Un cas de crampe des écrivains dystonique combinée à un syndrome du canal carpien à Ouagadougou (Burkina Faso)

Introduction

Focal hand dystonia and carpal tunnel syndrome are linked to impairment of the central and peripheral nervous systems, respectively. We report a singular case combining these two entities.

Case report

A 44-year-old right-handed male pharmacy employee whose daily activity for 18 years was to fill out and staple insurance vouchers and unload and store boxes of medicines, presented, almost concomitantly, the combination of right focal hand dystonia and homolateral carpal tunnel syndrome.

Conclusion

This observation corroborates the results of experimental studies on repetitive manual activity which would be a source of central and peripheral changes, affecting the hand somatotopy in the sensory-motor cortex and the descending control of basal ganglia, explaining dystonia, and leading to median nerve compression related to repeated microtrauma, inflammatory reactions and fibrosis of the carpal tunnel.     

Validation de l’échelle d’insight Q8 et évaluation de la conscience de la maladie chez 121 patients hospitalisés en psychiatrie

Insight (awareness of disorder) is an important domain for research and practice in psychiatry. Several instruments to measure insight are currently used. We present here a short scale (8 items with open question) applied to a sample of 121 psychiatric inpatients which permitted to validate this questionnaire and to demonstrate its easiness and rapidity. This insight Q8 scale shows that insight is higher in free hospitalization (compared to compulsary hospitalization), in mood disorders (compared to schizophrenia), in dépression (compared to schizophrenia and mania), in married patients (compared to widowed and single patients), in case of good cognitive functions (MMSE score) and antecedents of attempted suicide. Awareness must be taken into account in all major mental disorders. Therapeutic alliance, treatment compliance, prognosis and risk of relapes depend largely on this dimension.     

Le diagnostic précoce des troubles autistiques de l’enfant en pratique

The work of the Unit for screening and assessment of early developmental disorders of Caen University Hospital aims at detecting all the autistic children in an administrative area as early as possible. The ambition would be to diagnose autistic syndromes before the age of two and a half, so that early treatment may benefit autistic children. The Unit activity is built around young children and their families in collaboration with professionals. Using standard and internationally accepted means for screening, diagnosis and assessment brings a maximum objectivity and allows comparisons. In addition to its task of diagnosing and assessing developmental disorders, the Unit is permanently concerned with training and research. Supplying information and training concerned professionals is a fundamental in improving early screening. As for research, it is to be carried out without any aetiological a priori and with the aim of clarifying the risk factors of infantile autism.     

Diagnostic pitfalls in carpal tunnel syndrome

Background

This review focuses on the main aspects of positive and differential diagnosis of carpal tunnel syndrome (CTS) in different clinical situations encountered in daily practice.

State of the art

Authentic CTS can be discovered in situations, which alter the usual presentation or therapeutic management. This is the case for instance in pregnant women or in the elderly subject or with acute motor forms where CTS discloses a focal intratunnel disorder (neuroma, lipoma, arterial condition, bone disorder) or a general disease (hereditary neuropathy, amylosis). In certain situations, the clinical manifestations suggest a more proximal compression of the medial nerve (round pronator, Struthers arcade, or superficial flexor) or an inflammatory condition (mononeuritis, inflammatory demyelinising neuropathy). Locoregional disease may also be involved, for instance a plexus (thoracobrachial outlet syndrome, post-radiation plexitis) or radicular condition. The clinical presentation of diffuse polyneuropathy with initial manifestations involving the upper limb (ganglioneuropathies, polyradiculoneuritis, small-fiber neuropathies) may also be misleading. Finally central conditions can sometimes be confused with CTS.

Conclusion

A rigorous physical examination and an electroneuromyogram are determining to avoid diagnostic pitfalls.     

Au-delà de la peau,un regard porté… L’échographie et la pulsion scopique machinique

Throughout pregnancy, the pregnant woman pre-invests then invests the future child, feels it, to represent him, and see, watch the child. As the pregnant woman feels her unborn child, her story and her fantasies about him become rich and voluptuous. Shoring body is important and the introduction of ultrasound, it will then be able to rely on this image, on this look beyond the skin, both in the investment process of the baby, that level representations are the signs. A psychic creation's process supports the process of “creating organic” and, step-by-step, the child is there, in reality, beyond the womb. The ultrasound image is therefore helping to fantasizing, in a psychic creation's work, but sometimes it can also lead to freezing of the same fantasizing, to “freeze frame” of this creative work became a prisoner of madness maternal lethal. Indeed, the side of the destructiveness, the ultrasound image can become a fetish and a trauma, as will be shown in the case of a patient named Mary.     

