Leucine-Rich Glioma-Inactivated 1 Encephalitis: Broadening the Sphere

Background: Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a rare entity. Its typical features are seizures, faciobrachial dystonic seizures (FBDS), cognitive impairment, and personality changes. Case report: We report the case of a 66-year-old man with an unusual presentation, consisting of two types of FBDS, one starting in the foot and the other consisting of asynchronous myoclonic and dystonic jerks of the face triggered by noise and chin stimulation. The patient displayed no personality changes or cognitive impairment. Discussion: LGI1 encephalitis is a heterogeneous disease. Many different forms of FBDS may be observed, and these seizures can be the only symptom. This type of encephalitis should be suspected in presenting very frequent episodic events with dystonic features, regardless of the part of the body affected

Pregnancy after renal transplantation: a retrospective study at the military hospital of Tunis from 1992 to 2011

Introduction: Our study objective was to analyze the optimum conditions for pregnancy in kidney transplanted women. For that, we conducted a retrospective study was from 1992 to April 2011 about 17 pregnancies in 12 kidney transplanted patients followed in the Department of Obstetrics and Gynecology and Organ Transplant Unit of the Military Hospital of Tunis. Methods: We studied nephrological parameters and obstetric pathologies encountered during pregnancy and the potential impact of pregnancy on graft. Our main outcome measures were: time between renal transplantation and conception, birth of a living child, renal graft defect. Results: The mean age at the time of renal transplantation was 30.11 years. The average age at the time of conception is 34.23 years. The average time between renal transplantation and the occurrence of pregnancy was 46.94 months. More than 40% of pregnancies were not planned. Of the 17 pregnancies, 12 have advanced beyond the first trimester with 91.6% resulting in the birth of a living child. Toxemia was found in 60% of cases, low birth weight in 50%, preterm in 30% and intrauterine growth retardation in 20% of cases. Cesarean section was indicated in all cases. Graft survival was 90% with a mean of 6 years after delivery. Conclusion: Pregnancy in kidney transplanted patients is a high-risk pregnancy, but pregnancy does not appear to affect graft function through certain conditions

Management strategy in actinomycosis brain abscess

Key Clinical Message We reported herein a case of isolated cerebral actinomycosis in a 54‐year‐old immunocompetent man. Brain MRI showed a left frontal intra‐axial lesion and perilesional edema. We performed an open biopsy of the left frontal enhancing lesion. Intraoperative findings showed a yellowish, malleable, and capsulated lesion that was well defined with surrounding normal tissue within pus inside and lacked any necrotic content. MR spectroscopy showed a high level of choline, lactate, and lipid peaks with a choline/N‐Acetylaspartic acid ratio of 1.8. The diagnosis was confirmed histologically, and the patient was treated successfully for 3 months after surgical aspiration. Surgical management allowed to confirm the diagnosis with a shorten antibiotics, a rapid resolution of symptoms, and a complete recovery

Frequency and spectrum of hemochromatosis mutations in Tunisia

International audienceThe occurrence of the C282Y and H63D mutations of the HFE gene, responsible for toxic iron overload in the liver (hereditary hemochromatosis), was still unknown in Tunisia. We report the screening of 194 chromosomes from 97 randomly collected cord blood samples. The mutations were analyzed by PCR followed by DNA sequencing. The mild H63D and the severe C282Y mutations were found in 17.5+/-5.34% and 0.5+/-0.97% of the alleles, respectively. The allele frequency of the IVS 2+4 T --> C polymorphism is high (46.4+/-7.01%) in this population. Risk for homozygosity for the severe C282Y mutation is present in the Tunisian population at a low theoretical incidence. However, due to the relatively high rate of consanguinity in the country, liver pathology due to HH is not to be disregarded

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that CSF-B cells from NBD patients, but not RRMS, are the major source of intrathecal IL-10 as compared to T-CD4 cells. Moreover, we showed a lower expression of TGF-β and IL35, in the CSF cells of NBD patients as compared to the control group. Specific in vitro CpG stimulation of peripheral blood B cells from NBD patients resulted in a concomitant early mRNA expression of IL6 and IL10 but was limited to IL10 for RRMS patients. Furthermore, mRNA expression of IL-6 and IL-10 receptors was assessed and intriguingly IL6ST receptor subunit was significantly lower in NBD CSF, but not RRMS while IL10RB was increased in both. Deciphering the role of increased IL-10-producing B cells and IL10RB despite relapsing disease as well as the discordant expression of IL6 and IL6ST may pave the way for a better understanding of the pathophysiology of these neuro-inflammatory disorders

