[Bilateral diaphragmatic palsy due to Lyme neuroborreliosis]. / Une paralysie diaphragmatique bilatérale révélant une neuroborréliose de Lyme.

INTRODUCTION: Lyme disease is not uncommon and can sometimes progress to neurological complications. We report here an unusual case of bilateral diaphragmatic paralysis secondary to Lyme neuroborreliosis. CASE REPORT: A 79-year-old man was admitted to the intensive care unit for acute respiratory distress requiring intubation and the long-term use of nocturnal non-invasive ventilation. Three months beforehand he had been bitten by a tick and developed erythema migrans which was treated with Doxycycline for 10 days. This clinical presentation became complicated a few days later by the progressive onset of severe dyspnoea. At admission, chest radiography revealed bilateral elevation of the diaphragm. Pulmonary function tests revealed a severe restrictive disorder aggravated by decubitus. A diaphragmatic electromyogram showed bilateral axonal polyneuropathy of the phrenic nerves. IgG and IgM antibodies to Borrelia burgdorferi were detectable in serum and cerebrospinal fluid, leading to the diagnosis of Lyme disease. He was treated with intravenous ceftriaxone 2g per day for 21 days, leading to a substantial improvement in symptoms. CONCLUSION: In the presence of unilateral or bilateral diaphragmatic paralysis of undetermined aetiology, it seems relevant to perform Lyme serology in the blood and, in positive cases, to follow up with a lumbar puncture in order to detect intrathecal IgG synthesis.

Somatosensory evoked potentials in the assessment of peripheral neuropathies: Commented results of a survey among French-speaking practitioners and recommendations for practice.

BACKGROUND: Somatosensory evoked potentials (SSEPs) are increasingly performed for the assessment of peripheral neuropathies, but no practical guidelines have yet been established in this specific application. STUDY AIM: To determine the relevant indication criteria and optimal technical parameters for SSEP recording in peripheral neuropathy investigation. METHODS: A survey was conducted among the French-speaking practitioners with experience of SSEP recording in the context of peripheral neuropathies. The results of the survey were analyzed and discussed to provide recommendations for practice. RESULTS: SSEPs appear to be a second-line test when electroneuromyographic investigation is not sufficiently conclusive, providing complementary and valuable information on central and proximal peripheral conduction in the somatosensory pathways. CONCLUSIONS: Guidelines for a standardized recording protocol, including the various parameters to be measured, are proposed. CLINICAL RELEVANCE: We hope that these proposals will help to recognize the value of this technique in peripheral neuropathy assessment in clinical practice.

[Impact of a stroke-unit on rtPA use in a community hospital: a 3-year prospective study]. / Impact de la création d'une unité neurovasculaire sur l'utilisation du rtPA dans l'infarctus cérébral en centre hospitalier général : étude prospective sur trois ans.

INTRODUCTION: Although intravenous thrombolysis has been used for ischemic strokes since 2004 in our community hospital located in Pau (southwest of France), a specifically dedicated stroke-unit (SU) was created only recently in June 2010. We decided to collect prospective data to compare the use and efficacy of intravenous thrombolysis before and after the opening of this dedicated stroke unit. METHODS: Stroke patients with internal carotid artery territory involvement treated with intravenous thrombolysis were compared between two similar periods. The first period (called pre-SU period) stretched from January 2009 to June 2010. The second period (called SU period) stretched from June 2010 to October 2011. We collected prospectively all morbidity/mortality data as well as a modified Rankin score (mRS) three months later. RESULTS: During the pre-SU period, 21 strokes were treated with a mean NIHSS score of 15. Three months later, the mRS score was less than or equal to 2 for five patients, and greater than or equal to 3 for 12. A total of four patients died. In addition, two-thirds of patients (14 of 21) had suffered from notable complications at the initial phase of their stroke. During the SU period, 27 strokes were treated with a mean NIHSS score of 14. At 3 months, the mRS score less than or equal to 2 for 15 patients, and greater than or equal to 3 for nine other patients. A total of three patients died. During this second period, less than 50% of the patients (13 of 27) were not affected by any complication at the initial phase. Statistically, the results also show a better short-term (24 hours with NIHSS) and medium-term (3 months with NIHSS and mRS) clinical outcome for patients treated during the SU period. CONCLUSIONS: Instituting a dedicated stroke-unit helped improve outcome after ischemic strokes treated by intravenous thrombolysis. It also increased the number of patients and reduced the complications at the initial phase.

[Mutism and acute behavioral disorders revealing MELAS syndrome]. / Mutisme et troubles du comportement aigus révélant un syndrome MELAS.

