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Papel de la resistencia a la proteina C activada (R-PCA) en el ictus pediatrico

Papel de la resistencia a la proteina C activada (R-PCA) en el ictus pediatrico
Papel de la resistencia a la proteina C activada (R-PCA) en el ictus pediatrico

Introduction. Activated protein C resistance is a recently identified thrombophylic state which results from a mutation in the factor V gene and has been shown to be an important risk factor for peripheral venous thrombosis. We report a case of paediatric stroke in whom we have identified APC resistance. Clinical case. A boy presented acutely at the age of 6 year with a severe right sided headache, vomiting, unsteadiness and drowsiness which worsened over a period of 40 hours. Prior to this episode, he was neurologically and developmentally normal except for occasional headaches. CT showed low attenuation in the left cerebellar hemisphere, and occipital lobe associated with acute hydrocephalus. Excision biopsy of the left cerebellar cortex revealed inflammation and possible infarction. Although he remained in a 'locked-in' state with a flaccid quadriparesis for six months, he improved and was left with a left side hemiplegia, multiple cranial nerve palsies and a visual field defect. He represented at the age of thirteen years with transient ischaemic attacks and was found be heterozygous for the factor V Leiden mutation. Since he has been warfarinised, his symptoms have improved. Conclusions. Although cerebellar stroke in childhood is rare, it has been underdiagnosed in the past. As recurrence is common, patients should be fully investigated and followed up long term. Screening for new factor such as APC- resistance is recommended.

Activated protein C resistance, Cerebellar swelling, Posterior fossa stroke in childhood, Recurrent headaches
0210-0010
1589-1591
Cardo, E.
adb2bd0c-977c-4c6c-b149-83e80d013174
Campistol, J.
240730a2-b6c7-4948-9e69-dd7a82194eb2
Kirkham, F.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Cardo, E.
adb2bd0c-977c-4c6c-b149-83e80d013174
Campistol, J.
240730a2-b6c7-4948-9e69-dd7a82194eb2
Kirkham, F.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

Cardo, E., Campistol, J. and Kirkham, F. (1997) Papel de la resistencia a la proteina C activada (R-PCA) en el ictus pediatrico. Revista de Neurologia, 25 (146), 1589-1591.

Record type: Article

Abstract

Introduction. Activated protein C resistance is a recently identified thrombophylic state which results from a mutation in the factor V gene and has been shown to be an important risk factor for peripheral venous thrombosis. We report a case of paediatric stroke in whom we have identified APC resistance. Clinical case. A boy presented acutely at the age of 6 year with a severe right sided headache, vomiting, unsteadiness and drowsiness which worsened over a period of 40 hours. Prior to this episode, he was neurologically and developmentally normal except for occasional headaches. CT showed low attenuation in the left cerebellar hemisphere, and occipital lobe associated with acute hydrocephalus. Excision biopsy of the left cerebellar cortex revealed inflammation and possible infarction. Although he remained in a 'locked-in' state with a flaccid quadriparesis for six months, he improved and was left with a left side hemiplegia, multiple cranial nerve palsies and a visual field defect. He represented at the age of thirteen years with transient ischaemic attacks and was found be heterozygous for the factor V Leiden mutation. Since he has been warfarinised, his symptoms have improved. Conclusions. Although cerebellar stroke in childhood is rare, it has been underdiagnosed in the past. As recurrence is common, patients should be fully investigated and followed up long term. Screening for new factor such as APC- resistance is recommended.

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More information

Published date: October 1997
Alternative titles: The role of resistance to C active protein (R-PCA) in pediatric stroke
Keywords: Activated protein C resistance, Cerebellar swelling, Posterior fossa stroke in childhood, Recurrent headaches

Identifiers

Local EPrints ID: 429796
URI: http://eprints.soton.ac.uk/id/eprint/429796
ISSN: 0210-0010
PURE UUID: 010384dc-50c4-4e16-8ea0-0bc1e284fcec
ORCID for F. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 05 Apr 2019 16:30
Last modified: 23 Feb 2024 02:39

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Contributors

Author: E. Cardo
Author: J. Campistol
Author: F. Kirkham ORCID iD

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