Infarction of the right hemisphere in a patient with antiphospholipid antibody syndrome.
Hemicraniectomy as a surgical treatment for intracranial pressure following large ischemic lesions is widely practiced in selected patients. The antiphospholipid antibody syndrome (APS), a disorder characterized by recurrent arterial and venous thrombosis, is a very rare cause of space occupying ischemic lesions. We present a case of a 35 year old female diagnosed with APS who initially presented with small ischemic lesions and within days developed a massive near-total infarction of the right hemisphere. Because of central nervous system, skin and systemic manifestations Sneddon's syndrome and catastrophic antiphospholipid antibody syndrome (CAPS) remained a possible diagnoses. Sneddon's syndrome is a non-inflammatory occlusive arteriopathy of small and medium size arteries predominantly of the skin and brain, whereas the catastrophic antiphospholipid antibody syndrome is characterized by acute multi-organ system thrombosis of small and large vessels. In addition to the diagnostic criteria for APS a heterozygous factor V Leiden mutation was found in this patient, which may be a contributing risk factor for cerebral ischemia. When considering invasive decompressive procedures the neurosurgeon has to be aware of the poor prognosis of some forms of APS with systemic manifestations.
Finis
A
A
Department of Neurosurgery, University of Schleswig-Holstein, Campus Lübeck, Germany.
Ssenyonjo
H
H
Knopp
U
U
Koch
C
C
Seidel
G
G
Arnold
H
H
Giese
A
A
D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000473:pathology* / Q000503:physiopathology; D002540:Cerebral Cortex / Q000098:blood supply / Q000473:pathology* / Q000503:physiopathology; D002544:Cerebral Infarction / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D003399:Craniotomy; D003937:Diagnosis, Differential; D018450:Disease Progression; D004211:Disseminated Intravascular Coagulation / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D005165:Factor V / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D020767:Intracranial Thrombosis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D009154:Mutation / Q000235:genetics; D020769:Posterior Cerebral Artery / Q000002:abnormalities / Q000473:pathology; D012867:Skin / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology; D018860:Sneddon Syndrome / Q000175:diagnosis; D014057:Tomography, X-Ray Computed
C095381:factor V Leiden; D005165:Factor V