ISSN 0017-8748 doi: 10.1111/head.12298 Published by Wiley Periodicals, Inc.
Headache © 2014 American Headache Society
Images From Headache Hemiplegic Migraine: Neuroimaging Findings During a Hemiplegic Migraine Attack Gavin Sugrue, MD; Ferdia Bolster, MD; Ian Crosbie, MD; Eoin Kavanagh, MD
CASE A 19-year-old male presented with sudden onset right-sided weakness and slurred speech following a severe headache. His past medical history was relevant for a similar episode 3 years previously; however, he did not seek medical advice at this time. He denied any recent drug or alcohol use, or any recent head injury. He had no family history of migraine. Routine biochemical, hematological, and lumbar puncture test results were normal. On clinical exam, he had a dense right hemiparesis, inattention, and aphasia. An urgent noncontrast computerized tomography (CT) brain (Fig. 1) on admission demonstrated subtle asymmetrical sulcal effacement in the left cerebral hemisphere suggestive of cerebral edema. There was no intracranial hemorrhage and no evidence of an ischemic event. There was no enhancing lesion or abnormal enhancement following the administration of contrast. In light of these findings on CT, he underwent a magnetic resonance imaging (MRI) of the brain. T2-FLuid Attenuated Inversion Recovery (FLAIR)weighted images (Fig. 2) showed increased cortical hyperintensity and cortical swelling of the left cerebral hemisphere consistent with unilateral cerebral edema and were consistent with the clinical findings of right-sided weakness. Diffusion-weighted MR images (Fig. 3) show no abnormal signal intensity.
Fig 1.—Noncontrast CT of the brain demonstrating subtle asymmetrical sulcal effacement in the left cerebral hemisphere suggestive of cerebral edema. There was no evidence of intracranial hemorrhage or an ischemic event.
Post-contrast T1-weighted MR (Fig. 4) imaging demonstrated no abnormal enhancement. On day 2 of admission, he subsequently developed seizures, which spontaneously settled. The remained of his hospital admission was uncomplicated, and his neurological deficit had begun to The principal author takes full responsibility for the data presented in this study, analysis of the data, conclusions, and conduct of the research. The principal author had full access to those data and has maintained the right to publish any and all data independent of any third party.
From the Department of Radiology, Mater Misericordiae University Hospital, Dublin, Ireland.
Conflict of Interest: None.
716
Headache
717
A
B
Fig 2.—T2-FLAIR weighted MR images show increased cortical hyperintensity and cortical swelling of the left cerebral hemisphere consistent with unilateral cerebral edema.
improve at the time of discharge from hospital. He was followed up in the neurology outpatients; at 3 weeks, his right-sided hemiparesis was still evident but had significantly improved. At 3 months, a mild residual dysphasia remained. Follow-up T2-FLAIR MRI (Fig. 5) 3 months following initial presentation demonstrated no abnormality with complete radiological resolution of the cerebral cortical edema.
COMMENTARY Hemiplegic migraine is a rare type of migraine with aura. It has an incidence of 0.01%,1 with the familial and sporadic types occurring with equal frequency.
A
The pathogenesis of hemiplegic migraine is not well established; however, it is hypothesized that it arises from a wave of neuronal excitation in the gray matter that spreads across the cerebral cortex.2 The diagnosis of hemiplegic migraine is made clinically in accordance with international guidelines.3 Hallmark symptoms are intermittent episodes of motor weakness that spontaneously settle over time ranging from hours to weeks. Patients may experience visual and speech abnormalities, and seizures as in our case, but also paresthesia, numbness, fever, lethargy, and rarely death. However, if the diagnosis still remains uncertain, neuroimaging (CT brain, MRI
B
Fig 3.—(A) Diffusion-weighted MR image (Diffusion B 0) shows no abnormal signal intensity. (B) Diffusion-weighted MR image (Diffusion B1000) shows no abnormal signal intensity.
