phenprocoumon and Antiphospholipid-Syndrome

This study aimed to improve understanding and treatment of psychiatric symptoms in antiphospholipid syndrome (APS) and to present an approach to the medical management of patients presenting with obsessive-compulsive disorder (OCD) with suspected neurovascular pathology.. A 15-year-old boy presented with severe OCD of recent onset. An infarct of the caudate nucleus was identified as the initial presentation of primary APS. This case report includes a selective literature review of the neuropsychiatric correlates of APS.. The patient had OCD for 3 months with increasing symptoms resulting in admission for psychiatric reasons. After referral to the emergency department 3 weeks later, an infarct of the caudate nucleus was documented using magnetic resonance images of the brain, and APS was diagnosed based on additional laboratory findings. Anticoagulant treatment (enoxaparin and phenprocoumon) in this patient was effective in reducing obsessive-compulsive symptom severity.. OCD may present as a neuropsychiatric manifestation of APS. The present observations are consistent with a thrombotic mechanism for neurologic or psychiatric symptoms in APS. In general, routine medical workup for childhood OCD is not indicated, but a comprehensive psychiatric, medical, and family history taking and physical examination are essential, particularly if OCD is of recent onset. The role of anticoagulant therapy in neuropsychiatric manifestations of APS without the presence of a cerebral infarct requires further research.

Topics: Adolescent; Anticoagulants; Antiphospholipid Syndrome; Caudate Nucleus; Diagnosis, Differential; Enoxaparin; Humans; Magnetic Resonance Imaging; Male; Obsessive-Compulsive Disorder; Phenprocoumon; Psychiatric Status Rating Scales; Severity of Illness Index; Treatment Outcome

We report the case of a 28-year old woman with extensive red-black colored lesions of the skin on the left thigh, which appeared without trauma. The disease arrived during longterm coumarin therapy because of a deep vein thrombosis and an antiphospholipid syndrome. After consideration of the differential diagnoses and due to the typical clinical picture we made the diagnosis of coumarin necrosis. We review the clinical and therapeutic features for this rare complication.

Topics: Adult; Anticoagulants; Antiphospholipid Syndrome; Blood Coagulation Tests; Coumarins; Diagnosis, Differential; Drug Administration Schedule; Drug Eruptions; Drug Therapy, Combination; Female; Humans; Leg Dermatoses; Long-Term Care; Necrosis; Phenprocoumon; Skin; Venous Thrombosis; Vitamin K Deficiency

Topics: Abnormalities, Drug-Induced; Anticoagulants; Antiphospholipid Syndrome; Female; Heparin, Low-Molecular-Weight; Humans; Image Processing, Computer-Assisted; Imaging, Three-Dimensional; Infant, Newborn; Infant, Premature, Diseases; Maxillofacial Abnormalities; Phenprocoumon; Pregnancy; Pregnancy Complications; Pregnancy Trimester, First; Ultrasonography, Prenatal; Vitamin K

Movement disorders have only rarely been reported in association with antiphospholipid syndrome (APS). In such cases, chorea is the most common disorder observed, with occasional reports of hemidystonia, Parkinsonism, and hemiballism. We report here on 3 cases of APS (3 women ages 16, 46, and 56 years) who presented with movement disorders, including tics, tremor, myoclonus, and a corticobasal syndrome, never or rarely reported in association with this disease. Mild executive dysfunction was observed in all 3 patients. We also report the successful treatment of two of these patients with mild oral anticoagulation (INR 2-3). Movement disorders in APS seem more clinically heterogeneous than previously thought. Oral anticoagulation should be considered in the treatment of movement disorders associated with APS.

