Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 4, 2010, pp. 512-513

Neurology India, Vol. 58, No. 4, July-August, 2010, pp. 512-513

Editorial

Sporadic hemiplegic migraine in children

Pienczk-Reclawowicz Karolina, Pilarska Ewa, Lemka Malgorzata

Department of Developmental Neurology, Medical University of Gdansk
Correspondence Address:Department of Developmental Neurology, Medical University of Gdansk, Debinki Street 7, 80-952 Gdansk, pienczk@gumed.edu.pl

Date of Acceptance: 21-Jul-2010

Code Number: ni10140

PMID: 20739784

DOI: 10.4103/0028-3886.68659

Sporadic hemiplegic migraine (SHM) is a rare disorder, especially in the pediatric population. The only differentiating feature between sporadic hemiplegic migraine and familial hemiplegic migraine (FHM) is family history (first- or second-degree) of similar headaches. There are no characteristic clinical features that distinguish between the two. The diagnostic criteria for SHM have been established in 2004 by the International Headache Society. ^[1] However, the diagnosis remains problematic and one of exclusion as has been highlighted by Chakravarty and Sen ^[2] in this issue of the journal. For recognizing SHM, one should take into consideration other mimics, which include a host of disorders: stroke; postictal Todd′s palsy; the syndrome of headache, transient neurologic deficit and lymphocytosis of cerebrospinal fluid (HaNDL); mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) ^[3] ; and autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). ^[4] In younger children, SHM attacks need to be differentiated from alternating hemiplegia of childhood (AHC). ^[5] The prevalence of SHM in patients with Sturge-Weber syndrome is higher than in general population; however, the cause of this phenomenon is uncertain. ^[6]

The diagnosis of FHM may be supported with genetic testing. Three major mutations of genes (CACNA1A, ATP1A2 and SCNA1A genes) responsible for FHM occurrence encode three different ion channels. These mutations account for 50% to 70% of published FHM cases. Recent analysis by de Vries et al.[7] of 40 patients with SHM revealed that in some of the SHM patients also, these mutations may occur (most often, ATP1A2 mutation), thus suggesting common pathogenic mechanism for both FHM and SHM.

Jen et al.[8] suggested that hemiplegic migraine should be considered in every child with prolonged hemiplegic symptoms, as well as in adults. SHM in children may be complicated with persistent aura without cerebral infarction, ^[9] and dramatic complications may lead (however, infrequently) to migrainous infarction. ^[10],[11] The treatment of acute SHM attacks, as well as prevention, in children remains an unresolved issue due to rarity of the disease and lack of randomized controlled trials. Thus, the management is based on the management principles of migraine with aura.

To date, there is still scarcity of data on distinction of the course of SHM in children and adults. Reporting cases of SHM, especially those with unusual course, is of high importance. ^[2] Enriching the diagnosis of childhood-onset SHM with genetic testing would help to define its phenotypic variety.

References

1.	Headache Classification Committee. The international classification of headache disorders, 2nd ed. Cephalalgia 2004;24:1-160.  Back to cited text no. 1
2.	Chakravarty A, Sen A. Sporadic hemiplegic migraine in children: Report of two new cases. Neurol India 2010;58:648-50.   Back to cited text no. 2  [PUBMED]
3.	Ohno K, Isotani E, Hirakawa K. MELAS presenting as migraine complicated by stroke. Neuroradiology 1997;39:781-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.	Sathe S, DePeralta E, Pastores G, Kolodny EH. Acute confusional migraine may be a presenting feature of CADASIL. Headache 2009;49:590-6.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.	Swoboda KJ, Kanavakis E, Xaidara A, Johnson JE, Leppert MF, Schlesinger-Massart MB. Alernating hemiplegia of childhood or familial hemiplegic migraine?: A novel ATP1A2 mutation. Ann Neurol 2004;55:884-7.  Back to cited text no. 5
6.	Klapper J. Headache in Sturge-Weber syndrome. Headache 1994;34:521-2.  Back to cited text no. 6  [PUBMED]
7.	de Vries B, Freilinger T, Vanmolkot KR, Koenderink JB, Stam AH, Terwindt GM, et al. Systemic analysis of three FHM genes in 39 sporadic patients with hemiplegic migraine. Neurology 2007;69:2170-6.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.	Jen JC, Klein A, Boltshauser E, Cartwright MS, Roach ES, Mamsa H, et al. Prolonged hemiplegic episodes in children due to mutations in ATP1A2. J Neurol Neurosurg Psychiatry 2007;78:523-6.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.	Lemka M, Pienczk-Reclawowicz K, Pilarska E, Szmuda M. Cessation of sporadic hemiplegic migraine attacks after patent foramen ovale closure. Dev Med Child Neurol 2009;51:923-4.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.	Rossi LN, Penzien JM, Deonna TH, Goutieres F, Vasella F. Does migraine-related stroke occur in childhood? Dev Med Child Neurol 1990;32:1005-21.  Back to cited text no. 10
11.	Ribeiro RT, Pinto MM, Villa TR, Gamba LT, Tengan CH, de Souza Carvalho D. Migrainous infarction as a complication of sporadic hemiplegic migraine in childhood. Arq Neuropsiquiatr 2009;67:906-8.  Back to cited text no. 11

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