Diagnosis and immediate treatment of acquired von Willebrand syndrome revealed by recurrent cerebral hemorrhage.

We report the case of a French woman with acquired von Willebrand syndrome who presents recurrent subarachnoid and intra-cerebral hemorrhage since 2012. She had no family or personal bleeding history. In the biologic explorations, APTT was abnormally high with no anticoagulant drugs (it was normal, historically). Two monoclonal IgG and IgM kappa proteins were detected without any lymphoproliferative disorder. Intravenous infusion of immunoglobulin is very effective in AVWS with immunoglobulin G monoclonal gammapathie of undetermined significance. We had a satisfactory correction of coagulation factors for about 30 days. The exploration of APTT is surely essential for the diagnosis and treatment.

[Nemaline rod myopathy revealed by acute respiratory failure after an outpatient cataract surgery]. / Révélation d'une myopathie à bâtonnets de l'adulte par une insuffisance respiratoire aiguë après chirurgie ambulatoire de la cataracte.

We report the case of a 63-year-old patient admitted to the ICU for an acute respiratory failure one week after an outpatient cataract surgery that revealed a nemaline rod myopathy. We present this rare myopathy whose particularities are its aetiology, which can be inherited, mostly with a congenital onset, or sporadic, and the variability of the age at presentation. We discuss the exceptional onset of severe unknown underlying diseases in the context of outpatient surgery.

[Ocular manifestations of thrombotic thrombocytopenic purpura]. / Manifestations neuro-ophtalmologiques révélant un purpura thrombotique thrombocytopénique.

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 47-year-old woman, who presented fluctuating visual disturbances which had developed over the last six months. An antiphospholipid syndrome was suspected and intravenous heparin treatment was started. One week later, hemolytic anemia and renal insufficiency occurred. Severe deficiency of von Willebrand factor-cleaving protease was found and a diagnostic of TTP was made. The clinical outcome was favorable after treatment with plasmapheresis and fresh frozen plasma. Diagnosis, etiology and treatment of this life-threatening disease are discussed.

[Microcomputer processing of medical records]. / Traitement micro-informatique de dossiers médicaux.

The necessity of computers becoming undeniable, authors have brought up the project of a program for management and treatment of medical files on a hospital word scale. Using a monoposte microcomputer, characterized by its simplicity, autonomy and low cost, this system is to be considered among the centralized multi-ramified systems, whose results as far as using the medical parameters are unsatisfactory.

[Vascularization of the ulnar nerve of the arm]. / La vascularisation du nerf ulnaire au bras.

A review of 53 dissections associated with injections and radiographs was performed (by the authors) to specify the arteriovenous vascularization of the ulnar nerve of the arm. An arteriovenous pedicle is constantly to be found. The artery "collateralis ulnaris superior" arises from the inferior border of the "pectoralis major" at an average distance of 6,7 cm. The arterial depending vein most often directly rejoins the "vena brachialis" lower than "vena basilica" does, but usually more distally than the arterial emergence level. Thus constituted, the pedicle can be anastomosed, measuring generally more than 1 mm in diameter. The arterial distribution originates from an arcade parallel to the nerve from which arise some extremely fine ladderlike vessels. Finally, this pedicle vascularizes the whole brachial part of the nerve (roughly 23,6 cm).

[Proposal for a numerical prognostic evaluation in intracerebral hematoma]. / Proposition pour une évaluation pronostique chiffrée dans l'hématome intra-cérébral.

From 300 cases of spontaneous intracerebral hematomas, 5 parameters are extracted they are: Age, sex, level of consciousness, localisation of hematomas and risk factors. A value, varying from 1 to 5 is attributed to each parameter, the sum of values give a score for each patient. This score allows the clinician to predict, within the early 48 hours, the vital prognosis. Survival chances are: 80% if score between 5 and 8; 60% if score between 9 and 13; 40% if score between 14 and 17; 20% if score equal or superior to 18.