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Transient neurologic and ocular manifestations in primary thrombocythemia

Departments of Hematology, Neurology, and Pathology, University Hospital Dijkzigt, Erasmus University, Rotterdam, The Netherlands.

We report the presenting neurologic and visual symptoms in 17 patients with primary thrombocythemia. Poorly localizing symptoms occurred in 14 patients: transient unsteadiness in 13, dysarthria in eight, and scintillating scotomata in four. Nine patients had focal symptoms: transient monocular blindness in two patients, transient mono- or hemiparesis in six, and both of these in one patient. The attacks all had a sudden onset, occurred sequentially rather than simultaneously, lasted for a few seconds to several minutes, and were usually associated with a dull or pulsatile headache. A causal platelet-mediated arterial thrombosis was evident in 15 patients. There were no recurrences of thrombotic or ischemic events during long-term treatment of 12 patients with aspirin and during cytostatic reduction of platelet count to normal in seven patients. We conclude that low-dose aspirin and reduction of platelet count to normal are effective, but coumarin is ineffective, in the treatment and prevention of arterial thrombosis in thrombocythemia.

Address correspondence and reprint requests to Dr. Jan J. Michiels, Department of Hematology, University Hospital Dijkzigt, 40 Dr Molewaterplein, 3015 GD Rotterdam, The Netherlands.

Received July 7,1992. Accepted for publication in final form October 2, 1992.

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