Moya Moya Disease. About a Case Report

Kristel Jesus Fatima Nunes. 6 th year student of Medicine. Stefani Jesus Betania Nunes. 2 nd year student of Medicine.

SUMMARY

Moya Moya disease is a cerebrovascular disease first described in 1957 in Japan, characterized by progressive occlusion of both internal carotid arteries or their branches and offset by the development of a collateral vascular network. It is prevalent in East Asia (Japan, Korea) and very rare in our country and the world. Its etiology is unknown, although studies have linked the pathogenesis of genetic and environmental factors. Pediatric usually manifests as transient ischemic attacks or stroke, while in adults is more frequent cerebral hemorrhage. The case of schoolgirl 10 years of age who presented with headache of high intensity and left brachial monoparesis and after neuroradiological studies were diagnosed with Moya Moya disease.

Keywords: Moya Moya disease, cerebral ischemia, cerebral angiography

SUMARY

Moya Moya disease is a cerebrovascular disease in 1957 Described first in Japan. It's characterized by progressive occlusion of internal carotid Arteries Both Their branch with compensatory or Development of collateral vascular network. It is Prevalent in East Asia (Japan, Korea) and very rare in our country and the rest of the world. It's etiology is unknown, although the pathogenesis associated studies to Have Genetic and Environmental factors. In children, it you cause stroke or transient ischemic Attacks, while in adults is more common cerebral hemorrhage. We present the case of a schoolgirl 10 years WHO presented with headache of high intensity and left brachial monoparesis and After neuroradiological studies Moya Moya disease WAS Diagne.

Key words: Moya Moya disease, ischemic attacks, brain angiography

INTRODUCTION

Moya Moya disease first described in 1957 by Takeuchi and Shimizu in two Japanese children and whose name comes from the angiographic image that resembles the curling cigarette smoke, is a vascular stenosis or occlusion is characterized by slow and progressive arteries forming the circle of Willis, and in particular the region of the supraclinoid internal carotid with a predominance of middle cerebral artery and anterior cerebral can cause signs and symptoms consistent with stroke or transient ischemic-type permanent . (1,2)

It occurs most frequently in children than in adults and in females, with an age distribution as bimodal with a peak rate around 5 or 6 years and another around the fourth decade of life (3), reporting a high prevalence in populations of East Asia, especially Japan and Korea, and more rarely in other parts of the world. (4)

Although it is not yet clearly the cause of the disease have been published genetic and environmental implications in the development of this disease, including abnormalities of chromosomes 3, 6 and 17, and exposure to optical radiation of gliomas or craniopharyngiomas. (5)

The symptoms in children and adolescents is due to ischemic stroke, transient or permanent motor deficit manifested by seizures, movement disorders, and visual language. Are more common in adults hemorrhage from ruptured aneurysms or of fine vessels of the collateral circulation. (6)

The diagnosis is based on clinical and magnetic resonance imaging and magnetic resonance angiography and digital angiography nuclear. Once diagnosed, treatment is surgical and it must be established early in order to revascularize the affected area and prevent the recurrence of ischemic events. (7)

We present the case of a schoolgirl 10 years of age, was admitted to the hospital after presenting intensive headache and left brachial paresis monkey and neuroimaging study which revealed the typical image of cigarette smoke rings at the base of the brain characteristic of the Moya-Moya disease.

PRESENTATION

School female 10 years old, born and resident in Caracas, the current disease that starts October 20, 2010, when physically present after high-intensity headache in the occipital region without acalmia, why go to the emergency Hospital Universitario de Caracas, where he entered with the following physical exam:

Blood pressure: 120/85 mmHg. Heart rate: 70 beats per minute Respiratory rate: 21 breaths per minute. Eye: isochoric pupils with non-reactive right pupil, amaurosis and right eyelid ptosis, and blurred vision left. Cardiopulmonary and abdominal unchanged. Neurological: conscious, Glasgow: 11/15 points (motor response: 5 points, ocular response: 3-point verbal response: 3 points), tendon reflexes present, with hyperreflexia. Mono left brachial paresis.

Daros laboratory: hemoglobin 12.4 g / dL, hematocrit 42 mL / dL, platelets 440000/mm3, 13000/mm3 WBC, neutrophils 75%, lymphocytes 16%, TP: 12 "100% TTP: 26.5", fibrinogen : 220 mg%, clotting time: 6'min., bleeding time: 1 min. Protein C: 105% activity, protein S: 100% activity, antithrombin III: 115% activity. Glucose 95 mg / dL, creatinine 0.75 mg / dL, C3, C4, CH50, ANA, antiphospholipid antiADN and unaltered. VDRL and HIV (-).

