Oculomotor nerve schwannoma associated with ophthalmoplegic migraine.

PURPOSE: To describe a patient with an oculomotor nerve schwannoma who had symptoms of ophthalmoplegic migraine. METHODS: Case report. RESULTS: A 23-year-old woman had a history of recurrent headache accompanied by transient right oculomotor palsy since age 7 years. Ophthalmoplegic migraine was diagnosed. She was subsequently found to have a structural lesion of her right oculomotor nerve on magnetic resonance imaging. The magnetic resonance image characteristics were consistent with schwannoma originating from the oculomotor nerve. CONCLUSIONS: This case illustrates that an intrinsic lesion of the oculomotor nerve (schwannoma) may be associated with a painful relapsing-remitting oculomotor palsy mimicking the clinical syndrome of ophthalmoplegic migraine.

Impending status epilepticus and anxiety in a pregnant woman treated with levetiracetam.

Levetiracetam (LEV) has been considered to undergo no significant change in bioavailability during pregnancy; however, it was recently demonstrated to display modifications leading to a drop in its serum level. We describe a patient who displayed impending status epilepticus following a fall in her LEV level during the first trimester. The oral LEV dosage was increased, and phenytoin and benzodiazepines were transiently prescribed. She experienced severe anxiety and an unbearable fear over the deleterious consequences for her baby despite repeated, reassuring explanations. Her anxiety was so strong that she aborted electively shortly after leaving the hospital. This observation emphasizes the need for LEV level monitoring during pregnancy to prevent unexpected seizure relapses. The rapid increase in levetiracetam dosage in parallel with the loss of seizure control is suspected of facilitating the induction of significant psychiatric changes.

Als Marie Bonaparte sich taub stellte ...

Between 1927 and 1931 Marie Bonaparte had herself operated upon her clitoris three times. She did so against Freud's advice with whom she was in analysis. Among psychoanalysts these operations are still often regarded as "errors" or aberrations. But for Marie Bonaparte, who was in various ways familiar with physics and a somatic approach, surgery was the first choice, psychoanalysis only a possible alternative. She was not impressed by the skepticism of her colleagues, and adhered even more emphatically to her own strategy

Oncology clinicians' defenses and adherence to communication skills training with simulated patients: an exploratory study.

The aim of this exploratory study was to assess the impact of clinicians' defense mechanisms-defined as self-protective psychological mechanisms triggered by the affective load of the encounter with the patient-on adherence to a communication skills training (CST). The population consisted of oncology clinicians (N = 31) who participated in a CST. An interview with simulated cancer patients was recorded prior and 6 months after CST. Defenses were measured before and after CST and correlated with a prototype of an ideally conducted interview based on the criteria of CST-teachers. Clinicians who used more adaptive defense mechanisms showed better adherence to communication skills after CST than clinicians with less adaptive defenses (F(1, 29) = 5.26, p = 0.03, d = 0.42). Improvement in communication skills after CST seems to depend on the initial levels of defenses of the clinician prior to CST. Implications for practice and training are discussed. Communication has been recognized as a central element of cancer care [1]. Ineffective communication may contribute to patients' confusion, uncertainty, and increased difficulty in asking questions, expressing feelings, and understanding information [2, 3], and may also contribute to clinicians' lack of job satisfaction and emotional burnout [4]. Therefore, communication skills trainings (CST) for oncology clinicians have been widely developed over the last decade. These trainings should increase the skills of clinicians to respond to the patient's needs, and enhance an adequate encounter with the patient with efficient exchange of information [5]. While CSTs show a great diversity with regard to their pedagogic approaches [6, 7], the main elements of CST consist of (1) role play between participants, (2) analysis of videotaped interviews with simulated patients, and (3) interactive case discussion provided by participants. As recently stated in a consensus paper [8], CSTs need to be taught in small groups (up to 10-12 participants) and have a minimal duration of at least 3 days in order to be effective. Several systematic reviews evaluated the impact of CST on clinicians' communication skills [9-11]. Effectiveness of CST can be assessed by two main approaches: participant-based and patient-based outcomes. Measures can be self-reported, but, according to Gysels et al. [10], behavioral assessment of patient-physician interviews [12] is the most objective and reliable method for measuring change after training. Based on 22 studies on participants' outcomes, Merckaert et al. [9] reported an increase of communication skills and participants' satisfaction with training and changes in attitudes and beliefs. The evaluation of CST remains a challenging task and variables mediating skills improvement remain unidentified. We recently thus conducted a study evaluating the impact of CST on clinicians' defenses by comparing the evolution of defenses of clinicians participating in CST with defenses of a control group without training [13]. Defenses are unconscious psychological processes which protect from anxiety or distress. Therefore, they contribute to the individual's adaptation to stress [14]. Perry refers to the term "defensive functioning" to indicate the degree of adaptation linked to the use of a range of specific defenses by an individual, ranging from low defensive functioning when he or she tends to use generally less adaptive defenses (such as projection, denial, or acting out) to high defensive functioning when he or she tends to use generally more adaptive defenses (such as altruism, intellectualization, or introspection) [15, 16]. Although several authors have addressed the emotional difficulties of oncology clinicians when facing patients and their need to preserve themselves [7, 17, 18], no research has yet been conducted on the defenses of clinicians. For example, repeated use of less adaptive defenses, such as denial, may allow the clinician to avoid or reduce distress, but it also diminishes his ability to respond to the patient's emotions, to identify and to respond adequately to his needs, and to foster the therapeutic alliance. Results of the above-mentioned study [13] showed two groups of clinicians: one with a higher defensive functioning and one with a lower defensive functioning prior to CST. After the training, a difference in defensive functioning between clinicians who participated in CST and clinicians of the control group was only showed for clinicians with a higher defensive functioning. Some clinicians may therefore be more responsive to CST than others. To further address this issue, the present study aimed to evaluate the relationship between the level of adherence to an "ideally conducted interview", as defined by the teachers of the CST, and the level of the clinician' defensive functioning. We hypothesized that, after CST, clinicians with a higher defensive functioning show a greater adherence to the "ideally conducted interview" than clinicians with a lower defensive functioning.

