Aelia, Kaneez

Mustafa, Shahid

Zafar, Azra

English

eCommons@AKU

Pakistan Journal ofNeurological Sciences (PJNS)Volume 1 | Issue 2 Article 49-2006Sporadic Hemiplegic Migraine: A ChallengingDiagnosisAzra ZafarLiaquat National HospitalShahid MustafaLiaquat National HospitalKaneez AeliaLiaquat National HospitalFollow this and additional works at: https://ecommons.aku.edu/pjnsPart of the Neurology CommonsRecommended CitationZafar, Azra; Mustafa, Shahid; and Aelia, Kaneez (2006) "Sporadic Hemiplegic Migraine: A Challenging Diagnosis," Pakistan Journal ofNeurological Sciences (PJNS): Vol. 1 : Iss. 2 , Article 4.Available at: https://ecommons.aku.edu/pjns/vol1/iss2/4Migraine affects about 12% of the population in Westerncountries.1 Familial hemiplegic migraine (FHM) is the onlyvariety of migraine characterized by an autosomaldominant pattern of inheritance and transient hemiparesisfollowed by migraine headache.2 Some sporadic cases ofhemiplegic migraine with cerebellar signs have also beenr e p o r t e d .3 Sporadic hemiplegic migraine (SHM) hasclinical symptoms identical to FHM and distinct frommigraine with aura.4CASE REPORTA 20-year-old right-handed gentleman presented with a 2-day history of left-sided weakness (evolving to maximumdeficit over half an hour), followed by severe unilateralheadache, vomiting and altered level of consciousness.Weakness was preceded by numbness of the left half ofthe body, and blurring of vision. There was no history offever, neckache, sphincter dysfunction, abnormalmovement, or head trauma. Over the last 8 years, thepatient had experienced several similar episodes of lesssevere intensity, none lasting more than 4 hours.Frequency was variable ranging from 2 per month to onceevery 3-4 months. A brain CT scan and cerebrospinal fluidexamination done in 1993 were normal. Subsequent brainCTs repeated CT at variable intervals were also normal. Pas t medical , surg ica l and family his tory  wereunremarkable. He was unmarried, a non-smoker and didnot consume alcohol. He had quit schooling after the 10thgrade because of his illness. Medication use includedmultiple analgesics, including intravenous injections forheadache. On examination at the time of admission, he was openingeyes on painful stimuli, localizing with the right hand, andhad no verbal response. Power was 0/5 in the left half ofthe body with extensor plantar response on the left. Hehad a left facial nerve palsy of upper motor neuron type.He was moving the right side spontaneously, and therewere no signs of meningeal irritation. Fundi were normal.Sensory, cerebellar, and gait examination were deferred. Routine blood tests including complete blood count, urea,V O L .  1 ( 2 )   J U L - S E P  2 0 0 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  75C A S E  R E P O R TSPORADIC HEMIPLEGIC MIGRAINE: A CHALLENGINGDIAGNOSISAzra Zafar, Shahid Mustafa, Kaneez Aelia Department of Neurology, Liaqat National Hospital, Karachi, PakistanCorrespondence to: Dr. Zafar, Dept of Neurology, Liaqat National Hospital, Stadium Road, Karachi, Pakistan. Tel. (92-21) 450-2360. Email: thisisazra@yahoo.comABSTRACTMigraine is a common, chronic, incapacitating neurovascular disorder characterized by attacks of severe headache,autonomic nervous system dysfunction and, in some patients, an aura involving neurological symptoms. Familial hemiplegicmigraine (FHM) is characterized by the presence of hemiparesis and occasionally even severe coma during attacks.Sporadic hemiplegic migraine has also been described in the literature and has a clinical spectrum similar to FHM.Figure 1: Left facial nerve palsycreatinine, electrolytes, and random sugar were withinnormal limits. Anti-epileptic drug levels were in the sub-therapeutic range. Magnetic resonance imaging of thebrain (including diffusion-weighted studies) was normal. EEG showed severe right hemispheric dysfunction. Thepatient's family did not consent to CSF analysis. There wasno signif icant cl inical improvement after 3 days ofhospitalization. An initial diagnosis of