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How Rare Is Hemiplegic Migraine

Data Processing And Statistical Analysis

Hemiplegic Migraines Tied to Stroke

All data were processed and statistical analyses were performed using SPSS Base System 10.0 for Windows 98. The 2 test, MannWhitney test, KruskalWallis test, t test for independent samples and 95% confidence intervals were used as appropriate depending on the type of data. A 0.05 level of significance was used in single tests. When testing for multiple symptoms, a 0.001 level of significance was chosen in order to compensate for mass significance.

Consequences For Headache Classification Case Ascertainment And Clinical Diagnosis

In our families and in previous publications , there are several cases with reduced penetrance. It is therefore suggested to change the requirement for an affected firstdegree relative to a first or seconddegree relative.

Finally, it should be made clear in a comment that the presence of basilar migraine symptoms should not lead to a BM diagnosis, when concomitant with motor weakness, because BM symptoms are typically a part of FHM symptomatology. We suggest revision of IHS criteria for BM by adding an extra criterion: not associated with motor weakness, as shown in Table . The high frequency of BM symptoms in FHM suggests that pure BM patients, without motor weakness, may have the same genetic basis as FHM, as previously pointed out by others . This remains a hypothesis until genetic confirmation becomes available.

Table 4 Hemiplegic Migraine And Migraine With Unilateral Motor Symptoms

Clinical feature

Stereotyped sequence of symptoms. Weakness onset before headache

Cannot usually describe in detail. Weakness onset after headache. Give-way weakness


Severe, common during and between attacks

Headache frequency

Usually resolve within days

May be constant

Coexistence of hemiplegic migraine with other types of headache, such as short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing or short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms , has been reported .

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Acute And Emotional Stress

A recurring theme among various medical studies center on the role of stress in migraine, with it topping countless trigger lists. For hemiplegic migraine, stress comes in many formsacute stress during or after a stressful event as well as emotional tension. Evidence suggests that at least one-third of those with hemiplegic migraine cite various stressors as principal factors in the onset of attacks.1,2

How Is Hemiplegic Migraine Diagnosed

Hemiplegic Migraine Guide

Doctors diagnose hemiplegic migraine based on its symptoms.

Youre diagnosed with this type of headache if youve had at least two attacks of migraine with aura, weakness, and vision, speech, or language symptoms. These symptoms should go away after your headache improves.

Hemiplegic migraine can be hard to tell apart from other conditions, like stroke or mini-stroke . Its symptoms can also be similar to diseases like multiple sclerosis or epilepsy.

To rule out conditions with similar symptoms, your doctor will do tests like these:

  • A CT scan uses X-rays to create internal images of your body.
  • An MRI uses strong magnets and radio waves to make pictures inside your body.
  • An electroencephalogram measures the electrical activity in your brain.
  • An echocardiogram uses sound waves to create pictures of your heart.

If you have one or more family members with this type of migraine, you might want to have genetic testing.

However, most people with FHA will not test positive. Researchers have not yet found all the genes linked to this condition.

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Historical Note And Terminology

The discovery of genes associated with migraine and hemiplegic migraine enhanced our understanding of familial hemiplegic migraine . Familial hemiplegic migraine is linked to chromosome 19p13 in about 50% of families tested . Mutations in this gene also produce episodic ataxia type 2 , another autosomal dominant paroxysmal cerebral disorder characterized by acetazolamide-responsive attacks of cerebellar ataxia and migraine-like symptoms, interictal nystagmus, and cerebellar atrophy . A locus for EA-2 was mapped to chromosome 19p13 in the same interval as the FHM-1 locus . In 1996, Ophoff characterized a brain-specific P/Q-type Ca2+ channel 1-subunit gene, CACNA1A, which was implicated as a cause of both FHM-1 and EA-2 . CACNA1A mutations have a broad clinical spectrum due to different types of mutations .

Gardner and colleagues described a second locus in chromosome 1q33 related to a family with familial hemiplegic migraine features and a negative to CACNA1A mutations . Posteriorly, a dysfunction in the ATP1A2 gene on chromosome 1q21-23, encoding the Na+/K+pump, was associated with FHM-2 .

A heterozygous mutation in EAAT1 can lead to decreased glutamate uptake, leading to neuronal hyperexcitability that can cause seizures, hemiplegia, and episodic ataxia .

Identifying And Avoiding Triggers

Next, the persons migraine triggers must be identified and avoided. Each person may have a different combination of triggers. A migraine journal can be used to record your migraine symptoms, what time of day you have them, what occurred before the symptoms began, how long they last, and the severity. This will help you identify and later avoid your migraine triggers and your doctor accurately diagnose what type of migraine you have.

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Definition Of Basilartype Symptoms And Basilar Migraine

Basilartype symptoms were defined as simultaneous bilateral paresis and/or paresthaesiae, simultaneous visual symptoms in both the temporal and nasal fields of both eyes, dysarthria, vertigo, diplopia, tinnitus, decreased hearing, decreased level of consciousness, loss of balance when walking, drop attacks , crossed symptoms and symptoms switching from one side of the body to the other during the attack. The questions were based on the IHS criteria for BM .