Les antidépresseurs dans le traitement de l’état de stress post-traumatique

Posttraumatic stress disorder (PTSD) can be a prevalent, often chronic and disabling condition with major consequences for the individual and society. Diverse therapeutic approaches made the object of controlled and randomized studies: the pharmacological approach, the psychotherapic approach (essentially cognitivobehavioral), and the approaches combining pharmacology and psychotherapy. This article tries to review the available empirical data on antidepressant approaches to PTSD (selective serotonin re-uptake inhibitors, monoamine-oxydase inhibitors, tricyclic antidepressants, serotonin-norepinephrine re-uptake inhibitor) and to provide some recommendations for clinical practice. Although a comprehensive search of PubMed/Medline databases revealed a great number of case reports and open-label trials, we decided to focus primarily on evidence obtained from randomized controlled trials to determine the most effective antidepressant treatments for PTSD. Antidepressant pharmacotherapy seems to have positive effect on certain symptoms of PTSD but their short-term efficacy is partial and there remains unknown data on their long-term effectiveness. The available data supports antidepressant medication as the first-line pharmacotherapy for PTSD, with selective serotonin re-uptake inhibitors monotherapy having the strongest body of empirical support. Other potential options include, serotonin-norepinephrine re-uptake inhibitor, selective alpha-2 presynaptic serotoninergic inhibitors, monoamine-oxydase inhibitors or tricyclic antidepressants, and the combination with other pharmacotherapy in refractory PTSD cases. It seems to exist a certain consensus on the fact that the treatment should be continued for one prolonged length of time, exceeding largely the three months duration usually used in the controlled and randomized studies. A 12-month treatment seems indicated in chronic PTSD with residual symptoms.     

Place de l’électroencéphalogramme dans l’état de mal épileptique

Recordings of the electroencephalogram (EEG) play a major role in the management of patients with status epilepticus (SE). The EEG contributes to the diagnosis of SE, can be used to identify differential and syndromic diagnoses, and sometimes provides the etiologic diagnosis. EEG is helpful in monitoring therapeutic management and is an essential component of the follow up. The interpretation of the EEG in a patient with refractory SE is difficult, requiring clinical experience in this domain. We discuss the different modalities of EEG recording and their indications in emergency situations.     

Écriture du traumatisme ou traumatisme de l’écriture ? (deux femmes, deux histoires, deux écritures)

Writing workshops for adult out-patients are held once a week at the Centre d’Étude de l’Expression in the Clinique des Maladies Mentales de l’Encéphale. The author, a specialist in trauma for ten years, has been leading the workshop since 1997. A theoretical reflexion centred on psychological trauma and writing was elaborated through two clinical studies.     

La question éthique du respect de l’autonomie

To what degree does Alzheimer's disease impair the capacity for autonomy? This paper briefly describes and discusses the answers of three authors (Paul Appelbaum, Ronald Dworkin and Agnieszka Jaworska). Even if those answers are different, it is suggested that one could try to use each of them in order to respect the affected persons’ autonomy at the different stages of the disease.     

Apport d’une démarche électroclinique dans le diagnostic de l’épilepsie chez une population de sujets âgés confus

Background

Delusion symptoms often occur in old people; epilepsy is one of the main reasons behind these acute episodes. Current guidelines and recommendations from the Academy of Medicine have proposed a double clinical and electroencephalographic approach. Recently, a working group of French experts has issued an electro-clinical scale. The aim of our study was to compare the usual approach with the new one based on the electro-clinical score.

Method

All EEG requests performed since December 2008 in Bretonneau Hospital for elderly people aged over 75 years for delusion syndromes were retained for this study.

Results

One hundred and fifteen old patients from a geriatric-hospital (age 83.5 ± 6.06 years) were included in this protocol. The classical diagnostic process yielded the diagnosis of epilepsy for 50 subjects. The electro-clinical scale confirmed the diagnosis of epilepsy in 30 patients and ruled it out in 29 patients.

Conclusion

This study underscores the importance of evidence-based medicine for the diagnosis of epilepsy in old people and points out the underuse of the new technical tool, EEG-monitoring, for the management of these patients.     

Reconnaissance de l’émotion faciale et schizophrénie

A broad range of deficits in interpersonal skills characterizes schizophrenia. A natural way to tackle these deficits is to explore the ability of schizophrenic patients to process stimuli that have a well-established psychosocial content: faces, for instance. Schizophrenia deficits in facial recognition and discrimination have been studied extensively and most investigators have pointed out that patients with schizophrenia perform less well than non-patients and psychiatric controls in numerous facial paradigms, including facial identity, emotion and age recognition tests. The extent of the schizophrenic deficit suggests the alteration of a processing mechanism common to all kinds of facial information and the configural information extraction process has then been regarded as a probable candidate. Nevertheless, only a few studies directly tested the hypothesis. In what follows, we draw a general schema of the schizophrenia deficit in facial processing, next we present a series of studies investigating the putative implication of configural information in the abnormal processing of facial emotion in the disease.     

Syndromes dysautonomiques du visage avec signe et syndrome d’Harlequin : étude de trois cas et revue de la littérature

Introduction

Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome.

Patients and methods

We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body.

Discussion

We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed.

Conclusion

Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.     

