We present in this article a twelve -year- old girl with generalized chorea and chronic migraine headache. Her examination showed generalized chorea with hypotonia. Brain magnetic resonance images showed multiple old ischemic strokes in the frontal and occipital regions, with flow void appearance in the basal ganglia region. Brain magnetic resonance angiography showed collateral channels at the base of the brain with narrowing of the arteries of circle of Willis which the classic picture of Moyamoya disease .
Key words: Moyamoya Disease ,chorea, stroke .
Arabic Abstract :
الحركة اللاإراديه الرقصيه كعرض لمرض المويامويا
في هذه الورقة نعرض حالة طفلة تبلغ من العمر اثنا عشر عامًا تعاني من حركات رقصيه، وصداع الشقيقة
المزمن ، ولقد أجريت لها أشعة الرنين المغناطيسي للدماغ والشرايين ، فاتضح وجود جلطات متعددة في الدماغ
وضيق في الشرايين ، ممَّا أثبت تشخيص مرض المويامويا .
Moyamoya disease is a chronic cerebrovascular disease characterized by severe
bilateral stenosis, or occlusion of the arteries around the circle of Willis with prominent collateral circulation [1,2]. Involuntary movement disorder including chorea is not a common clinical presentation in Moyamoya disease .
This case report describes a child presented with chorea and chronic migraine headache diagnosed as Moyamoya disease. It also includes a review of literature related to similar cases.
Case report :
A twelve- year-old girl presented to the emergency department with a history of abnormal movements for a five-months duration. The abnormal movements involved the upper limbs, lower limbs and trunk. She confirmed a history of slurred speech, Gait difficulty with frequent falling attacks and numbness on the right side of the body. The symptoms initially were fluctuating and then became progressive. There was no history of other types of abnormal movements. she denied any history of loss of consciousness, convulsion, memory disturbance, or symptoms of cranial nerves involvement. She is a product of normal delivery with normal natal, antenatal, and post natal history, She had one attack of febrile convulsion when she was four year old . At the age of six years, she started to have a migraine headache with aura once a month and this was sometimes complicated by transient right hemiparesis. She had a history of frequent tonsillitis. There was not history of rheumatic heart disease, skin rash, psychological problems, joint pain or jaundice. She denied any drug ingestion apart from Propanolol for migraine headache. She is an intermediate school student with good school performance. She lives in Tabook. There was not history of travel abroad. No family history of similar illness. Her mother and father are first degree relatives .On examination, she was afebrile with normal blood pressure, respiratory rate and pulse rate. Cardiac, chest, gastrointestinal, rheumatological systems examination was unremarkable, Neurological examination revealed normal higher functions. She was dysarthric with no cranial nerves involvement. Motor examination revealed restless movements which involve upper limbs, lower limbs and trunk ,the movements were rapid, jerky, irregular, low amplitude and flit from one part of the body to another which is typical of chorea, there was weakness in the right side of grade 4/5, hyptonia , normal deep tendon reflexes and the plantar responses were down going bilaterally. Her gait was unsteady. Finger to nose test revealed no dysmetria, although there were involuntary movements during the maneuver.
The following investigations were done to rule out any possible causes of chorea which included thyroid function ,antistreptolysin O titer , throat culture, lactate ,pyruvate ,ceruplasmin level , erythrocyte sedimentation rate , C reactive protein, anti-nuclear antibodies, anti -DNA titers, anticardiolipin ,alpha fetoprotein ,echocardiogram and blood film. All were within normal limits. The electroencephalogram showed sharp activity over the left frontotemporal area that spread to right side occasionally. Cranial MRI revealed right frontal and occipital region encephalomalacia, which may indicates old ischemic stroke also showed flow void appearance around the basal ganglia. Further evaluation by magnetic resonance angiography demonstrated collateral channels at the base of the brain with stenosis of the arteries around circle of Willis which is typical of Moyamoya disease( see figure 1and 2). The following laboratory tests were done to rule out any other possible causes of ischemic stroke ;Protein C and S ,homocyteine, anti thrombin III level , Factor IV Leiden mutation, sickle cell screen ,all were within normal limits.
After we reached the diagnosis of Moyamoya disease, the patient was started on antiplatelets aspirin 81 mg once per day and haloperidol 1.5 mg twice per day ,next follow up she showed mild improvement but still she had gait difficulty, she was referred to neurosurgery for possible surgical intervention ,but she did not come back
Moyamoya disease was described initially in Japan in the sixties as (wavering puff of smoke ). It was believed to be restricted only to Japan however there have now been reports from various parts of the world.
