Childhood Migraine Variants Clinical Presentation
- Author: Wendy G Mitchell, MD; Chief Editor: Amy Kao, MD more...
Variant migraine episodes may be independent of actual head pain. Other symptoms may predominate and be significantly more troublesome.
Hemiplegic migraine presents with hemiplegia or hemiparesis, with or without a speech or language disturbance, which clears in minutes to hours. Headache may be less dramatic than the hemiplegia. Other migrainous symptoms (eg, nausea, vomiting, and photophobia) are present to varying degrees. Hemiplegia may precede or accompany the headache.
Hemiplegic migraine is often familial, is dominantly inherited, and is linked to chromosome 19 in some studies.[11, 12] An alternative locus has been described on chromosome 1.[13, 14] A defect in the gene for the calcium channel is documented in some families (affecting the voltage-dependent P/Q-type calcium channel alpha-1A [CACNA1A] subunit).[15, 16] An increased risk of stroke exists in families with this disorder.
Cerebral autosomal dominant arteriopathy and subcortical infarcts and leukoencephalopathy (CADASIL), an autosomal dominant disorder in which multiple subcortical strokes may lead to dementia in early adulthood, sometimes presents in adolescence as atypical hemiplegic migraine. The CADASIL mutation also localizes to chromosome 19.
Additional genetic associations have been found including with mutations in SCN1a, ATP1A2, and PRRT2 genes. All of these mutations include variable phenotypes with epilepsy, paroxysmal dyskinesias, episodic ataxia, and hemiplegic migraine seen in affected families. Families with the same mutation may manifest differing combinations of symptoms, and the same symptoms may be due to different mutations.[7, 9, 10]
The diagnosis cannot be made during the first episode, though it may be suspected in the presence of a positive family history. Exclude more serious causes of headache with hemiplegia or hemiparesis, including intracranial hemorrhage, mass, infection, and stroke. With repeated stereotyped episodes and complete clearing between episodes, the diagnosis can be made with more confidence, particularly in the presence of a positive family history.
The differential diagnosis of repeated episodes includes alternating hemiplegia of childhood, unobserved partial seizures with postictal paralysis, and mitochondrial cytopathies, particularly the mitochondrial encephalomyopathy, lactic acidosis, and stroke syndrome (MELAS). On occasion, hemiplegic migraine may be associated with reversible unilateral cerebral edema on imaging.
Although confusional migraine is more common in younger children, it sometimes presents in postpubertal adolescents or adults. Occasionally, a child whose episodes began in the prepubertal years continues to have episodes into adolescence. The child has a period of confusion and disorientation, with or without agitation, followed by vomiting, which is relieved by sleep. Headache may not be prominent or may be elicited only retrospectively.
So called "Alice-in-Wonderland syndrome" may be a special case of confusional migraine. The patient subjectively experiences the sensation of their own body being either larger or smaller than normal, and objects and people around them appearing larger or smaller, combining both somesthetic and visual perceptual disturbances. The same clinical syndrome can also be associated with a variety of viral illnesses, including influenza and EBV infection, but if it occurs repeatedly may be a migraine aura.
Making the diagnosis during the first episode is difficult; it is possible only after the episode has resolved fully. Acute differential diagnosis of a single episode includes all types of encephalopathy or encephalitis, toxic ingestion, intoxication, and an unobserved seizure with postictal agitation.
A child with abdominal migraine may complain of episodic pain, nausea, and vomiting. The headache may be minimal or absent. An aura may precede the pain but is not frequent. Symptoms are relieved by sleep and antiemetic or antimigraine therapies. The diagnosis is difficult to make during the first episode.
Cyclic vomiting of childhood, which can be associated with a mitochondrial cytopathy, may be a severe variant of abdominal migraine.
A patient with basilar migraine may have an aura followed by dizziness, vertigo, syncope, and dysarthria. Headache may be minimal or absent. This variant is observed most frequently in adolescent girls. The differential diagnosis includes cardiogenic or vasovagal syncope, inner ear disease, somatoform disorder, and posterior-fossa tumors.
