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Hemiplegic Migraine

Hemiplegic Migraines

Hemiplegic migraine is an unusual variant of migraine. Persons with this form of migraine have headaches associated with the loss of motor strength on one side of the body. A migraine is a recurrent throbbing headache. Typically, the pain only affects one side of the head. While classic migraine is often accompanied by nausea and disturbed vision, with a hemiplegic migraine, the person may also have speech difficulties or confusion in addition to the unilateral weakness


Presentation

Paraplegic migraine typically exhibits the same stages, as typical migraines, with the addition of the one-sided weakness. Attacks may be initiated by environmental triggers or stress.

  • Prodrome: Many persons experience vague symptoms as much as 24 hours before the onset of an attack. These may include fatigue, depression, irritability, photophobia, phonophobia, and difficult sleeping.
  • Aura: The aura phase precedes that actual headache. There are focal neurological symptoms that may persist up to an hour. These symptoms include visual, sensory, or language disturbances. Normally, the headache will start within 30 minutes after the aura.
  • Headache: After resolution of the aura symptoms, the migraine headache begins. The migraine headache is described as a unilateral throbbing. It may be associated with nausea, vomiting, photophobia, or phonophobia. If not treated, the headache persists up to 72 hours. With hemiplegic migraine, the headache is accompanied by hemiparesis or hemiplegia. As with other typical migraines, persons may have visual disturbances (flickering lights, spots, or loss of vision) or problems with speech.
  • Resolution: Once the acute headache and associated symptoms subside, the person enters the resolution phase, frequently noted by a deep sleep. Sometimes persons describe a migraine hangover (malaise, fatigue, and headaches brought on by coughing or head movement).

The diagnosis of familial hemiplegic migraine (FHM) requires the diagnosis of HM in two or more persons from the same family. Approximately half of the cases of hemiplegic migraine will have family members that have been identified as having the disorder. Those who present with symptoms of hemiplegic migraine but without a known familial connection are said to have sporadic hemiplegic migraine (SHM). Although the cause of SHM is unknown, it is probably the result of spontaneous gene mutations.

