Sporadic hemiplegic migraine

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.

Cause

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

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
• Stroke
• Systemic lupus erythematosus
• Metabolic disorders
• Inherited disorders such as mitochondrial myopathy
• Functional neurological disorder

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

References

1. "sporadic hemiplegic migraine".
2. (25 February 2003). "Evidence for a separate type of migraine with aura: sporadic hemiplegic migraine.". Neurology.
3. (January 2014). "Sporadic Hemiplegic Migraine With Permanent Neurological Deficits". Headache: The Journal of Head and Face Pain.
4. "Orphanet: Familial or sporadic hemiplegic migraine".
5. (1 April 2003). "Sporadic Hemiplegic Migraine: A Separate Entity". Pediatric Neurology Briefs.
6. (10 October 2000). "CACNA1A gene de novo mutation causing hemiplegic migraine, coma, and cerebellar atrophy.". Neurology.
7. (2011). "Sporadic Hemiplegic Migraine with Seizures and Transient MRI Abnormalities". Case Reports in Neurological Medicine.
8. (December 2004). "Sporadic Hemiplegic Migraine". Cephalalgia.