epilepsy (made inthe past at another facility) was questioned. On the basisof a detailed history, exclusion of metabolic abnormalities,and normal neuroimaging, a diagnosis of sporadichemiplegic migraine was tentatively made. He was started on NSAIDs, flunarizine and valproic acid.Strength on the affected side gradually improved to 3/5,he became fully conscious and oriented, and he wasdischarged on above medications. After 3 days, he wasagain hospitalized with severe headache, vomiting,imbalance, and drowsiness. He was given steroids forstatus migrainosus and intravenous verapamil (5 mg over5 minutes), followed by oral verapamil (120 mg twice aday). Within 48 hours his power returned to normal andthe headache subsided completely. Repeat EEG alsoshowed improvement. A final diagnosis of SHM wasreached. DISCUSSIONMigraine is characterized by episodes of headache thatare often throbbing, frequently unilateral, and may besevere. In migraine without aura (previously known ascommon migraine), attacks are usually associated withnausea, vomiting, and sensitivity to l ight, sound ormovement. A combination of features is required for theV O L .  1 ( 2 )   J U L - S E P  2 0 0 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  76Figure 2: Left-sided hemiparesisFigure 3: T2-weighted MRI of the brain (normal study)Figure 4: EEG showing right hemispheric dysfunctionFigure 5: Repeat EEG showing some improvementdiagnosis, though not all features are present in everyat tack or every patient. Any severe and recurrentheadache is most likely to be a form of migraine and to beresponsive to anti-migranous therapy. In 15% of patients,migraine attacks are usually preceded or accompanied bytransient focal neurological symptoms; such patients havemigraine with aura (previously known as clas sicmigraine).5Hemiplegic migraine was initially described in 1910 as atype of  migraine consis ting of recurrent headacheassociated with transient  hemipares is .6 F a m i l i a lhemiplegic migraine (FHM) is a rare autosomal dominantform of migraine with aura in which some degree ofhemiparesis is present during attacks. In FHM, the auratypically lasts longer than in migraine with aura andusually comprises visual, sensory, aphasic and motors y m p t o m s .7 However, patients with similar cl inicalsymptoms but without other affected family membershave been reported, and the term sporadic hemiplegicmigraine has been used.8 Based on the literature, twosubforms of FHM families exist - pure FHM in 80% andFHM fami lies with cerebel lar symptoms in 20%.9Mutations in the gene  CACNA1A, which encodes aneuronal calcium channel, are present in 50% familieswith hemiplegic migraine including those with cerebellarsigns.2 Eight mutations in CACNA1A have been identifiedin 18 families affected by hemiplegic migraine and twopatients with SHM.1 0 Genetic studies have revealed denovo mutations or incomplete penetrance of CACNA1A inpatients with SHM.2 , 3 thereby suggesting that SHM maybe part of the spectrum of FHM syndromes.FHM is a disorder with an interesting and complexsymptomatology. Motor aura symptoms are obligatory.Furthermore, 69% have concurrence of basilar migraine,with basilar-type symptoms during attacks. Duringepisodes, 95% patients always experience a headache,4% experience headache in some attacks, and 1 % neverexperience headache. Other associated symptoms includecerebellar ataxia associated with progressive cerebellaratrophy and epilepsy. FHM has a younger age of onsetcompared with migraine with aura.9 SHM has clinical symptoms identical to FHM. In SHM,presence of motor aura symptoms is essential. Othercommon aura symptoms are sensory (98%), visual (91%),and some form of aphasia (81%). Up to 72% of patientswith SHM have concurrence of basilar migraine. Headachealmost always occurs in close temporal relation to theaura, and SHM has an age aof onset younger than 45y e a r s .4 The most differential diagnoses of SHM typicallyincludes epilepsy (post-ictal weakness following seizure, orTodd's phenomenon), transient ischemic