The catastrophic antiphospholipid antibody syndrome: An unusual presentation of spontaneous acute intracranial haemorrhage

Ganapathy, Sibhi and Nair, Rajesh and Nagaraj, Anmol and Menon, Girish (2015) The catastrophic antiphospholipid antibody syndrome: An unusual presentation of spontaneous acute intracranial haemorrhage. Journal of Neurology & Stroke, 3 (4). pp. 1-6. ISSN 2373-6410

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Abstract

Background: Catastrophic Antiphospholipid syndrome (CAPS) is a scarcely understood entity with a high mortality approaching 50%The rapid deterioration is triggered by a deadly cytokine storm, which is difficult to recognize and almost impossible to treat in time. Various regimens have been advocated, which include administration of monoclonal anti-phospholipid antibodies and pulses of high dose methyl prednisolone. However, these treatment modalities are only effective if the disease is diagnosed early and instituted. Clinical presentation: We present an unusual case of multiple spontaneous intracranial haematomas in a young individual, with evidence of severe refractory coagulopathy in a background of multisystem dysfunction. A retrospective analysis of the subsequent rapid deterioration and resultant fatality provided us with important learning points for early diagnosis and management of triggers which can initiate and lead to a cascade of events that eventually end in death. Conclusion: CAPS is a fatal disease which should be considered when dealing with spontaneous intracerebral bleeds with multisystem dysfunction in the background of coagulation derangements. Rapid treatment with steroids and monoclonal antibodies may be helpful in halting the progression of the disease and eventually help in repressing a fatal outcome. Identifying the trigger factors, that usually initiate the downhill cascade, are a priority, and avoiding such provocative factors is an important aspect of management. This will help in the prevention of morbidity and eventual mortality in such patients.

Item Type: Article
Uncontrolled Keywords: Catastrophic Antiphospholipid Syndrome; Spontaneous Acute Intracranial Haemorrhage; Refractory coagulopathy; Intracranial haematomas; Spontaneous intracerebral bleeds.
Subjects: Medicine > KMC Manipal > Neuro Surgery
Depositing User: KMC Library
Date Deposited: 16 Mar 2017 09:59
Last Modified: 16 Mar 2017 09:59
URI: http://eprints.manipal.edu/id/eprint/148485

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