Falling
  • After 5 h application of LPS in vitro, both WT and R192Q KI cultures demonstrated significant increase in macrophage activation, very large rise in TNFα mRNA content, and ambient protein levels together with fall in TNFα precursor, suggesting potent release[ncbi.nlm.nih.gov]
  • 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]
  • When Claire was 5, she had a mild fall that left her feeling dizzy. That dizziness quickly progressed to her being unable to use the right side her body. She couldn’t walk or talk.[chop.edu]
  • I practically fall into the bathroom, and throw up my food from the day into the toilet, and press my head onto the cold tile floor. The cold helps.[themighty.com]
  • Numbness or weakness on one side of the body (Hemiplegia) Once side of the face can fall which looks like a stroke Headache Visual disturbance and aura (sparkles, shimmers) Numbness or tingling in extremities Slurred speech or trouble speaking Fever Confusion[migraine.ie]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: November 30, 2018[patientslikeme.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: November 23, 2018[patientslikeme.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]
  • The symptoms of prodrome may include: lowered mood levels, especially depression or feelings of well-being and euphoria fatigue poor concentration and comprehension lowered intellect levels Summary Migraine.[healthcentral.com]
  • This forum is for fibromyalgia and Chronic Fatigue Immune Deficiency Syndrome (CFS/CFIDS).[neurotalk.psychcentral.com]
Vomiting
  • A 10-year-old boy with psychomotor developmental delay and cerebellar vermis atrophy developed right hemiplegia with vomiting, unconsciousness, convulsions, and late-onset fever.[ncbi.nlm.nih.gov]
  • The most characteristic were recurrent attacks of blurred vision, paresthesias and hemiparesis often accompanied by speech disturbances and followed by severe headache with vomiting.[ncbi.nlm.nih.gov]
  • Safe drug treatment options include NSAIDs, antiemetics (for nausea and vomiting) and narcotic analgesics.[diamondheadache.com]
Tinnitus
  • Two or more aura symptoms of the followingtypes: Dysarthria Vertigo Tinnitus Decreased hearing Double vision Ataxia Decreased level of consciousness Simultaneous bilateral visual symptoms in both the temporal and nasal field of both eyes Simultaneous[brain.oxfordjournals.org]
  • Other auras may include ringing in the ears (tinnitus), tingling in the arm or face, speaking difficulties, changes in smell (such as strange odors) or taste, or simply having a “funny feeling.”[my.clevelandclinic.org]
  • The prodromal symptoms reflect brainstem dysfunction: altered level of consciousness, dysarthria, tinnitus, ataxia, diplopia, symptoms in both the temporal and nasal fields of both eyes, and peripheral dysesthesias, followed by occipital headache.[physio-pedia.com]
  • Benign paroxysmal vertigo of childhood Benign paroxysmal vertigo of childhood (BPVC) is characterized by brief episodes of vertigo and disequilibrium lasting for hours, without headache, aura, hearing loss, or tinnitus.[emedicine.medscape.com]
Photophobia
  • Her motor symptom was always followed by development of nausea and photophobia lasting several hours. Computerized tomography of the brain and electroencephalography were normal. She was treated with carbamazepine, aspirin, and codeine.[ncbi.nlm.nih.gov]
  • 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]
  • […] other neurological symptoms and vegetative disorders especially pertraining to the gastric tract. aura focal transient neurological symptoms appearing directly during migraine preceding period or migraine itself visual changes: bright lights, scotoma, photophobia[prezi.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]
Diplopia
  • Searchable terms included basilar migraine, vertigo, dysarthria, diplopia, hemiplegia/hemiparesis, facial droop, weakness, confusion, altered consciousness, confusion, ataxia, and aphasia, as well as all triptans.[n.neurology.org]
  • 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]
  • Table 5 Basilar‐type symptoms experienced by 101* of 147 patients with familial hemiplegic migraine % n Definite brainstem symptoms Diplopia 51 52 Decreased hearing 48 48 Crossed symptoms 1 1 Bi‐cortical and/or brainstem symptoms Dysarthria 73 74 Loss[brain.oxfordjournals.org]
  • , 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]
  • TIA must be ruled out, as emboli from the carotid artery can cause similar symptoms. [3] Results in pain around the eye and paralysis in the distribution of the third, fourth, and sixth cranial nerve and can produce diplopia (double vision).[physio-pedia.com]
Aura
  • Hemiplegic migraine is a rare form of migraine with aura characterized by motor aura. Although auras in hemiplegic migraine are typically complex with two or more aura symptoms, neglect has been rarely described.[ncbi.nlm.nih.gov]
  • In these kindreds, we propose that hemiplegic aura is only a severe and complex form of typical aura.[ncbi.nlm.nih.gov]
  • We found cerebral hypoperfusion in the relevant cortical areas within the first hour after onset of aura symptoms. This report supports the concept that migraine aura across the migraine spectrum is caused by similar mechanisms.[ncbi.nlm.nih.gov]
  • To report biphasic changes in cerebral blood flow (CBF) in the acute phase of hemiplegic migraine with prolonged aura (HMPA), in which aura symptoms lasted longer than 24 h, in three patients with familial hemiplegic migraine (FHM) carrying a p.H916L[ncbi.nlm.nih.gov]
  • In contrast to patients suffering from migraine with aura and without aura, CGRP infusion did not induce more migraine-like attacks in FHM patients without known mutations compared to controls.[ncbi.nlm.nih.gov]
Headache
  • Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are also very rare conditions that are classified[ncbi.nlm.nih.gov]
  • All reported a positive therapeutic response, as their average headache frequency decreased by 92% (30 to 2.5 headache days/month); Visual Analog Score by 44% (9.5 to 5.3); frequency of hemiplegic episodes by 96% (7.5 to 0.25 hemiplegic episodes/month[ncbi.nlm.nih.gov]
  • A 10-year-old girl presented with episodic vascular headache and transient left sided hemiparesis, which persisted for a few hours and was clinically labelled as sporadic hemiplegic migraine according to International Headache Society criteria.[ncbi.nlm.nih.gov]
  • To avoid missing FHM1, a detailed clinical history including headache or hemiplegia is essential.[ncbi.nlm.nih.gov]
  • There was no difference in the incidence of CGRP-induced delayed headaches between the groups (P .18).[ncbi.nlm.nih.gov]
Stroke
  • Two patients with signs of acute ischemic stroke were evaluated for thrombolysis and turned out to suffer from familial hemiplegic migraine.[ncbi.nlm.nih.gov]
  • Roughly 10 to 15 percent of all first strokes are the result of a broken blood vessel in the brain, according to the American Heart Association and American Stroke Association Stroke Council.[livestrong.com]
  • Again there was little clinical or imaging evidence of new or old stroke.[bmj.com]
  • The word Hemiplegia comes from the Greek – ‘Hemi’ meaning ‘half’ and ‘Plegia’ from either ‘plēgē’ meaning ‘stroke’ or ‘plēssein’ meaning ‘to strike’.[migraine.ie]
  • A 44-year-old male was admitted with suspected stroke because of confusion and aphasia. Initial gadolinium-based perfusion MRI showed a decrease in cerebral blood flow and an increase in capillary flow disturbances within the left hemisphere.[ncbi.nlm.nih.gov]
Vertigo
  • Hemiplegic migraine symptoms include temporary paralysis down one side of the body, vertigo or difficulty walking, double vision or blindness, hearing impairment and numbness around the mouth, leading to trouble speaking or swallowing.[medical-dictionary.thefreedictionary.com]
  • Searchable terms included basilar migraine, vertigo, dysarthria, diplopia, hemiplegia/hemiparesis, facial droop, weakness, confusion, altered consciousness, confusion, ataxia, and aphasia, as well as all triptans.[n.neurology.org]
  • The other symptoms include Vertigo Ataxia (Lack of coordination of muscle movements) Coma Visual disturbance Problem in speech Trouble swallowing Loss of balance Epilepsy (Group of neurological diseases) The FHM and SHM share the same symptoms.[migrainerelief.co]
  • (sparkles, shimmers) Numbness or tingling in extremities Slurred speech or trouble speaking Fever Confusion, brain fog and in extreme and very rare cases possible unconsciousness Ataxia – Muscles not working properly Nausea and/or vomiting Dizziness/Vertigo[migraine.ie]
  • […] pins-and-needles feeling, often moving from your hand up your arm Numbness on one side of your body, which can include your arm, leg, and half of your face Weakness or paralysis on one side of your body Loss of balance and coordination Dizziness or vertigo[webmd.com]
Paresthesia
  • The most characteristic were recurrent attacks of blurred vision, paresthesias and hemiparesis often accompanied by speech disturbances and followed by severe headache with vomiting.[ncbi.nlm.nih.gov]
  • Aged 32 she suffered of right hemisomatic sudden-onset paresthesias, hypoesthesia and hyposthenia and the patient was genetically investigated for sporadic hemiplegic migraine.[ncbi.nlm.nih.gov]
  • Sporadic Hemiplegic Migraine migraine headache hemiplegic migraine migraine variant associated by paralysis or temporary weakness on one side of the body (lasts from 1 up to several days) accompanied by numbness, tingling sensation (paresthesia) and other[prezi.com]
  • […] aura symptoms of the followingtypes: Dysarthria Vertigo Tinnitus Decreased hearing Double vision Ataxia Decreased level of consciousness Simultaneous bilateral visual symptoms in both the temporal and nasal field of both eyes Simultaneous bilateral paresthesias[brain.oxfordjournals.org]

