Sporadic hemiplegic migraine (SHM) is a form of

hemiplegic migraine Symptoms and signs Hemiplegia (Greek 'hemi' = Half), is 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 sid ...

headache isolated cases of which are observed. It is a

rare disease A rare disease is any disease that affects a small percentage of the population. In some parts of the world, an orphan disease is a rare disease whose rarity means there is a lack of a market large enough to gain support and resources for discove ...

. It is considered to be a separate type of migraine.

Presentation

Sporadic hemiplegic migraine (SHM) has clinical symptoms identical to

familial hemiplegic migraine Familial hemiplegic migraine (FHM) is an autosomal dominant type of hemiplegic migraine that typically includes weakness of half the body which can last for hours, days, or weeks. It can be accompanied by other symptoms, such as ataxia, coma, ...

(FHM) and distinct from

migraine with aura An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is a seizure. Epileptic and migraine auras are due to the involvement of specific areas of the brain, which are those that determine the symptom ...

. By definition the neurodeficits are supposed to be reversible. However, some cases with permanent neurological deficits have also been noted.

Cause

CACNA1A, ATP1A2 and SCNA1 appear to be the genes that cause the condition of ''sporadic hemiplegic migraine''.

Diagnosis

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

Investigations

MRI features can be suggestive of cortical infarction and edema.

Differential diagnosis

Differential diagnoses can be: *

Todd's paresis Todd's paresis (or postictal paresis/paralysis, "after seizure") is focal weakness in a part or all of the body after a seizure. This weakness typically affects appendages and is localized to either the left or right side of the body. It usually s ...

Stroke A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...

Systemic lupus erythematosus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Comm ...

Metabolic disorder A metabolic disorder is a disorder that negatively alters the body's processing and distribution of macronutrients, such as proteins, fats, and carbohydrates. Metabolic disorders can happen when abnormal chemical reactions in the body alter the ...

s * Inherited disorders such as

mitochondrial myopathy Mitochondrial myopathies are types of myopathies associated with mitochondrial disease. On biopsy, the muscle tissue of patients with these diseases usually demonstrate "ragged red" muscle fibers. These ragged-red fibers contain mild accumulati ...

Functional neurological disorder A functional neurologic disorder or functional neurological disorder (FND) is a condition in which patients experience neurological symptoms such as weakness, movement disorders, sensory symptoms and blackouts. Symptoms of functional neurological ...

Treatment

Epidemiology

Prevalence is estimated to be 0.005%. 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.

References

External links

{{Headache Channelopathies Headaches Rare diseases