DOI: 10.1148/radiol.2452041596
(Radiology 2007;245:600-603.)
© RSNA, 2007
Case 125: Hemiplegic Migraine1
Maria Politi, MD,
Panagiotis Papanagiotou, MD,
Iris Q. Grunwald, MD and
Wolfgang Reith, MD, PhD
1 From the Clinic for Diagnostic and Interventional Neuroradiology, University Hospital of the Saarland, Kirrbergestr D-66421 Homburg/Saar, Germany. Received September 26, 2004; revision requested November 30; revision received January 16, 2005; accepted February 1; final version accepted May 4.
Address correspondence to M.P. (e-mail: mariapoliti{at}hotmail.com).
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HISTORY
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A 15-year-old boy was admitted to our hospital with acute weakness in the left upper and lower extremities. He was febrile (39.1°C) and somnolent. Two hours prior to arrival, a migraine attack with visual aura began. The patient had experienced a transient attack of right upper and lower extremity weakness 6 years earlier.
Findings of routine biochemical and hematologic examinations and lumbar puncture were normal. Test results were negative for mitochondrial disorders. Magnetic resonance (MR) images were obtained.
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IMAGING FINDINGS
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T2-weighted images showed diffuse cortical swelling and mild cortical hyperintensity of the right hemisphere. These findings were most likely caused by cortical edema (Fig 1). Diffusion-weighted images (Fig 2) and MR angiograms (Fig 3) were normal.
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Figure 1a: Transverse fast spin-echo T2-weighted MR images (repetition time msec/echo time msec, 4030/108) through the level of the (a) occipital lobe and (b) lateral ventricles show diffuse cortical swelling and mild cortical hyperintensity of the right hemisphere. These findings are most likely caused by cortical edema. Note how the appearance of the subarachnoid space differs between the two hemispheres.
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Figure 1b: Transverse fast spin-echo T2-weighted MR images (repetition time msec/echo time msec, 4030/108) through the level of the (a) occipital lobe and (b) lateral ventricles show diffuse cortical swelling and mild cortical hyperintensity of the right hemisphere. These findings are most likely caused by cortical edema. Note how the appearance of the subarachnoid space differs between the two hemispheres.
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During the next week, the patient developed aphasia and experienced a seizure, while hemiparesis persisted. MR imaging was repeated. Increased cortical swelling of the right hemisphere was seen on T2-weighted MR images (Fig 4). After 15 days, symptoms had fully reversed, and T2-weighted images were normal (Fig 5).
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Figure 4a: Transverse fast spin-echo T2-weighted MR images (4030/108) through the level of the (a) frontal, temporal, and occipital lobes and (b) lateral ventricles show increased cortical swelling in the right hemisphere.
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Figure 4b: Transverse fast spin-echo T2-weighted MR images (4030/108) through the level of the (a) frontal, temporal, and occipital lobes and (b) lateral ventricles show increased cortical swelling in the right hemisphere.
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DISCUSSION
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Hemiplegic migraine is a rare type of migraine with aura characterized by recurrent episodes of varying degrees of focal weakness associated with migraine (1). According to International Headache Society criteria, hemiplegic migraine is diagnosed clinically on the basis of an accurate history, while routine biochemical and hematologic examinations, as well as lumbar puncture and imaging findings, help to exclude other more common disorders (2). Attacks of hemiplegic migraine may manifest with acute neurologic symptoms and signs; thus, the differential diagnosis includes acute brain infarction, vasculitis, focal infections, or less common conditions, such as the syndromes of (a) mitochondrial encephalomyopathy, lactic acidosis, and strokelike symptoms (or MELAS), (b) cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (or CADASIL), and (c) transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (or HaNDL) (1,2).
