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A rare clinical association of antiphospholipid antibody syndrome associated with recurrent benign intracranial hypertension with features of motor neuron disease.


Greg David V. Dayrit,
Virgilio Gerald H. Evidente,
Rogelio Libarnes

Related Institution

Institute of Neurosciences - St. Luke's Medical Center

Department of Neurology - Cardinal Santos Medical Center

Publication Information

Publication Type
Publication Sub Type
Case report
Philippine Journal of Internal Medicine
Publication Date
May-June 2003


BACKGROUND: Antiphospholipid antibody syndrome (APAS) is a rare autoimmune disorder presenting with systemic and neurologic features associated with antiphospholipid antibodies. The neurological complications of the antiphospholipid antibody syndrome include migraine, stroke, chorea, dementia, transverse myelopathy, and rarely motor neuron disease.
OBJECTIVES: To discuss the rare clinical association of primary APAS with recurrent benign intracranial hypertension (BIH) with associated late features of focal motor neuron disease.
CASE SUMMARY: A 36 year old Filipino male had a 5-year history of recurrent headache with visual obscurations. On fundoscopy, bilateral papilledema was noted. Brain MRI showed foci of increased T2 signal in the subcortical white matter. MRA and MRV were normal. Serial lumbar punctures revealed markedly elevated opening pressures; CSF showed mildly increased protein. The patient was treated with diuretics, acetazolamide, steroids, anti-inflammatory agents, and topiramate at different times with fair success. Yearly large volume lumbar taps relieved him of severe breakthrough headaches.
A few years later, the patient developed proximal left arm weakness, atrophy, and cramps. EMG revealed a proximal, axonal process of both upper extremities, in a pattern suggestive of focal anterior horn cell disease. Cervical MRI only revealed a small disc protrusion at C5-C6. Serological abnormalities included elevated ESR, (+) ANA, and (+) anticardiolipin antibodies and lupus anticoagulant. Work-up for SLE and connective tissue disorders were negative
SUMMARY: Recurrent benign intracranial hypertension with or without focal motor neuron signs should alert the clinician to the possibility of APAS. Benign intracranial hypertension may occur even without venous cerebral thrombosis in the clinical spectrum of APAS.

Physical Location

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