Abstract
Hemiplegic migraine is a rare form of migraine characterized by aura with unilateral paralysis; however, studies on its treatment are limited. A 39-year-old man with migraine headaches and myofascial pain syndrome was referred to our hospital with transient right hemiplegia, after presenting to an outside emergency department with headache and right hemiplegia 2 days prior. Computed tomography, magnetic resonance imaging, and blood test results revealed no abnormalities. The symptoms resolved spontaneously; he was referred to our hospital. Based on the International Classification of Headache Disorders, Third Edition criteria, he was diagnosed with sporadic hemiplegic migraine. Propranolol was added to his regular regimen as prophylactic treatment, which resulted in reduction in his migraine frequency. Over the next 2 years, no recurrent paralysis occurred. Hemiplegic migraines should be considered in patients with migraine exhibiting transient hemiplegia without obvious intracranial abnormalities. Prophylactic treatment with propranolol could be effective in treatment of hemiplegic migraine.
Introduction
Hemiplegic migraine (HM), a rare form of migraine characterized by aura with unilateral paralysis, can occur within families (familial hemiplegic migraine (FHM)) or sporadically (sporadic hemiplegic migraine (SHM)). 1 The International Classification of Headache Disorders, Third Edition (ICHD-3) criteria state that such patients experience migraine characterized by aura, fully reversible motor weakness, and fully reversible visual, sensory, and/or speech symptoms. Although paralysis occurs rarely bilateral, the affected side may alternate. 2 Given there are no specific diagnostic clinical or radiological examinations for HM, a diagnosis is primarily made based on patient medical history and the ICHD-3 criteria. Paralysis typically lasts for <72 h. However, it may last for days or weeks. 3 HM-related symptoms resemble those of stroke; HM has been misdiagnosed as transient ischemic attack (TIA), resulting in inappropriate thrombolytic therapy administration, particularly with presence of ischemic stroke risk factors.4,5
To date, no randomized controlled trials have evaluated therapeutic medications (acute or prophylactic) for HM. Case reports and small studies have indicated the efficacy of verapamil and propranolol as prophylactic treatments of HM. 5 However, studies on the prophylactic efficacy of propranolol are limited.6,7 This report describes a patient with SHM presenting with right hemiplegia, who showed improvements with additional administration of prophylactic propranolol.
Case report
A 39-year-old Japanese man with a history of migraine headache and myofascial pain syndrome was referred to our hospital with transient right hemiplegia. Twenty days prior to his presentation, he experienced weakness in his right upper and lower limbs, followed by left occipital pain that resolved with loxoprofen. He had no history of sleep disturbances, excessive physical or mental stress, or head trauma prior to the onset of symptoms. Two days prior to presentation, he experienced weakness in his right upper and lower limbs and a tense feeling on the left side of his mouth while at work. He was found unconscious by a colleague and transported to a local emergency department. He subsequently experienced a left occipital headache, wherein a physical examination indicated right upper and lower limb muscle weakness and sensory disturbance on the ulnar side of the right upper arm. Noncontrast head computed tomography and noncontrast head magnetic resonance imaging (MRI) (Figure 1) and blood test results showed no obvious abnormalities; his symptoms gradually improved. The patient was discharged and then referred to our hospital as the etiology of his symptoms remained unknown. On the first visit, his vital signs were stable (axillary temperature, 36.6°C; blood pressure, 139/91 mmHg; pulse, 70 beats/minute), and his symptoms were mild except for a left parietal headache and fatigue. He was regularly taking lomerizine hydrochloride (5 mg twice daily), diazepam (5 mg once daily), meloxicam (10 mg once daily), and goshuyuto extract (Japanese herbal medicine, 2.5 g twice daily) for his underlying disease. Physical examination revealed no obvious abnormalities in the cranial, motor, or sensory nerves, except for a decreased pain sensation in the left frontal region. Additionally, no abnormal skin lesions or muscle atrophy were observed. Blood test results showed no abnormalities (Table 1) and electroencephalography showed no findings suggestive of epilepsy. No genetic testing was performed. Based on the ICHD-3 criteria, the patient was diagnosed with SHM. Upon the patient’s visit to our hospital, his hemiplegia had significantly improved. As a result, our initial treatment plan was focused on managing his remaining migraine headaches. The patient was taking lomerizine hydrochloride as prophylaxis for migraine headaches. However, the migraine was poorly controlled. Therefore, propranolol hydrochloride (10 mg twice daily) was added to his regular regimen; sumatriptan succinate was initiated for acute headache treatment. The migraine frequency decreased significantly, as did his headache intensity (pain score, 2/10). No recurrence of paralysis occurred approximately 2 years following propranolol initiation. Moreover, the symptoms were sufficiently controlled, without impact on daily activities.
Computed tomography (CT) and magnetic resonance imaging (MRI) during hemiplegic symptoms. (a) Axial noncontrast enhanced CT image shows no findings of edema, infarction, or hemorrhage in the cerebral cortex or medulla. (b) Axial diffusion-weighted MRI shows no hyperintense lesion consistent with acute infarction or inflammation. (c) Axial fluid-attenuated inversion recovery MRI demonstrates no abnormalities.
