Sporadic hemiplegic migraine

This condition is inherited in an autosomal dominant manner

Sporadic hemiplegic migraine (SHM) is a form of hemiplegic migraine headache isolated cases of which are observed.[1] It is a rare disease. It is considered to be a separate type of migraine.[2]

## Contents

* 1 Presentation
* 2 Cause
* 3 Diagnosis
* 3.1 Investigations
* 3.2 Differential diagnosis
* 4 Treatment
* 5 Epidemiology
* 6 References
* 7 External links

## Presentation

Sporadic hemiplegic migraine (SHM) has clinical symptoms identical to familial hemiplegic migraine (FHM) and distinct from migraine with aura. By definition the neurodeficits are supposed to be reversible. However, some cases with permanent neurological deficits have also been noted.[3]

## Cause

CACNA1A, ATP1A2 and SCNA1 are genes associated with hemipeligic migraines, although many cases are considered idiopathic with no known cause. [4]

## Diagnosis

Diagnostic criteria require motor symptoms and at least one visual, sensory, or speech symptom, resembling basilar migraine.[5] They may also be associated with cerebellar signs.[6]

### Investigations

MRI features can be suggestive of cortical infarction and edema.[7]

### Differential diagnosis

Differential diagnoses can be:[5]

* Todd's paresis
* Stroke
* Systemic lupus erythematosus
* Metabolic disorders
* Inherited disorders such as mitochondrial myopathy
* Functional neurological disorder

## Treatment

## Epidemiology

Prevalence is estimated to be 0.005%.[8] 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. Females outnumber males, 4 to 1. Only 3% have attacks after age 52.[2]

## References

1. ↑ "sporadic hemiplegic migraine" (in en). https://ghr.nlm.nih.gov/condition/sporadic-hemiplegic-migraine. Retrieved 19 June 2017.
2. ↑ 2.0 2.1 Thomsen, LL; Ostergaard, E; Olesen, J; Russell, MB (25 February 2003). "Evidence for a separate type of migraine with aura: sporadic hemiplegic migraine.". Neurology 60 (4): 595–601. doi:10.1212/01.WNL.0000046524.25369.7D. PMID 12601098.
3. ↑ Schwedt, Todd J.; Zhou, Jiying; Dodick, David W. (January 2014). "Sporadic Hemiplegic Migraine With Permanent Neurological Deficits". Headache: The Journal of Head and Face Pain 54 (1): 163–166. doi:10.1111/head.12232. PMID 24117121.
4. ↑ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Familial or sporadic hemiplegic migraine" (in en). https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=569. Retrieved 2 October 2019.
5. ↑ 5.0 5.1 Millichap, J Gordon (1 April 2003). "Sporadic Hemiplegic Migraine: A Separate Entity". Pediatric Neurology Briefs 17 (4): 32. doi:10.15844/pedneurbriefs-17-4-8.
6. ↑ Vahedi, K; Denier, C; Ducros, A; Bousson, V; Levy, C; Chabriat, H; Haguenau, M; Tournier-Lasserve, E et al. (10 October 2000). "CACNA1A gene de novo mutation causing hemiplegic migraine, coma, and cerebellar atrophy.". Neurology 55 (7): 1040–2. doi:10.1212/WNL.55.7.1040. PMID 11061267.
7. ↑ Bhatia, Harsha; Babtain, Fawzi (2011). "Sporadic Hemiplegic Migraine with Seizures and Transient MRI Abnormalities". Case Reports in Neurological Medicine 2011: 258372. doi:10.1155/2011/258372. PMID 22937333.
8. ↑ Thomsen, LL; Olesen, J (December 2004). "Sporadic Hemiplegic Migraine". Cephalalgia 24 (12): 1016–1023. doi:10.1111/j.1468-2982.2004.00788.x. PMID 15566415.

## External links

Classification|

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

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External resources|

* Orphanet: 569

* v
* t
* e

Diseases of the nervous system, primarily CNS (G04–G47, 323–349)

Inflammation|

| Brain|

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

|

Spinal cord|

* Myelitis: Poliomyelitis
* Demyelinating disease
* Transverse myelitis
* Tropical spastic paraparesis
* Epidural abscess

Both/either|

* Encephalomyelitis
* Acute disseminated
* Myalgic
* Meningoencephalitis

Brain/
encephalopathy|

| Degenerative|

| Extrapyramidal and
movement disorders|

* Basal ganglia disease
* Parkinsonism
* PD
* Postencephalitic
* NMS
* PKAN
* Tauopathy
* PSP
* Striatonigral degeneration
* Hemiballismus
* HD
* OA

* 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|

* Tauopathy
* Alzheimer's
* Early-onset
* Primary progressive aphasia
* Frontotemporal dementia/Frontotemporal lobar degeneration
* Pick's
* Dementia with Lewy bodies
* Posterior cortical atrophy

* Vascular dementia

Mitochondrial disease|

* Leigh syndrome

Demyelinating|

* autoimmune
* Multiple sclerosis
* Neuromyelitis optica
* Schilder's disease
* hereditary
* Adrenoleukodystrophy
* Alexander
* Canavan
* Krabbe
* ML
* PMD
* VWM
* MFC
* CAMFAK syndrome
* Central pontine myelinolysis
* Marchiafava–Bignami disease
* Alpers' disease

Episodic/
paroxysmal|

| Seizure/epilepsy|

* Focal
* Generalised
* Status epilepticus
* Myoclonic epilepsy

|

Headache|

* Migraine
* Familial hemiplegic
* Cluster
* Tension

Cerebrovascular|

* TIA
* Amaurosis fugax
* Transient global amnesia
* Acute aphasia

* Stroke
* MCA
* ACA
* PCA
* Foville's
* Millard–Gubler
* Lateral medullary
* Medial medullary
* Weber's
* Lacunar stroke

Sleep disorders|

* Insomnia
* Hypersomnia
* Sleep apnea
* Obstructive
* Congenital central hypoventilation syndrome
* Narcolepsy
* Cataplexy
* Kleine–Levin
* Circadian rhythm sleep disorder
* Advanced sleep phase disorder
* Delayed sleep phase disorder
* Non-24-hour sleep–wake disorder
* Jet lag

CSF|

* Intracranial hypertension
* Hydrocephalus/NPH
* Choroid plexus papilloma
* Idiopathic intracranial hypertension
* Cerebral edema
* Intracranial hypotension

Other|

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

Spinal cord/
myelopathy|

* Syringomyelia
* Syringobulbia
* Morvan's syndrome
* Vascular myelopathy
* Foix–Alajouanine syndrome
* Spinal cord compression

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
* SMA-PCH
* SMA-LED
* SMA-PME
* Progressive muscular atrophy
* Progressive bulbar palsy
* Fazio–Londe
* Infantile progressive bulbar palsy

* both:
* Amyotrophic lateral sclerosis

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