Attempt to establish an experimental animal model of moyamoya disease using immuno-embolic material--histological changes of the arterial wall resulting from immunological reaction in cats.

In this study, we investigated the relationship between intimal thickening of the internal carotid artery (ICA) and immunological reaction, and between occlusion of the ICA and development of basal collateral vessels in moyamoya disease. Rod-shaped lactic acid-glycolic acid copolymer (LGA-50) and N-acetylmuramyl-L-alanyl-D-isoglutamine (muramyl dipeptide: MDP), and immuno-embolic material, were injected into cats unilaterally via the common carotid artery. Histological changes of duplication of the internal elastic lamina could be seen mainly in the terminal portion of the ICA in the animals injected with rod-shaped LGA-50 containing MDP. No angiographic changes were seen in any of the animals. These findings suggest that the immunological reaction induced by MDP caused histological changes in the intima of the ICA similar to those observed in moyamoya disease. This experimental study, however, could not clarify the development of the basal collateral vessels.</p

GEN-O-MA project: an Italian network studying clinical course and pathogenic pathways of moyamoya disease—study protocol and preliminary results

Background: GENetics of mOyaMoyA (GEN-O-MA) project is a multicenter observational study implemented in Italy aimed at creating a network of centers involved in moyamoya angiopathy (MA) care and research and at collecting a large series and bio-repository of MA patients, finally aimed at describing the disease phenotype and clinical course as well as at identifying biological or cellular markers for disease progression. The present paper resumes the most important study methodological issues and preliminary results. Methods: Nineteen centers are participating to the study. Patients with both bilateral and unilateral radiologically defined MA are included in the study. For each patient, detailed demographic and clinical as well as neuroimaging data are being collected. When available, biological samples (blood, DNA, CSF, middle cerebral artery samples) are being also collected for biological and cellular studies. Results: Ninety-eight patients (age of onset mean ± SD 35.5 ± 19.6 years; 68.4% females) have been collected so far. 65.3% of patients presented ischemic (50%) and haemorrhagic (15.3%) stroke. A higher female predominance concomitantly with a similar age of onset and clinical features to what was reported in previous studies on Western patients has been confirmed. Conclusion: An accurate and detailed clinical and neuroimaging classification represents the best strategy to provide the characterization of the disease phenotype and clinical course. The collection of a large number of biological samples will permit the identification of biological markers and genetic factors associated with the disease susceptibility in Italy

Intermittent Hemiplegia in a Boy with Primary Moyamoya Disease: A Case Report from Iran

How to Cite This Article: Bidaki R, Zarepur E. Intermittent Hemiplegia in A Boy With Primary Moyamoya Disease; A Case Report from Iran. Iran J Child Neurol. Spring 2017; 11(2):65-68. AbstractMoyamoya is a rare chronic progressive occlusive cerebrovascular disease. Its manifestation varies from stroke, progressive learning impairment and transient ischemic attack to headache and seizure. There is no accepted medical treatment and surgery usually, is needed. We report here a case of 8 yr old boy referred to psychiatrist outpatient. An eight yr old boy with intermittent hemiplegia was brought to Imam Ali Clinic, Yazd, Iran in 2015 because his headache and medical problem began from 6 yr old. Stress and excitement exacerbated his condition. His first attack was at the age of 6 yr old. During attack, he had incontinence, severe headache, alogia, pallor, claudication and left hemiplegia (Left lower limb). Magnetic resonance angiography (MRA) was done and our diagnosis was moyamoya disease. Moyamoya is a mysterious disease and psychiatrists should consider it in differential diagnosis of alogia and plegia. Acute management of this disease is mainly symptomatic. Nowadays, surgery is a good choice and early diagnosis of this disease can change our patient’s life. References 1. Hallemeier CL, Rich KM, Grubb RL, Chicoine MR, Moran CJ, Cross DT, et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 2006;37(6):1490-6.2. Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20(3):288-99.3. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, et al. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 1997;99 Suppl 2:S1-5.4. Duan L, Bao XY, Yang WZ, Shi WC, Li DS, Zhang ZS, et al. Moyamoya disease in China: its clinical features and outcomes. Stroke 2012;43(1):56-60.5. Im SH, Cho CB, Joo WI, Chough CK, Park HK, Lee KJ, et al. Prevalence and epidemiological features of moyamoya disease in Korea. J Cerebrovasc Endovasc Neurosurg 2012;14(2):75-8.6. Lutterman J, Scott M, Nass R, Geva T. Moyamoya syndrome associated with congenital heart disease. Pediatrics 1998;101(1 Pt 1):57-60.7. Peerless SJ. Risk factors of moyamoya disease in Canada and the USA. Clin Neurol Neurosurg 1997;99 Suppl 2:S45-8.8. Abuzayed B, Khreisat W, Maaiah W, Agailat S. Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult. J Neurosci Rural Pract 2014;5(2):176-9.9. Yamauchi T, Houkin K, Tada M, Abe H. Familial occurrence of moyamoya disease. Clin Neurol Neurosurg 1997;99 Suppl 2:S162-7.10. Kim JS. Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis. J Stroke 2016;18(1):2-11.11. Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base 2005;15(1):15-26.12. Yamada I, Suzuki S, Matsushima Y. Moyamoya disease: comparison of assessment with MR angiography and MR imaging versus conventional angiography. Radiology 1995;196(1):211-8.13. Nakakita K, Tanaka S, Fukuda A, Fujii C, Kohama A, Miyasato H. [Nontraumatic acute subdural hematoma caused by the rupture of transdural anastomotic vessels in moyamoya disease]. No Shinkei Geka 1994;22(6):561-5.14. Mughal DK, Nissirios KS, Puri MR. A 30-Year-Old Female with Moyamoya Disease and Associated Depression. Psychiatr Ann 2014;44(12):549-551.15. Kim T, Oh CW, Bang JS, Kim JE, Cho WS. Moyamoya Disease: Treatment and Outcomes. J Stroke 2016;18(1):21-30.