Hematological and biochemical test results.
Discussion
This case report highlights two educational findings. First, HM should be considered in patients with migraine who present with transient unilateral motor paralysis without obvious intracranial abnormalities on imaging. The differential diagnoses for HM are broad and include stroke, TIA, epilepsy (paralysis following a seizure or Tod’s phenomenon), reversible cerebral vasoconstriction syndrome, carotid artery dissection, meningitis/encephalitis, tumors, and hysteria.2,8 HM is classified into FHM and SHM. However, both types have similar frequency, combination, and duration of aura and cannot be distinguished based on symptoms.2,8 In the acute phase, urgent and thorough investigations are required to identify the underlying cause, such as stroke or TIA. TIA and migraine symptoms can be distinguished, based on their onset, duration, and recurrence patterns. 9 TIAs present with acute onset motor or sensory deficits, hemianopsia, monocular blindness, aphasia, or dysarthria, with symptoms subsiding within minutes to an hour and potentially recurring over days or weeks. Headaches may occur, typically during the neurological phase. In contrast, migraines involve sequential symptom development, beginning with visual disturbances, followed by somatosensory issues, lasting for 20–30 min or longer, recurring over years. Migraines typically include headaches following neurological symptoms, accompanied by additional symptoms such as nausea, vomiting, and sensitivity to light and sound. A meta-analysis investigating the association between stroke and migraine reported that intravenous thrombolytic therapy was performed in 6.65% of patients without acute ischemic stroke; 17.91% of the patients had migraine with aura. 4 HM may be difficult to distinguish from stroke through physical examination owing to the possibility of increased deep tendon reflexes and sensory-motor deficits that may appear primarily in the upper extremities during a migraine attack. 5 The timing of the headache can provides useful information for distinguishing between HM and cerebral hemorrhage. Headaches associated with cerebral hemorrhage develop abruptly, whereas HM typically progresses gradually with aura symptoms. 10 A diagnosis of epilepsy may be made in patients without headache or in pediatric patients. 10 Even in such patients, a detailed history can be used to distinguish convulsive seizures (sudden, usually <1 min) from HM aura (progressive, >20 min). MRI can be useful for assessing migraine with aura mimicking stroke. Conventional imaging findings of HM have, sometimes, revealed cortical edema without infarction. 11 Arterial spin-labeling perfusion analysis could help in identifying patients with HM by revealing associated hypoperfusion during the aura state and hyperperfusion during the headache state.11,12
Second, propranolol could be a potential prophylactic treatment for preventing recurrence of HM, specifically when combined with calcium channel blockers. Confirming propranolol’s efficacy may be useful for the therapeutic diagnosis of migraines. No randomized controlled trials have evaluated the therapeutic efficacy of HM (acute therapy or prophylaxis). Currently, drugs used for acute therapy include verapamil, nimodipine, ketamine, triptans, pulse steroids, and hypertonic saline. 5 The use of triptans in the acute setting is controversial and historically contraindicated. 3 However, a 2007 Finnish retrospective study reported a mean response rate of 6.9 (scale, 1–10; with 10 being excellent) for triptans in patients with HM, indicating that triptans are a safe and effective treatment in most patients with HM. 13 Medications used for prophylactic treatment include verapamil, acetazolamide, flunarizine, lamotrigine, memantine, dextromethorphan, onabotulinum toxin A, calcitonin gene-related peptide antagonists, topiramate, and propranolol.3,5 The prophylactic effect of propranolol for HM has rarely been reported or adequately investigated.6,7 Some specialists avoid using beta-blockers as prophylactic therapy in patients with HM or basilar migraines due to concerns that these drugs may limit compensatory vasodilatory capacity. 14 However, the guidelines related to typical migraine treatment indicate that beta-blockers, such as propranolol, have established efficacy in migraine prophylaxis. 15 Based on our patient’s experience, we believe that addition of propranolol to the lomerizine hydrochloride regimen could be a potential treatment option for HM prophylaxis. Our case might contribute to the existing literature by highlighting the potential of beta-blockers and calcium channel blockers for the treatment of migraine headaches, thereby expanding possible therapeutic options for HM. Further studies regarding concomitant medication use for migraine prophylaxis are needed.
Conclusion
HM should be considered in patients with migraine who present with transient unilateral motor palsy without obvious intracranial abnormalities. Prophylactic treatment with propranolol could be effective in the treatment of SHM.
Footnotes
Acknowledgements
None.
Author contributions
H.M. conceived the idea and wrote the original draft of the manuscript. T.K. and T.A. developed the theory of this case report. H.M., T.K., T.A., H.O., and H.H. discussed the case and commented on the manuscript. H.M., T.K., and T.A. revised and edited the manuscript. All authors gave final approval prior to submission of the manuscript.
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Ethics approval
Our institution does not require ethical approval for reporting individual cases or case series.
Informed consent
Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.
