Botulinum toxin therapy improves masseter spasticity in Amyotrophic Lateral Sclerosis.

Fifteen ALS patients, with troublesome symptoms linked to masseter spasticity, benefited from BoNT-A injections in each masseter. Based on the medical records of patients, the effect of the first injection was assessed one month later. We retrospectively collected information for 12 patients. Eight of them reported a beneficial effect after the injection for the following symptoms: trismus, tongue, lip and cheek biting, and jaw clonus. Five patients indicated that dental care was easier after injection. Our study showed that injections of BoNT-A unequivocally reduced masseter spasticity in ALS patients who subsequently enjoyed greater comfort in their daily living.

The "Neurological Hat Game": A fun way to learn the neurological semiology.

INTRODUCTION: In-class courses are deserted by medical students who tend to find it more beneficial to study in books and through online material. New interactive teaching methods, such as serious games increase both performance and motivation. We developed and assessed a new teaching method for neurological semiology using the "Hat Game" as a basis. METHODS: In this game, two teams of second-year medical students are playing against one another. The game is played with a deck of cards. A neurological symptom or sign is written on each card. Each team gets a predefined period of time to guess as many words as possible. One member is the clue-giver and the others are the guessers. There are three rounds: during the first round, the clue-giver uses any descriptive term he wants and as many as he wants to make his team guess the maximum number of words within the allocated time. During the second round, the clue-giver can only choose one clue-word and, during the third round, he mimes the symptom or sign. The team that has guessed the most cards wins the game. To assess the efficacy of this learning procedure, multiple choices questions (MCQs) were asked before and after the game. Exam results of second-year students on their final university Neurology exam were analyzed. A satisfaction survey was proposed to all participating students. RESULTS: Among 373 students, 121 volunteers (32.4%) were enrolled in the "Neurology Hat Game" and 112 attended the game. One hundred and seven of the 112 students completed the MCQs with a significant improvement in their responses after the game (P<0.001). The 112 students who completed the satisfaction self-administered questionnaire were very satisfied with this funny new teaching method. CONCLUSIONS: Teaching neurological semiology via the "Hat Game" is an interesting method because it is student-centered, playful and complementary to the lecturer-centered courses. A randomized controlled study would be necessary to confirm these preliminary results.

Simulated fate of catchment-derived sediment on the Great Barrier Reef shelf.

Numerical experiments using a 3D model of fine sediment transport in the Great Barrier Reef region indicate deposition of the bulk mass of catchment sediments from river plumes within a few tens of kilometres from river mouths. A very fine fraction of easily resuspended catchment sediment has a capacity to propagate over much greater distances reaching out into the mid-shelf and outer-shelf regions. The model suggests such particles, instrumental to the development of low density flocs in the marine environment, can play a critical role in altering optical properties of water masses over the shelf during wet years. The mid-term (4 year) impact of Great Barrier Reef catchments on the probability of suspended sediment concentration exceeding the ecologically significant trigger value of 2 mg/L is confined to inshore regions adjacent to river mouth locations.

Comparative Study of MRI Biomarkers in the Substantia Nigra to Discriminate Idiopathic Parkinson Disease.

BACKGROUND AND PURPOSE: Several new MR imaging techniques have shown promising results in patients with Parkinson disease; however, the comparative diagnostic values of these measures at the individual level remain unclear. Our aim was to compare the diagnostic value of MR imaging biomarkers of substantia nigra damage for distinguishing patients with Parkinson disease from healthy volunteers. MATERIALS AND METHODS: Thirty-six patients and 20 healthy volunteers were prospectively included. The MR imaging protocol at 3T included 3D T2-weighted and T1-weighted neuromelanin-sensitive images, diffusion tensor images, and R2* mapping. T2* high-resolution images were also acquired at 7T to evaluate the dorsal nigral hyperintensity sign. Quantitative analysis was performed using ROIs in the substantia nigra drawn manually around the area of high signal intensity on neuromelanin-sensitive images and T2-weighted images. Visual analysis of the substantia nigra neuromelanin-sensitive signal intensity and the dorsolateral nigral hyperintensity on T2* images was performed. RESULTS: There was a significant decrease in the neuromelanin-sensitive volume and signal intensity in patients with Parkinson disease. There was also a significant decrease in fractional anisotropy and an increase in mean, axial, and radial diffusivity in the neuromelanin-sensitive substantia nigra at 3T and a decrease in substantia nigra volume on T2* images. The combination of substantia nigra volume, signal intensity, and fractional anisotropy in the neuromelanin-sensitive substantia nigra allowed excellent diagnostic accuracy (0.93). Visual assessment of both substantia nigra dorsolateral hyperintensity and neuromelanin-sensitive images had good diagnostic accuracy (0.91 and 0.86, respectively). CONCLUSIONS: The combination of neuromelanin signal and volume changes with fractional anisotropy measurements in the substantia nigra showed excellent diagnostic accuracy. Moreover, the high diagnostic accuracy of visual assessment of substantia nigra changes using dorsolateral hyperintensity analysis or neuromelanin-sensitive signal changes indicates that these techniques are promising for clinical practice.

