Sporadic hemiplegic migraine and CREST syndrome

Abstract

Hemiplegic migraines are characterized by attacks of migraine with aura accompanied by transient motor weakness. There are both familial and sporadic subtypes, which are now recognized as separate entities by the International Classification of Headache Disorders, edition II (ICHD-II). The sporadic subtype has been associated with other medical conditions, particularly rheumatological diseases. We report the case of a woman with sporadic hemiplegic migraine associated with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Since there is a close relationship between migraine and Raynaud's phenomenon, it could be speculated that the sporadic hemiplegic migraines in our patient might be secondary to CREST syndrome. [PUBLICATION ABSTRACT]

J Headache Pain (2010) 11:171173 DOI 10.1007/s10194-010-0188-1

BRIEF REPORT

Sporadic hemiplegic migraine and CREST syndrome

Martin Pablo Grecco Miguel Pieroni

Marcela Otero Jorge Luis Ferreiro

Mara de Lourdes Figuerola

Received: 29 October 2009 / Accepted: 6 January 2010 / Published online: 4 February 2010 Springer-Verlag 2010

Abstract Hemiplegic migraines are characterised by attacks of migraine with aura accompanied by transient motor weakness. There are both familial and sporadic subtypes, which are now recognised as separate entities by the International Classication of Headache Disorders, edition II (ICHD-II). The sporadic subtype has been associated with other medical conditions, particularly rheumatological diseases. We report the case of a woman with sporadic hemiplegic migraine associated with CREST syndrome (calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Since there is a close relationship between migraine and Raynauds phenomenon, it could be speculated that the sporadic hemiplegic migraines in our patient might be secondary to CREST syndrome.

Keywords Migraine disorders Hemiplegic migraine

Migraine with auras CREST syndrome

Introduction

Hemiplegic migraines are characterised by fully reversible motor auras and have been recognised as a distinct group by the International Classication of Headache Disorders, edition II (ICHD-II) [1]. They present with two subtypes: genetic (familial hemiplegic migraine) and sporadic

(sporadic hemiplegic migraine) [1, 2]. The latter has been associated with a number of medical conditions, such as tumours, vascular disorders and autoimmune diseases [3]. We report the case of a woman with sporadic hemiplegic migraine associated with CREST syndrome (calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia).

Case report

A 34-year-old woman was referred to our clinic with a presumptive diagnosis of transient ischemic attack (TIA). She presented with episodes of transient hemiparesis that fully resolved without sequelae and were followed by severe headache. These episodes consisted of progressive left-sided weakness occurring in a stepwise pattern: rst affecting the face, then the arm and nally the leg. Some of the episodes were preceded by sensory disturbances and, on other occasions, by scintillating scotomas. The symptomatology lasted no more than 30 min and was followed by an intense contralateral headache with a migrainous pattern lasting from 12 to 36 h. The patient admitted having experienced similar episodes since adolescence, with a once-a-year frequency over the past 5 years. During the past year these attacks had become more frequent, increasing to one per month.

Her past medical history was remarkable for CREST syndrome, which rst appeared at age 15 with Raynauds phenomenon. She later developed skin induration in her hands and feet, calcinosis cutis and facial telangiectasias. These latter symptoms, which appeared approximately 15 years after Raynauds phenomenon, led to the diagnosis of CREST syndrome. The presence of anti-centromere antibodies conrmed the diagnosis. She had no family

M. P. Grecco (&) M. Pieroni M. Otero J. L. Ferreiro

Neurology, Hospital de Clnicas, Av. Crdoba 2351, 1120 Buenos Aires, Argentinae-mail: [email protected]

M. de L. FiguerolaNeurology, Hospital de Clnicas, CEDIE-CONICET, Buenos Aires, Argentina

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history of migraine or any other neurological illnesses. Her usual medications included prednisone, nifedipine and aspirin. She was also taking levothyroxine for hypothyroidism. She had never taken oral contraceptives and was not on hormone replacement therapy.

The neurological examination was normal, and the laboratory panel showed a raised erythrocyte sedimentation rate of 48 mm/h and a mild normocytic anaemia (haemoglobin = 11.5 mg/dL). She had previously tested negative for anti-neutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), anti-DNA and anti-cardiolipin antibodies, hepatitis B and C, HIV and syphilis. Magnetic resonance imaging (T1-weighted, T2-weighted and FLAIR sequences) showed only a few punctiform hyperintense white matter lesions on T2-weighted images. Diffusion-weighted imaging (DWI) taken between 12 and 24 h after the hemiplegic attacks on two occasions were normal. Cerebral MRI angiography was also normal. Cerebrospinal uid (CSF) analysis did not reveal any abnormalities. The electroencephalogram (EEG) showed no evidence of focal or general discharges. A transesophageal echocardiogram ruled out a patent foramen ovale (PFO). She was started on prophylactic treatment with topiramate 25 mg/day, reaching a nal dose of 75 mg BID. After 9 months of follow-up, there was nearly a 50% reduction in both the frequency and severity of the migraine attacks with aura, and she did not experience further episodes of hemiplegic migraine.

