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1.
Article in English | MEDLINE | ID: mdl-38693627

ABSTRACT

BACKGROUND: In recent decades, progress has been made in the care of people with polyhandicap/profound intellectual and multiple disabilities (PIMD) through a better understanding of the pathophysiology and the development of new care management and rehabilitation strategies adapted to these extreme pathologies. Although there is a lack of knowledge about the health status and care management of the oldest people, a better understanding of the natural course of life of people with polyhandicap/PIMD would consequently allow the optimisation of preventive and curative care management strategies. Few robust data on mortality and life expectancy have been documented for this population in France. Our aims are to estimate the median survival time and assess the factors associated with mortality in people with polyhandicap/PIMD receiving care in France. METHODS: This study included people with polyhandicap/PIMD, followed by the French national cohort 'Eval-PLH' since 2015. These individuals were included in specialised rehabilitation centres and residential institutions. The people included in the first wave of the cohort (2015-2016) were eligible for the present study. Vital status on 1 January 2022 (censoring date) was collected in two ways: (1) spontaneous reporting by the participating centre to the coordinating team and (2) systematic checking on the French national death platform. According to the vital status, survival was calculated in years from the date of birth to the date of death or from the date of birth to the censoring date. The factors associated with mortality were evaluated using the Cox proportional regression hazards model. RESULTS: Data from 780 individuals aged between 3 and 67 years were analysed. At the censoring date, 176 (22.6%) had died, and the mean survival was 52.8 years (95% confidence interval: 51.1-54.5). Mortality was significantly associated with a progressive aetiology, recurrent pulmonary infections, drug-resistant epilepsy and a higher number of medical devices. CONCLUSIONS: This study shows for the first time the survival and impact of factors associated with mortality in people with polyhandicap/PIMD in France.

2.
Arch Pediatr ; 29(4): 292-299, 2022 May.
Article in English | MEDLINE | ID: mdl-35249798

ABSTRACT

BACKGROUND: Hospitalized children and young adults with polyhandicap (PLH) often present with behavioral and relational disorders that are mainly related to their difficulties in communicating and interacting with their environments. Educational support is rarely provided to these patients. An intensive multimodal educative program could help in reducing behavioral disorders and in improving the quality of life of healthcare workers, including nurses and auxiliary nurses. METHODS: A multicenter, randomized controlled trial compared the impact of the usual practice of an educative program (1 h a week) to a multimodal intensive educative program (5 h a week) at 12 months. Patients aged 3-25 with PLH defined by the combination of five criteria (motor deficiency, severe-to-profound mental impairment, daily life dependence, restricted mobility, onset of cerebral lesion at younger than 3 years, and at least one behavioral disorder per week [withdrawn behavior, unexplained crying, teeth grinding, self-injury, aggression, stereotypy, or merycism]) were included. The primary outcome was the evolution of the predominant behavioral disorder between study inclusion and 12 months. Healthcare workers completed questionnaires about chronic stress, coping strategies, and quality of life at study inclusion and at 12 months. RESULTS: Overall, 60 patients were included. Despite a tendency toward reduced teeth grinding, withdrawn and self-injury behaviors, the intervention was not significantly effective: The median duration of continuous behavioral disorders (stereotypy, unexplained crying, withdrawn behavior, and teeth grinding) did not differ between groups. The median frequency of the discontinuous behavioral disorders (self-injury) did not differ between groups. Considering each disorder separately, there was a decrease in teeth grinding, self-injury, and autistic-like traits in the intervention group, although it did not reach statistical significance. This study also suggested decreased depersonalization feelings by healthcare workers. CONCLUSION: Although the study did not show a significant reduction in behavioral disorders in patients with PLH, these results encourage further evaluation of educational management, particularly in regard to patients with self-injury and with withdrawn and teeth-grinding behaviors.


Subject(s)
Intellectual Disability , Quality of Life , Adaptation, Psychological , Child , Child, Hospitalized , Health Personnel , Humans , Surveys and Questionnaires , Young Adult
3.
Rev Med Liege ; 77(1): 39-44, 2022 Jan.
Article in French | MEDLINE | ID: mdl-35029339

ABSTRACT

Summmary : Teratomas are the most common histologic type of germ cell tumors in pediatrics. There are two types of teratomas, mature, benign and immature, malignant. Initial diagnosis is essential for optimal management. This work, based on a clinical case, aims to review the clinical, radiological, biological and histological characteristics allowing them to be differentiated.


Les tératomes sont le type histologique le plus fréquent des tumeurs germinales en pédiatrie. Il existe deux types de tératomes, matures, bénins et immatures, malins. Le diagnostic initial est primordial pour une prise en charge optimale. Ce travail, basé sur un cas clinique, a pour but de reprendre les caractéristiques cliniques, radiologiques, biologiques et histologiques permettant de les différencier.


