Moya moya disease

Moyamoya disease (MS) is a vascular disease characterized by a progressive stenosis (until occlusion) of the terminal portion of both internal carotid arteries, which may include anterior cerebral artery (ACA) and medium (ACM) associated an abnormal vascular network called moyamoya vessels. It is a rare disease in children, so the aim of this publication is to put it in mind as a differential diagnosis in boxes origenvascular symptomatology, guiding the mind-directed study to your search. This becomes more important when you consider that is a progressive disease, which diagnosed early, can monitor your progress and avoid clinical deterioration that can be disabling, or even fatal consequences.

Takaku4 Susuki and in 1969 nominated this clinical entity, "smoke volcanoes" in Japanese, moyamoya, due to the appearance with angiographic images. Its etiology is still unknown, although epidemiological studies suggest genetic factors in its pathogenesis.

In children it is usually presented as transient ischemic attacks and strokes, with progression can be slow, with intermittent events, or fulminant with rapid neurological compromise. Diagnosis is based on clinical and radiological findings that show the characteristic internal carotid artery stenosis with abundant collateral vessels. The evolution without treatment is inevitably progression and clinical vascular compromise.

Cerebral revascularization surgery improves long-term prognosis, the various techniques described are intended to prevent ischemic damage greater increase collateral blood flow through the external carotid.

Below we describe our experience with two patients who were diagnosed with MS in Pediatric Critical Patient Unit of Clinica Santa Maria (UPCP-CSM) between January 1998 and December 2007. During this period have graduated 17 patients with stroke brain (AVE), a total of 5864 hospitalized patients, MS corresponding to 11% of stroke in patients between 15 days and 17 years, with an incidence of 0.2% per center per year.

Its etiology has not yet been clarified; There is growing evidence of the involvement of a genetic factor. It has been linked to several different genes located on chromosomes. D353050 reference to markers located in the 3p24.2-268 chromosome, D175939 chromosome 17q25, also in other parts of the same chromosome region contained in the 9-CM, D 175,785 to D1758369 is made, and on chromosome 6 in D6S44110. Polygenic inheritance autosomal dominant pathway low penetrancia11 is proposed. The family presentation, although it has been reported mainly in Japanese population, cases have also been reported in other parts of the world.12. The description of a G / C heterozygous genotype in position -418 in the promoter of tissue inhibitor of metalloproteinase 2 (TIMP2) could be a genetic factor predisposing to familial MS

Several studies of clinical, laboratory and pathological have tried to define its pathogenesis. It described an intimate thickened HERE samples of patients with moyamoya, techniques immune reaction suggestive of overexpression of factor-1 (alpha), inducing hypoxia and endoglinas in the intima of the ACM. Along with the aforementioned genetic factors, environmental factors also play a role, as suggested by Ulrich, who reports that 3.5% of children who had received radiotherapy for primary brain tumors, especially in the vicinity of the circle of Willis, developed MS in the follow-up, which was related more to those with type 1 neurofibromatosis and those receiving more than 5000 cGy of radiation. Other studies have been directed to seek soluble adhesion molecules in cerebrospinal fluid (CSF) of patients, suggesting an inflammatory process in the etiology of this enigmatic disease, that would be supported by the description of an MS by angiography in a 20- years, eight months after submitting a pneumococcal meningitis. It has also been associated with apoptosis, evidenced by the detection of activated caspase-3 (for technical immunoreaction) in the tunica media of the ACM with thinning of the vessel wall and the description of a high proportion of endothelial anticélulas serum antibody MS patients. On the other hand, publications that report the persistent trigeminal artery and the artery Bernasconi-Cassinari, both embryonic arteries, allowing suspect an early onset of the disease. 

In my personal opinion Moyamoya disease is a disorder that occurs in children, and causes various problems in the child's brain.

it is important that we know as nurses provide care to patients who suffer because it is very difficult to bring the symptoms for both parents and for the child.

Recovered of http://www.elenciclopedista.com.ar/enfermedad-de-moyamoya/ on 20th november, at 18:40 

By Rebeca Guzmàn