Sporadic hemiplegic migraine

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Sporadic hemiplegic migraine
Autosomal dominant - en.svg
This condition is inherited in an autosomal dominant manner
Classification and external resources
ICD-10 G43.1
OMIM 141500
Orphanet 569

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 (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]



Diagnostic criteria require motor symptoms and at least one visual, sensory, or speech symptom, resembling basilar migraine.[4] They may also be associated with cerebellar signs.[5]


MRI features can be suggestive of cortical hyper intensity and edema.[6]

Differential diagnosis[edit]

Differential diagnoses can be:[4]



Prevalence is estimated to be 0.005%.[7] 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]


  1. ^ "sporadic hemiplegic migraine". Genetics Home Reference. Genetics Home Reference. Retrieved 19 June 2017. 
  2. ^ a b 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. PMID 12601098. doi:10.1212/01.WNL.0000046524.25369.7D. 
  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. PMC 4220590Freely accessible. doi:10.1111/head.12232. 
  4. ^ a b 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. 
  5. ^ 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. PMID 11061267. doi:10.1212/WNL.55.7.1040. 
  6. ^ Bhatia, Harsha; Babtain, Fawzi (2011). "Sporadic Hemiplegic Migraine with Seizures and Transient MRI Abnormalities". Case Reports in Neurological Medicine. 2011: 1–4. doi:10.1155/2011/258372. 
  7. ^ Thomsen, LL; Olesen, J (December 2004). "Sporadic Hemiplegic Migraine". Cephalalgia. 24 (12): 1016–1023. doi:10.1111/j.1468-2982.2004.00788.x.