Subject(s)
Ovarian Neoplasms , Teratoma , Child , Humans , Ovarian Neoplasms/diagnosis , Ovarian Neoplasms/therapy , Teratoma/diagnosis , Teratoma/therapy
4.
Arch Pediatr ; 28(5): 374-380, 2021 Jul.
Article in English | MEDLINE | ID: mdl-33994267

ABSTRACT

AIM: Little is known about the clinical profile of COVID-19 infection in polyhandicapped persons. This study aimed to describe the characteristics of this infection among individuals with polyhandicap. METHOD: This was a retrospective observational study. Polyhandicap was defined by the combination of motor deficiency, profound mental retardation, and age at onset of cerebral lesion younger than 6 years. A positive COVID-19 status was considered for patients with a positive COVID-19 laboratory test result, or patients presenting with compatible symptoms and living in an institution or at home with other patients or relatives who had laboratory-confirmed COVID-19 infection. Data collection included sociodemographic data, clinical and paraclinical characteristics, as well as the management and treatment for COVID-19 infection. RESULTS: We collected 98 cases, with a sex ratio of 0.98 and a mean age of 38.5 years (3 months to 73 years). COVID-19 infection was paucisymptomatic in 46% of patients, 20.6% of patients presented with dyspnea, while the most frequent extra-respiratory symptoms were digestive (26.5%) and neurological changes (24.5%); 18 patients required hospital admission, four adults died. The mean duration of infection was longer for adults than for children, and the proportion of taste and smell disorders was higher in older patients. CONCLUSION: These findings suggest that PLH persons often develop paucisymptomatic forms of COVID-19 infection, although they may also experience severe outcomes, including death. Clinicians should be aware that COVID-19 symptoms in PLH persons are often extra-respiratory signs, mostly digestive and neurologic, which may help in the earlier identification of COVID-19 infection in this particular population of patients.


Subject(s)
COVID-19/complications , COVID-19/diagnosis , Intellectual Disability/complications , Motor Disorders/complications , Adolescent , Adult , Aged , Child , Child, Preschool , Female , France , Humans , Infant , Male , Middle Aged , Retrospective Studies , Young Adult
5.
Rev Neurol (Paris) ; 177(6): 683-689, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33069376

ABSTRACT

OBJECTIVES: Providing a new tool, based on the point of view of experts in polyhandicap, which assesses the global severity of the health status of polyhandicapped persons is necessary. We present herein the initial validation of the polyhandicap severity scale (PSS). METHODS: The initial development of the tool was undertaken in two steps: item selection and validation process. The final set included 10 items related to abilities and 17 items related to comorbidities and impairments. The patient selection criteria were as follows: age>3 years, age at onset of cerebral lesion under 3 years old, with a combination of motor deficiency and profound intellectual impairment, associated with restricted mobility and everyday life dependence. External validity, reproducibility (20 patients), responsiveness (38 patients), and acceptability were explored. RESULTS: During the 18-month study period, a total of 875 patients were included. Two scores were calculated: an abilities score and a comorbidities/impairments score (higher score, higher severity). The 2 scores were higher for: older patients, patients with a progressive etiology, patients with more devices and more medications, patients with higher dependency and lower mobility. Indicators of reproducibility and responsiveness were satisfactory. The mean time duration of fulfilling was 22minutes (standard deviation 5). CONCLUSIONS: Quantifying the health severity of polyhandicapped persons is necessary for both healthcare workers and health decision makers. The polyhandicap severity scale provides the first reliable and valid measure of the health severity status for children and adults.


Subject(s)
Health Status , Nervous System Diseases , Child, Preschool , Comorbidity , Health Personnel , Humans , Psychometrics , Reproducibility of Results , Surveys and Questionnaires
6.
Rev Neurol (Paris) ; 176(5): 370-379, 2020 May.
Article in English | MEDLINE | ID: mdl-31959355

ABSTRACT

INTRODUCTION: A better understanding of the natural course of the health status of patients with polyhandicap may optimize preventive and curative care management. From a large sample of patients aged from 3 to 25 years, we reported the description of their health status. METHODS: This was an 18-month cross-sectional study including patients aged from 3 to 25 years with a combination of severe motor deficiency and profound intellectual impairment. The patients were recruited from 4 specialized rehabilitation centers, 9 residential facilities, and a pediatric/neurological department. The following data were collected: polyhandicap etiology, health status (impairments, comorbidities, and neurodevelopmental status), medical devices, and rehabilitation procedures. RESULTS: A total of 545 patients were included (n=80 [3-5 years], n=166 [6-11 y], n=155 [12-17 y], and n=144 [18-25 y]). The etiology of polyhandicap was unknown for 11.5% of the cases. Behavioral disorders and (orthopedic and digestive) comorbidities were more frequent in the oldest age classes. The neurodevelopmental status of the patients was close to those of a 5- to 7-month-old child without progression across age. Gastrostomy was the most frequent device needed by the patients. DISCUSSION/CONCLUSION: Early detection and management of impairments and comorbidities may improve the disease course of the patients.