INTRODUCTION: MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a rare genetic mitochondrial disease which can cause cerebral (cerebrovascular accident, migraine, mental deterioration..), sensorial (bilateral symmetrical deafness) and peripheral (muscular involvement, neuropathy) disorders potentially associated with diabetes, renal or cardiac disorders, or growth retardation. Eighty percent of the patients have the 3243 A>G mutation in the leucine RNA transfer gene. Clinical manifestations leading to discovery of the mutation can be extremely varied, affecting patients of different age groups. CLINICAL CASE: We report the case of a 49-year-old man who presented acute fits of confusion followed by mutism and praxic disorders. History taking revealed recently diagnosed type 2 diabetes, axonal neuropathy, and bilateral symmetrical deafness requiring hearing aids. The initial MRI showed FLAIR sequences with bi-parietal abnormalities, no signs of recent stroke on the DW/B10000 sequences, and basal ganglia calcifications. Blood tests and morphological findings ruled out a vascular origin. Search for lactic acidosis remained constantly negative in blood samples despite positive cerebrospinal fluid samples (N×3). The 3243 A>G mitochondrial DNA mutation was identified. The neuropsychological evaluation revealed a serious dysexecutive syndrome with a major impact on the patient's self sufficiency. CONCLUSION: Neurocognitive disorders are not common in MELAS syndrome. Brain MRI results and the presence of extra-neurological signs can be helpful for diagnosis.

[Ischemic strokes related to benign primitive cardiac tumours]. / Infarctus cérébraux révélant une tumeur bénigne primitive cardiaque.

BACKGROUND: We report three cases of ischemic cardioembolic strokes related to benign primary cardiac tumours (two fibromas and one fibroelastoma). CASE REPORTS: This is a retrospective study over a five years period (from December 2004 to December 2009) in a French community hospital. Data on hospital strokes were obtained from the informatics department. Three benign primary cardiac tumours were found as the cause of acute neurological manifestations: a 45-year-old woman with a fibroelastoma revealed by a brain infarction, a 29-year-old man with a myxoma revealed by a transient ischemic attack, and a 46-year-old woman with a myxoma revealed by a brain infarction. Rankin scores performed at least 18 months after cardiac tumour surgery were respectively of 0, 0 and 2. CONCLUSION: Our study confirms that this is a rare event even if those tumours seem to have a high embolic potential (myxomas). Anyway, long-term functional outcome seems to be good.

[Deep-brain stimulation of the internal pallidum in multiple system atrophy]. / Stimulation cérébrale profonde du pallidum interne dans l'atrophie multisystématisée.

INTRODUCTION: The experience with deep-brain stimulation (DBS) in multiple-system atrophy (MSA) is sparse and generally disappointing. DBS is currently not recommended in MSA and its use is often related to a misdiagnosis. OBSERVATION: We describe the outcome of bilateral DBS of the internal pallidum in a 46-year-old woman suffering from MSA that initially resembled Parkinson's disease with prominent levodopa-induced dyskinesias. DBS of the left internal pallidum was performed in 1998 after a ten-year clinical course and improved dyskinesias. Six months later, the right side was implanted. A few months after the second surgery, the patient progressively developed signs of cerebellar and dysautonomic impairment and MSA was diagnosed. CONCLUSION: Our observation confirms the ineffectiveness of DBS of the internal pallidum in MSA and even suggests a harmful effect. DBS remains contra-indicated in atypical parkinsonism.

[Lambert-Eaton Myasthenic Syndrome associated with vocal cord carcinoma]. / Syndrome myasthénique de Lambert-Eaton associé à un carcinome épidermoïde de la corde vocale.

INTRODUCTION: Lambert-Eaton Myasthenic Syndrome (LEMS) is an autoimmune channelopathy in which patients produce autoantibodies directed against voltage-gated calcium channels. LEMS is paraneoplastic in 50% of patients, most frequently associated with small cell lung carcinoma. We describe a case of paraneoplastic LEMS associated with a vocal cord carcinoma. OBSERVATION: A 64-year-old man developed in five months muscle weakness affecting gait. Clinical examination showed proximal muscular deficiency, areflexia and dysphonia. Electrophysiologic study showed potentiation greater than 500% after post exercise facilitation and 76 percent increment response at high-rate repetitive nerve stimulation (20Hz). Diagnosis of LEMS was confirmed by electrophysiologic study and anti-voltage gated calcium channel antibodies (90pM, positive value greater or equal to 70pM). Left vocal cord lesion histology showed epidermoid carcinoma. A combination of vocal cord tumor removal by endoscopy and treatment by pyridostigmine, 3-4 diaminopyridine and intravenous human immunoglobulin improved neurological symptoms. CONCLUSION: Paraneoplastic syndromes in association with cancers of the larynx and hypopharynx are unusual. Only two cases are reported with LEMS associated with larynx carcinoma. We describe an unusual case of LEMS associated with a left vocal cord carcinoma.