Workup

Because of the association with neurologic symptoms with either FHM or SHM, it is imperative to exclude other causes that may need treatment. Persons with a transient ischemic attack (TIA) present with stroke-like symptoms that last less than 24 hours. Like FHM or SHM, there is usually a unilateral weakness that may be associated with visual or speech disturbances.

TIAs may be secondary to carotid disease that can be excluded using carotid ultrasound to look for atherosclerotic disease. Electrocardiogram (EKG) and EKG rhythm monitoring may identify an irregular heart beating that may allow clots to form that can embolize to the brain causing neurologic symptoms. Echocardiography or ultrasonography of the heart can examine for prior cardiac muscle damage, as from a heart attack, that may allow clot to adhere to the muscle wall and be dislodged resulting in brain embolism. Sometimes, the neurologic findings can persist even up to or greater than 24 hours. In this setting, computerized tomography (CT scan) of the brain can exclude cerebral stroke or hemorrhage.

In the presence of symptoms directing at hemiplegic migraine, it is important to exclude other neurologic diseases. The SHM and FHM diagnoses are then based on an evaluation of the patients’ symptoms combined with a full family history. Genetic testing is now available for persons with hemiplegic migraine. If a person has a family member with hemiplegic migraine, or if testing is positive for the presence of genetic defects that have been linked to HM, the person is said to have the familial form of the disease. It would be imperative to pass that information on to family members.

