Hemiplegic migraine

Medical condition

Hemiplegic migraine

Other names| Familial hemiplegic migraine or sporadic hemiplegic migraine[1]
Hemiplegic migraine is inherited via autosomal dominant manner

Hemiplegic migraine is a type of migraine headache characterized by motor weakness affecting only one side of the body, accompanied by aura. There is often an impairment in vision, speech, or sensation. It can run in the family, called familial hemiplegic migraine, or in a single individual, called sporadic hemiplegic migraine. The symptoms can be similar to a stroke, and may be precipitated by minor head trauma. People with FHM are advised to avoid activities that may trigger their attacks.

## Symptoms and signs

[edit]

Hemiplegia (Greek 'hemi' = Half), is a condition that affects one side of the body. Signs of a hemiplegic migraine attack are similar to what would be presented in a stroke that typically includes sudden severe headache on one side of the brain, weakness of half the body, ataxia and aphasia which can last for hours, days or weeks. [2]

## Cause

[edit]

Symptoms of weakness during a Hemiplegic migraine attack are a result of motor aura. The aura displayed during a migraine episode is believed to be caused by a phenomenon known as cortical spreading depression.

For those not at risk due to a family history of Hemiplegic migraines, the current medical discussion indicates that genetic factors may still play a role. Some scientific studies have indicated that a percentage of those who suffer from Hemiplegic migraines may have mutations of the CACNA1A and the ATP1A2 genes.[3]

## Diagnosis

[edit]

### Classification

[edit]

The ICHD classification and diagnosis of migraine distinguish 6 subtypes of hemiplegic migraine.[4] Familial hemiplegic migraine (FHM) can be loosely divided into two categories: with and without cerebellar signs. Cerebellar signs refer to ataxia, sometimes episodic and other times progressive, that can accompany FHM1 mutations and is caused by degeneration of the cerebellum. These cerebellar signs result in a phenotypic overlap between FHM and both episodic ataxia and spinocerebellar ataxia. This is unsurprising as subtypes of these disorders (FHM1, EA2 and SCA6) are allelic, i.e., they result from mutations in the same gene. The other forms of FHM seem to be distinguishable only on the basis of their genetic cause.[citation needed]

### Familial hemiplegic migraine

[edit]

Main article: Familial hemiplegic migraine

Familial hemiplegic migraine is a form of hemiplegic migraine headache that runs in families.[5] Hemiplegic migraine is inherited via autosomal dominant manner.

### Sporadic hemiplegic migraine

[edit]

Main article: Sporadic hemiplegic migraine

There are also non-familial cases of hemiplegic migraine, termed sporadic hemiplegic migraine. These cases seem to have the same causes as the familial cases and represent de novo mutations. Sporadic cases are also clinically identical to familial cases with the exception of a lack of family history of attacks.[6]

## Screening

[edit]

Prenatal screening is not typically done for FHM, however it may be performed if requested. As penetrance is high, individuals found to carry mutations should be expected to develop signs of FHM at some point in life.[citation needed]

## Management

[edit]

Further information: Migraine treatment

People with FHM are encouraged to avoid activities that may trigger their attacks. Minor head trauma is a common attack precipitant, so FHM sufferers should avoid contact sports. Acetazolamide or standard drugs are often used to treat attacks, though those leading to vasoconstriction should be avoided due to the risk of stroke.[citation needed]

## Epidemiology

[edit]

Migraine itself is a very common disorder, occurring in 15–20% of the population. Hemiplegic migraine, be it familial or spontaneous, is less prevalent, 0.01% prevalence according to one report.[7] Women are three times more likely to be affected than males.[citation needed]

## References

[edit]

1. ^ "Orphanet: Familial or sporadic hemiplegic migraine". Orphanet. 2024-05-28. Retrieved 2024-06-11.
2. ^ "What is hemiplegia? | HemiHelp: for children and young people with hemiplegia (hemiparesis)". www.hemihelp.org.uk. Archived from the original on 2013-03-05. Retrieved 2019-07-09.
3. ^ Kumar, Anil; Samanta, Debopam; Emmady, Prabhu D.; Arora, Rohan (2023), "Hemiplegic Migraine", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30020674, retrieved 2023-12-26
4. ^ Website The International Classification of Headache Disorders 3rd edition (Beta version). Retrieved 29. August 2016.
5. ^ "familial hemiplegic migraine". Genetics Home Reference. Retrieved 19 June 2017.
6. ^ "sporadic hemiplegic migraine". Genetics Home Reference. Retrieved 19 June 2017.
7. ^ Lykke Thomsen, L; Kirchmann Eriksen, M; Faerch Romer, S; Andersen, I; Ostergaard, E; Keiding, N; Olesen, J; Russell, MB (June 2002). "An epidemiological survey of hemiplegic migraine". Cephalalgia. 22 (5): 361–375\. doi:10.1046/j.1468-2982.2002.00371.x. PMID 12110112. S2CID 22040734.