In this case, diffusion-weighted images and MR angiograms were normal, enabling us to rule out an acute brain infarction. Vasculitis was not a probable diagnosis because of the episode 6 years earlier and the nearly holohemispheric and unilateral nature of the process. Furthermore, lumbar puncture findings were normal. There was no tumor, obvious lesion, or sign of infection, such as abscess or encephalitis, on MR images. An infection was not probable because the blood analysis, biochemical examination, and lumbar puncture findings were normal. Furthermore, lumbar puncture revealed no signs of infection or less common disorders, such as HaNDL syndrome. MR imaging revealed no signs of other rare disorders, such as MELAS or CADASIL syndromes. MELAS syndrome was also excluded because test results were negative for mitochondrial disorders. Moreover, the finding of cortical swelling, which was the only imaging finding in this case, was in accordance with the imaging findings of hemiplegic migraine, as described in the literature (3–7).
In this case, no other disorder was detected, motor weakness was seen after the headache, the patient had experienced a transient attack of hemiparesis in the past, and the imaging examinations revealed mild cortical swelling. On the basis of the above combined signs and symptoms, the diagnosis of sporadic hemiplegic migraine was the most probable one. Motor weakness had fully reversed after 15 days; thus, the International Headache Society criteria for hemiplegic migraine (2) were fulfilled.
Attacks may include fever, lethargy, aphasia, confusion, scintillating scotoma, hemianopsia, hemisensory symptoms, cerebellar ataxia, epilepsy, loss of consciousness, coma, or—in rare instances—death. Unilateral symptoms may switch sides between attacks. More commonly, weakness is reported in the upper limbs rather than in the lower limbs. When the attack is finished, the neurologic deficit usually resolves fully; however, permanent neurologic sequelae—including dementia, hemiplegia, progressive cerebral dysfunction, and even death—may occur (2). Attacks may begin at 5 years of age, and, in general, onset occurs before 20 years of age. Attacks may be provoked by mild head trauma or angiography. The frequency of attacks varies, ranging from two to more than 100 attacks per lifetime.
There are two forms of hemiplegic migraine: the familial form and the sporadic form. Both forms share a similar spectrum of clinical presentations and should be thought of as similar but separate disorders (2). In the familial form of hemiplegic migraine, the transmission is autosomal dominant. Familial hemiplegic migraine is divided into two subtypes according to the genetic mutation. In the first type, there is a point mutation in the CACNA1A gene on chromosome 19. In the second type, there is a mutation in the ATP1A2 gene on chromosome 1. For sporadic hemiplegic migraine, only the first mutation is reported. A wide range of aurae and clinical phenotypes can be seen in family members who bear the same mutation (8).
The pathogenesis of migraine is incompletely understood. The neuronal hyperexcitability followed by depression of normal activity spreads slowly from the site of initiation at a rate of 2–6 mm per minute, resulting in aura. The cortical spreading depression activates the trigeminal nucleus caudalis, and both the trigeminal and the parasympathetic systems cause dilatation of the extracerebral circulation, particularly of the meningeal arteries, producing the headache. The progressive damage to the periaqueductal gray matter may explain some aspects of central sensitization or change in phenotypic expression of the disorder (9).
Several examinations have been performed in patients with hemiplegic migraines while the headache or symptoms are still present in an attempt to understand the pathogenesis of migraine (3). It is reported that before aura symptoms development, cerebral blood flow is reduced at the occipital pole; this may explain the visual symptoms. The hypoperfusion gradually spreads anteriorly and is followed by a period of hyperperfusion that usually outlasts the headache (3,4). T2-weighted images obtained during hyperperfusion usually show diffuse cortical swelling and edema contralateral to the hemiparesis. The affected regions do not correspond to a singular vascular territory, and the lesion does not enhance on T1-weighted images after contrast medium administration. Diffusion-weighted images might show reversible decrease in water diffusion in the hemisphere contralateral to the hemiparesis, and perfusion MR images might show increased perfusion at the same area (5,6); however, neither finding was seen in this case. The presence of decreased diffusion for a long time suggests that cortical edema may be caused by prolonged neuronal depolarization and is not of ischemic origin (5).