These results rule out more common etiologies of ischemic events in childhood such as infections, metabolic disorders, connective tissue diseases and hematological diseases

Computed tomography performed in plain skull admission cortical brain sample density hypo temporo-occipital right. Subsequently, cerebral angiography is indicated, that reports: right cortical infarct, occlusion of supraclinoid segment, A1 and M1 of both internal carotid and the presence of multiple proximal and distal side supplying them partially affected territories angiographic appearance of Moya Moya disease.

The school remains hospitalized until November 15, 2010, when it is transferred to the Maternal and Child Center (University Hospital), which is a center of high complexity, where the treatment is performed neurosurgical revascularization through Encefaloduroarteriosinangiosis technique, employing external carotid artery superficial temporal branch, as the donor artery.

DISCUSSION

Moya Moya disease is rare in Western countries, but Japan has a high prevalence and incidence of 0.3 per 100000 population being more frequent in children and females. The age and sex of our patient are consistent with those described in the literature. (2-5)

In 1979, the Ministry of Health of Japan established diagnostic criteria for moya moya disease, which are:

a) stenosis or occlusion of the intracerebral portion of the internal carotid artery, middle cerebral artery or anterior cerebral artery, b) abnormal vascularization around the stenosis; c) bilateral findings, d) absence of another cause.

The reported patient presented the clinical and paraclinical findings that coincide with those reported in the literature. (6, 7)

Approximately 60% of pediatric patients presented with focal seizures, associated with transient ischemic attacks or strokes, but a 6% presented headache, probably due to vasodilation of collateral vessels to stimulate meningeal nociceptors in the dura as our patient. (8)

The definitive diagnosis of Moya Moya disease is made by angiography, evidence shows the characteristic findings described by Suzuki and Takaku (2) and, depending on its stage, will serve to set the therapeutic strategy.

Suzuki and Takaku (1969) described the natural evolution angiography Moya Moya disease as a:

1. Bilateral stenosis of the internal carotid artery in its suprasellar portion. 2. Increased carotid stenosis. Moya Moya emerging at the base of the skull. 3. Prominent development of anastomotic vessels. Time of diagnosis of the disease in most patients. 4. Commitment of all the vessels of the circle of Willis



5. Development of neo-extracranial revascularization. 6. Irrigation of the cerebral hemispheres from intraextracraneal anastomosis. (1, 2) The reported patient was in stage 3. Following extensive cerebral infarction, the possibility of permanent neurologic sequelae is high, so treatment should be installed early. Present because no pharmacological treatment exists to reverse or prevent progression of the disease, surgical treatment is recommended in patients with cerebral ischemic events, progressive or repeated, which aim to stop cerebral ischemic injury by increasing collateral blood flow to hypoperfused areas of the cerebral cortex, using the external carotid circulation as a donor.

In the case of our patient, surgical treatment was encefaloduroarteriosinangiosis was used, which is an indirect surgical technique in which dissects the superficial temporal artery, and sutured to the cut edge of the dura.

Bibliography? A

1. Takeuchi K, Shimizu K. Bilateral internal carotid Hypogenesis of arteries. No to Shinkei 1957; 9:37-43 2. Suzuki J, Takaku A. Cerebrovascular "Moya-Moya" Disease. Disease Showing Abnormal Net-Like Vessels in Base of Brain. Arch Neurol. 1969, 20:288-299. 3. Marin L. Quintana 20 years experience in the management of Moya-Moya disease. Rev Chil neurocan. 2004, 23:30-36. 4. Gonzalez G, Russo ME, Campistol J, Costa G, Navarro Balbuena R, R Crosa et al. Moya-Moya in non-Asian child population. Expansion of casuistry. Arch Pediatr Urug. 2008, 79 (4) :291-302. 5. Ajler P M, Brocanelli M A, G S. Ajler Moya-Moya disease: a case report. Rev Arg neurocan. 2005, 19:146-148. 6. Uchino KI, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington State and California. Neurology. 2005, 65: 956-8. 7. Kuroda S, Nanba R, Ishikawa T. Clinical manifestation of infantile moyamoya disease. No Shinkei Geka. 2003, 31: 1073-8. 8. ER Smith MD, MD Scott RM: Surgical management of Moyamoya syndrome, Skull base and interdisciplinary approach 2005, 15 (1) :15-26.



Source: medical portals.

Article published in: doctors today