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Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome

  • Neurology of Systemic Diseases (J. Biller, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Understanding of antiphospholipid antibody syndrome (APS), associated neurological manifestations, and disease-directed treatment has grown considerably over the last decade. Herein, we critically review the current and high-yield literature related to the pathophysiology, neurological presentations, and management of APS with particular emphasis on the rare and more fatal subset of APS, catastrophic antiphospholipid syndrome (CAPS).

Recent Findings

APS may manifest with a variety of neurologic syndromes, with cerebrovascular disease representing the most commonly encountered presentation. Diagnostic evaluation and treatment are often tailored to the specific presentation, with suspicion and testing for antiphospholipid antibodies recommended when neurologic presentations occur atypically or in younger individuals. In CAPS, which is more rapidly progressive with multiorgan involvement, potential alternative microangiopathic syndromes should be carefully considered in the differential diagnosis. To date, anticoagulation with vitamin K antagonists remains the mainstay of therapy in APS while triple therapy with anticoagulation, corticosteroids, and plasma exchange is standard of care in CAPS. Immunotherapy has shown early promise in refractory cases.

Summary

APS is an autoimmune clinical syndrome with neurologic presentations classically characterized by vascular thrombosis, though recent understandings suggest additional direct immune-mediated phenomena. Our understanding of the underlying pathogenic mechanisms of APS continues to grow and will continue to influence our therapeutic approaches.

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Abbreviations

AIS:

Acute ischemic stroke

APS:

Antiphospholipid antibody syndrome

aPL:

Antiphospholipid antibodies

CAPS:

Catastrophic antiphospholipid syndrome

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

CVT:

Cerebral venous thrombosis

DOAC:

Direct oral anticoagulant

EEG:

Electroencephalogram

ICH:

Intracerebral hemorrhage

INR:

International normalized ratio

MRI:

Magnetic resonance imaging

MS:

Multiple sclerosis

NAION:

Non-arteritic ischemic optic neuropathy

NMOSD:

Neuromyelitis optica spectrum disorders

SAH:

Subarachnoid hemorrhage

SLE:

Systemic lupus erythematosus

TIA:

Transient ischemic attack

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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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    Rafid Mustafa

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Mustafa, R. Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome. Curr Neurol Neurosci Rep 22, 589–600 (2022). https://doi.org/10.1007/s11910-022-01228-0

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