On the other hand, T2- or diffusion-weighted images may be normal (3,5,6). Contrary to MR angiographic findings in this case, a mild dilatation of intracranial vessels contralateral to the hemiparesis has been reported (5,6). Phosphorus 31 spectroscopy reveals low magnesium levels in the posterior brain areas in patients with hemiplegic migraine compared with magnesium levels in healthy individuals. The low magnesium levels may contribute to neuronal hyperexcitability and thus to the pathogenesis of hemiplegic migraine. Alternatively, changes in magnesium concentration may reflect unsuccessful attempts by the brain to maintain homeostasis by means of magnesium fixation in the cell to stabilize excitable membranes and by means of gating excitatory receptors (10).
The cerebral consumption of oxygen is normal; thus, migraines are not caused by ischemic alternations (5). Finally, positron emission tomography reveals glucose hypometabolism, suggesting primary neuronal dysfunction (7). The changes usually resolve spontaneously without radiologic or clinical evidence of irreversible damage.
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FOOTNOTES
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Published online
Authors stated no financial relationship to disclose.
| Part one of this case appeared 4 months previously and may contain larger images.
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References
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- Schoenen J, Sandor PS. Headache with focal neurological signs or symptoms: a complicated differential diagnosis. Lancet Neurol 2004;3(4):237–245. [CrossRef][Medline]
- Black DF. Sporadic hemiplegic migraine. Curr Pain Headache Rep 2004;8(3):223–228. [CrossRef][Medline]
- Oberndorfer S, Wober C, Nasel C, et al. Familial hemiplegic migraine: follow-up findings of diffusion-weighted magnetic resonance imaging (MRI), perfusion-MRI and [99mTc] HMPAO-SPECT in a patient with prolonged hemiplegic aura. Cephalalgia 2004;24(7):533–539. [CrossRef][Medline]
- Olesen J, Friberg L, Olsen TS, et al. Timing and topography of cerebral blood flow, aura, and headache during migraine attacks. Ann Neurol 1990;28(6):791–798. [CrossRef][Medline]
- Butteriss DJ, Ramesh V, Birchall D. Serial MRI in a case of familial hemiplegic migraine. Neuroradiology 2003;45(5):300–303. [Medline]
- Masuzaki M, Utsunomiya H, Yasumoto S, Mitsudome A. A case of hemiplegic migraine in childhood: transient unilateral hyperperfusion revealed by perfusion MR imaging and MR angiography. AJNR Am J Neuroradiol 2001;22(9):1795–1797.[Abstract/Free Full Text]
- Gutschalk A, Kollmar R, Mohr A, et al. Multimodal functional imaging of prolonged neurological deficits in a patient suffering from familial hemiplegic migraine. Neurosci Lett 2002;332(2):115–118.[CrossRef][Medline]
- Moskowitz MA, Bolay H, Dalkara T. Deciphering migraine mechanisms: clues from familial hemiplegic migraine genotypes. Ann Neurol 2004;55(2):276–280.[CrossRef][Medline]
- Welch KM. Contemporary concepts of migraine pathogenesis. Neurology 2003;61(8 suppl 4):S2–S8. [Abstract/Free Full Text]
