OBJECTIVES At the end of this presentation, students should be able to Define Moyamoya disease and its epidemiology Causes of moyamoya disease Symptoms and signs of moyamoya disease How to Assess the patients with Moyamoya disease Diagnosis of moyamoya disease Treatments and management of patient with moyamoya disease Conclusion Reference
INTRODUCTION Moyamoya disease is the rare, chronic and progressive cerebrovascular disease characterized by stenosis or occlusion of internal carotid arteries at the base of the skull including its proximal branches. To compensate for the narrowing arteries, the brain create collateral blood vessels in an attempt to deliver oxygen-rich blood to the areas of brain. these tiny collateral vessels when seen on angiogram have hazy, firm appearance.
INTRO........... the Japanese were first to describe the condition and they named it MOYAMOYA which means puff of smoke Moyamoya collaterals are more fragile than normal blood vessels so they can break and bleed into brain causing hemorrhage. Moyamoya usually affects both sides of the brain and is accompanied by brain aneurysms.
EPIDEMILOGY OF MOYAMOYA DISEASE The highest number of cases are seen in East Asia, with more women affected than men. The incidence of Moyamoya disease is 0.94 and 2.3 per 100,000 individuals in Japan and South Korea respectively. The disease is mostly diagnosed in children aged 5 to 10 and in adult aged 30 to 50.
CAUSES OF MOYAMOYA DISEASE The exact cause of moyamoya disease is unknown, inheritance{family history} may play a role. Medical conditions include sickle cell anemia, Down syndrome and other genetic disorders, also Autoimmune disease such as Graves disease and systematic lupus erythematosus has been associated with an elevated risk of moyamoya disease.
CONT................ Moyamoya-like changes can also occur in patients who have undergone radiation or radiotherapy to the head and neck such as in treatment of pituitary tumors and craniopharyngiomas.
SYMPTOMS AND SIGNS OF MOYAMOYA DISEASE Ischemic stroke Hemorrhagic stroke Transient ischemic stroke[mini-stroke] Seizures Cognitive or learning impairment Headache
PHYSICAL ASSESSMENT Physical assessment begins with the general appearance the nurse assess the skin of the patient, level of consciousness, distress level and breathing of the patient. Basing on patient with moyamoya disease the following are features expected to be observed when the nurse use tools of inspection, palpation, auscultation and percussion
INSPECTION General appearance of patient with moyamoya disease are Enlarged eyes: the eyes may appear larger than normal due to increased pressure in the brain caused by narrowed or blocked blood vessels. It can lead to vision problem and even blindness if left untreated. Droopy eyelids: the eyelids may appear droopy or puffy due to the swelling of the tissue around the eye
INSPECTION Weakness or paralysis of the limbs: patient may experience weakness or paralysis of the arms and legs which can worsen over time if left untreated. Seizures: are common complication of moyamoya disease due to abnormal blood flow to the brain
PALPATION Palpation should be performed over the carotid artery and their branches in the neck and head. The nurse should feel for any areas of decreased or absent pulse as well as any areas of tenderness or swelling. Palpation should be performed on both sides of the neck and head, pay attention to any sensation of stenosis or occlusion such as feeling of resistance.
AUSCULTATION On listening with stethoscope to patient with moyamoya disease to various areas of the chest, including but not limited to pericardium and lung fields. By carefully listening to abnormal sounds like murmurs or gallops and changes in the heart and lung function.
PERCUSSION Percussion can be used to evaluate for signs of increased intracranial pressure(ICP) and potential neurological deficits
DIAGNOSIS OF MOYAMOYA DISEASE Magnetic resonance imaging[MRI]. this test uses magnetic field and radio waves to produce detailed picture of the brain. Magnetic resonance angiogram[MRA]. This test uses magnetic field and pulses of radio waves energy CT scan. it uses special x-rays equipment to create images of internal organ.
TREATMENT AND MANAGEMENT OF PATIENTS WITH MOYAMOYA DISEASE There are no medications capable of reversing the artery narrowing in moyamoya disease. Instead treatment focuses on reducing the risks of repeated strokes through arterial bypass or creation of a new blood supply to the affected areas of the brain. MEDICATIONS For those experiencing strokes and transient ischemic attack(TIA) can be given
CONT…………………………………………… Anticoagulants to prevent blood clots and reduce risk of stroke example Aspirin. Antiplatelet agents to prevent aggregation and reduce risk of stroke. Blood pressure-lowering medications to reduce the risk of stroke and slow the progression of disease. Seizure medication to control seizures
CONT……….. Cognitive and behavioral therapy to improve cognitive function and quality of life. SURGICAL INTERVENTION Surgery is generally recommended for moyamoya patients with recurrent or progressive TIA or stroke example Revascularization procedures to restore blood flow to the brain. The procedure can be direct or indirect.
CONT……………… Endovascular stenting or bypass surgery to improve blood flow to the brain. Brain tissue removal to relieve pressure and improve symptoms. LIFESYTLE MODIFICATION Regular exercise to improve overall health and reduce risk of complications
CONCLUSION Moyamoya disease is rare and complex neurological disorder that affect internal carotid artery and its branches. Early diagnosis and treatment of the disease are critical to prevent long-term cognitive decline and improve outcomes.
REFERENCE https://www.ninds.nih.gov.Disorders//All-Disorders/Mayomayo-Disease-Information-Page Suzuki J, Takatu A. Cerebrovascular " moyamoya ” disease. Disease showing abnormal net-like vessels in base of brain. AMERICAN HEART ASSOCIATION