Topics: Adolescent; Antiphospholipid Syndrome; Brain; Cerebral Infarction; Diagnosis, Differential; Dyskinesias; Electroencephalography; Electromyography; Female; Follow-Up Studies; Frontal Lobe; Humans; Lupus Erythematosus, Systemic; Magnetic Resonance Imaging; Middle Aged; Movement Disorders; Myoclonus; Neurologic Examination; Neuropsychological Tests; Occipital Lobe; Phenindione; Phenprocoumon; Sneddon Syndrome; Spinocerebellar Degenerations; Tics; Tourette Syndrome; Tremor; Warfarin

We report on a 32-year old female patient with primary antiphospholipid syndrome (PAPS) and several thromboembolic events despite stable doses of oral anticoagulation, good patient compliance and maintained INR values of >3. Over the preceding 3 years the patient had presented a wide spectrum of manifestations of APS, including recurrent venous and arterial thromboses, cardiac, gynecological (HELLP syndrome), neurological involvements, livedo reticularis, a mild thrombocytopenia and the most feared manifestation of the catastrophic antiphospholipid syndrome (CAPS). Life-threatening bilateral subdural bleeding occurred while she was anticoagulated. The clinical features appeared to be refractory to oral anticoagulation with phenprocoumon. They were life threatening on each occasion and she developed repetitive episodes of organ damage with cardiac insufficiency (NYHA III), pulmonary hypertension and other residual defects. Even during heparinization recurrent thromboembolism supervened as well as livedo reticularis of the extremities. Lupus anticoagulants (LAC), anticardiolipin (aCL) antibodies and anti-beta(2)-glycoprotein-1 (beta(2)GPI) titers were all markedly elevated. This case report shows that recurrent episodes of thrombosis can occur despite seemingly adequate anticoagulation in patients with CAPS.

Topics: Administration, Oral; Adult; Anticoagulants; Antiphospholipid Syndrome; Female; Hematoma, Subdural; Humans; International Normalized Ratio; Phenprocoumon; Pregnancy; Recurrence; Thromboembolism

Recurrent thromboembolism despite oral anticoagulation is primarily suspicious of overt or occult neoplasia. We report on a man (age: 67 years) who presented with severe thrombophilia which was only controlled when the patient was set on a combined anticoagulation with low molecular weight heparin in supratherapeutic dosage and phenprocoumon with a target INR of 2.0. Despite repeated evaluation over about two years, a malignant tumour could never be demonstrated. However, the patient suffered in addition to a protein S deficiency from an antiphosphospholipid syndrome and a chronic myelomonocytic leukaemia. We postulate that the accepted strong thrombogenicity of antiphosphospholipid syndrome was further increased by protein S deficiency and a possibly procoagulatory effect of the abnormal monocytes explaining the severe thrombophilia resistant to standard therapeutic anticoagulation with a vitamin K antagonist and usual therapeutic doses of low molecular weight heparin, respectively.

Topics: Aged; Anticoagulants; Antiphospholipid Syndrome; Blood Coagulation Tests; Heparin, Low-Molecular-Weight; Humans; Male; Phenprocoumon; Pulmonary Embolism; Recurrence; Thrombosis; Tomography, X-Ray Computed

Over a period of several months a 33-year-old man had recurrent pulmonary emboli. No thromboses could be demonstrated in the peripheral venous system. Transoesophageal echocardiography showed two spherical space-occupying structures in the right ventricle which were removed operatively under the suspected diagnosis of multilobular myxomas. However, their histological examination revealed pure thrombi that had grown by apposition. This unusual findings of right-ventricular thrombi could not be explained pre- and intraoperatively by any local thrombi-favouring changes in the right heart. Tests of clotting mechanisms demonstrated lupus anticoagulant (kaolin-clotting-time mixture test: LA index 21.7 [normal: < 15]), as well as an increased IgG cardiolipin antibody concentration of 19.3 U/l). As no underlying disease was discovered, the diagnosis was by definition primary antiphospholipid syndrome. No further thrombo-embolism has occurred during continuing oral anticoagulation with phenprocoumon.

Topics: Adult; Antiphospholipid Syndrome; Diagnosis, Differential; Heart Diseases; Heart Neoplasms; Heart Ventricles; Humans; Lupus Coagulation Inhibitor; Male; Myxoma; Phenprocoumon; Pulmonary Embolism; Recurrence; Thrombosis