Hemiplegic migraine: presentation of two cases and review of clinical and imaging findings

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1 Hemiplegic migraine: presentation of two cases and review of clinical and imaging findings Poster No.: R-0193 Congress: 2014 CSM Type: Scientific Exhibit Authors: K. Marripudi, J. Freebody, J. Evans, R. Lee, D. Crimmins, E. Reyneke, J. Hanson; GOSFORD/AU Keywords: Edema, Education, MR, Neuroradiology brain DOI: /ranzcr2014/R-0193 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply RANZCR/AIR/ACPSEM's endorsement, sponsorship or recommendation of the third party, information, product or service. RANZCR/AIR/ ACPSEM is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold RANZCR/AIR/ACPSEM harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies,.ppt slideshows,.doc documents and any other multimedia files are not available in the pdf version of presentations. Page 1 of 14

2 Aim To describe the clinical and radiologic features in two patients with hemiplegic migraine (combination of migraine-type headache with aura and short-term neurological signs). To review the spectrum of imaging findings and relevant differentials. Methods and materials Patient 1: 24-year-old female with long history of common migraine presented with twoday history of occipital headache, left sided hemiplegia and paraesthesia, dysarthria and confusion. Laboratory tests (including LP) were unremarkable. She was initially treated with acyclovir and prednisone, subsequently with antiepileptics and supportive measures. Symptoms completely resolved over 2-3 weeks. Patient 2: 75-year-old female, with history of similar previous episodes, presented with headache, right sided weakness, dysphasia, and seizure. She was treated with continuing anti-epileptic medication and supportive measures. Symptoms gradually resolved over 4 weeks, with no residual deficit. Results Results: Patient 1: Non contrast CT brain was normal. MRI (day 5) showed cortical swelling and increased signal throughout the right hemisphere on T2/FLAIR sequences (Fig. 1 on page 4). Diffusion-weighted imaging showed minor cortical hyperintensity without restricted diffusion (Fig. 2 on page 4, Fig. 3 on page 5). TOF MRA showed normal intracranial arteries (Fig. 4 on page 6). There was mild increased vascularity on post iv gadolinium T1 sequences. Repeat MRI (day 14) showed progressive right hemisphere swelling, left sided shift of midline structures and uncal herniation (Fig. 5 on page 7, Fig. 6 on page 8) Repeat MRI (6 weeks) showed subtle residual cortical signal abnormalities, and no infarction (Fig. 7 on page 9). Patient 2: Non contrast CT brain was normal. Repeat CT (day 7) showed left cerebral swelling. MRI (day 10) showed cortical swelling and increased signal involving most of the left hemisphere on T2/FLAIR sequences (Fig. 8 on page 10). There was minor Page 2 of 14

3 cortical hyperintensity on diffusion-weighted imaging, without restricted diffusion (Fig. 9 on page 11). Discussion: Hemiplegic migraine is a rare type of migraine with aura, characterised by the presence of motor weakness, which may occasionally last up to several days, and then resolve without sequelae. Patients with at least one first- or second-degree relative, who present with aura characterised by motor weakness, fulfill the criteria for familial hemiplegic migraine, whereas patients without a family history have sporadic hemiplegic migraine (1). The pathogenesis of migraine is not completely understood. Neuronal hyperexcitability, followed by depression of normal activity, spreads slowly from the site of initiation resulting in aura. The cortical spreading depression activates the trigeminal nucleus caudalis, and both the trigeminal and the parasympathetic systems cause dilatation of the meningeal arteries, triggering the headache (2). 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 (3, 9). Attacks of hemiplegic migraine may present with acute neurologic symptoms and signs - the differential diagnosis includes acute brain infarction, vasculitis, focal infections, or less common conditions, such as the syndromes of mitochondrial encephalomyopathy, lactic acidosis, and stroke like symptoms (or MELAS), cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (or CADASIL), and transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (or HaNDL) (3, 4 and 9). Before the aura begins cerebral blood flow is reduced at the occipital pole; this may be responsible for the visual symptoms. Hypoperfusion gradually spreads anteriorly and is followed by a period of hyperperfusion that usually outlasts the headache (5,6 and 9). T2-weighted images obtained during hyperperfusion usually show diffuse cortical swelling and edema contralateral to the hemiparesis and not confined to one vascular territory. The region does not usually enhance on T1-weighted images after contrast administration. Diffusion-weighted images may show reversible decrease in water diffusion and perfusion MR images may show increased perfusion in the same area (7,8 and 9). The persistent time-course of decreased diffusion suggests that cortical edema may be caused by prolonged neuronal depolarization and is not of ischemic origin (7). Page 3 of 14

4 Images for this section: Fig. 1: (case 1) T2-weighted imaging at the level of lateral ventricles demonstrates increased signal and cortical swelling and sulcal effacement of the right hemisphere. Page 4 of 14

5 Fig. 2: (case 1) Axial Diffusion-weighted sequence demonstrates minor right cortical hyperintensity (b=1000, TE/TE: 5800/96 ms). Page 5 of 14

6 Fig. 3: (case 1) Corresponding ADC map with no restricted diffusion. Page 6 of 14

7 Fig. 4: (case 1) TOF MRA demonstrates no abnormalities with the vessels. Page 7 of 14

8 Fig. 5: (case 1) Day 14, T2-weighted axial sequence with progressively increasing cortical swelling on the right and midline shift to the left. Page 8 of 14

9 Fig. 6: (case 1) T2-weighted coronal BLADE sequence on day 14 through the level of third ventricle shows cortical swelling and sulcal effacement on the right with left sided midline shift and uncal herniation. Page 9 of 14

10 Fig. 7: (case 1) Axial FLAIR sequence at 6 weeks shows subtle residual right cortical signal abnormalities and no infarction. Page 10 of 14

11 Fig. 8: (case 2) Axial FLAIR sequence demonstrates increased signal involving most of the left hemisphere. Page 11 of 14

12 Fig. 9: (case 2) Axial Diffusion-weighted imaging shows subtle increased left hemisphere cortical signal but there was no restricted diffusion. Page 12 of 14

13 Conclusion Diagnosis of hemiplegic migraine is based on clinical features. The role imaging is to rule out other conditions which may mimic the diagnosis, and to distinguish between persistent aura and migrainous infarction. Personal information References 1.Mourand I, Menjot de Champfleur N, Carra-Dallière C, Le Bars E, Roubertie A,Bonafé A, Thouvenot E. Perfusion-weighted MR imaging in persistent hemiplegic migraine. Neuroradiology (2012); 54: Welch KM. Contemporary concepts of migraine pathogenesis. Neurology 2003; 61 (8 suppl 4): S2-S8. 3. Black DF. Sporadic hemiplegic migraine. Curr Pain Headache Rep 2004; 8(3): Schoenen J, Sandor PS. Headache with focal neurological signs or symptoms: a complicated differential diagnosis. Lancet Neurol 2004; 3(4): 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): 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): Butteriss DJ, Ramesh V, Birchall D. Serial MRI in a case of familial hemiplegic migraine. Neuroradiology 2003; 45(5): Page 13 of 14

14 8. 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): Politi M, Papanagiotou P, Grunwald I, Reith W, Hemiplegic migraine, Diagnosis please, case: 125. Radiology: Volume 245: Number 2-November Page 14 of 14