Angiografisches Moyamoya bei Glykogenose Typ IA – Management bei einer erwachsenen Patientin
Fallbericht mit Schwerpunkt auf der Behandlung: beidseitige Encephalo-Duro-Arterio-Myo-Synangiose (EDAMS)

Zusammenfassung

Angiografisches Moyamoya ist eine seltene zerebrovaskuläre Erkrankung mit Häufung in Asien. Progrediente Obstruktion der hirnversorgenden Arterien führt zu zerebralen Ischämien, meist bereits im frühen Kindesalter und ist verbunden mit ausgeprägter Kollateralisation, welche in der Angiografie den Aspekt von Rauch (japan.: moya moya) imitiert. Als operative Therapieoptionen existieren direkte und indirekte Anastomoseverfahren, wobei prospektive, randomisierte Studien zur Therapiewahl bislang fehlen. Bei einer 23-jährigen Glykogenose Typ IA-Patientin mit transitorischer ischämischer Attacke wurde die Erstdiagnose eines beidseitigen angiografischen Moyamoya gestellt. Die Koinzidenz beider seltener Erkrankungen wurde bereits in zwei Fallberichten publiziert; es scheint sich hierbei um einen echten Risikofaktor zu handeln. Durch beidseitige Enzephalo-Duro-Arterio-Myo-Synangiose (EDAMS) bei jeweils proximaler ACI-Stenose wurde eine klinisch und radiografisch hervorragendes Ergebnis erzielt.

Abstract

Angiographic Moyamoya is a rare cerebrovascular disease most frequent in asia. Its characateristics are recurrent ischemic attacks due to progressive occlusion of ICA branches. Angiography reveals fine arterial collateralisation reminding of ascending smoke (”moyamoya” in japanese). Neurosurgical treatment strategies include direct and indirect reanastomosation procedures. Randomised trials for comparison of clinical outcome and long term survival remain missing. A 23 years old female with glycogenosis type IA was first diagnosed bilateral angiographic moyamoya with bilateral proximal stenosis of ICA after transient ischemic attack (TIA). Coincidence of both rare diseases moyamoya and glycogenosis has previously been reported in three cases, so that this metabolic dysfunction presumably is a true risk factor for moyamoya. In our case, excellent angiographic and functional results were achieved by bilateral, consecutive Enzephalo-Duro-Arterio-Myo-Synangiosis (EDAMS).

Moyamoya - Glykogenose Typ I - v. Gierke-Erkrankung - Glykogenspeicherkrankheit - Risikofaktor

moyamoya - glycogenosis type I - Gierkes‘ disease - glycogen storage disease - risk factor

