[Ebola virus disease: Clinical presentation, prognosis and treatment]. / Maladie à virus Ebola : présentation clinique, aspects pronostiques et principes thérapeutiques.

The clinical spectrum of Ebola virus disease (EVD) ranges from very serious forms with organ failure and death within days to paucisymptomatic forms and perhaps even asymptomatic. The authors propose a focus on the clinical manifestations of EVD, on prognosis and on therapeutic aspects (excluding resuscitation). This work extracts from the literature the main data gathered during the 2014-2015 epidemic that raged in Guinea Conakry and Sierra Leone. These two countries, even if they are separated by a border, are one and the same population base. The characteristics of the epidemic in Liberia have not been analyzed. The authors have treated EVD patients in the health workers treatment center of Conakry and enrich this work about their personal experience.

Inappropriate bradycardia in Ebola virus disease.

BACKGROUND: As part of French assistance for the outbreak of Ebola virus disease in west Africa, a military treatment center for infected healthcare workers was deployed in Conakry, Guinea. Although some cases of bradycardia have been reported since the first Ebola outbreak, they have never been documented to our knowledge. We studied heart rhythm in patients with Ebola virus disease to analyze inappropriate bradycardia and discuss its mechanism. METHODS: Nine patients who tested positive for Ebola were admitted in March 2015. Baseline clinical data were noted at admission and twice a day during follow-up, and laboratory analyses (with troponin testing) were performed. RESULTS: At admission, patients had no or moderate tachycardia (pulse = 82 ± 27 bpm). Among them, a 32-year-old midwife admitted on her fourth day of symptoms had marked bradycardia: 43 bpm. ECG showed sinus bradycardia with no conduction disturbances or repolarization anomalies; findings were similar for the three other patients with bradycardia (< 60 bpm). During follow-up, her pulse gradually increased, as it did for the other three; all four recovered. DISCUSSION: Despite several factors likely to promote tachycardia, we observed no or only moderate tachycardia in all patients with Ebola. In our study, ECG recorded sinus rhythm, without significant node dysfunction or atrioventricular block. In the absence of any evidence of myocarditis, we discuss the possibility of a central nervous system cause, associated with encephalitis. CONCLUSION: We observed relative or marked bradycardia in our patients infected with Ebola. We hypothesize that its causal mechanism was encephalitis.

[Impact of headache among studied military population in Afghanistan deployed in the Kabul military field hospital]. / Impact des céphalées parmi le personnel militaire déployé au sein de l'hôpital médico-chirurgical de Kaboul.

INTRODUCTION: Headaches are a common reason for consultation with a prevalence of 30%. Few data exist for military personnel, including in situations of war operations. The main objective of this work was to measure the evolution of the impact of headache in such a context. MATERIALS AND METHODS: Two hundred and one personnel deployed in the Kaïa military field hospital in Afghanistan were recruited. A questionnaire designed to recognize headaches, supported by two quality of life scales (MIDAS and HIT-6) and a stress questionnaire were filled out before departure and upon return from missions. DISCUSSION: Sixty-three patients with headache were initially identified, of whom 52 remained symptomatic during the mission. The average total score of MIDAS before departure was 4 days and fell to 1.4 days upon return, with a mean measured change of 3.3 days. For HIT-6, the mean total score was 51.2 points initially and 51.9 points at the end of the mission with a mean change of-0.3 points. Nine patients without headache initially became symptomatic: MIDAS and HIT-6 were not affected. CONCLUSION: Thus, the impact of headache in the particular context of presence in a theater of operations was low: improved MIDAS score and the lack of influence on the HIT-6 score are underlined.

[Guillain-Barré syndrome and anti-hepatitis A vaccination]. / Syndrome de Guillain-Barré et vaccination contre l'hépatite A.

The Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuritis. The pathophysiology remains unknown but the existence of triggering factors such as external antigens is regularly suspected. We report the case of a 30-year-old patient with a past medical history of Graves disease, who presented with GBS within the month after receiving an anti-hepatitis A vaccination. GBS rarely happens after a hepatitis A vaccination. However, the responsibility of this vaccine should be considered in the clinical presentation of an acute polyradiculoneuritis.

[Anticonvulsant hypersensitivity syndrome and lamotrigine-associated anticonvulsant hypersensitivity syndrome]. / Syndrome d'hypersensibilité aux antiépileptiques. Cas particulier de la lamotrigine.