A Moroccan woman with severe electrolyte disorder

The case of a Moroccan woman, age 25, who came into the emergency department with clinical tetany, is presented. She had experienced muscle spasms and paresthaesia of the upper limb over the previous few days. She had also experienced major diarrhoea for the previous 3 weeks. Investigations revealed a severe electrolyte disorder.

Predictors of HIV-protection behaviour in HIV-positive men who have sex with casual male partners: a test of the explanatory power of an extended Information-Motivation-Behavioural Skills model.

This prospective study applies an extended Information-Motivation-Behavioural Skills (IMB) model to establish predictors of HIV-protection behaviour among HIV-positive men who have sex with men (MSM) during sex with casual partners. Data have been collected from anonymous, self-administered questionnaires and analysed by using descriptive and backward elimination regression analyses. In a sample of 165 HIV-positive MSM, 82 participants between the ages of 23 and 78 (M=46.4, SD=9.0) had sex with casual partners during the three-month period under investigation. About 62% (n=51) have always used a condom when having sex with casual partners. From the original IMB model, only subjective norm predicted condom use. More important predictors that increased condom use were low consumption of psychotropics, high satisfaction with sexuality, numerous changes in sexual behaviour after diagnosis, low social support from friends, alcohol use before sex and habitualised condom use with casual partner(s). The explanatory power of the calculated regression model was 49% (p<0.001). The study reveals the importance of personal and social resources and of routines for condom use, and provides information for the research-based conceptualisation of prevention offers addressing especially people living with HIV ("positive prevention").

Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.

Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while receiving corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclophosphamide, which led to a dramatic improvement in her clinical condition and respiratory function tests. The increase in vital capacity was one of the highest among 35 published cases of SLS. Thus, restoring a near-normal lung function is an achievable goal in SLS, and the use of rituximab, with or without concomitant cyclophosphamide, certainly deserves further study in this setting.

Early occurrence of lung adenocarcinoma and breast cancer after radiotherapy of a chest wall sarcoma in a patient with a de novo germline mutation in TP53.

We report a 26-year-old female patient who was diagnosed within 4 years with chest sarcoma, lung adenocarcinoma, and breast cancer. While her family history was unremarkable, DNA sequencing of TP53 revealed a germline de novo non-sense mutation in exon 6 p.Arg213X. One year later, she further developed a contralateral ductal carcinoma in situ, and 18 months later a jaw osteosarcoma. This case illustrates the therapeutic pitfalls in the care of a young cancer patient with TP53 de novo germline mutations and the complications related to her first-line therapy. Suggestion is made to use the less stringent Chompret criteria for germline TP53 mutation screening. Our observation underlines the possibly negative effect of radiotherapy in generating second tumors in patients with a TP53 mutation. We also present a review of six previously reported cases, comparing their cancer phenotypes with those generally produced by TP53 mutations.

