MOYAMOYA DISEASE

1
MOYAMOYA DISEASE

• BS. CK1. DANG NGOC DUNG

2
INTRODUCTION

• Moyamoya disease is a unique chronic progressive
  cerebrovascular disease characterized by
  bilateral stenosis or occlusion of the arteries
  around the circle of willis with prominent
  arterial collateral circulation
• The annual incidence of MM is 0.35 to 0.94 per
  100.000 population. the prevalence of MM is 3.2
  to 10.5 per 100.000 population (Japan)
• Asian 0.28 per 100.000 population
• Age distribution in child 10 14 y.old
• Male/Female 1/1.8 to 1/2.2

3
INTRODUCTION

• Moyamoya disease was first described in Japan in
  1957 (Suzuki)
• Many similar cases have subsequently been
  reported, mainly in Japan and other Asian
  countries. the disease is found less frequently
  in North America and Europe

4
ETIOLOGY

• Unknown
• Familial occurrence of approximately 10 of cases
• Familial Mm.D has been linked to chromosomes
  3P24.2-P26, 6Q25, 8Q23, 12P12, and 17Q25

5
CLINICAL FEATURES

• Transient ischemic attack (TIA)
• Ischemic stroke
• Hemorrhagic stroke
• Epilepsy
• In children, symptomatic episodes of ischemia may
  be triggered by exercise, crying, coughing,
  straining, fever or hyperventilation

6
NEUROIMAGING

• CT scan infarction may involve cortical and
  subcortical regions. In the patients with
  parenchymal hemorrhage, cranial CT usually show a
  high density area indicating blood in the basal
  ganglia, thalamus and/or ventricular system
• CTA can also demonstrate the abnormal vessels of
  MM.D, including the collateral MM vessels in the
  basal ganglia
• MRI acute ischemic brain lesions, in some cases,
  dilated collateral vessels
• MRA, DSA

7
DIAGNOSIS

• The diagnosis of MM.D is based upon the
  characteristic angiographic appearance of
  bilateral stenoses affecting the distal internal
  carotid arteries proximal circle of willis
  vessels, along with the presence of prominent
  basal collateral vessels

8
ACUTE MANAGEMENT

• Acute management is mainly symtomatic and
  directed towards reducing elevated intracranial
  pressure, improving cerebral blood flow, and
  controlling seizures. In patients with
  intracerebral hemorrhage, ventricular drainage,
  and/or hematoma, removal is often required.

9
SECONDARY PREVENTION

• There is no curative treatment for MM.D
• Secondary prevention for patients with
  symptomatic MM syndrome is largely centered on
  surgical revascularization techniques

10
(No Transcript)
11
(No Transcript)
12
(No Transcript)
13
(No Transcript)
14
(No Transcript)
15
(No Transcript)
16
THANK YOU