Migraine aura without headache
Migraineurs of any age may experience an aura with or without the typical headache. In some, the headache may be minimal while neurologic symptoms predominate. Visual symptoms without subsequent headache are fairly frequent. These include scintillating scotomata, formed visual hallucinations (usually stereotyped, in a single visual field), micropsia, and tunnel vision.
The differential diagnosis includes occipital epilepsy, with or without an identifiable lesion. If episodes never are accompanied by headache, the diagnosis is speculative.
Auditory hallucinations as migraine auras are infrequent but can occur. Sensory dysesthesias (usually hemisensory numbness or tingling) similar to a more typical migraine aura may occur without subsequent head pain.
Benign paroxysmal vertigo of childhood
Given the high proportion of children with a family history of migraine, benign paroxysmal vertigo of childhood is sometimes considered a migraine variant. This syndrome is not uncommon and frequently is followed by the development of more typical migraine headaches later in childhood.
The syndrome presents with brief episodes of vertigo. Toddlers may be unable to verbalize the symptoms, but they typically cling to the parent and look frightened. Older children often verbalize that they are “moving.” Headache does not follow the attack.
Ophthalmoplegic migraine may begin in childhood. Acute disorders of eye movement, unilateral abnormal pupillary response, or Horner syndrome may precede or accompany the headache.
As with all vascular headache syndromes, the neurologic examination between episodes typically yields completely normal findings. If persistent neurologic signs (eg, hemiparesis, visual changes, sensory loss) are evident between episodes, strongly consider and investigate alternative diagnoses.
Migraineurs have a higher risk of stroke, and patients with hemiplegic migraine may be at even higher risk. Abdominal migraine (cyclic vomiting syndrome) may cause significant dehydration.
Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013 Jul. 33 (9):629-808. [Medline].
Gelfand AA, Thomas KC, Goadsby PJ. Before the headache: infant colic as an early life expression of migraine. Neurology. 2012 Sep 25. 79(13):1392-6. [Medline].
Romanello S, Spiri D, Marcuzzi E, Zanin A, Boizeau P, Riviere S. Association between childhood migraine and history of infantile colic. JAMA. 2013 Apr 17. 309(15):1607-12. [Medline].
Hansen JM, Hauge AW, Ashina M, Olesen J. Trigger factors for familial hemiplegic migraine. Cephalalgia. 2011 Sep. 31(12):1274-81. [Medline].
Haan J, Terwindt GM, Maassen JA, et al. Search for mitochondrial DNA mutations in migraine subgroups. Cephalalgia. 1999 Jan. 19(1):20-2. [Medline].
Zaki EA, Freilinger T, Klopstock T, Baldwin EE, Heisner KR, Adams K, et al. Two common mitochondrial DNA polymorphisms are highly associated with migraine headache and cyclic vomiting syndrome. Cephalalgia. 2009 Jul. 29(7):719-28. [Medline].
Frosk P, Mhanni AA, Rafay MF. SCN1A mutation associated with intractable myoclonic epilepsy and migraine headache. J Child Neurol. 2013 Mar. 28(3):389-91. [Medline].
Marini C, Conti V, Mei D, Battaglia D, Lettori D, Losito E, et al. PRRT2 mutations in familial infantile seizures, paroxysmal dyskinesia, and hemiplegic migraine. Neurology. 2012 Nov 20. 79(21):2109-14. [Medline]. [Full Text].
Silveira-Moriyama L, Gardiner AR, Meyer E, King MD, Smith M, Rakshi K. Clinical features of childhood-onset paroxysmal kinesigenic dyskinesia with PRRT2 gene mutations. Dev Med Child Neurol. 2013 Apr. 55(4):327-34. [Medline].
De Cunto A, Bensa M, Tonelli A. A case of familial hemiplegic migraine associated with a novel ATP1A2 gene mutation. Pediatr Neurol. 2012 Aug. 47(2):133-6. [Medline].