[Normal pressure hydrocephalus: A review and practical aspects]. / Hydrocéphalie à pression normale: mise au point et aspects pratiques.

Idiopathic normal pressure hydrocephalus is a chronic disorder affecting the elderly. It is defined by Adams and Hakim's triad in addition to ventricular dilation visible by brain imaging and normal cerebrospinal fluid pressure during lumbar puncture. The objective of this review was to propose a standard of care for idiopathic normal pressure hydrocephalus based on an extensive literature review conducted on 459 articles published over the last 10 years. Those articles were obtained by searching for the keywords "normal pressure hydrocephalus" in the PubMed database and selecting all the articles published in English or in French. The diagnosis of idiopathic normal pressure hydrocephalus is difficult because of commonly associated diseases, such as Alzheimer's disease and microangiopathy. Brain MRI is one of the key procedures to assist in the diagnosis of idiopathic normal pressure hydrocephalus. Indeed, the presence of certain MRI features is highly predictive of a positive tap test and shunt responsiveness. Nevertheless, tap test remains the standard of care for diagnosis. Continuous cerebrospinal fluid drainage test is an alternative because it improves the sensitivity of diagnosis (but is a more complicated test to perform). Alzheimer's biomarkers dosing in the cerebrospinal fluid seems interesting when diagnosis remains uncertain: the presence of Alzheimer's profile of the biological markers is predictive of a lower response to the tap test.

Dapsone for thrombocytopenic purpura related to human immunodeficiency virus infection.

PURPOSE: The purpose of this study was to determine the effect of dapsone on platelet count in patients with human immunodeficiency virus (HIV)-related autoimmune thrombocytopenia. PATIENTS AND METHODS: Eleven patients with HIV-related thrombocytopenia received dapsone (50 to 125 mg/day) for 2 to 43 months. Patients with the acquired immunodeficiency syndrome were not enrolled. RESULTS: Of the 11 patients, six developed platelet counts above 50 X 10(9)/L and did not require any other specific therapy. No significant side effects were observed. CONCLUSION: We conclude that dapsone may be effective in some patients with HIV-related thrombocytopenia.

Increase in plasma concentration of plasminogen activator inhibitor, fibrinogen, von Willebrand factor, factor VIII:C and in erythrocyte sedimentation rate with age.

Elderly patients have previously been shown to have an increased plasma concentration of tissue plasminogen activator (t-PA) antigen (t-PA Ag). Since the concentration of t-PA Ag depends on both free t-PA and t-PA complexed with inhibitors, mainly plasminogen activator inhibitor (PA inhibitor), we have investigated the relationship between the plasma concentration of PA inhibitor and age in 20 elderly and 20 young individuals. Elderly individuals showed a slight increase in PA inhibitor, in parallel with increase on others, acute-phase proteins, fibrinogen, von Willebrand factor, factor VIII:C, and the erythrocyte sedimentation rate. The increase in PA inhibitor as well as other acute-phase proteins in the elderly may be significant in relation to the increased incidence of thrombotic disease.

New parameters in erythrocyte counting. Value of histograms.

In this report we rate a new, third-generation automated hematology system (Technicon Instruments H-1) that can furnish a full range of values, including erythrocyte parameters and a leukocyte differential count. Particular attention is focused on erythrocyte morphometric parameters, including measurement of cell size and hemoglobin content on a cell-by-cell basis. We assess the usefulness of new parameters derived from these measurements, such as mean corpuscular volume and red blood cell distribution width, which characterize cell size, and mean corpuscular hemoglobin concentration, and hemoglobin distribution width, which characterize cell hemoglobinization in evaluating normal and abnormal subjects. The value of these parameters in classifying anemias is assessed in our patient population that includes those with iron deficiency anemias and thalassemias, as well as other forms of anemia.

[Peripheral facial paralysis and post-antirabies-vaccination polyneuroradiculitis (author's transl)]. / Paralysies faciales périphériques et polyradiculonévrites post-vaccino-rabiques. A propos d'un cas de diplégie faciale avec dissociation albumino-cytologique.

A case of facial diplegia with albuminocytologic dissociation of cerebrospinal fluid after antirabies vaccination prompts the author to attempt a retrospective study of post-antirabies-vaccination neuropathies, with facial or extensive involvement. Fifty-seven cases are considered. Irrespective of vaccine type, certain clinical similarities emerge, notably the age of occurrence (adult), the early onset of the paralysis (during vaccine therapy or in the following week), and the prognosis (good survival rate, severity of functional impairment varying with extent of involvement). The author attributes the main pathogenic role to the peripheral myelinic basic protein contained in varying proportions in conventional vaccines, the corollary being that such accidents should disappear completely with the new vaccination based on tissue culture material. A therapy programme comprising the discontinuation of vaccine therapy and administration of corticoids is proposed.