attack or stroke,metabolic abnormalities associated with focal deficits(hypercapnia, hypoglycemia, hyponatremia, hypocalcemia,hepatic fai lure and renal fai lure), meningitis orencephalitis, carotid dissection, anti-phospholipid antibodysyndrome, SLE, and orni thine transcarbamylasedeficiency. Inherited disorders associated with migraine headachethat may include hemiparesis include cerebral autosomaldominant arteriopathy with subcortical infarcts andleukoencephalopathy (CADASIL); mitochondrial myopathy,encephalopathy, lactic acidosis and stroke like episodes(MELAS); hereditary hemorrhagic telengiectesia (HHT);hereditary cerebral amyloid angiopathy; familial cerebralcavernous malformation; and benign familial infantileconvulsions.4Current therapeutic recommendations are based onisolated case repor ts and inc lude propranolo l,1 1p h e n y t o i n ,1 2 f l u n a r i z i n e ,1 3 and verapamil.8 S u c c e s s f u lresponses of FHM to intravenous verapamil have beenr e p o r t e d .1 4 Verapamil, administered either orally orintravenously, is also effective for SHM.15 Our patient hadno further episodes after starting oral verapamil 240 mgdaily, and was symptom-free at one-year follow up. SHMis a relatively rare disorder which should be considered inpat ients presenting wi th episodes of  reversibleneurological deficits with headaches; a therapeutic trial ofcalcium channel blocker (verapamil) may be administered.REFERENCES1. Stewart WF, Lipton RB, Celentano DD, Reed ML. Prevalence of migraine headache in the United States: relation to age, income, race, and other sociodemographic factors. JAMA 1992; 267: 64-9 2. Ducros A, Denier C, Joutel A, Cecillon M, Lescoat C,Vahedi K, et al. The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel. N Engl J Med 2001; 3456: 17-243. Vahedi K, Denier C, Durcos A, Bousson V, Levy C, Chabriat H, et al. CACNA1A gene denovo mutation causing hemiplegic migraine, coma and cerebellar atrophy. Neurology 2000; 55: 1040-2 4. Thomsen LL, Ostergaard E, Olesen J, Russell MB. Evidence for a separate type of miograine with aura:sporadic hemiplegic migraine. Neurology 2003; 60: 595-601 5. Goadsby PJ, Lipton RB Ferrari MD. Migraine - current understanding ant treatment. N Engl J Med2002; 346: 257-67 6. Clark JM. On recurrent motor paralysis in migraine, V O L .  1 ( 2 )   J U L - S E P  2 0 0 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  77with report of a family in which recurrent hemiplegia accompanied the attacks. BMJ 1910; 1534-15387. Benatar M, Ford CM. Familial hemiplegic migraine: more than just a headache. Neurology 2005; 64: 592-3 8. Razavi M, Razavi B, Fattal D, Afifi A, Adams HP. Hemiplegic migraine induced by exertion. Arch Neurol 2000; 57: 1363-5 9. Thomsen LL, Eriksen MK, Roemer SF, Andersen I, Olesen J, Russell MB. A population-based study of familial hemiplegic migraine suggests revised diagnostic criteria. Brain 2002; 125: 1379-91 10. Battistini S, Stenirri S, Piatti M, Gelfi C, Righetti G, Rocchi R, et al. A new CACNA1A gene mutation in acetazolamide responsive familial hemiplegic migraine and ataxia. Neurology 1999; 53: 38-43 11. Lai C, Ziegler DK, Lansky LL, Torres F. Hemiplegic migraine in childhood: diagnostic and therapeutic aspects. J Pediatr 1982; 101: 696-699 12. Ross RT. Hemiplegic migraine. Can Med Assoc J1958; 78: 10-1613. Tobita M, Hino M, Ichikawa N, Takase S, Ogawa A. A case of hemiplegic migraine treated with flunarizine. Headache 1987; 27: 487-48814. Yu W, Horowitz SH. Familial hemiplegic migraine and its abortive therapy with intravenous verapamil. Neurology 2001; 57: 1732-173315. Yu W, Horowitz SH. Treatment of sporadic hemiplegic migraine with calcium channel blocker verapamil. Neurology 2003; 60: 120-121V O L .  1 ( 2 )   J U L - S E P  2 0 0 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  78

Sporadic Hemiplegic Migraine: A Challenging Diagnosis

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Sporadic Hemiplegic Migraine: A Challenging Diagnosis

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