Introduction: Catastrophic Antiphospholipid syndrome (CAPS) is a little understood entity having a high mortality approaching 50%The rapid deterioration is caused by a deadly cytokine storm which is difficult to recognize and treat in time. Various regimens such as monoclonal antiphospholipid antibodies and high dose methyl prednisolone infusions have been found to be effective against the disease, but are of use only if detected early.
Methods: We present an unusual case of multiple spontaneous intracranial haematomas , with evidence of severe refractile coagulopathy in a background of multisystem dysfunction. The subsequent rapid deterioration and resultant fatality, when analysed retrospectively provide important clues for diagnosis and management of triggers which can initiate the series of events that eventually end in death.
Results: The rapid sequence of events resulted in the death of the patient despite all effort to save him. This event presents a valuable lesion to understand the nature and presentation of the disease
Conclusions: CAPS therefore is a deadly disease which should be kept in mind when dealing with spontaneous intracerebral bleeds with multisystem dysfunction and coagulation derangements. Rapid treatment with steroid and monoclonal antibodies may be lifesaving and help avoid complications. Avoidance of triggers is an important aspect of management that helps in prevention of patient deterioration and death
Patient Care: by familiarising more and more neurosurgeons with the phenomenon of CAPS, appropriate care may be instituted early to avoid poor outcomes
Learning Objectives: by the conclusion of this session, participants should be able to :
1. understand the pathogenesis and progression of CAPS
2. Appreciate the various clinical presentations of CAPS as well as a diagnostic algorithm
3. familiarise themselves with management options as well as avoidance of triggers