Treatment

The first goal of treatment is to confirm the diagnosis by excluding other causes of the neurologic symptoms. Although there is disagreement about the safety and effectiveness of the usual migraine medications, they are frequently used in the treatment of FHM and SHM.

Treatment for FHM and SHM is not standardized. Some doctors use triptans (serotonin receptor agonists) as the drug of choice to treat hemiplegic migraines. Other medications may be employed to treat and prevent migraine attacks. Medication using an anticonvulsant or a calcium channel blocker may reduce the frequency of migraine attacks. Calcium channel blockers improve blood flow. Two drugs from this category, Verapamil and Flunarizine, are used frequently in the treatment of hemiplegic migraine.

Prognosis

For many people, migraine headaches will go into remission and sometimes they will stop completely. However, the symptoms may be very severe. Up to 90% of persons are unable to function normally on the day of the migraine attack. More than 80% will have abnormal sensitivity to noise and light. Up to 30% will need bed rest for a period after the acute attack. Migraine is a chronic disorder, but with treatment the number and severity of attacks may be reduced. Many physicians use traditional therapies for migraines. For FHM and SHM, calcium channel blockers may be useful in particular.

Etiology

The nervous system consists of the brain and the spinal cord (the central nervous system) and the nerves outside the spinal cord (the peripheral nervous system). Communication within the nervous system is carried out by the use of signals (electrical and chemical) that allow passage of these impulses from one nerve cell to the next. As the impulse arrives at the end of the nerve, it signals a gate to open releasing chemical messengers to tell the next nerve cell or muscle cell how to act. These chemicals that signal the next nerve cell are called neurotransmitters.

In the case of migraine, the neurotransmitters are released abnormally, signalling the next nerve cell or muscle in error. Researchers have identified three genes (CACNA1A, ATP1A2, and SCN1A) that when defective are associated with hemiplegic migraine. Mutations of these three genes result in the inability to make a specific protein needed for the communication between nerve cells. Thus, the neurons have trouble either releasing or binding up neurotransmitters causing the nerves to conduct in an abnormal manner. Typically, persons with familial hemiplegic migraine (FHM) have inherited the mutated gene from a single parent who was also symptomatic. The aberrant genes for FHM are found on chromosomes designated as 1 and 19. With the genetic mutations, the calcium channel doesn’t open or close properly. This inability to regulate calcium results in the neurons firing rapidly [1].

Effectiveness of calcium channel blockers has been found sometimes in preventing symptoms caused by FHM. A significant familial risk factor for migraine has long been apparent. This has been demonstrated in twin studies where the association for migraine in monozygotic twins is greater than that for dizygotic twins [2]. Persons without any family history are said to have the sporadic form of the disease. Sporadic hemiplegic migraine is associated with mutations in some genes (ATP1A2 and CACNA1A). Protein products of these genes transport charged ions across cellular membranes. The transport of these ions has a critical role in the conduction of message between nerve cells in the brain and the rest of the nervous system.

Epidemiology

The prevalence of sporadic hemiplegic migraine worldwide is not known. In Denmark, 1 in 10,000 people are estimated to have hemiplegic migraine. In case of FHM, the condition appears to be prevalent in about 50% of family members having a parent suffering from this disease. For sporadic hemiplegic migraine there is no family history for the condition.

Sex distribution
Age distribution

Pathophysiology

Hemiplegic migraine typically progresses through the same stages of symptoms as in other migraine headaches. Persons initially have a prodrome phase with malaise occurring hours before the onset of migraine event. Immediately before the migraine, persons describe an aura. With the hemiplegic variant, persons still describe severe headaches that may last for up to 72 hours if left untreated. In addition, persons have a one-sided paralysis or paresis with sever weakness. After the acute phase, persons move into the resolution phase and may have symptoms that have been described as a migraine hangover.

It is now apparent that genetic disorders account for hemiplegic migraine. Four different missense mutations have been identified in the α1A subunit of the P/Q-type on chromosome 19 that are deemed responsible for familial hemiplegic migraine (FHM) in some families [3]. This mutation results in a faulty calcium channel mediated transmission of the electrical signal from neuron to neuron or muscle cell. FHM has an autosomal dominant inheritance. In addition, defects in chromosome 19 have been identified in some families with typical migraine [4] [5].