718
April 2014
Fig 4.—T1-weight MR image post contrast demonstrates no abnormal enhancement.
brain, magnetic resonance angiogram), electroencephalogram, and lumbar puncture can rule out other potential differential diagnosis that include cerebral vascular event; tumor; vasculitis; mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episode; cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy; and epilepsy. Neuroimaging in acute attacks of hemiplegic migraine is often normal. However, imaging may reveal cerebral cortical hyperintesity and edema4,5 contralateral to the hemiparesis, as seen in this case. These abnormalities seen on imaging, alike the neurological deficit, often resolve within weeks to months after an attack. There have been no randomized control trials to evaluate treatment options. Case reports and anecdotal evidence suggestive treatment with verapamil, flunarizine, lamotrigine, and acetazolamide. Avoidance of triptans and beta blockers6 has been suggested. In conclusion, hemiplegic migraine is a rare condition, and the diagnosis is often made clinically. However, if the diagnosis is not clear, neuroimaging can exclude other important diagnoses, in particular a stroke or tumor. Characteristic neuroimaging findings
Fig 5.—T2-FLAIR MR image 3 months following initial presentation demonstrates no abnormality, with complete radiologic resolution of the cerebral cortical edema.
have been described and supportive of a diagnosis of hemiplegic migraine.
REFERENCES 1. Lykke Thomsen L, Kirchmann Eriksen M, Faerch Romer S, et al. An epidemiological survey of hemiplegic migraine. Cephalalgia. 2002;22:361-375. 2. Welch KM. Contemporary concepts of migraine pathogenesis. Neurology. 2003;61(Suppl. 4)S2-S8. 3. Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders. Cephalalgia. 2004; 24(Suppl. 1):9-150. 4. Cha YH, Millett D, Kane M, Jen J, Baloh R. Adultonset hemiplegic migraine with cortical enhancement and oedema. Cephalalgia. 2007;27:1166-1170. 5. Dreier JP, Jurkat-Rott K, Petzold GC, et al. Opening of the blood-brain barrier preceding cortical edema in a severe attack of FHM type II. Neurology. 2005; 64:2145-2147. 6. Evans RW, Lipton RB. Topics in migraine management: a survey of headache specialists highlights some controversies. Neurol Clin. 2001;19:1.
Headache © 2014 American Headache Society
Images From Headache Hemiplegic Migraine: Neuroimaging Findings During a Hemiplegic Migraine Attack Gavin Sugrue, MD; Ferdia Bolster, MD; Ian Crosbie, MD; Eoin Kavanagh, MD
CASE A 19-year-old male presented with sudden onset right-sided weakness and slurred speech following a severe headache. His past medical history was relevant for a similar episode 3 years previously; however, he did not seek medical advice at this time. He denied any recent drug or alcohol use, or any recent head injury. He had no family history of migraine. Routine biochemical, hematological, and lumbar puncture test results were normal. On clinical exam, he had a dense right hemiparesis, inattention, and aphasia. An urgent noncontrast computerized tomography (CT) brain (Fig. 1) on admission demonstrated subtle asymmetrical sulcal effacement in the left cerebral hemisphere suggestive of cerebral edema. There was no intracranial hemorrhage and no evidence of an ischemic event. There was no enhancing lesion or abnormal enhancement following the administration of contrast. In light of these findings on CT, he underwent a magnetic resonance imaging (MRI) of the brain. T2-FLuid Attenuated Inversion Recovery (FLAIR)weighted images (Fig. 2) showed increased cortical hyperintensity and cortical swelling of the left cerebral hemisphere consistent with unilateral cerebral edema and were consistent with the clinical findings of right-sided weakness. Diffusion-weighted MR images (Fig. 3) show no abnormal signal intensity.
Fig 1.—Noncontrast CT of the brain demonstrating subtle asymmetrical sulcal effacement in the left cerebral hemisphere suggestive of cerebral edema. There was no evidence of intracranial hemorrhage or an ischemic event.
Post-contrast T1-weighted MR (Fig. 4) imaging demonstrated no abnormal enhancement. On day 2 of admission, he subsequently developed seizures, which spontaneously settled. The remained of his hospital admission was uncomplicated, and his neurological deficit had begun to The principal author takes full responsibility for the data presented in this study, analysis of the data, conclusions, and conduct of the research. The principal author had full access to those data and has maintained the right to publish any and all data independent of any third party.
