A Rare Association of Protein S Deficiency and Mineralizing Angiopathy in a Toddler

A bstract A developmentally normal two-year-old boy presented with left-sided hemiparesis following a trivial trauma. Computed tomography (CT) showed thin linear calcification in the right gangliocapsular region suggestive of mineralizing angiopathy (MA) of lenticulostriate arteries. Cerebral angiography was normal. Of the thrombophilia work-up, he was found to have protein S (PS) deficiency. The child showed complete resolution of symptoms with aspirin and regular physiotherapy. Stroke in children is uncommon and arises from a diverse range of causes. MA is a relatively newer cause identified. Trivial head trauma and cytomegalovirus infection are recognized as predisposing factors of MA. PS deficiency is a well-known risk factor for venous thromboembolic stroke. However, an association of PS deficiency and MA is never reported in the literature before. This rare association should be studied further to prove causation. CT of the brain in a pediatric stroke with a history of trivial head trauma is crucial for the identification of MA. Due to its complex and not fully understood nature, it is essential to conduct a comprehensive investigation into the underlying causes, with particular emphasis on the thrombophilia profile.

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Publication Details

Journal
Assam Journal of Internal Medicine
Published
2025-01-01
DOI
https://doi.org/10.4103/ajoim.ajoim_29_24
Primary Topic
Blood Coagulation and Thrombosis Mechanisms
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article
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article

A Rare Association of Protein S Deficiency and Mineralizing Angiopathy in a Toddler

Thirunavukkarasu Arun Babu, K. S. Aswanth
Assam Journal of Internal Medicine
Blood Coagulation and Thrombosis Mechanisms
article

A Rare Association of Protein S Deficiency and Mineralizing Angiopathy in a Toddler

Thirunavukkarasu Arun Babu, K. S. Aswanth
article en

Abstract

A bstract A developmentally normal two-year-old boy presented with left-sided hemiparesis following a trivial trauma. Computed tomography (CT) showed thin linear calcification in the right gangliocapsular region suggestive of mineralizing angiopathy (MA) of lenticulostriate arteries. Cerebral angiography was normal. Of the thrombophilia work-up, he was found to have protein S (PS) deficiency. The child showed complete resolution of symptoms with aspirin and regular physiotherapy. Stroke in children is uncommon and arises from a diverse range of causes. MA is a relatively newer cause identified. Trivial head trauma and cytomegalovirus infection are recognized as predisposing factors of MA. PS deficiency is a well-known risk factor for venous thromboembolic stroke. However, an association of PS deficiency and MA is never reported in the literature before. This rare association should be studied further to prove causation. CT of the brain in a pediatric stroke with a history of trivial head trauma is crucial for the identification of MA. Due to its complex and not fully understood nature, it is essential to conduct a comprehensive investigation into the underlying causes, with particular emphasis on the thrombophilia profile.

Assam Journal of Internal MedicineVol. 15(1)
All India Institute of Medical Sciences Raipur (IN), All India Institute of Medical Sciences Bhopal (IN), All India Institute of Medical Sciences (IN)
Openalex Percentile: Top 81%
Blood Coagulation and Thrombosis Mechanisms
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A Rare Association of Protein S Deficiency and Mineralizing Angiopathy in a Toddler — Thirunavukkarasu Arun Babu, K. S. Aswanth · Assam Journal of Internal Medicine (2025) | TGRS Research Map | TGRS