Sporadic hemiplegic migraine

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]

Sporadic hemiplegic migraine
This condition is inherited in an autosomal dominant manner

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 appear to be the genes that cause the condition of sporadic hemiplegic migraine.[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 hyper intensity and edema.[7]

Differential diagnosis

Differential diagnoses can be:[5]

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]

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References
  1. "sporadic hemiplegic migraine". Genetics Home Reference. Genetics Home Reference. Retrieved 19 June 2017.
  2. 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. PMC 4220590. PMID 24117121.
  4. RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Familial or sporadic hemiplegic migraine". www.orpha.net. Retrieved 2 October 2019.
  5. 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; Bousser, MG (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. PMC 3420796. 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.
Classification
External resources
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