Jurkat-Rott K, Freilinger T, Dreier JP, et al. Variability of familial hemiplegic migraine with novel A1A2 Na+/K+-ATPase variants. Neurology. 2004 May 25. 62(10):1857-61. [Medline].
Swoboda KJ, Kanavakis E, Xaidara A, et al. Alternating hemiplegia of childhood or familial hemiplegic migraine? A novel ATP1A2 mutation. Ann Neurol. 2004 Jun. 55(6):884-7. [Medline].
Ducros A, Joutel A, Vahedi K, et al. Mapping of a second locus for familial hemiplegic migraine to 1q21-q23 and evidence of further heterogeneity. Ann Neurol. 1997 Dec. 42(6):885-90. [Medline].
Gardner K, Barmada MM, Ptacek LJ, Hoffman EP. A new locus for hemiplegic migraine maps to chromosome 1q31. Neurology. 1997 Nov. 49(5):1231-8. [Medline].
Beauvais K, Cave-Riant F, De Barace C, et al. New CACNA1A gene mutation in a case of familial hemiplegic migraine with status epilepticus. Eur Neurol. 2004. 52(1):58-61. [Medline].
Terwindt G, Kors E, Haan J, et al. Mutation analysis of the CACNA1A calcium channel subunit gene in 27 patients with sporadic hemiplegic migraine. Arch Neurol. 2002 Jun. 59(6):1016-8. [Medline].
Asghar SJ, Milesi-Hallé A, Kaushik C, Glasier C, Sharp GB. Variable manifestations of familial hemiplegic migraine associated with reversible cerebral edema in children. Pediatr Neurol. 2012 Sep. 47(3):201-4. [Medline].
Lanska JR, Lanska DJ. Alice in Wonderland Syndrome: somesthetic vs visual perceptual disturbance. Neurology. 2013 Mar 26. 80(13):1262-4. [Medline].
Golomb MR, Sokol DK, Walsh LE, et al. Recurrent hemiplegia, normal MRI, and NOTCH3 mutation in a 14-year-old: is this early CADASIL?. Neurology. 2004 Jun 22. 62(12):2331-2. [Medline].
Lewis DW, Ashwal S, Dahl G, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2002 Aug 27. 59(4):490-8. [Medline].
Lewis D, Ashwal S, Hershey A, Hirtz D, Yonker M, Silberstein S. Practice parameter: pharmacological treatment of migraine headache in children and adolescents: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology. 2004 Dec 28. 63(12):2215-24. [Medline].
Evans RW, Taylor FR. "Natural" or alternative medications for migraine prevention. Headache. 2006 Jun. 46(6):1012-8. [Medline].
Ahonen K, Hamalainen ML, Rantala H, Hoppu K. Nasal sumatriptan is effective in treatment of migraine attacks in children: A randomized trial. Neurology. 2004 Mar 23. 62(6):883-7. [Medline].
Evers S, Rahmann A, Kraemer C, et al. Treatment of childhood migraine attacks with oral zolmitriptan and ibuprofen. Neurology. 2006 Aug 8. 67(3):497-9. [Medline].
Cloarec R, Bruneau N, Rudolf G, Massacrier A, Salmi M, Bataillard M. PRRT2 links infantile convulsions and paroxysmal dyskinesia with migraine. Neurology. 2012 Nov 20. 79(21):2097-103. [Medline].
Gardiner AR, Bhatia KP, Stamelou M, Dale RC, Kurian MA, Schneider SA, et al. PRRT2 gene mutations: from paroxysmal dyskinesia to episodic ataxia and hemiplegic migraine. Neurology. 2012 Nov 20. 79(21):2115-21. [Medline]. [Full Text].
Langhagen T, Schroeder AS, Rettinger N, Borggraefe I, Jahn K. Migraine-related vertigo and somatoform vertigo frequently occur in children and are often associated. Neuropediatrics. 2013 Feb. 44(1):55-8. [Medline].