De Fusco et al. have shown that the gene that codes for the alpha2 subunit of the Na+/K+ pump (ATP1A2), is associated with familial hemiplegic migraine type 2 (FHM2). It is linked to chromosome 1q23 [6]. This gene mutation results in a loss of function of a single allele of ATP1.

Prevention

There is disagreement regarding the treatment of hemiplegic migraines. Doctors use both triptans (serotonin receptor agonists), anticonvulsants, and calcium channel blockers to interrupt acute migraines and prevent future attacks.  Verapamil and Flunarizine are both used to treat hemiplegic migraine.

Summary

Hemiplegic migraine is a migraine variant that includes neurologic symptoms (one-sided weakness or paralysis) that may mimic a transient ischemic attack (TIA) or stroke symptoms that completely resolve within 24 hours. It is a rare condition which is frequently linked to an underlying genetic abnormality. It has been diagnosed more frequently in the U.K. Symptoms are typical for migraine with the addition that temporary paralysis, hemiplegia, is associated with the debilitating headache. Two categories of the disease have been described. Familial hemiplegic migraine (FHM) is associated with a strong family history and involves defects in at least three different genes. About 50% of children born to a parent with FHM will develop this disorder. Sporadic hemiplegic migraine (SHM) is diagnosed when all symptoms of hemiplegic migraine are present without a family link to the disease. It is believed that this variant of the disease is caused by some ‘sporadic’ gene mutations.

Patient Information

Hemiplegic migraine is an important variant of migraine disorder. This migraine presents like other migraines with well-known stages.

  • Prodrome: A collection of vague symptoms that occur up to 24 hours before the onset of the headache. These include fatigue, depression, irritability, and difficult sleeping.
  • Aura: Visual, sensory, and/or language disturbances. Usually, the migraine begins within 30 minutes of the onset of the aura.
  • Headache: A migraine is described as a one-sided throbbing pain. It may be associated with nausea, vomiting, or light sensitivity. Without treatment, the headache persists up to 72 hours . With hemiplegic migraine, the headache is accompanied by one-sided weakness or paralysis. Persons may have speech disturbances or visual symptoms (flickering lights, spots, or loss of vision).
  • Resolution: Once the headache and other symptoms subside, the person enters a resolution phase characterized by deep sleep. Occasionally there is a migraine hangover marked by “the blahs,” tiredness, and head pain brought on by coughing or head movement.

Because of the association of neurologic symptoms with the headaches, it is important to seek medical attention to exclude other causes of the one-sided weakness, such as a stroke. Testing may include imaging studies of the brain, heart, and neck arteries. The diagnosis of familial hemiplegic migraine (FHM) depends on the observation of two or more people in the same family with one-sided weakness associated with migraine headache. Approximately one-half of the cases of hemiplegic migraine will have family members that have been diagnosed with the disorder.

Preventive medications: Your doctor may suggest an anticonvulsant medication or a calcium channel blocker to prevent future attacks. Some herbal treatments have been proposed [7].

References

Article

  1. Tepper SJ. Understanding Migraine and Other Headaches. University Press of Mississippi; 2004: 23.
  2. Honkasalo ML, Kaprio J, Winter T, et al. Migraine and concomitant symptoms among 8167 adult twin pairs. Headache. 1995;35:70-78.
  3. Ophoff RA, Terwindt GM, Vergouwe GM, et al. Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations in the Ca2+ channel gene CACNL1A4. Cell.1996;87:543-552.
  4. May A, Ophoff, RA, Terwindt GM, et al. Familial hemiplegic migraine locus on chromosome 19p13 is involved in common forms of migraine with and without aura. Hum Genet. 1995; 96(5):604-608.
  5. Nyholt DR, Lea RA, Goadsby PJ, et al. Familial typical migraine: linkage to chromosome 19p13 and evidence for genetic heterogeneity. Neurology. 1998;50:1428-1432.
  6. De Fusco M, Marconi R, Silvestri L, et al. Haploinsufficiency of ATP1A2 encoding the Na+/K+ pump alpha2 subunit associated with familial hemiplegic migraine type 2. Nat Genet. 2003;33(2):192-196.
  7. Lipton RB, Göbel H, Einhäupl KM, et al. Petasites hybridus root (Butterbur) is an effective preventive treatment for migraine. Neurology. 2004; 63:2240-2244.

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Last updated: 2018-06-22 04:20