Protein C and protein S deficiency associated cerebral venous thrombosis: one case report.

Objective To report one case with protein C deficiency (PCD) and protein S deficiency (PSD) associated cerebral venous thrombosis (CVT), and to explore diagnostic and therapeutic strategies. Methods and Results A 27-year-old male patient presented with positional headache, worsened in supine positio...

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Publicado en:Chinese Journal of Contemporary Neurology & Neurosurgery Vol. 25; no. 6; pp. 523 - 530
Autores principales: HUANG Zhan, ZHU Ming-fang, MENG Qi, TAN Lei-lei, ZHANG Jie-wen, HUANG Yue
Formato: case study diagnostic images Journal Article
Publicado: Chinese Journal of Contemporary Neurology & Neurosurgery Jun2025
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jun2025
      vid: 25
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      pub: Chinese Journal of Contemporary Neurology & Neurosurgery
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        186295679
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        10.3969/j.issn.1672-6731.2025.06.011
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        atl: Protein C and protein S deficiency associated cerebral venous thrombosis: one case report.
      aug:
        au:
          HUANG Zhan
          ZHU Ming-fang
          MENG Qi
          TAN Lei-lei
          ZHANG Jie-wen
          HUANG Yue
        affil: Department of Neurology, Zhengzhou University People's Hospital; He'nan Provincial People's Hospital, Zhengzhou 450003, He'nan, China
      sug:
        subj:
          Blood Protein Disorders Etiology
          Cerebral Veins Pathology
          Sinus Thrombosis, Intracranial Diagnosis
          Sinus Thrombosis, Intracranial Therapy
          Male
          Adult
          Headache
          Brain Radiography
          Blood Proteins Analysis
          Cerebrospinal Fluid Analysis
          Genetic Screening
          Mutation
          Heparin Administration and Dosage
          Anticoagulants Administration and Dosage
          Monitoring, Intracranial Pressure
          Nerve Growth Factors Therapeutic Use
          Analgesia
          Adult: 19-44 years
          Male
      ab: Objective To report one case with protein C deficiency (PCD) and protein S deficiency (PSD) associated cerebral venous thrombosis (CVT), and to explore diagnostic and therapeutic strategies. Methods and Results A 27-year-old male patient presented with positional headache, worsened in supine position and relieved upon standing. Imaging findings showed thrombosis in the right transverse sinus, sigmoid sinus, and confluence of sinus. Laboratory tests showed significantly reduced protein C and protein S activities (49% and 51%) and elevated cerebrospinal fluid (CSF) pressure (> 330 mm H2O). Genetic test identified a frameshift mutation in the PROC gene [c.574_577del (p.Val192Serfs*5)] and missense mutation in the PROS1 gene [c.1915T > G (p.Cys639Gly) and c.301C > T (p.Arg101Cys)]. After receiving heparin followed by non-vitamin K oral anticoagulants (NOACs), alongside intracranial pressure reduction, neurotrophic therapy, and analgesia treatment, the patient's headache symptom resolved, and at 4-month follow-up reexamination showed CSF pressure normalized (135 mm H2O), with protein C and protein S activities rising to 53% and 54.10% (still below normal ranges). No headache recurrence was observed. Conclusions PCD and PSD are critical etiological factors for CVT. NOACs effectively improve venous reflux and avoid warfarin-related coagulation dysfunction, representing a preferred therapeutic option. Screening for coagulation protein activity and genetic analysis in young patients is essential to guide precise anticoagulation management.
      pubtype: Academic Journal
      doctype:
        case study
        diagnostic images
        Journal Article
      ougenre: Article
    language: Chinese
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