Anticonvulsant hypersensitivity syndrome (AHS) is defined by the association of high fever, cutaneous rash and multiorgan-system abnormalities (incidence, one in 1000 to one in 10,000 exposures). Fatal complications are described in 10%. This reaction usually develops 1 to 12 weeks after initiation of an aromatic anticonvulsant. Drug rash with eosinophilia and systemic symptoms (DRESS) can be discussed as differential diagnosis. Several hypotheses have been put forward to explain the pathogenesis of AHS. These include accumulation of toxic metabolites, antibody production and viral infection. The one based on toxic metabolites has found the greatest acceptance due to the fact that it can be proven by an in vitro test, the lymphocyte toxicity assay. In vivo, skin biopsies show characteristic findings of erythema multiform or typical leucocytoclastic angitis. The patch-test is positive in 80% of the cases. Lamotrigine-associated anticonvulsant hypersensitivity syndrome (LASH) is rare and was described in 1998. We report two new cases demonstrating the two particular configurations of apparition of LASH found in the 14 cases from the review of literature (Pubmed: anticonvulsant hypersensitivity syndrome - lamotrigine): high doses of lamotrigine (or lamotrigine in very young or old patients), and lamotrigine associated with another anti-epileptic (phenobarbital or sodium valproate). We discuss the links between DRESS after lamotrigine and LASH as illustrated in a new case.

[Idiopathic intracranial hypertension as a side effect of doxycycline]. / Hypertension intracrânienne bénigne secondaire à la prise de doxycycline.

Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment. We report a 26-year-old female with benign intracranial hypertension due to tetracycline, revealed by headaches and gradual visual loss. Standard investigations were unremarkable and favourable outcome after therapeutic lumbar puncture confirmed the diagnosis.

[Interferon-beta retinopathy]. / Rétinopathie liée à l'interféron-beta 1a sous-cutané

INTRODUCTION: Interferon-alpha associated retinopathy is an ocular complication of hepatitis C treatment well established in the literature. But, there are far fewer reports on multiple sclerosis related interferon-beta retinopathy. CASE REPORT: A 58-year-old male while receiving subcutaneous interferon-beta 1a 44microg thrice a week since 2001 for multiple sclerosis developed blurred vision. Visual acuity remained stable throughout the course of surveillance. Cotton wool spots were found on fundus exam. The retinopathy disappeared without specific therapy 2 months after discontinuing interferon injections. The diagnosis of interferon-beta 1a retinopathy was retained due to the lack of any other etiology. CONCLUSION: An ophthalmological examination including a fundus examination to search for a retinopathy should be undertaken when new ocular symptoms develop in a multiple sclerosis patient receiving interferon. An adverse event linked to interferon can be discussed and favored if the retinopathy resolves after interferon withdrawal.

[Neurological damage of brain tumor therapy]. / Complications neurologiques des traitements des tumeurs cérébrales.

Damage to the central nervous system induced by treatment of brain tumors is common and impairs the patient quality-of-life. Neurotoxicity is induced by synergistic effects of different cytotoxic treatments such as radiotherapy and chemotherapies administered concurrently or sequentially. Recent progress in the management of brain tumors has led to new neurotoxicities. The growing concern about the neuropsychological performance of patients has disclosed another type of brain damage which has been largely neglected to date. Neurological toxicity can be acute, requiring dose adaptation or a change of drugs. But it also often occurs late and can be irreversible. To date, treatments have been ineffective. The early diagnosis of neurotoxicity is thus a major challenge. Numerous clinical studies suggest an individual sensitivity which is not only related to age or vascular status, but also to genetic predisposition that remains to be detailed. Understanding the mechanisms of personal susceptibilities would be helpful in designing more tailored treatments. In this review we address the question of adverse effects of brain radiation as well as those of chemotherapy protocols which are particularly toxic for the central nervous system that is, methotrexate, platin and aracytin.

[Spontaneous low cerebospinal fluid pressure syndrome. A case report and literature review]. / Syndrome d'hypotension spontanée du liquide cérébrospinal. Revue de la littérature à propos d'un cas.