Basilar migraine was confirmed in patients having at least two of the following symptoms: simultaneous bilateral paresis and/or paraesthesiae, simultaneous visual symptoms in both the temporal and nasal fields of both eyes, dysarthria, vertigo, double vision, tinnitus, decreased hearing or decreased level of consciousness. Loss of balance when walking is not necessarily ataxia, and the presence of loss of balance was therefore not used to diagnose BM. The permanent ataxia present in two families was unrelated to attacks, and was therefore not counted. The questions about drop attacks, crossed symptoms and symptoms switching from one side to the other are not part of the IHS criteria of BM and therefore were not used to diagnose BM.

Prevention And Risk Factors

Migraine Headaches : About Hemiplegic Migraines

Attacks of hemiplegic migraine often start in childhood or young adulthood. Youre more likely to have this type of headache if it runs in your family.

If one of your parents has hemiplegic migraine, you have a 50 percent chance of also getting these headaches, according to the Stroke Association.

You may not be able to prevent hemiplegic headaches if they run in your family. However, you can take medication to help reduce the number of headaches you get.

Another way to help prevent these migraine attacks is to avoid any factors that trigger your headaches.

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Familiar Or Sporadic Hemiplegic Migraine


Familial or sporadic hemiplegic migraine

  • Visual disturbance
  • Sensory loss
  • Difficulty with speech

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.


80%-99% of people have these symptoms
Focal motor
30%-79% of people have these symptoms
Cerebral edema
5%-29% of people have these symptoms
Alien limb phenomenon
1%-4% of people have these symptoms
Amaurosis fugax
Percent of people who have these symptoms is not available through HPO
Extreme sensitivity of the eyes to light Light hypersensitivity


Hemiplegic Migraine: An Unusual Type Of Migraine With Aura

Toronto, Canada


Since she was 13, Terri has been experiencing migraine without aura approximately 1-2 times per month. 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 . However, over the last 18 months, she has had five more dramatic episodes in which she experienced pronounced weakness/paralysis on one side of her body together with sensory symptoms and/or visual symptoms .

The weakness can last up to 12 hours and has led to Emergency Department visits on each occasion, with co-workers, family members, and Terri herself concerned that she could be suffering a stroke. CT scans and MRIs of her brain have been normal, ruling out a stroke. She was sent for tests of her heart and the blood vessels in her neck as well tests to determine if her blood was prone to form clots. All came back normal.



A rare and more dramatic subtype of migraine with aura is hemiplegic migraine. This is an unusual migraine disorder that affects only a very small segment of the population. With hemiplegic migraine, the aura consists of weakness or paralysis of one side of the body occurring in association with any of the visual, sensory, or speech disturbances described above. The aura in hemiplegic migraine is more prolonged and can last anywhere from 5 minutes to 24 hours .


Table #1

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Diagnosing Hemiplegic Migraine With 6 Symptoms

Hemiplegic Migraine is a type of Migraine with aura that includes motor weakness. Although plegic is commonly understood to mean paralysis, in many cases, there is only partial weakness. Muscle weakness is the major presenting symptom of HM, and, it sometimes even persists for weeks following the headache phase.

The first step in diagnosing an attack of weakness or other neurologic symptoms is to rule out dangerous causes like stroke or transient ischemic attack. Even if you have a history of Migraine, if you develop sudden weakness for the first time, you should go to the Emergency Room immediately to be evaluated. Once a neurologist has given you a diagnosis of Hemiplegic Migraine, you do not need to seek medical care with each attack, but when in doubt, it is better to err on the safe side if you are having any new symptoms.

  • Aura consisting of either:
  • Fully reversible visual, sensory, and/or speech/language symptoms.
  • At least 2 of the 4 characteristics:
  • Spreads gradually over at least 5 minutes.
  • 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.
  • The diagnosis of HM is only made when physicians are unable to better account for symptoms by another ICHD-3 diagnosis.
  • Hemiplegic Migraine: An Overview

    How rare are hemiplegic migraines ?

    Migraine is an inherited neurological disorder which causes a collection of symptoms including headache, brain fog, nausea or vomiting, and sensitivities to light, sound, smells and touch. A more unusual type of migraine, Hemiplegic Migraine, may also cause weakness or paralysis on one side of the face, the arm or leg or all down one side. The weakness is twice as likely to be in the upper arm as in the leg according to one study. This can occur as a part of the aura phase and be quite transient or it can persist as the headache comes on. The weakness can last anything between half an hour to several days. Occasionally the headache comes on first.

    There are two main types of hemiplegic migraine.

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    Migraine Causes And Symptoms

    A migraine has a usual cause of environmental and genetic factors. About two-third of migraineurs have a Migraine in their genetics. The nerve and blood vessels of the brain are believed to be involved in migraine.

    A migraine headache is a result of unusual changes within the brain. It causes severe head pain that is often accompanied by sensitivity to light, sound, or smells.

    Common Symptoms Include

    • Sensitivity to light or sound
    • Nausea
    • Vomiting
    • When usually severe pain happens on one side of the head that some individuals described as pounding

    Most typical migraines are distinguished by the fact that whether its accompanied by the aura or not.