[Multiple system atrophy: prognostic factors in the "MSA-Aquitaine" cohort]. / Atrophie multisystématisée: survie et facteurs pronostiques dans la cohorte "MSA-Aquitaine".

INTRODUCTION: Multiple system atrophy (MSA) is a common cause of atypical parkinsonism, of poor prognosis. MSA is associated with short survival but data stemming from clinical or pathological studies are sparse and contrasted. Factors predicting survival in MSA are not fully established. We investigated the survival and prognostic factors of MSA in the cohort "MSA-Aquitaine". METHODS: This was a retrospective study of an unselected cohort of patients included throughout Aquitaine based on the Consensus Conference statement concerning MSA diagnostic criteria, with prospective follow-up on mortality. All patients received a standard clinical examination and disease history was collected through medical records and interviews of patients. Survival was ascertained by telephonic calls. RESULTS: From 1 November 1998 to 1 April 2002, we diagnosed 86 patients (43 men and 43 women) with "probable" or "possible" MSA. Median survival from study inclusion was 2.4 years and was 10.2 years from clinical onset, very similar to the other series. Low age at study, diabetes, dysphagia, Hoehn and Yahr stage 5 can predict shorter survival in patients with MSA. CONCLUSION: We confirm that the prognosis for MSA patients is poor and that some factors may predict shorter survival.

[Bullous localization of a lupus erythematosus induced by radiotherapy]. / Lupus érythémateux bulleux: rôle localisateur de la radiothérapie.

INTRODUCTION: Connective diseases induced or exacerbated by radiotherapy are not frequent. We report a particular erosive erythematosus lupus with lesions in the precise distribution of radiation therapy given for a breast cancer. OBSERVATION: An 80 year-old woman presented with painful erosive skin lesions of her breast which had been treated by irradiation 9 years before. The patient also had rheumatoid arthritis. A skin biopsy showed keratinocyte necrosis, acantholysis and a dermal lymphocytic infiltrate under the basal cell layer. Direct immunofluorescence showed granular deposition of IgG, IgM and C3 along the basal cell layer. Antinuclear antibodies were positive at a titre of 1: 1,000. Erythematosus lupus diagnosis was established and annular lesions secondarily appeared on her neck, back, and arms. A treatment with hydroxychloroquine and topical corticosteroïds was effective in 3 months. DISCUSSION: The unusual erosive lesions and their localization on a previously irradiated site suggest the role of X-rays in our observation, despite their late appearance after radiotherapy. Radiotherapy and erythematosus lupus affect the same target structures (basal cells and small dermal capillaries) and could have additive effects. The presence of rheumatoid arthritis in the past medical history may have exaggerated this complication.

Evidence for a functional renin-angiotensin system in full-term fetoplacental unit.

This investigation was performed to study the renin-angiotensin system in the human fetoplacental circulation. Full-term placentas from uncomplicated pregnancies were studied within 30 min of delivery. The umbilical artery and vein to a single placental cotyledon were cannulated and the artery perfused with RPMI media (0.764 ml/min). Angiotensin II caused a dose-dependent increase in perfusion pressure that was blunted by the administration of the competitive angiotensin II receptor antagonist saralasin. The properties of human placental angiotensin II receptors were further defined in binding studies performed on a crude membrane fraction of placental cotyledons. In experiments performed at 22 degrees C, saturable binding reached steady state at 30 min and was linear with protein concentration. Scatchard analysis of binding data indicated a single class of high-affinity binding sites. The potency order to competitive binding of analogues and antagonists of angiotensin II was [Ile5]angiotensin II = [Sar1, Ala8]-angiotensin II greater than [Val5]angiotensin II greater than angiotensin III greater than angiotensin II-(3-8) hexapeptide. Further evidence for the physiological significance of angiotensin II binding sites was provided by measurements of the circulating components of the renin-angiotensin system in umbilical venous blood (n = 7). Plasma renin activity, angiotensin I, angiotensin-converting enzyme activity, angiotensin II, and aldosterone were each present in elevated amounts. These experiments provide evidence for an active renin-angiotensin system in the human fetal circulation that may modulate placental perfusion and function under physiological conditions.

Discordant anencephalus in a Pergonal-induced triplet pregnancy.

A patient with a triplet pregnancy had one infant affected by anencephalus. This pregnancy resulted from ovulation stimulation by gonadotropins. Several cases of association between anencephalus and induction of ovulation by clomiphene have been reported. The association with gonadotropins, however, has been reported only once. The event was prenatally diagnosed by ultrasound studies in the first and second trimesters. The association of anencephalus with ovulation stimulation is discussed briefly.