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that CSF-B cells from NBD patients, but not RRMS, are the major source of intrathecal IL-10 as compared to T-CD4 cells. Moreover, we showed a lower expression of TGF-β and IL35, in the CSF cells of NBD patients as compared to the control group. Specific in vitro CpG stimulation of peripheral blood B cells from NBD patients resulted in a concomitant early mRNA expression of IL6 and IL10 but was limited to IL10 for RRMS patients. Furthermore, mRNA expression of IL-6 and IL-10 receptors was assessed and intriguingly IL6ST receptor subunit was significantly lower in NBD CSF, but not RRMS while IL10RB was increased in both. Deciphering the role of increased IL-10-producing B cells and IL10RB despite relapsing disease as well as the discordant expression of IL6 and IL6ST may pave the way for a better understanding of the pathophysiology of these neuro-inflammatory disorders

Utilization Patterns of Amantadine in Parkinson’s Disease Patients Enrolled in the French COPARK Study

Introduction: Immediate-release (IR) amantadine has been marketed for Parkinson’s disease (PD) therapy for 50 years, while two novel extended-release formulations have only recently reached the market in the US. Objectives: The aim of this study was to describe amantadine IR utilization patterns in the French COPARK cohort, at baseline and after 2 years of follow-up. Methods: Overall, 683 PD patients from the COPARK survey were evaluated. All patients were assessed in a standardized manner (demographics, treatments, Unified Parkinson’s Disease Rating Scale [UPDRS], Hospital Anxiety and Depression Scale, Pittsburg Questionnaire and health-related quality-of-life scales (Short Form-36 [SF-36], 39-item Parkinson’s Disease Questionnaire [PDQ-39]). Longitudinal data were only available for 401/683 patients (59%) with a median (P25–75) follow-up period of 23 months (18–31). Patients were assessed in the same way as in the baseline visit. Results: At baseline, amantadine was prescribed to 61/683 (9%) patients (median dose 200 mg/day, range 100–300 mg/day). Amantadine was initiated after a median of 7 years from PD diagnosis, and its prescription was correlated with the presence of dyskinesia (logistic regression odds ratio [OR] 3.72, 95% confidence interval [CI] 1.95–7.08) and hallucinations (UPDRS I.2) [OR 1.57, 95% CI 1.08–2.29]. After 2 years, the amantadine prescription increased from 33 (8%) patients at baseline to 54 (14%) patients in the subset of 401 patients analysed twice (p = 0.001). Among the 33 patients receiving amantadine at baseline, 9 (27%) stopped amantadine, 5 (15%) increased the dose, 6 (18%) reduced the dose and 13 (40%) stayed at the same doses. Treatment was initiated in 30/54 new patients (55%). Patients who started amantadine or increased its dose (n = 35) had more levodopa-induced dyskinesias at baseline (OR 7.02, 95% CI 3.09–15.90) and higher Mini-Mental State Examination score at follow-up (OR 1.37, 95% CI 1.06–1.79). Undergoing deep brain stimulation was related to stopping or downtitrating amantadine (OR 22.02, 95% CI 4.24–114.44; n = 15). Conclusions: In this cohort, amantadine was used in 10% of patients. Its use increased during follow-up, despite the fact that one-third of patients who received amantadine at baseline stopped taking it. Amantadine prescription was mainly correlated with the presence of dyskinesia.Fil: Rascol, Olivier. Université Paul Sabatier; FranciaFil: Negre Pages, Laurence. Université Paul Sabatier; FranciaFil: Damier, Philippe. Universite Lille; FranciaFil: Delval, Arnaud. Universite de Nantes; FranciaFil: Derkinderen, Pascal. Universite Lille; FranciaFil: Destée, Alain. Universite de Nantes; FranciaFil: Fabbri, Margherita. Université Paul Sabatier; FranciaFil: Meissner, Wassilios G.. Universite Victor Segalen Bordeaux Ii; FranciaFil: Rachdi, Amine. Université Paul Sabatier; FranciaFil: Tison, Francois. Universite Victor Segalen Bordeaux Ii; FranciaFil: Perez Lloret, Santiago. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Cardiológicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Cardiológicas; Argentin

Excessive buccal saliva in patients with Parkinson’s disease of the French COPARK cohort

