Koinzidenz von ischämischer Enzephalopathie und essentieller Thrombozythämie: zerebrale kortikale Venenthrombose ?

Publication

Koinzidenz von ischämischer Enzephalopathie und essentieller Thrombozythämie: zerebrale kortikale Venenthrombose ?

Journal Paper/Review - Nov 8, 2010

Hundsberger Thomas, Müller Stefanie, Sommacal Andreas, Fretz Christian, Tettenborn Barbara

Units

Neurologie, Medizinische Onkologie und Hämatologie, Pathologie, Radiologie und Nuklearmedizin

Keywords

Essential thrombocythaemia; ischaemic encephalopathy; cortical vein
thrombosis; brain biopsy; cerebral vasculitis

Link

http://www.sanp.ch/pdf/2010/2010-07/2010-07-062.PDF

Contact

Thomas Hundsberger

Citation

Hundsberger T, Müller S, Sommacal A, Fretz C, Tettenborn B. Koinzidenz von ischämischer Enzephalopathie und essentieller Thrombozythämie: zerebrale kortikale Venenthrombose ?. S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2010; 161:262-265.

Type

Journal Paper/Review (Deutsch)

Journal

S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2010; 161

Publication Date

Nov 8, 2010

Pages

262-265

Publisher

www.sanp.ch

Brief description/objective

We here report a 39-year-old women with an ischaemic encephalopathy in temporal association with the first diagnosis of an essential thrombocythaemia.Diagnosis was difficult due to cognitive impairment, a long lasting period of progressive symptoms, a former febrile infection, relevant weight loss and ambiguous findings in brain imaging. Meningeal and brain biopsy obtained the diagnosis of an ischaemic encephalopathy and reliably delineated this finding from an infectious or malignant disease of the brain. According to the persistent thrombocytosis subsequent bone marrow biopsy revealed a JAK-2 positive essential thrombocytosis. In the absence of any other pathology we suspected a subacute cortical vein thrombosis as the most likely cause of the ischaemic encephalopathy triggered by the underlying essential thrombocythaemia. After initial anticoagulation she was therefore treated with aspirin for prophylaxis of further vascular events and hydroxyurea for cytoreduction. Follow up examination revealed good recovery from neurological symptoms.