Prise en charge thérapeutique du syndrome de Gilles de la Tourette chez l’enfant et chez l’adulte

Tourette's syndrome is a neuro-psychiatric disorder which starts in childhood and is characterized by the association of motor and vocal tics of which the frequency, type and location vary in time. The numerous ways of neurotransmission of the physiopathology of this illness have been tested. Several psychiatric comorbidities have been described at length among which the attention deficit hyperactivity disorder and obsessional compulsive disorder are the most frequent. The decision to initiate a treatment must take into account numerous factors and notably the repercussions of the pathology on the social and occupational life of the patient which rest on an individual valuation of the illness. Care must first start with psychoeducation allowing a better understanding of the syndrome by the patients and their family. Medicinal therapeutics then intervenes to treat the weak to severe tics affecting the daily life of the patients. The choice of the molecule will differ according to the severity of the illness, to the age of the patient and to the comorbid associations. Psychotherapies such as behavior therapy and supportive therapy are an alternative to pharmacologic treatment. They can be employed alone and may be sufficient for some patients. They can be also associated with classic therapeutics in order to increase therapeutic effectiveness. Finally, deep cerebral stimulation may constitute a new and interesting way, but it is in its developmental stage and needs to be assessed. This treatment is restricted for the time being to cases resistant to the classical treatments and to patients meeting the requirements of eligibility for such treatment.     

Utilisation du lorazépam injectable dans le traitement de l’état de mal épileptique : étude comparative au sein d’hôpitaux pédiatriques francophones

Background

Injectable lorazepam (IL) is marketed in many countries but in France is only available within the framework of a compassionate use program for refractory status epilepticus. This study aims to evaluate the differences of pediatric use and status of IL in the hospitals of the Mother-Child French-speaking Network (Réseau mère-enfant de la francophonie, i.e., RMEF).

Methods

Inclusion criteria are: firstly, RMEF member; secondly, one site per town; thirdly, all the Assistance publique-Hôpitaux de Paris hospitals. After a phone-recruitment in each selected hospital, a survey was sent by e-mail. The data collected concerned the number of beds in the hospital, the official status of IL, its place in the therapeutic strategy, inhospital consumption in 2008 (in milligram) and the therapeutic alternatives.

Results

Among the 18 hospitals selected, 17 were contacted and 12 (70%) replied. IL is not marketed in Tunisia and Lebanon. In Switzerland, Canada and Belgium, IL is marketed and used in all the polled hospitals (6.2 to 48.0 mg per bed). In France, only the Robert Debré Hospital uses it (3.2 mg per bed). In the countries where it is marketed, IL was firstly prescribed for the studied indication. In the other countries, injectable diazepam was the first line treatment (six out of eight hospitals).

Discussion/conclusion

France is the only country where IL is available though not marketed. The pharmacokinetic data favor use of IL instead of its principal therapeutic alternative (injectable diazepam) but no currently available evidence concludes that IL is superior to diazepam in the management of pediatric status epilepticus. The official indication of IL in France (last intention) is in contradiction with its use in the countries where it is marketed and with the data of the literature in favor of the first intention. This works presents the first evaluation on the use of IL in pediatric status epilepticus in the RMEF hospitals. It highlights the discrepancies in the management of status epilepticus in comparable pediatric hospitals.     

Prise en charge d’un état de mal épileptique de l’enfant (nouveau-né exclu)

Convulsive status epilepticus in childhood is a life threatening condition with serious risk of neurological sequelae which constitutes a medical emergency. Clinical and experimental data suggest that prolonged seizures can have immediate and long-term adverse consequences on the immature and developing brain. So the child who presents with a continuous generalized convulsive seizure lasting greater than five minutes should be promptly treated. The outcome is mainly determined by the underlying etiology, age and duration of status epilepticus. In children the mortality from status epilepticus ranges from 3 to 5% and the morbidity is two-fold higher. Mortality and morbidity are highest with status epilepticus associated with central nervous system infections, which is the most important cause of status epilepticus. There are few evidence-based data to guide management decisions for the child with status epilepticus. Immediate goals are stabilization of airways, breathing and circulation and termination of seizures. Benzodiazepines remain the first-line drugs recommended for prompt termination of seizures. As intravenous lorazepam is not available in France, we suggest clonazepam as the best choice for initial therapy. Rectal diazepam or buccal midazolam remain important options. Intravenous phenytoin/fosphenytoin and phenobarbital are the second-line drugs. Phenytoin is being increasingly substituted by fosphenytoin, but pediatric data are scarce and fosphenytoin is not authorized for use in France below five years old. In children, phenytoin is often preferred to phenobarbital, even though no comparative studies have demonstrated a better efficacy. To manage status epilepticus refractory to a benzodiazepine and administration of phenytoin and/or phenobarbital, many pediatricians today prefer high-dose midazolam infusion rather than thiopental to minimize serious side effects from barbiturate anesthesia. There is no benefit/risk ratio to support the use of propofol for children with refractory status epilepticus.