INTRODUCTION: Spontaneous low cerebrospinal fluid pressure syndrome is a spontaneous intracranial hypotension pressure due to a cerebrospinal fluid leak without any known dural effraction. It is clinically characterised by postural headaches relieved by supine position. We report a 38-year-old patient with this syndrome and review the literature. EXEGESIS: The diagnosis is sometimes difficult in atypical presentation of the syndrome and can lead to incapacitating chronic headache and rarely to complications. Cerebral magnetic resonance imaging has dramatically improved identification, diagnosis and management of this syndrome. Treatment is mainly based on blood patch realisation. Cerebrospinal fluid leak probably due to a spontaneous defect in the dural mater is suspected to be the main mechanism of this syndrome without any history of lumbar puncture or penetrating trauma. CONCLUSION: Early diagnosis, often easy on the basis of clinical characteristics of the headache may avoid complications.

[Listeria monocytogenes abscess of the brain]. / Abcès du tronc cérébral à Listeria monocytogenes.

INTRODUCTION: Listeriosis commonly involves the central nervous system. Meningoencephalitis and rhomboencephalitis are the most frequent manifestations. Brain abscesses are rare. CASE REPORT: We report the case of a 63-year-old man treated with steroids for a long period; he was hospitalized for hemiparesis, confusion and fever. Clinical examination revealed meningeal signs, right hemiparesis and Parinaud syndrome. Initial CT scan was normal. The CSF contained 520 white cells/mm3 with predominance of polymorphonuclear neutrophils. An acute meningo- rhombencephalitis in an immunodepressed patient was suggested. The diagnosis of listeriosis was confirmed by blood cultures. Amoxicillin and gentamycin were started. The outcome on day 4 was severe with coma and tetraparesis. Brain MRI revealed a left peduncle abscess which descended deep into the brain reaching the internal capsule. The final clinical outcome involved residual right hemiparesis and left oculomotor nerve (III) palsy. CONCLUSION: Brain stem abscess is an uncommon form of listerial central nervous system infection. Listeria monocytogenes infection should be considered in patients with altered cell-mediated immunity that develop local neurologic deficits, a diagnosis which pursued rapidly with repeated blood cultures. Successful treatment requires early antibiotic therapy with ampicillin and gentamycin.

Myoclonus-dystonia: clinical and electrophysiologic pattern related to SGCE mutations.

OBJECTIVE: To clarify the clinical and neurophysiologic spectrum of myoclonus-dystonia patients with mutations of the SGCE gene. METHODS: We prospectively studied 41 consecutive patients from 22 families with documented mutations of the SGCE gene. The patients had a standardized interview, neurologic examination, and detailed neurophysiologic examination, including surface polymyography, long-loop C-reflex studies, and EEG jerk-locked back averaging. RESULTS: We noted a homogeneous electrophysiologic pattern of myoclonus of subcortical origin with short jerks (mean 95 msec, range 25 to 256 msec) at rest, during action, and during posture; there were no features of cortical hyperexcitability (specifically no abnormal C-reflex response and no short-latency premyoclonic potential on back-averaging studies). Myoclonus was either isolated or associated with mild to moderate dystonia, and predominated in the neck/trunk or proximal upper limbs in most cases. We found that 22% of the patients had a spontaneous improvement in their dystonia before reaching adulthood and that hypotonia can occasionally be a presenting symptom of the disorder. CONCLUSION: We describe the myoclonus in patients with mutations in the SGCE gene and characterize the electrophysiologic pattern of this myoclonus. This pattern may help to improve the sensitivity of molecular tests and to define homogeneous populations suitable for inclusion in therapeutic trials.

[Eulenburg's paramyotonia congenita]. / Paramyotonie congénitale d'Eulenburg.

INTRODUCTION: Paramyotonia congenita is an autosomal dominant sodium channelopathy, caused by mutations in gene coding for muscle voltage-gated sodium channel alpha subunit. CASE REPORT: We report the case of a 38-year-old man who described since childhood muscle stiffness with attacks ok weakness induced by two provocative stimuli: cold exposure and exercise. It primarily concerned eyelids and hands, occasionally limbs. Family history suggested an autosomal dominant mode of transmission. Clinical examination revealed myotonia at the thenar eminence percussion. Generalized myotonic discharges were observed on electromyography. Molecular diagnosis reported an Arg1448Cys mutation in exon 24 in gene coding for muscle voltage-gated sodium channel alpha subunit (SCN4A) in chromosome 17. CONCLUSION: Paramyotonia congenita is not evolutive. Treatment is essentially preventive. Some medications could be proposed: membrane stabilizing agents like antiarrhythmic drugs (mexiletine, tocainide), or the carbonic anhydrase inhibitor (acetazolamide). Precautions may be taken during general anaesthesia because of diaphragm myotonia risk.