Literatur

• 1 Aoki N, Kagawa M, Wanifuchi H. et al . An adult case of moyamoya disease associated with marked advance of occlusive lesion in the bilateral carotid system.  No Shinkei Geka. 1989;  17 399-403
• 2 Berzsenyi M D, Brady B M, O’Donnell C J. et al . Cerebrovascular disease in type 1a glycogen storage disease.  J Clin Neurosci. 1998;  5 93-94
• 3 Crowell R M, Yasargil M G. End-to-side anastomosis of superficial temporal artery to middle cerebral artery branch in the dog.  Neurochirurgia (Stuttg). 1973;  16 73-77
• 4 Fujiwara F, Yamada H, Hayashi S. et al . A case of adult moyamoya disease showing fulminant clinical course associated with progression from unilateral to bilateral involvement.  No Shinkei Geka. 1997;  25 79-84
• 5 Fukui M. Current state of study on moyamoya disease in japan.  Surg Neurol. 1997;  47 138-143
• 6 Fukui M, Kono S, Sueishi K. et al . Moyamoya disease.  Neuropathology. 2000;  20 (Suppl) S61-S64
• 7 Goutières F, Bourgeois M, Trioche P et al. Moyamoya disease in a child with glycogen storage disease type ia.  Neuropediatrics. 1997;  28 133-134
• 8 Hallemeier C L, Rich K M, Grubb R LJ. et al . Clinical features and outcome in north American adults with moyamoya phenomenon.  Stroke. 2006;  37 1490-1496
• 9 Kagawa R, Okada Y, Moritake K. et al . Magnetic resonance angiography demonstrating adult moyamoya disease progressing from unilateral to bilateral involvement--case report.  Neurol Med Chir. 2004;  44 183-186
• 10 Kang K H, Kim H S, Kim S Y. Quantitative cerebrovascular reserve measured by acetazolamide-challenged dynamic ct perfusion in ischemic adult moyamoya disease: initial experience with angiographic correlation.  AJNR Am J Neuroradiol. 2008;  29 1487-1493
• 11 Kawaguchi T, Fujita S, Hosoda K. et al . Multiple burr-hole operation for adult moyamoya disease.  J Neurosurg. 1996;  84 468-476
• 12 Keddad K, Thérond P, Motta C. et al . Alterations in erythrocyte membrane fluidity and fatty acid composition in glycogen storage diseases.  Biochim Biophys Acta. 1996;  17 61-65
• 13 Khan N, Schuknecht B, Boltshauser E. et al . Moyamoya disease and moyamoya syndrome: experience in europe; choice of revascularisation procedures.  Acta Neurochir. 2003 ;  145 1061-1071 (discussion 1071)
• 14 Kim D, Kang S, Yoo D. et al . Surgical results in pediatric moyamoya disease: angiographic revascularization and the clinical results.  Clin Neurol Neurosurg. 2007;  109 125-131
• 15 Kuroda S, Ishikawa T, Houkin K. et al . Incidence and clinical features of disease progression in adult moyamoya disease.  Stroke. 2005;  36 2148-2153
• 16 Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives.  Lancet Neurol. 2008;  7 1056-1066
• 17 Marfaing-Koka A, Wolf M, Boyer-Neumann C. et al . Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia.  J Pediatr Gastoenterol Nutr. 2008;  37 566-570
• 18 Natori Y, Ikezaki K, Matsushima T. et al . Angiographic moyamoya – its definition, classification, and therapy.  Clin Neurol Neurosurg. 1997;  99 (Suppl 2) S168-S172
• 19 Ogata T, Yasaka M, Inoue T. et al . The clinical features of adult unilateral moyamoya disease: does it have the same clinical characteristics as typical moyamoya disease?.  Cerebrovasc Dis. 2008;  26 244-249
• 20 Oka K, Yamashita M, Sadoshima S. et al . Cerebral haemorrhage in moyamoya disease at autopsy.  Virchows Arch A Pathol Anat Histol. 1981;  392 247-261
• 21 Oka Y, Kusunoki K, Nochide I. et al . A case of adult moyamoya disease progressed after vascular reconstructive surgery.  No Shinkei Geka. 2000;  28 373-378
• 22 Ozen H. Glycogen storage diseases: new perspectives.  World J Gastroenterol. 2007;  13 2541-2553
• 23 Peerless S J. Risk factors of moyamoya disease in canada and the usa.  Clin Neurol Neurosurg. 1997;  99 Suppl 2 S45-48
• 24 Saeki N, Silva M N, Kubota M. et al . Comparative performance of magnetic resonance angiography and conventional angiography in moyamoya disease.  J Clin Neurosci. 2000;  7 112-115
• 25 Shirane R, Mikawa S, Ebina T. A case of adult moyamoya disease showing progressive angiopathy on cerebral angiography.  Clin Neurol Neurosurg. 1999;  101 210-214
• 26 Sunder T R. Moyamoya disease in a patient with type i glycogenosis.  Arch Neurol. 1981;  38 251-253
• 27 Suzuki J, Takaku A. Cerebrovascular „moyamoya” disease. Disease showing abnormal net-like vessels in base of brain.  Arch Neurol. 1969;  20 288-299
• 28 Takeshita I, Tsukamoto H, Yamaguchi T. et al . A progressive occlusion of the internal carotid arteries in a case of adult-onset moyamoya disease.  Fukuoka Igaku Zasshi. 1995;  86 367-372
• 29 Takeuchi K SK. Hypogenesis of bilateral internal carotid arteries.  No To Shinkei. 1957;  9 37-43
• 30 Tomida M, Muraki M, Yamasaki K. Angiographically verified progression of moyamoya disease in an adult. Case report.  J Neurosurg. 2000;  93 1055-1057
• 31 Trioche P, Francoual J, Capel L. et al . Apolipoprotein E polymorphism and serum concentrations in patients with gloycogen storage disease type Ia.  J Inherit Metabol Dis. 2000;  23 107-112
• 32 Wanifuchi H, Takeshita M, Aoki N. et al . Adult moyamoya disease progressing from unilateral to bilateral involvement.  Neurol Med Chir (Tokyo). 1996;  36 87-90
• 33 Yamashita M, Oka K, Tanaka K. Histopathology of the brain vascular network in moyamoya disease.  Stroke. 1983;  14 50-58
• 34 Yekeler E, Dursun M, Emeksiz E. et al . Prediction of premature atherosclerosis by endothelial dysfunction and increased intima-media thickness in glycogen storage disease types ia and iii.  Turk J Pediatr. 2007;  49 115-119
• 35 Yonekawa Y, Ogata N, Kaku Y. et al . Moyamoya disease in europe, past and present status.  Clin Neurol Neurosurg. 1997;  99 (Suppl 2) S58-60
• 36 Yoshida Y, Yoshimoto T, Shirane R. et al . Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: an extensive follow-up study.  Stroke. 1999;  30 2272-2276
• 37 Zipfel G J, Sagar J, Miller J P. et al . Cerebral hemodynamics as a predictor of stroke in adult patients with moyamoya disease: a prospective observational study.  Neurosurgical Focus. 2009;  26 (4) E6