    Table 3 Diagnostic Criteria For Sporadic Hemiplegic Migraine

    A. Fulfills criteria for 1.2.3 hemiplegic migraine B. No first- or second-degree relative fulfills criteria for 1.2.3 hemiplegic migraine

    Pelzer and colleagues reviewed the clinical characteristics of 281 patients with familial hemiplegic migraine or sporadic hemiplegic migraine . Patients with autosomal dominant mutations in CACNA1A, ATP1A2, or SCN1A were significantly more likely to have familial hemiplegic migraine, attacks triggered by mild head trauma, severe weakness, brain stem features, brain edema, or confusion during attacks. Only patients with mutations had either mental retardation or progressive ataxia.

    The true prevalence of motor symptoms in migraine outside of tertiary headache centers is unclear . Questionnaire-based studies of hemiplegic migraine, such as a study of 293 patients in Finland with familial hemiplegic migraine, found only 7% had mutations in CACNA1A, ATP1A2, and SCN1A genes. These patients reported significantly higher headache severity, unilateral pain, and disability than migraine with aura controls.

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    Medical Tests To Diagnose

    Get emergency medical help right away if you have any of these symptoms. Do not assume that you are having a hemiplegic migraine. It could be a stroke.

    A CT scan or an MRI of your head can show signs of a stroke. Tests of your heart and the blood vessels in your neck can rule out symptoms caused by blood clots.

    If you have a family member with similar symptoms, your doctor may want to do genetic testing. Familial hemiplegic migraine means it runs in your family, and you could pass it on to your children. People who don’t have problems with those four genes have sporadic hemiplegic migraine .

    Enhancing Healthcare Team Outcomes

    Hemiplegic Migraine

    A hemiplegic migraine is a very rare migraine headache accompanied by unilateral weakness. It can be very upsetting to the patient and their family. Typically an interprofessional approach involving a nurse experienced in headache education working with a clinical headache specialist to educate the patient and family will result in the best outcome.

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    Aphasic Symptoms Or Other Speech Disturbances

    The characteristics of the speech disturbances are shown in Table .

    Only those with problems in the production of language and problems in understanding what other people say were included as having aphasia. Eight individuals had dysarthria but no aphasia. The mean duration of the aphasia was 3 h 7 min . Fiftyeight per cent had prolonged duration of the aphasic aura symptoms.

    Table 1 Diagnostic Criteria For Hemiplegic Migraine

    A. At least 2 attacks fulfilling criteria B and C B. Aura consisting of both of the following:

    Fully reversible motor weakness Fully reversible visual, sensory, and/or speech/language symptoms

    C. At least 2 of the following 4 characteristics:

    At least 1 aura symptom spreads gradually over more than or equal to 5 minutes, and/or 2 or more symptoms occur in succession Each individual nonmotor aura symptom lasts 5 to 60 minutes, and motor symptoms last less than 72 hours At least 1 aura symptom is unilateral The aura is accompanied, or followed, within 60 minutes by headache

    D. Not better accounted for by another ICHD-3 diagnosis, and transient ischemic attack and stroke have been excluded

    Familial hemiplegic migraine. Familial hemiplegic migraine is migraine with aura including motor weakness, and at least 1 first- or second-degree relative has migraine aura including motor weakness.

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    What Is A Hemiplegic Migraine

    A hemiplegic migraine is a rare, serious type of migraine headache. The term hemiplegia refers to paralysis on one side of the body, which is a temporary symptom of a hemiplegic migraine. The symptoms of a hemiplegic migraine can mimic a stroke.


    Hemiplegic migraines are categorized into two types:

    • Familial hemiplegic migraines At least one other family member has the condition
    • Sporadic hemiplegic migraines No known family history of the condition


    The main feature of a hemiplegic migraine is an aura that involves numbness, weakness or paralysis on one side of the body. Possible additional symptoms include the following:

    • Dizziness
    • Visual disturbances, such as seeing wavy lines or spots
    • Language difficulties
    • Confusion or memory loss
    • Loss of consciousness

    Auras are the hallmark of hemiplegic migraines and are typically more severe and last longer than with other types of migraines. They can last for hours before slowly resolving. Most symptoms go away completely within a few days however, difficulties with concentration and memory loss can last from weeks to months.

    Typically, migraine headache pain, which is throbbing pain on one or both sides of the head, occurs after the aura phase however, it can also occur before or with the aura. With some hemiplegic migraines, headache pain may not develop. Symptoms can vary from one hemiplegic migraine to the next.



    • Stress or intense emotions

    Two Types Of Hemiplegic Migraine

    Rare Disease Day 2014

    Hemiplegic Migraine is divided into two types:

    Familial Hemiplegic Migraine and Sporadic Hemiplegic Migraine . Like most Migraine types, both types of HM often begin in childhood.

    Researchers know about four different genes related to familial Hemiplegic Migraine: CACNA1A, ATP1A2, SCN1A, and possibly PRRT2. Mutations in these genes result in over-excitable nerves. Familial Hemiplegic Migraine is most often diagnosed by looking at symptoms, family history, and ruling out other disorders, but genetic testing is also available.

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