Discussion

Hemiplegic migraines have been recognised as a separate entity by the ICHD-II (2004), comprising both familial (FHM) and sporadic (SHM) subtypes [1, 2]. The sporadic form is dened as a migraine with aura including motor weakness but with no rst or second degree relative with aura including motor weakness [1]. SHM clinical characteristics are very similar to those of FHM, consisting of motor aura usually followed by other typical auras (mostly visual and sensory) and headache [2, 4, 5]. Motor decits are usually unilateral, and most of them resolve in less than an hour. A large subset of patients also met the clinical criteria for basilar migraine during the attacks [1, 2, 4]. The main differential diagnoses include transient ischemic attacks (TIA), stroke and epilepsy [1, 2, 6]. Other entities with a similar clinical presentation, albeit uncommon, include MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and HaNDL (transient headache with neurological decits and CSF lymphocytosis) [6]. The prognosis is generally good with complete recovery after the attacks. The frequency of the attacks tends to decrease with age, and the pattern becomes similar to migraine with aura. There are also reports in the

literature describing cases of SHM attacks secondary to an underlying medical condition such as systemic lupus erythematosus (SLE), anti-phospholipid syndrome (APS), SturgeWeber syndrome, hemifacial atrophy and tumours, among others [3]. This last group is also known as symptomatic sporadic hemiplegic migraine (symptomatic SHM) [3].

There are no uniform recommendations regarding treatment, although drugs such as propranolol, verapamil, unarizine and naloxone have been used with variable degrees of response [2, 4]. It has been suggested that triptans and ergotamine derivatives are not useful and are even detrimental [2]. With regard to disease management, it seems reasonable to initiate preventive treatment only in those patients who have frequent and/or severe attacks. For the management of our patient, we started prophylactic treatment, taking into account both the frequency and intensity of her migraine attacks. She was started on topiramate given its favourable side effects prole in comparison with other treatments such as calcium channel blockers or naloxone in a young woman with a CREST syndrome as an associated pathology. She was also prescribed naproxen to relieve acute attacks.

To our knowledge this is the rst case of symptomatic SHM associated with CREST syndrome. CREST syndrome is considered a subtype of limited scleroderma, consisting of calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia [7]. The pattern of cutaneous involvement is limited to the face and distal extremities, sparing the trunk. Raynauds phenomenon is usually the rst symptom and is followed years later by skin thickening of the hands (sclerodactyly) as well as the other manifestations of the disease. Systemic involvement (e.g., lung brosis and kidney failure) tends to be less severe than in systemic sclerosis. Other associated conditions are pulmonary arterial hypertension, primary biliary cirrhosis and hypothyroidism. The presence of anti-centromere antibodies is a highly specic marker, though not very sensitive (50%). In contrast to those with systemic sclerosis, the prognosis is generally better for patients with CREST [7].

Many reports in the literature associate Raynauds syndrome with migraine [8, 9]. Compared to the general population, the prevalence of migraine is 24 times higher in patients with primary Raynauds syndrome. In addition, Raynauds syndrome appears to be more prevalent in patients with migraine [10]. There is also an increased prevalence of migraine in other diseases associated with Raynauds, namely, SLE [11].

The involvement of small vessels is considered to be a relevant factor in the physiopathology of Raynauds and CREST syndrome [7]. The mechanisms proposed to explain this involvement include an imbalance in

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vasomotor tone reactivity and changes in the architecture of the vessel walls, leading to obliterative brosis [7, 12]. These changes could sensitise the trigeminal vascular system, which is considered to play a major role in the physiopathology of migraine. Based on this we could speculate that the migraine attacks in our patient might be related to her CREST syndrome, although a casual association cannot be ruled out.

Acknowledgments The authors would like to thank Dr. Pablo A. Lpez, Dr. Francisco Paulin and Dr. Mara Elina Grecco for their collaboration. Dr. Figuerola is Senior Researcher from CONICET.

Conict of interest None.

References

1. Headache Classication Subcommittee of the International Headache Society (2004) The International Classication of Headache Disorders, 2nd edn. Cephalalgia 24 (Suppl 1):9160

2. Thomsen LL, Ducros A (2005) Sporadic and familial hemiplegic migraines. In: Olesen J, Tfelt-Hansen P, Goadsby P (eds) The

headaches, 3rd edn. Lippincott, Williams and Wilkins, Philadelphia, pp 5835853. Vetvik KR, Dahl M, Russell MB (2005) Symptomatic sporadic hemiplegic migraine. Cephalalgia 25:10931095

4. Thomsen LL, Olesen J (2004) Sporadic hemiplegic migraine. Cephalalgia 12:10161023

5. Thomsen LL, Eriksen M, Faerch S et al (2002) An epidemio-logical survey of hemiplegic migraine. Cephalalgia 5:361375

6. Bhatia R, Desai S, Tripathi M et al (2008) Sporadic hemiplegic migraine: report of a case with clinical and radiological features. J Headache Pain 6:385388

7. Varga, J (2008) Systemic sclerosis (scleroderma) and related disorders. In: Fauci A, Braunwald E, Kasper D et al (eds) Harrisons principles of internal medicine, 17th edn. McGrawHill, pp 20962106

8. Kaiser RS (1992) Raynauds disease in migraineurs: one entity or two? Headache 32(9):463465

9. Geraud G, Fabre N, Soulages X et al (1986) Migraine and the idiopathic Raynaud phenomenon. Rev Neurol (Paris) 142:638640

10. Zahavi I, Chagnac A, Hering R et al (1984) Prevalence of Raynauds phenomenon in patients with migraine. Arch Intern Med 144(4):742744

11. Bernatsky S, Pineau CA, Lee JL, Clarke AE (2006) Headache, Raynauds syndrome and receptor agonists in systemic lupus erythematosus. Lupus 12:671674

12. Gabrielli A, Awedimento EV, Krieg T (2009) Scleroderma. N Engl J Med 360(19):19892003

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