Protein S Deficiency and an Adult Case with Moyamoya Syndrome that Presented with Primary Intraventricular Haemorrhage

Çevik, Betül; Acu, Berat; Aksoy, Dürdane; Kurt, Semiha

doi:10.5152/balkanmedj.2014.13028

Cited by 4 publications

(4 citation statements)

References 5 publications

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“…This discrepancy may partly be explained by the aggressive reversal of anticoagulation during the hyper-acute phase in the subjects, which can prevent hematoma expansion or re-bleeding. There are many reports demonstrating that clinical improvement can be achieved through the reversal of thrombocytopenia and coagulopathy [ 30 , 48 – 50 ]. Recently, patients with severe coagulation factor deficiency or severe thrombocytopenia have been recommended to receive appropriate factor replacement therapy or platelets, respectively [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

Factors Associated with Clinical Outcomes in Patients with Primary Intraventricular Hemorrhage

Lee

Park

et al. 2017

Med Sci Monit

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BackgroundPrimary intraventricular hemorrhage (PIVH) is an uncommon type of intracerebral hemorrhage. Owing to its rarity, the clinical and radiological factors affecting outcomes in patients with PIVH have not been widely studied.Material/MethodsWe retrospectively reviewed 112 patients (mean age 53 years) treated for PIVH at our institution from January 2004 to December 2014. Clinical and radiological parameters were analyzed 3 months after initial presentation to identify factors associated with clinical outcomes, as assessed by the Glasgow Outcome Scale (favorable ≥4, unfavorable <4).ResultsOf the 99 patients who underwent angiography, causative vascular abnormalities were found in 46%, and included Moyamoya disease, arteriovenous malformation, and cerebral aneurysm. At 3 months after initial presentation, 64% and 36% of patients were in the favorable and unfavorable outcome groups, respectively. The mortality rate was 19%. However, most survivors had no or mild deficits. Age, initial Glasgow Coma Scale (GCS) score, simplified acute physiology score (SAPS II), modified Graeb score, and various radiological parameters reflecting ventricular dilatation were significantly different between the groups. Specifically, a GCS score of less than 13 (p=0.015), a SAPS II score of less than 33 (p=0.039), and a dilated fourth ventricle (p=0.043) were demonstrated to be independent predictors of an unfavorable clinical outcome.ConclusionsIn this study we reveal independent predictors of poor outcome in primary intraventricular hemorrhage patients, and show that nearly half of the patients in our study had predisposing vascular abnormalities. Routine angiography is recommended in the evaluation of PIVH to identify potentially treatable etiologies, which may enhance long-term prognosis.

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Section: Discussionmentioning

confidence: 99%

Factors Associated with Clinical Outcomes in Patients with Primary Intraventricular Hemorrhage

Lee

Park

et al. 2017

Med Sci Monit

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“…Abnormal thrombophilia profile in moyamoya disease is reported in the literature. There have been reports of moyamoya disease with protein c and protein s deficiency [ 3 , 4 ]. One case report of homocystinuria with moyamoya was also described in the literature [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Hyperhomocysteinemia in a Patient with Moyamoya Disease

Meena

Maheshwari

2018

Case Reports in Neurological Medicine

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Moyamoya disease is a chronic progressive cerebrovascular disease characterized by bilateral occlusion or stenosis of arteries around circle of Willis. We report a case of 18-year-old female presented with recurrent episodes of headache and vertigo. On cerebral angiography, the patient was diagnosed to have moyamoya disease. On further evaluation, thrombophilia profile showed increased homocysteine level. The patient was treated conservatively with cobalamin and aspirin and advised for revascularization. According to the literature, there are few case reports of moyamoya disease with thrombotic disorders. Hence, we are reporting this interesting and rare case.

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“…We found five papers (5%) that described an association between moyamoya and metabolic disorders, including mucolipidosis II [ 36 ], protein S deficiency [ 37 ], CD59 deficiency [ 38 ], severe hyperhomocysteinemia [ 39 ], and lysinuric protein intolerance [ 40 ].…”

Section: Reviewmentioning

confidence: 99%

Western Moyamoya Phenotype: A Scoping Review

Miller¹,

Unda²,

Holland³

et al. 2021

Cureus

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Moyamoya, a rare angiographic finding, is characterized by chronic and progressive stenosis at the terminal end of the internal carotid artery, followed by collateralization of the cerebral vasculature at the base of the skull. Coined by Suzuki and Takaku in 1969, the term “moyamoya” means a “puff of smoke” in Japanese, a reference to the angiographic appearance of moyamoya collateralization. Moyamoya is most commonly found in East Asian countries, where much governmental and civilian effort has been expended to characterize this unique disease process. However, despite its rarity, the occurrence of moyamoya in Western countries is associated with significant divergence regarding incidence, gender, sex, age at diagnosis, clinical presentation, and outcomes. Here, we attempted to review the Western literature on moyamoya presentation using the PubMed database to characterize the Western phenotype of moyamoya. We were guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews (PRISMA-ScR). We reviewed papers generated from a search with keywords “moyamoya case report,” those reported from a Western institution, and those reported on a relevant association. Our scoping review demonstrated various clinical associations with moyamoya. Moreover, we summarized the demographic profile and clinical symptomatology, as well as reported disease associations to better elucidate the Western phenotype of moyamoya.

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Protein S Deficiency and an Adult Case with Moyamoya Syndrome that Presented with Primary Intraventricular Haemorrhage

Cited by 4 publications

References 5 publications

Factors Associated with Clinical Outcomes in Patients with Primary Intraventricular Hemorrhage

Factors Associated with Clinical Outcomes in Patients with Primary Intraventricular Hemorrhage

Hyperhomocysteinemia in a Patient with Moyamoya Disease

Western Moyamoya Phenotype: A Scoping Review

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