Severe Arterial Cerebral Thrombosis in a Patient with Protein S Deficiency (Moderately Reduced Total and Markedly Reduced Free Protein S): A Family Study

Girolami, Antonio; Simioni, Paolo; Ar, Lazzaro; Cordiano, I

doi:10.1055/s-0038-1646544

Cited by 81 publications

(37 citation statements)

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“…Ischemic stroke has been reported as a rare manifestation of protein S deficiency. Girolami et al 15 and Sie et al 16 were among the first who reported the association of familial deficiency of protein S as a cause of ischemic stroke in young. Wiesel et al .17 studied 105 patients with protein S deficiency, out of which 14 had arterial thrombotic accidents involving the central nervous system or the myocardium, while most studies revealed a weaker association between the two.…”

Section: Discussionmentioning

confidence: 99%

Protein S Deficiency: Ischemic Stroke in Young adult A Case Report

Rahman¹,

Ahmed²,

Uddin³

et al. 2013

Bangla. J. Neuroscience

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Stroke is the third most common cause of mortality in Westernised countries and Accounts for 12% of all deaths in the UK. The economic cost of stroke is enormous. Twelve per cent of first strokes occur in patients under 45 years of age, of which approximately 50% are ischaemic in nature. Stroke in young adult poses a major health problem. The causes of ischaemic stroke in young adults are many and diverse.Such patients usually require more extensive investigations in order to find an underlying cause than more elderly patients. Principal causes are cardioembolism, premature atherosclerosis, haematological and immunological disorders and migraine. Thrombophilic factors have been implicated in 4-8% of the young adult strokes worldwide. Protein S deficiency is a rare cause of ischemic stroke in young population. Only a few sporadic cases have been described in the literature. We are reporting a case of protein S deficiencyrelated ischemic stroke in a 40-year-old man. Early diagnosis and targeted approach can help such patients to prevent recurrent thrombotic episodes.Bangladesh Journal of Neuroscience 2012; Vol. 28 (1): 59-62

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Section: Discussionmentioning

confidence: 99%

Protein S Deficiency: Ischemic Stroke in Young adult A Case Report

Rahman¹,

Ahmed²,

Uddin³

et al. 2013

Bangla. J. Neuroscience

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show abstract

“…Studies from Indian scenario and other countries shows conflicting reports limit the reliability of this association. Girolami et al [10] and Sie et al [11] were among the first who reported the association of familial deficiency of protein S as a cause of ischemic stroke in young. Mayer et al [12] also supported the fact that acquired deficiency of free protein S is not a major risk factor for ischemic stroke.…”

Section: Discussionmentioning

confidence: 99%

Ischemic Stroke in Young due to Combined Protein C and S Deficiency

Patel¹,

Sachan²,

Gupta³

et al. 2013

JCR

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Abstract:WHO defines stroke as an event caused by the interruption of the blood supply to the brain, usually because a blood vessel bursts or is blocked by a clot. This cuts off the supply of oxygen and nutrients, causing damage to the brain tissue [1]. Globally, stroke is the third commonest cause of mortality [2] and the fourth leading cause of disease burden [3]. Ischemic stroke is the most common cerebrovascular disease, most often due to atherothrombotic diseases and uncommonly by disorders of hypercoagulation. Disorders of coagulation leading to thrombotic disorders are approximately 1% of all ischemic strokes and 4-8% of young strokes. Similarly combined deficiency of protein C and S can lead to hypercoagulable state and rarely present as cerebrovascular accident. We describe here a rare case of 27 year old male who presented with right middle cerebral artery territory infarct due to combined protein C and S deficiency.Key words: Stroke, Blood Coagulation, Protein C, Infarction, Middle Cerebral Artery, Brain. IntroductionCerebrovascular diseases are one of the common causes of high morbidity and mortality all over the world and out of all cerebrovascular diseases most of them are ischemic strokes (85%). Stroke in young individual poses a major problem as these young family members are the major income earner of the family. Abraham et al [4] from Vellore, South India reported that 25% cases of the stroke were less than 40 years of age. Other Indian studies have highlighted a high incidence 24-35% of stroke in young population [5]. Atherothrombotic diseases are most common cause for ischemic stroke, however disorder of coagulation although uncommon but can lead to hypercoagulability resulting in ischemic stroke. Case ReportA 27 year old male presented with complains of weakness in left side of body since last 10 days. General physical examination was unremarkable. Neurological examination revealed findings consistent with left-sided hemiparesis. No precipitating factors such as obesity, diabetes, hypertension, valvular heart disease, chronic drug intake, intravenous drug abuse and smoking were present. Family history was negative for vascular events

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“…In this case report, the patient presented at 11 months of age. Girolami et al and Sie P et al first described familial deficiency of protein S as a cause of ischaemic stroke in young people 4,5 . Proteins C and S are 2 vitamin K-dependent plasma proteins working in concert as a natural anticoagulant system.…”

Section: Discussionmentioning

confidence: 99%