Sporadic hemiplegic migraine: report of a case with clinical and radiological features
© Springer-Verlag 2008
Received: 12 May 2008
Accepted: 18 August 2008
Published: 23 September 2008
A case of visual hallucination, headache and left hemiparesis is reported. The patient had a history of recurrent attacks of similar semiology for the previous 15 years. MRI brain revealed a cortical hyperintensity on T2W, FLAIR and diffusion weighted imaging (DWI) in the right cerebral hemisphere with a normal ADC (apparent diffusion coefficient) map and MR angiogram. Detailed workup for MELAS was negative. A diagnosis of sporadic hemiplegic migraine was made and he was managed conservatively. He made a gradual complete recovery over 2 weeks. He was discharged on flunarizine for prophylaxis and has remained asymptomatic over the ensuing 4 months. This interesting condition is reviewed and discussed herein.
Sporadic hemiplegic migraine is a rare subtype of migraine headache with aura defined as migraine attacks associated with some degree of motor weakness/hemiparesis during the aura phase and where no first- or second-degree relative (parent, sibling or child) has identical attacks . The earliest description of this syndrome has been by Whitty et al.  in 1953. Bradshaw et al. in 1965 reported a series of 37 cases of hemiplegic migraine. However, not all patients in the series had recurrent episodes of hemiplegia . Thomsen et al.  reported a population search based prevalence study in Denmark, which found 105 patients with sporadic hemiplegic migraine at a prevalence rate of 0.01%. Based on their findings, sporadic hemiplegic migraine was assigned a separate subtype in the revised ICHD 2004,  with nearly 200 cases described so far, still there is sparse data regarding the radiologic findings, pathophysiology and treatment of acute attack as well as prevention of SHM. We describe here the clinical and radiologic findings in a patient with sporadic hemiplegic migraine.
He was managed conservatively and made a gradual and complete recovery over the next 10 days. Flunarizine in a dose of 10 mg per day was started for migraine prophylaxis and he did not have any further episodes of headaches or focal deficit over a 4-month follow-up. MRI study repeated after 4 weeks of symptom onset showed resolution of changes (Fig. 1f–h).
Possible differential diagnosis of recurrent hemiplegia
Transient ischemic attacks and recurrent strokes
Complex partial seizure with and without secondary generalization (with post-ictal Todd’s palsy)
One of the migraine with aura subtypes:
(a) Hemiplegic migraine.(sporadic or familial)
(b) Basilar-type migraine
(c) Migrainous infarction (complication of migraine)
Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome
Pseudomigraine with lymphocytic pleocytosis with transient neurologic deficits
Alternating hemiplegia of childhood
Most patients with sporadic hemiplegic migraine have ‘typical’ aura symptoms (visual, sensory and/or aphasic) associated with motor weakness during the acute attack [3, 4]. About three-fourth of patients with sporadic hemiplegic migraine also fulfill the criteria for basilar-type migraine during the acute attack, but all such patients should be diagnosed as hemiplegic migraine rather than basilar-type migraine as per ICHD criteria [1, 3]. The auras are more prolonged in sporadic hemiplegic migraine as compared to a typical migraine with aura . The acute hemiplegic attack is always associated with severe headache while typical migraine with aura may occur without headache also [1, 3]. Absence of infarction on imaging helps in differentiating it from migrainous infarction or stroke associated with migraine [1, 3].
The data on imaging abnormalities described in sporadic hemiplegic migraine as well as familial hemiplegic migraine is sparse. The findings described consist of either restricted diffusion, normal or increased diffusion based on DWI and ADC values involving a hemisphere opposite to the side of deficit [5–7] associated with normal T2W, T1W images, angiography and perfusion studies have shown hyperperfusion . Magnetic resonance spectroscopy has suggested reduced myoinositol/creatinine ratio, reduced N-acetylaspartate (NAA) on the affected side with normal choline/creatinine ratio. All these abnormalities have been found to be resolving gradually over 4–12 weeks . These findings have been thought to represent a fully resolving diffusion metabolic abnormality without any vaso-occlusion, which could be due to the pathology at the cellular level. The restricted diffusion is correlated with cell swelling as a result of ATPase pump dysfunction [5, 7]. Similarity of imaging findings in sporadic and familial hemiplegic migraine is suggestive of a possible common pathophysiology involving both the conditions which at present is thought to be due to a spreading depolarizing wave of neuro-electric or metabolic stimulus leading to prolonged neuronal depolarization. Interestingly, however, in the present case, the abnormality was in the form of hyperintense signal apparent on T2W and FLAIR imaging also, with hyperintense signal on DWI but no abnormalities on ADC map. These changes could possibly suggest vasogenic edema or a metabolic change as evidenced by complete reversibility on follow-up, both clinically and radiologically.
Treatment strategy at present is only on the basis of case reports of effective treatment with Flunarizine, Naloxane and Verapamil [8–10]. At present, SHM is treated on the same lines as familial hemiplegic migraine. Sympathomimetic drugs like ergotamine derivatives and triptans are avoided in acute attacks of hemiplegic migraine due to the fear of vasospasm leading to permanent sequelae. Similarly, beta-blockers are not used for prophylaxis in patients with hemiplegic or basilar-type migraine for the risk of prolonged aura or migrainous infarction. However, the most important part in the management of hemiplegic migraine, and especially in SHM, is that it is considered a diagnosis of exclusion, only after a thorough workup for other conditions has been carried out. At the same time, it is also important to keep SHM as a possibility during evaluation of patients with recurrent TIAs or epilepsy, especially if there is a history of hemiplegic episodes, which recover without any residual sequelae.
Conflict of interest
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