]

• Tingling

  Terri, who is now 21, also gets migraine with visual aura a few times per year and, on several occasions, has experienced migraine with a sensory aura (numbness/tingling up one arm and into her face). [headaches.org]

  It also causes temporary weakness, numbness and tingling, and paralysis on one side of the body. These symptoms start before the headache. “Hemiplegia” means paralysis. Hemiplegic migraine affects a small number of people who get migraine with aura. [healthline.com]

  Sometimes the patient experiences numbness, tingling, headache, and trouble speaking. The two main types of hemiplegic migraine are familial and sporadic. [headacheandmigrainenews.com]

  The symptoms of these terrible migraines can make them appear similar to strokes or epileptic episodes; these symptoms will include: Confusion Tingling Paralysis on just one side of the body Numbness Difficulty moving limbs Nausea Aside from the symptoms [blog.themigrainereliefcenter.com]

  […] weeks and include reversible hemiplegia (motor weakness/paralysis on one side of the body) and at least one of the following: Reversible visual symptoms such as seeing flickering lights and spots or loss of vision Reversible sensory symptoms such as tingling [diamondheadache.com]

• Motor Symptoms

  and motor symptoms lasts up to 72 hours at least one symptom on one side only the visual, sensory or motor symptom accompanied or followed by a headache within 1 hour For a correct HM diagnosis, all other potential reasons for the symptoms must be ruled [medicalnewstoday.com]

  Each non-motor symptom lasts 5-60 min and motor symptoms last < 72 hours. At least one symptom is restricted to one side of the body. The aura is accompanied within 60 minutes by a headache. [migraineagain.com]

  Diagnostic criteria require motor symptoms and at least one visual, sensory, or speech symptom, resembling basilar migraine. [pediatricneurologybriefs.com]

  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]

  Neurotherapeutics 4:274–284 CrossRef PubMed CAS Google Scholar Young WB, Gangal KS, Aponte RJ, Kaiser RS (2007) Migraine with unilateral motor symptoms (MUMS): A case control study. [link.springer.com]

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]

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]

Treatment of hemiplegic migraine can be challenging. [americanmigrainefoundation.org]

The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]

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]

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]

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]

BMJ 330:63–65 CrossRef PubMed Google Scholar Kirchmann M (2006) Migraine with aura: new understanding from clinical epidemiological studies. [link.springer.com]

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]

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]

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]

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]

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]