MOYA MOYA DISEASE

MOYA MOYA DISEASE ---- A CASE REPORT DR AMIT KUMAR GHOSH NEUROSURGEON Institute of Neuroscience,Kolkata: 

MOYA MOYA DISEASE ---- A CASE REPORT DR AMIT KUMAR GHOSH NEUROSURGEON Institute of Neuroscience,Kolkata -

CLINICAL PRESENTATION : 

CLINICAL PRESENTATION 4 years, male child with normal developmental milestones Recurrent ischaemic strokes---- right sided weakness with slurring of speech on April and October 2007 -- treated elsewhere Recovered completely, but not investigated CT scan of brain was done on April 2007 : small left MCA infarct On January 2008, he presented with left sided weakness and slurring of speech

EXAMINATION : 

EXAMINATION General examination –normal Neurological examination – conscious, aphasic left UMN 7 th nerve paresis left hemiparetic (grade 2)

PowerPoint Presentation: 

LEFT COMMON CAROTID INJECTION ACA MCA PUFF OF SMOKE ACA MCA ICA ECA THINNED OUT INCRACRANIAL VESSELS WITH PUFF OF SMOKE COLLATERALS

PowerPoint Presentation: 

LEFT VERTEBRAL INJECTION

PowerPoint Presentation: 

RIGHT CAROTID INJECTION ACA MCA ACA MCA THINNED OUT INCRACRANIAL VESSELS WITH PUFF OF SMOKE COLLATERALS

TREATMENT : 

TREATMENT MANAGEMENT: MRI brain not possible due to financial constraints Initially Aspirin, physiotherapy given BILATERAL Enchephalo-duro-myo-arterio-synangiosis was done on 25/01/08

PowerPoint Presentation: 

LEFT TEMPORAL CRANIOTOMY AND ENCEPHALO- MYO-DURO- ARTERIAL SYNANGIOSIS PERFORMED

-: 

- Post operatively Child improved neurologically and at discharge was independently ambulant

PowerPoint Presentation: 

Moyamoya disease is an extremely rare disorder in most parts of the world except in Japan, where the overall incidence is higher (0.35 per 100,000). The pathogenesis of Moyamoya disease is unknown Moyamoya disease is characterized by progressive intracranial vascular stenoses of the circle of Willis LITERATURE REVIEW

PowerPoint Presentation: 

It is a disease that tends to affect children and adults in the third to fourth decades of life. In children it tends to cause strokes or seizures. In adults it tends to cause bleeding or strokes. The clinical features are cerebral ischaemia (strokes), recurrent transient ischaemic attacks,haemorrhage, convulsions and/or migraine-like headaches.

PowerPoint Presentation: 

The diagnosis is initially suggested by CT, MRI, or angiogram . The name derives from its angiographic image-- the "puff of smoke," refers to the appearance of multiple collateral vessels seen on angiography.

PowerPoint Presentation: 

There is no Medical treatment for Moya Moya disease . The role of Aspirin: thought by some to reduce the incidence of microemboli from the micro-collaterals Surgery is the Mainstay of treatment to prevent furthur Ischemic and Hemorrhagic events

PowerPoint Presentation: 

Several surgical approaches have been suggested in treating moyamoya disease These approaches are generally divided into two groups: Direct Revascularisation - STA-MCA anastomosis Indirect Revascularisation- -- EDAMS (encephalo-duro-arterio-myo-synangiosis) EMS (encephalomyosynangiosis), EDAS (encephaloduroarteriosynangiosis), multiple burr holes procedure Superior cervical ganglionectomy Cervical pericarotid sympathectomy

PowerPoint Presentation: 

With the direct STA-MCA surgery, increased blood supply is immediate After the in-direct EDAMS, EDAS, EMS, and multiple burr holes surgeries, symptoms may seem to improve almost immediately and it will take probably 6-12 months before new vessels (blood supply) can develop sufficiently. Post-operative angiography is best to be done between 6 months and 1 year The development of EDAMS was an attempt to increase collateral circulation from both the middle meningeal artery and the superficial temporal artery

PowerPoint Presentation: 

A definite conclusion about whether to choose EDAMS or another procedure to treat moyamoya disease is unclear . Clinical results of EDAMS show a greater reduction of the ischemic attacks in moyamoya disease, and angiograms taken after surgery show extensive collateral vessels on the ischemic brain. Therefore, EDAMS seems to be one of the more effective treatments for moyamoya disease.

PowerPoint Presentation: 

References Kuriyama S, Kusaka Y, Fujimura M, et al (2008). "Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey". Stroke 39 (1): 42–7. Wakai K, Tamakoshi A, Ikezaki K, et al (1997). "Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey". Clin Neurol Neurosurg 99 Suppl 2: S1–5. Surgical Treatment of Moyamoya Disease: Operative Technique for Encephalo-duro-arterio-myo-synangiosis, Its Follow-up, Clinical Results, and Angiograms; Kinugasa, Kazushi M.D.; Mandai, Shinya M.D.; Kamata, Ichiro M.D.; Sugiu, Kenji M.D.; Ohmoto, Takashi M.D. Volume 32(4), April 1993, p 527–531

THANK YOU: 

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