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A 66-year-old-male presented to the hospital with a six-week history of progressive muscle weakness, truncal radicular pain, dysphagia, hypophonia and bulbar weakness.  The neurological exam was notable for asymmetric L sided proximal weakness in the extremities, hyporeflexia and ataxia.

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Initial MRI brain and cervical spine were unremarkable. CSF analysis revealed pleocytosis with lymphocytic predominance and elevated protein, suggestive of central nervous system infection. Borrelia Burgdorferi IgG and IgM were positive in both CSF and serum confirming neuroborreliosis, and the patient was treated with ceftriaxone. The patient subsequently developed a deep venous thrombosis in the Left lower extremity followed by pulmonary embolism and encephalopathy. Repeat MRI brain demonstrated bilateral multifocal cerebral and cerebellar acute infarcts and petechial hemorrhage in the temporal lobe. Further workup revealed an occlusion of the right posterior cerebral artery on CT angiography. There was no evidence of endocarditis or thrombus on echocardiogram. CT abdomen identified no malignancy but revealed an additional splenic infarct. Hypercoagulable workup showed positive lupus anticoagulant antibody. Given intravascular thrombosis involving the brain, lungs and spleen within one week in the presence of positive lupus anticoagulant antibody, catastrophic antiphospholipid syndrome was suspected. Patient was treated with a course of intravenous immunoglobulin and plasmapheresis with significant neurological improvement.  

The classic manifestation of neuroborreliosis consists of radiculoneuritis, cranial neuritis and lymphocytic meningitis. Less commonly, peripheral neuropathy and cerebral vasculitis are associated with neuroborreliosis. We present a rare case of catastrophic antiphospholipid syndrome in the setting of systemic inflammatory response to neuroborreliosis. Our patient responded well to the combined therapy of anticoagulation, plasma exchange and intravenous immunoglobulin. This case highlights that neuroinfection can precipitate catastrophic antiphospholipid syndrome and a timely diagnosis and treatment is crucial to reduce the mortality of this fatal disease.