We describe excessive buccal saliva (EBS) prevalence in patients with Parkinson’s Disease (PD) and controls of the COPARK study, its changes between “ON” and OFF” conditions and over time, its impact on Health-related Quality of life (HRQoL), and factors associated with this condition. We studied 671 ambulatory PD patients and 177 age/sex-matched controls. We defined “sialorrhea” as UPDRS item #6 (salivation) = 1 or 2; and “drooling” as item #6 = 3 or 4. SCOPA-Aut drooling score (item #2) was also available in a subset (45%) of the cohort. HRQoL was assessed by the PDQ-39 and SF-36 scales. Twenty-four months’ follow-up data were available in 401/671 patients. EBS as assessed by UPDRS was present in 38% of PD patients in the “ON” condition (“Sialorrhea”: 35%; “drooling”: 3%). There were also more PD patients reporting “drooling” than controls according to the SCOPA-Aut (49% vs 19%, p < 0.01). UPDRS salivation score was worse in the “OFF” vs “ON” condition in PD patients with motor fluctuations (0.90 ± 0.94 vs 0.54 ± 0.79, p < 0.01). UPDRS salivation score worsened after ~ 24 months of follow-up (0.47 ± 0.70 vs 0.64 ± 0.81, p < 0.01). Worse PDQ-39 scores were observed in PD patients with EBS in bivariate but not in multivariate analyses. EBS was directly related to PD duration and severity, male gender, dysphagia, hypomimia, and autonomic dysfunction (logistic regression). EBS was more frequent in PD patients than controls, worsened in the “OFF” condition and after ~ 24 months of follow-up, moderately affected HRQoL, and was correlated with indices of bradykinesia, dysphagia, and autonomic dysfunction.Fil: Rascol, Olivier. Université de Toulouse; Francia. Inserm; FranciaFil: Negre Pages, Laurence. Hôpital La Colombière; FranciaFil: Damier, Philippe. Universite de Nantes; Francia. Inserm; FranciaFil: Delval, Arnaud. Université de Lille; Francia. Inserm; FranciaFil: Derkinderen, Pascal. Universite de Nantes; Francia. Inserm; FranciaFil: Destée, Alain. Université de Lille; Francia. Inserm; FranciaFil: Fabbri, Margherita. Università di Torino; ItaliaFil: Meissner, Wassilios G.. Universite de Bordeaux; Francia. Chu Hôpitaux de Bordeaux; Francia. University of Otago; Nueva ZelandaFil: Rachdi, Amine. Universite Paul Sabatier Toulouse; Francia. Inserm; FranciaFil: Tison, Francois. Chu Hôpitaux de Bordeaux; Francia. Universite de Bordeaux; Francia. Centre National de la Recherche Scientifique; FranciaFil: Perez Lloret, Santiago. Pontificia Universidad Católica Argentina "Santa María de los Buenos Aires"; Argentina. Universidad Abierta Interamericana; Argentina. Universidad de Buenos Aires; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentin

Excessive buccal saliva in patients with Parkinson’s Disease of the French COPARK cohort

Abstract: Introduction: We describe excessive buccal saliva (EBS) prevalence in patients with Parkinson’s Disease (PD) and controls of the COPARK study, its changes between “ON” and OFF” conditions and over time, its impact on Health-related Quality of life (HRQoL), and factors associated with this condition. Methods: We studied 671 ambulatory PD patients and 177 age/sex-matched controls. We defined “sialorrhea” as UPDRS item #6 (salivation)=1 or 2; and “drooling” as item #6=3 or 4. SCOPA-Aut drooling score (item #2) was also available in a subset (45%) of the cohort. HRQoL was assessed by the PDQ-39 and SF-36 scales. Twenty-four months follow-up data was available in 401/671 patients. Results: EBS as assessed by UPDRS was present in 38% of PD patients in the “ON” condition (“Sialorrhea”: 35%; “drooling”: 3%). There were also more PD patients reporting “drooling” than controls according to the SCOPA-Aut (49% vs 19%, p<0.01). UPDRS salivation score was worse in the “OFF” vs “ON” condition in PD patients with motor fluctuations (0.90±0.94 vs 0.54±0.79, p<0.01). UPDRS salivation score worsened after 24 months of follow up (0.47±0.70 vs 0.64±0.81, p<0.01). Worse PDQ-39 scores were observed in PD patients with EBS in bivariate but not in multivariate analyses. EBS was directly related to PD duration and severity, male gender, dysphagia, hypomimia, and autonomic dysfunction (logistic regression). Conclusions: EBS was more frequent in PD patients than controls, worsened in the “OFF” condition and after 24 months of follow-up, moderately affected HRQoL and was correlated with indices of bradykinesia, dysphagia, and autonomic dysfunction