- Boska MD, Welch KM, Barker PB, Nelson JA, Schultz L. Contrasts in cortical magnesium, phospholipid and energy metabolism between migraine syndromes. Neurology 2002;58(8):1227–1233. [Abstract/Free Full Text]
Congratulations to the 60 individuals and three resident groups that submitted the most likely diagnosis (hemiplegic migraine) for Diagnosis Please, Case 125. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses
- Todd Aho, MD, Detroit, Mich
- Erhan Akpinar, Ankara, Turkey
- Jason B. Ashley, MD, London, Ontario, Canada
- Asim K. Bag, MD, Birmingham, Ala
- Kenneth F. Baliga, MD, Rockford, Ill
- Eric L. Bressler, MD, Minnetonka, Minn
- Daniel F. Broderick, MD, Jacksonville, Fla
- Marcelo Cabrini, Lomas de Zamora, Argentina
- Antonio A. Cavalcanti, MD, São Paulo, Brazil
- Michael H. Childress, MD, Silver Spring, Md
- Alex T. Chuang, MD, Dallas, Tex
- Walter T. Depaulaneto III, MD, Rio de Janeiro, Brazil
- Seyed A. Emamian, MD, PhD, Rockville, Md
- Shella Farooki, Dublin, Ohio
- Virginia Fattal Jaef, MD, Rosario, Argentina
- Brett D. Ferdinand, MD, Livingston, NJ
- Akira Fujikawa, MD, Setagaya, Tokyo, Japan
- Rajneesh Galwa, Jaipur, India
- Ram P. Galwa, MD, Chandigarh, India
- Gilles Genin, Annecy, France
- Vidisha V. Ghole, MD, Irving, Tex
- Mark G. Goldshein, MD, Andover, Mass
- Francisco Jose Gonzalez, Santander, Spain
- Dan G. Gridley, MD, Phoenix, Ariz
- Flavius F. Guglielmo, MD, Basking Ridge, NJ
- Waleed M. Ibrahim, MD, Columbus, Ohio
- Kiriakos Kalampoukas, MD, Halandri, Greece
- Shinichi Kan, MD, Sagamihara-shi, Kanagawa, Japan
- Stefanos Lachanis, Athens, Greece
- Alexis Lacout, MD, Paris, France
- Mario A. Laguna, MD, Milwaukee, Wis
- Martin Lecompte, MD, Manotick, Ontario, Canada
- Myron M. Levitt, MD, Scotch Plains, NJ
- David A. Lisle, MBBS, Brisbane, Australia
- Patricia A. Lowry, MD, Chattanooga, Tenn
- Waldir H. Maymone, MD, Rio de Janeiro, Brazil
- Ronan A. McDermott, MBBCh, Dublin, Ireland
- Nikolaos Michailidis, Thessaloniki, Greece
- Manabu Minami, MD, PhD, Yokohama, Japan
- Robert L. Mittl, Jr, MD, Charlotte, NC
- Sankar R. Mondal, MD, Nassau, Bahamas
- Thomas Moser, MD, Strasbourg, France
- Tetsuo Nakayama, MD, Osaka, Japan
- Bonnie E. O'hayon, MD, Toronto, Ontario, Canada
- Anietie E. Okon, MD, North Liberty, Iowa
- Klaus Orth, Aachen, Germany
- Marc G. Ossip, MD, Toronto, Ontario, Canada
- Yeliz Pekcevik, Izmir, Turkey
- Ilias Primetis, MD, Athens, Greece
- Daniel C. Rappaport, MD, Toronto, Ontario, Canada
- Eytan Raz, BS, Roma, Italy
- Tsutomu Sakamoto, MD, Tokyo, Japan
- Anthony J. Scuderi, MD, Johnstown, Pa
- Matthew P. Shapiro, MD, Charlottesville, Va
- Taro Shimono, MD, Osaka, Sayama, Japan
- Subramanian Subramanian, MD, New Delhi, India
- Kouichi Sugiyama, Numazu, Japan
- Douglas L. Teich, MD, Brookline, Mass
- Eleni Vafeiadou, Thessaloniki, Greece
- Joe Yut, Olathe, Kan
Resident group responses
- Santa Casa da Misericórdia do Rio de Janeiro Radiology Residents, Rio de Janeiro, Brazil
- University of Pennsylvania Radiology Residents, Philadelphia, Pa
- Virginia Commonwealth University Radiology Residents, Richmond, Va
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HISTORY
IMAGING FINDINGS