From the Department of Radiology, Mater Misericordiae University Hospital, Dublin, Ireland.
Conflict of Interest: None.
716
Headache
717
A
B
Fig 2.—T2-FLAIR weighted MR images show increased cortical hyperintensity and cortical swelling of the left cerebral hemisphere consistent with unilateral cerebral edema.
improve at the time of discharge from hospital. He was followed up in the neurology outpatients; at 3 weeks, his right-sided hemiparesis was still evident but had significantly improved. At 3 months, a mild residual dysphasia remained. Follow-up T2-FLAIR MRI (Fig. 5) 3 months following initial presentation demonstrated no abnormality with complete radiological resolution of the cerebral cortical edema.
COMMENTARY Hemiplegic migraine is a rare type of migraine with aura. It has an incidence of 0.01%,1 with the familial and sporadic types occurring with equal frequency.
A
The pathogenesis of hemiplegic migraine is not well established; however, it is hypothesized that it arises from a wave of neuronal excitation in the gray matter that spreads across the cerebral cortex.2 The diagnosis of hemiplegic migraine is made clinically in accordance with international guidelines.3 Hallmark symptoms are intermittent episodes of motor weakness that spontaneously settle over time ranging from hours to weeks. Patients may experience visual and speech abnormalities, and seizures as in our case, but also paresthesia, numbness, fever, lethargy, and rarely death. However, if the diagnosis still remains uncertain, neuroimaging (CT brain, MRI
B
Fig 3.—(A) Diffusion-weighted MR image (Diffusion B 0) shows no abnormal signal intensity. (B) Diffusion-weighted MR image (Diffusion B1000) shows no abnormal signal intensity.
718
April 2014
Fig 4.—T1-weight MR image post contrast demonstrates no abnormal enhancement.
brain, magnetic resonance angiogram), electroencephalogram, and lumbar puncture can rule out other potential differential diagnosis that include cerebral vascular event; tumor; vasculitis; mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episode; cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy; and epilepsy. Neuroimaging in acute attacks of hemiplegic migraine is often normal. However, imaging may reveal cerebral cortical hyperintesity and edema4,5 contralateral to the hemiparesis, as seen in this case. These abnormalities seen on imaging, alike the neurological deficit, often resolve within weeks to months after an attack. There have been no randomized control trials to evaluate treatment options. Case reports and anecdotal evidence suggestive treatment with verapamil, flunarizine, lamotrigine, and acetazolamide. Avoidance of triptans and beta blockers6 has been suggested. In conclusion, hemiplegic migraine is a rare condition, and the diagnosis is often made clinically. However, if the diagnosis is not clear, neuroimaging can exclude other important diagnoses, in particular a stroke or tumor. Characteristic neuroimaging findings
Fig 5.—T2-FLAIR MR image 3 months following initial presentation demonstrates no abnormality, with complete radiologic resolution of the cerebral cortical edema.
have been described and supportive of a diagnosis of hemiplegic migraine.
REFERENCES 1. Lykke Thomsen L, Kirchmann Eriksen M, Faerch Romer S, et al. An epidemiological survey of hemiplegic migraine. Cephalalgia. 2002;22:361-375. 2. Welch KM. Contemporary concepts of migraine pathogenesis. Neurology. 2003;61(Suppl. 4)S2-S8. 3. Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders. Cephalalgia. 2004; 24(Suppl. 1):9-150. 4. Cha YH, Millett D, Kane M, Jen J, Baloh R. Adultonset hemiplegic migraine with cortical enhancement and oedema. Cephalalgia. 2007;27:1166-1170. 5. Dreier JP, Jurkat-Rott K, Petzold GC, et al. Opening of the blood-brain barrier preceding cortical edema in a severe attack of FHM type II. Neurology. 2005; 64:2145-2147. 6. Evans RW, Lipton RB. Topics in migraine management: a survey of headache specialists highlights some controversies. Neurol Clin. 2001;19:1.
![[Electroencephalographic expression of hemiplegic migraine].](/assets/img/hold.png)