Experimental study of the pathogenesis of moyamoya disease: histological changes in the arterial wall caused by immunological reactions in monkeys.

Moyamoya disease is a progressive vascular disorder of unknown etiology. Theories of inflammatory and immunologic mechanisms have been proposed as the pathogeneses. We have designed a new method of administering N-acetylmuramyl-L-alanyl-D-isoglutamine (MDP) for experimental induction of moyamoya disease using an intravascular interventional technique combined with rod-shaped embolic materials made from lactic acid-glycolic acid copolymer. The embolic materials containing MDP were repeatedly injected into the right internal carotid artery of monkeys in the embolic group. Intravenous injections of MDP solution alone were performed in the intravenous group. Histological examination of the arteries demonstrated reduplication and lamination of the internal elastic laminae, which corresponded with findings of moyamoya disease in both groups. These histological changes occurred not only in the intracranial arteries on the embolization side, but also in the contralateral intracranial and even extracranial arteries. The changes were more prominent in the intravenous group than in the embolic group. We conclude that the systemic humoral factors induced by MDP in this study may be important in the pathogeneses of moyamoya disease. Our observations suggest that moyamoya disease is a systemic vascular disease and has an etiologic factor affecting both intracranial and extracranial arteries</p

Moyamoya Disease, a Rare Cause of Recurrent Strokes in an African Sickle Cell Child: Does hydroxyurea have a Role in this Context?

Background: Neurological complications are a significant cause of morbidity and mortality in sickle cell patients with reported incidence of stroke in Africa as high as 1·3/100 patient per year [1,2]. There is an association between sickle cell disease (as well as other hemoglobinopathies) and Moyamoya disease [3]. Data on the occurrence of this condition in African sickle patient are scare. Likewise the role of hydroxyurea among patients with both sickle cell anemia and Moyamoya disease in preventing stroke has not yet been studied in Africa. Case presentation: In the present report, we describe an African child who had a recurrent stroke. She was later diagnosed as having Moyamoya disease while already receiving hydroxyurea. Conclusion: Moyamoya disease is a rare condition associated with recurrent stroke in African sickle children. The role of hydroxyurea in this context is still unclear

A Case of Moyamoya Disease in a Girl with Thyrotoxicosis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea

Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome.

Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS), in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness) at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA). Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease

Moyamoya disease: an elusive diagnosis

Moyamoya disease is an idiopathic vasculopathy, affecting vessels of Circle of Willis.1 It usually manifests as stroke, but can also cause seizures and cognitive impairment.2 Ischemic strokes are common in children and hemorrhagic strokes in adults.1 We describe our experience with moyamoya disease in four patients who presented with ischemic strokes, at an academic tertiary care center and emphasize that this diagnosis should be considered in young patients, especially children, who present with stroke

Clinical and radio-angiographic features of paediatric moyamoya disease in Bangladesh

Background: Moyamoya disease is a cerebrovascular arteriopathy of unknown origin characterized by progressive stenosis followed by occlusion of the cerebral arteries. Studies on moyamoya disease, especially in children in Bangladesh, are rare. We aimed to determine the clinical and neuroimaging features of moyamoya disease, particularly angiographic features. Methods: Forty children diagnosed with moyamoya disease were consecutively recruited from Bangabandhu Sheikh Mujib Medical University Hospital, Dhaka, Bangladesh. Each patient underwent a medical history and physical examination focusing on stroke, magnetic resonance imaging, and magnetic resonance angiography scans of the brain. In some instances, electroencephalogram and digital subtraction angiography were also performed. Results: Of the 40 patients, 22 experienced their first-ever stroke (median age, 84 months), and 18 had recurrent strokes (median age, 90 months). Common symptoms included hemiparesis, headache, seizure, and speech disorder. The commonly affected vessels were the internal carotid and middle cerebral arteries. Cortical involvement was found in 82.5% of cases. Bilateral involvement was observed in 37.5% of the patients, most of whom were in the Suzuki stage III. Conclusion: Hemiparesis, headache, seizure, and speech disorder were the common manifestations. Most patients reported late (Suzuki stages III and IV), indicating an advanced stage.  Early detection is necessary, considering the severity of the disease and its inherent tendency for recurrence.