## External links

[edit]

Classification|

* ICD-10: ICD-10: G43.1
* OMIM: 141500

|

External resources|

* Orphanet: 569

* v
* t
* e

Diseases of the nervous system, primarily CNS

Inflammation|

| Brain|

* Encephalitis
* Viral encephalitis
* Herpesviral encephalitis
* Limbic encephalitis
* Encephalitis lethargica
* Cavernous sinus thrombosis
* Brain abscess
* Amoebic

|

Brain and spinal cord|

* Encephalomyelitis
* Acute disseminated
* Meningitis
* Meningoencephalitis

Brain/
encephalopathy|

| Degenerative|

| Extrapyramidal and
movement disorders|

* Basal ganglia disease
* Parkinsonism
* PD
* Postencephalitic
* NMS
* PSP
* CBD
* NBIA
* PKAN
* Striatonigral degeneration
* Hemiballismus
* Huntington's disease
* Olivopontocerebellar atrophy

* Dyskinesia
* Dystonia
* Status dystonicus
* Spasmodic torticollis
* Meige's
* Blepharospasm
* Athetosis
* Chorea
* Choreoathetosis
* Myoclonus
* Myoclonic epilepsy
* Akathisia

* Tremor
* Essential tremor
* Intention tremor
* Restless legs
* Stiff-person

|

Dementia|

* Alzheimer's
* Early-onset
* Primary progressive aphasia
* Frontotemporal dementia/Frontotemporal lobar degeneration
* Lewy body dementia
* Posterior cortical atrophy

* Creutzfeldt–Jakob disease

* Vascular dementia

Mitochondrial disease|

* Leigh syndrome

Demyelinating|

* Autoimmune
* Inflammatory
* Multiple sclerosis
* For more detailed coverage, see Template:Demyelinating diseases of CNS

Episodic/
paroxysmal|

| Seizures and epilepsy|

* Focal
* Generalised
* Status epilepticus
* For more detailed coverage, see Template:Epilepsy

|

Headache|

* Migraine
* Cluster
* Tension
* For more detailed coverage, see Template:Headache

Cerebrovascular|

* TIA
* Stroke
* For more detailed coverage, see Template:Cerebrovascular diseases

Other|

* Sleep disorders
* For more detailed coverage, see Template:Sleep

CSF|

* Intracranial hypertension
* Hydrocephalus
* Normal pressure hydrocephalus
* Choroid plexus papilloma
* Idiopathic intracranial hypertension
* Cerebral edema
* Intracranial hypotension

Other|

* Brain herniation
* Reye syndrome
* Hepatic encephalopathy
* Toxic encephalopathy
* Hashimoto's encephalopathy
* Static encephalopathy

Both/either|

| Degenerative|

| SA|

* Friedreich's ataxia
* Ataxia–telangiectasia

|

MND|

* UMN only:
* Primary lateral sclerosis
* Pseudobulbar palsy
* Hereditary spastic paraplegia

* LMN only:
* Distal hereditary motor neuronopathies
* Spinal muscular atrophies
* SMA
* SMAX1
* SMAX2
* DSMA1
* Congenital DSMA
* Spinal muscular atrophy with lower extremity predominance (SMALED)
* SMALED1
* SMALED2A
* SMALED2B
* SMA-PCH
* SMA-PME
* Progressive muscular atrophy
* Progressive bulbar palsy
* Fazio–Londe
* Infantile progressive bulbar palsy

* both:
* Amyotrophic lateral sclerosis

* v
* t
* e

Headache

Primary|

| ICHD 1|

* Migraine
* Familial hemiplegic
* Retinal migraine

|

ICHD 2|

* Tension
* Mixed tension migraine

ICHD 3|

* Cluster
* Chronic paroxysmal hemicrania
* SUNCT

ICHD 4|

* Hemicrania continua
* Thunderclap headache
* Sexual headache
* New daily persistent headache
* Hypnic headache

Secondary|

| ICHD 5|

* Migralepsy

|

ICHD 7|

* Ictal headache
* Post-dural-puncture headache

ICHD 8|

* Hangover
* Medication overuse headache

ICHD 13|

* Trigeminal neuralgia
* Occipital neuralgia
* External compression headache
* Cold-stimulus headache
* Optic neuritis
* Postherpetic neuralgia
* Tolosa–Hunt syndrome

Other|

* Eye strain
* Orthostatic
* Vascular

*[v]: View this template
*[t]: Discuss this template
*[e]: Edit this template