Moyamoya disease is a clinical entity characterized by bilateral occlusion of the internal carotid arteries and the development of collateral arteries. The etiology
of Moyamoya disease has not been clarified. Family history of Moyamoya disease was apparent in 10% of patients in Japan.  It can occur as a primary disease or as syndrome in association with a variety of conditions(e.g. neurofibromatosis, Down syndrome, vascuilitis, radiation, sickle cell anemia, congenital heart disease, type 1 glycogenosis), yet its pathogenesis remains unexplained. Histopathological studies revealed stenosis and occlusion of the main arterial trunk, usually due to fibro cellular thickening of intima [5,6]. The diagnostic criteria classify the adult cases displaying bilateral occlusive lesion as definitive Moyamoya disease and those exhibiting unilateral involvement as a probable Moyamoya disease[5,7]. The highest incidence of this disease is in the first decade of life. Initial symptoms manifest as transient ischemic attack , cerebral infarction ,and intracerebral hemorrhage , or occasional seizure. An ischemic event is the usual clinical presentation in childhood Moyamoya disease . Whereas the hemorrhagic stroke is more common in adulthood, chorea has rarely been described. The episodic symptoms of Moyamoya disease may be precipitated by hyperventilation and changes in body temperature .
Several medical treatments (anticoagulation, antiplatlet drugs and corticosteroid) have been tried without benefit. Surgical therapy for Moyamoya disease is recommended in most of the patients , particularly ,in light of the ineffectiveness of medical therapy. Surgical treatment is intended to restore the circulation for the ischemic brain area to prevent ischemic stroke and TIA . Surgical therapy of Moyamoya disease can be categorized as: direct revascularization (superficial temporal artery to middle cerebral artery by pass or, middle meningeal artery bypass) and indirect revascularization (encephaloduroarteriosyangiosis -EDA,
Encephalomoysynangiosis EMO, enceohaloarteriosynangiosisESA ,duraplexy and
surgery (STA-MCA by pass with EDAMS) for pediatric Moyamoya disease was
effective in reducing the risk of post operative ischemic attacks compared with
indirect surgery .
Chorea is uncommon abnormal movement, the term chorea is derived from the Greek chorea, meaning " dance". This condition consists of irregular, unpredictable ,brief and jerky movements that flit from one part of the body to another in a random sequence .It can be seen in a wide range of disorders These disorders are classified as follows : inherited disorders, drug induced diseases, systemic diseases ,rheumatic diseases ,vascular-related diseases ,tumor related disorders and other rare causes. Chorea is a comparatively rare clinical feature of Moyamoya disease and syndrome . It has been suggested that 6% of Moyamoya disease have chorea[12,13]. Other types of abnormal movements have been described in patients with Moyamoya disease. Choreitic movement in this disease usually wax and wane and are often triggered by excitement ,emotion, strain or crying. They may be unilateral or bilateral and often appear alternatively in the left and right side.
The pathophysiology of chorea is related to basal ganglia hypoperfusion which is explained well by marked improvement after by pass surgery of Moyamoya disease .
In the English language literature we found 21 case reports describing cases of Moyamoya disease with initial presentation of chorea[3,8,11,12,13,14,16,17,18,19,20,21,22,25,26,27] . We made a summary of these case reports, comparing the age, sex and race (see table 1). The age of onset was variable from one year to fifty five years. The sex distribution showed female predominance with 76%, most of the patients were Japanese
Regarding the issue of migraine headache in our case is not clear if it is related to Moyamoya disease or not, the history of transient right side weakness during some of migraine headache attacks raised the possibility of hemiplegic migraine,but transient hemiparesis can be a picture of Moyamoya disease ,In English language literatures we found four cases of moyamoya disease have been reported with initial presentation of migraine like headache[4,15,23,24] , the age onset was variable from (4 - 49years) , all of them were females.
Association of migraine and Moyamoya disease, although existing, is infrequent . Therefore, the point of contention over whether it is worth screening patients with migraine headaches for Moyamoya disease must be addressed.
Finally , as we noticed in the previous reviews, chorea as presentation of Moyamoya disease is uncommon but existing. Accordingly, Moyamoya disease should be included in the differential diagnosis of patients presenting with chorea for possible early intervention in order to prevent further disability.
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Figure 1: magnetic resonance angiogram of the brain, lateral view demonstrates stenosis around the circle of Willis(short arrows) with development of collateral vessels(long arrows) which is a classic of Moyamoya disease.
Figure 2:magnetic resonance angiogram of the brain,posterior view demenostrates stenosis around the circle of willis (short arrows)with prominent collateral circulation(long arrows).
Table 1:summary of the reported cases of Moyamoya disease with chorea presentation :