[Detection of platelet antibodies in idiopathic thrombopenic purpura]. / Détection des anticorps antiplaquettaires dans le purpura thrombopénique idiopathique.

The authors report the results of Dixon's assay modified by Follea in 24 cases of idiopathic thrombocytopenic purpura. The level of immunoglobulin G bound to platelet membrane was increased in 91 p. 100 of patients in the acute phase of the disease. An inverse correlation was demonstrated between platelet-bound antibody levels and platelet count as well as platelet survival. In all cases of refractory idiopathic thrombocytopenic purpura, the assay was positive and the mean level was higher. When the platelet count improved after prednisone therapy or after splenectomy, the level decreased. Platelet antibody determination seems to be useful for predicting the course of the disease. Patients with normal immunoglobulin G bound to platelet might have only C3, IgM or IgA. Dixon's assay is not specific to idiopathic thrombopenic purpura, since it is positive in other types of thrombocytopenia, but immunoglobulin G bound to platelet probably represents specific antiplatelet antibodies.

[Extreme hypogammaglobulinemia disclosing large granular lymphocytes with hepatosplenorenal infiltration]. / Hypogammaglobulinémie extrême révélant une leucémie à grands lymphocytes granuleux avec infiltration hépato-spléno-rénale.

We report a case of large granular lymphocytosis, or chronic "natural killer" lymphocytosis, a newly described entity. We were able to demonstrate the proliferative character of the disease by the finding of karyotype abnormalities. This case was remarkable for the pre-existence, for at least three years, of severe hypogammaglobulinaemia, for the very slow course of the proliferative process and for the progressive and tumoral infiltration of the spleen and liver, then kidney.

[Acquired hemophilia caused by antifactor VIIIc antibodies. Apropos of 3 cases]. / "Hémophilie acquise" par anticorps antifacteur VIII c. A propos de 3 observations.

In connection with three observations, we discuss clinical and biological characteristics of "acquired haemophilia". We insist on therapeutics problems: in all the cases corticosteroid and or immunosuppressive drugs are indicated: haemorrhagic accidents treatment depend on the clinical state severity and on the inhibitor concentration: indications of substitutive therapy, plasmapheresis and activated concentrate are considered.

[A new case of Urbach-Wiethe disease. Electron-microscopy study]. / Un nouveau cas de maladie d'Urbach Wiethe. Etude en microscopie électronique.

A new case of Urbach Wiethe disease is reported. The diagnosis of this disorder, despite the existence of typical histological connective tissue deposits, was recognized late. The authors review the morphological characteristics of the disease, emphasizing that atypical clinical presentations may occur which underline the importance of the histological examination.

[Cushing's disease successfully treated by transphenoidal hypophysectomy since eight years (author's transl) (proceedings)]. / Maladie de Cushing traitée par hypophysectomie transphénoïdale. Guérison avec 8 ans de recul

The authors show a case of true Cushing's disease by bilateral adrenal hyperplasia without pituitary tumor which is cured for eight years by only treated with transphenoidal hypophysectomy. They think that reaching by surgery, the nervous afferences from hypothalamus to hypophysis could play a part in ameliorating the pathologic relations which existed between two centers.

[Treatment of idiopathic thrombocytopenic purpura and thrombocytopenia associated with the HIV virus using anti-Rhesus immunoglobulins]. / Traitement du purpura thrombocytopénique idiopathique et des thrombocytopénies liées au VIH par les immunoglobulines anti-Rhésus.

Fourteen Rhesus-positive patients with idiopathic thrombocytopenic purpura or thrombocytopenia associated with human immunodeficiency virus (HIV) infection were treated with intravenous Rhesus antibodies. The mean total dose was 6,800 micrograms administered over a 3 to 6 days' period. A significant rise in platelet count was observed in 13 of the 14 Rhesus-positive patients, but not in 3 Rhesus-negative patients used as controls. This rise was usually transient, lasting less than 3 weeks, but it persisted for more than 3 months in 2 cases. Treatment was well tolerated; transient signs of haemolysis appeared in 11 patients who did not require transfusion. Rhesus antibodies are quick-acting, the number of platelets being doubled as early as the second day of treatment. Interaction between antibody-coated red blood cells and macrophages is a possible mode of action and a feasible therapeutic approach in selected patients with autoimmune of HIV-mediated thrombocytopenia.

[Anemia and dysmyelopoiesis with marker chromosome and transferrin receptor anomaly]. / Anémie et dysmyélopoïèse avec chromosome marqueur et anomalie du récepteur de la transferrine.

A 64-year old man presented with microcytic hypochromic aplastic acquired anemia without iron depletion. His bone marrow was hypercellular with dyserythropoiesis and no stainable iron deposits. 59 Fe incorporation by erythroblasts was reduced, and the karyotype revealed an aneuploidy with marker chromosome. After study of transferrin receptor with specific antibody, we conclude that the receptor presents a functional defect.