Rigshospitalet

healthcare πŸ“ Copenhagen, Denmark
Rigshospitalet
3
EM Publications
1
EM Researchers

Associated Institutions

University of Copenhagen
related
Glostrup Hospital
child
Copenhagen University Hospital
parent
Danish Multiple Sclerosis Center
child
Capital Region of Denmark
parent

Publications

[Hemichorea caused by polycythemia vera].

Sodemann NW, Brask-Thomsen PK, van Kooten Niekerk PB
Ugeskrift for laeger β€’

Polycythaemia vera (PV) is a chronic myeloproliferative disorder. PV should be suspected with hemoglobin >10.2/9.9 mmol/L (m/f) and/or PV-related features (aquagenic pruritus, erythromelalgia, atypical thrombosis, splenomegaly, thrombocytosis or leukocytosis). This case report highlights the connection between PV and neurological manifestations, including rare symptoms like chorea. An 82-year-old woman presented with sudden choreiform movements, dysarthria, and behavioral changes. Investigations ruled out primary neurological disease and confirmed PV. With treatment, the neurological symptoms resolved.

The prostaglandin E1 analog misoprostol reduces symptoms and microvascular arteriovenous shunting in erythromelalgia-a double-blind, crossover, placebo-compared study.

MΓΈrk C, Salerud EG, Asker CL, Kvernebo K
The Journal of investigative dermatology β€’

Based on previous experience with parenteral prostanoids, we studied the effect of misoprostol treatment, an orally administered prostaglandin E1 analog, in patients with erythromelalgia. Treatment with placebo was followed by treatment with misoprostol (0.4-0.8 mg per d), both for 6 wk. The patients (n=21) and a study nurse who administered the trial were blinded. The endpoints were change in pain and need for cooling and global assessment of the treatment. Following central body heat provocation, global skin perfusion, capillary morphology, and change in pain were also recorded before and after each treatment period. Results were compared with data from healthy control subjects (n=11) that did not undergo treatment. Clinical safety and tolerability evaluation included physical examinations, clinical laboratory tests, and monitoring of adverse events. All clinical outcome measures were significantly better after treatment with misoprostol (p<0.01) as compared with placebo treatment and after a 3- mo follow-up without treatment. The heat-induced increase in global perfusion after misoprostol treatment was similar to the control group and significantly lower when compared with baseline (p<0.01) and placebo treatment (p<0.05), respectively. This study demonstrates that misoprostol is clinically superior to placebo in patients with erythromelalgia. The results of the perfusion studies may imply that the mechanism of action of the beneficial effect of misoprostol is reduced microvascular arteriovenous shunting in affected skin.

[The chronic Philadelphia chromosome-negative myeloproliferative syndrome II. Clinical findings, diagnostics and treatment].

Hasselbalch HC, Mourits-Andersen HT, Jensen BA, Jensen MK, Nielsen OJ
Ugeskrift for laeger β€’

Polycythaemia vera (PV) and essential thrombocytosis (ET) are clinically characterised by non-specific neurologic symptoms, peripheral circulatory disturbances (acrocyanosis, wounds, erythromelalgia) or abdominal symptoms. The treatment of PV includes phlebotomy, antiaggregation and cytoreduction. In ET, the primary treatment is also low-dose aspirin except for patients presenting with a haemorrhagic diathesis. Hydroxyurea may be associated with an increased risk of acute leukaemia or myelodysplasia. Therefore alpha-interferon and anagrelide should be considered in younger patients. Early cytoreductive therapy is advocated in patients with idiopathic myelofibrosis (IMF) to inhibit further progression of bone marrow fibrosis and further expansion of myeloid metaplasia in the spleen and liver. Treatment with androgens (danazol) and glucocorticoids may improve severe anaemia and thrombocytopenia. In younger patients, allogeneic bone marrow transplantation should be considered.