Persistence of retinal function after intravitreal melphalan injection for retinoblastoma.

BACKGROUND: The risk/benefit profile of intravitreal melphalan injection for treatment of active vitreous seeds in retinoblastoma remains uncertain. We report clinical and electroretinography results after 6 months of one patient who has shown a favorable initial clinical response to intravitreal melphalan injections for treatment of refractory vitreous seeds. METHODS: Clinical case report. PATIENT: The patient presented at age 17 months with bilateral retinoblastoma [OD: International Classification (ICRB) group E, Reese-Ellsworth (R-E) class Vb; OS: ICRB D, R-E Vb] with no known prior family history. The right eye was enucleated primarily. The patient received systemic chemotherapy and extensive local treatment to the left eye. Ten months later, she presented with recurrent disease, including fine, diffuse vitreous seeds. Tumor control was established with intra-arterial chemotherapy and local treatment. Subsequent recurrence was treated with further intra-arterial chemotherapy, local treatment, and plaque radiotherapy with iodine-125. Persistent free-floating spherical vitreous seeds were treated with 4 cycles of intravitreal melphalan injection via the pars plana, with doses of 30, 30, 30, and 20 μg. RESULTS: After 6 months of follow-up, the left eye remained free of active tumor. Visual acuity was 20/40. Photopic ERGs amplitudes were unchanged compared with those recorded prior to the intravitreal injection treatments. CONCLUSIONS: Intravitreal melphalan injection for refractory spherical vitreous seeds of retinoblastoma with favorable tumor response is compatible with good central visual acuity and preservation of retinal function as indicated by photopic ERG recordings.

Analyse de la prise en charge médicale et de l'impact économique des patients ayant présentés des coûts extrêmes dans un hôpital universitaire [Analysis of the medical management and the economic impact of patients who have represented extreme costs at a university hospital]

Les coûts de traitement de certains patients s'avèrent extrêmement élevés, et peuvent faire soupçonner une prise en charge médicale inadéquate. Comme I'évolution du remboursement des prestations hospitalières passe à des forfaits par pathologie, il est essentiel de vérifier ce point, d'essayer de déterminer si ce type de patients peut être identifié à leur admission, et de s'assurer que leur devenir soit acceptable. Pour les années 1995 et 1997. les coûts de traitement dépassant de 6 déviations standard le coût moyen de la catégorie diagnostique APDRG ont été identifiés, et les dossiers des 50 patients dont les coûts variables étaient les plus élevés ont été analysés. Le nombre total de patients dont I'hospitalisation a entraîné des coûts extrêmes a passé de 391 en 1995 à 328 patients en 1997 (-16%). En ce qui concerne les 50 patients ayant entraîné les prises en charge les plus chères de manière absolue, les longs séjours dans de multiples services sont fréquents, mais 90% des patients sont sortis de l'hôpital en vie, et près de la moitié directement à domicile. Ils présentaient une variabilité importante de diagnostics et d'interventions, mais pas d'évidence de prise en charge inadéquate. En conclusion, les patients qualifiés de cas extrêmes sur un plan économique, ne le sont pas sur un plan strictement médical, et leur devenir est bon. Face à la pression qu'exercera le passage à un mode de financement par pathologie, les hôpitaux doivent mettre au point un système de revue interne de I'adéquation des prestations fournies basées sur des caractéristiques cliniques, s'ils veulent garantir des soins de qualité. et identifier les éventuelles prestations sous-optimales qu'ils pourraient être amenés à délivrer. [Auteurs] Treatment costs for some patients are extremely high and might let think that medical care could have been inadequate. As hospital financing systems move towards reimbursement by diagnostic groups, it is essential to assess whether inadequate care is provided, to try to identify these patients upon admission, and make sure that their outcome is good. For the years 1995 and 1997, treatment costs exceeding by 6 standard deviations the average cost of their APDRG category were identified, and the charts of the 50 patients with the highest variable costs were analyzed. The total number of patients with such extreme costs diminished from 391 in 1995 to 328 in 1997 (-16%). For the 50 most expensive patients, long stays in several services were frequent, but 90% of these patients left the hospital alive, and about half directly to their home. They presented an important variation in diagnoses and operations, but no evidence for inadequate care. Thus, patients qualified as extreme from an economic perspective cannot be qualified as such from a medical perspective, and their outcome is good. To face the pressure linked with the change in financing system, hospitals must develop an internal review system for assessing the adequacy of care, based on clinical characteristics, if they want to guarantee good quality of care and identify potentially inadequate practice.