Subject(s)
Disabled Persons/statistics & numerical data , Health Status , Intellectual Disability/complications , Intellectual Disability/epidemiology , Adolescent , Adult , Age Factors , Child , Child, Preschool , Comorbidity , Cross-Sectional Studies , Female , France/epidemiology , Humans , Male , Young Adult
7.
Rev Neurol (Paris) ; 176(1-2): 92-99, 2020.
Article in English | MEDLINE | ID: mdl-31255322

ABSTRACT

INTRODUCTION: Polyhandicap is defined as the combination of severe mental impairment and severe motor deficit resulting in reduced mobility and an extreme reduction in autonomy. Over the last 20years, care management for these patients has become more structured, however, their care pathway is not always optimal. OBJECTIVE: To describe/compare the health characteristics, treatment and history of the care pathways of subjects who received care before and after 1990. METHOD: Multicentre cross-sectional study, population studied: patients with polyhandicap: (i) causal brain damage<3years, (ii) severe mental impairment, (iii) motor disability, (iv) reduced mobility, (v) extreme restriction of autonomy. DATA COLLECTED: clinical and medical, care procedures, treatments, history of care pathways. RESULTS: Patients are divided into 2 groups: 545 patients who received care after 1990 and 330 before 1990. Older patients present more recurrent urinary infections, slow transit, behavioural disorders and pain, and are prescribed a greater number of drugs. For those who received care before 1990, the age of admission to an establishment is lower, with one-third receiving a consultation dedicated to the transition from paediatric to adult teams. DISCUSSION/CONCLUSION: The care sector for patients with polyhandicap makes it possible to meet their needs throughout their lives, however, there is still progress to be made in terms of formalisation and of coordinating the care pathway in order to facilitate the transition from paediatric to adult services/establishments.


Subject(s)
Abnormalities, Multiple/therapy , Critical Pathways , Disabled Persons , Health Status , Abnormalities, Multiple/epidemiology , Adolescent , Adult , Aged , Child , Child, Preschool , Combined Modality Therapy/methods , Combined Modality Therapy/standards , Comorbidity , Critical Pathways/history , Critical Pathways/standards , Critical Pathways/trends , Cross-Sectional Studies , Disabled Persons/history , Disabled Persons/statistics & numerical data , Female , France/epidemiology , History, 20th Century , History, 21st Century , Humans , Intellectual Disability/complications , Intellectual Disability/epidemiology , Intellectual Disability/therapy , Male , Middle Aged , Motor Disorders/complications , Motor Disorders/epidemiology , Motor Disorders/therapy , Nervous System Diseases/complications , Nervous System Diseases/epidemiology , Nervous System Diseases/therapy , Young Adult
8.
J Intern Med ; 286(5): 583-595, 2019 11.
Article in English | MEDLINE | ID: mdl-31361936

ABSTRACT

BACKGROUND: Most risk factors for lymphoma identified so far relate to immunosuppression, but its aetiology remains unclear. OBJECTIVES: We investigated whether Bacillus Calmette-Guérin (BCG) vaccination is associated with lymphoma, overall and separately for Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL). METHODS: A cohort of 400 611 subjects born in the province of Québec, Canada, between 1970 and 1974 was used. Information on BCG vaccination was extracted from the Quebec BCG Vaccination Registry. Lymphomas cases were individuals who had ≥2 health encounters, medical visits or hospitalizations, for lymphoma within 2 months or who were identified through the Quebec Tumor Registry. Cox proportional hazard regression was used to estimate hazard ratios (HRs) and 95% confidence interval (CI), adjusting for potential confounders. RESULTS: A total of 178 335 (46.0%) subjects were BCG-vaccinated, and 1478 (0.38%) cases of lymphomas were ascertained. Amongst them, 922 were identified as NHL and 421 as HL. After adjustment, no association was observed between BCG vaccination and either lymphoma (any type) (HR = 1.03, 95% CI: 0.96-1.11) or NHL (HR = 0.99, 95% CI: 0.86-1.13). For HL, nonproportional hazards were observed. Before the age of 18, the risk of HL was elevated amongst vaccinated individuals (HR = 2.26, 95% CI: 1.39-3.69). However after 18 years of age, no association was found (HR = 0.93, 95% CI: 0.75-1.15). CONCLUSION: Bacillus Calmette-Guérin vaccination may increase the risk of HL before 18 years of age, but residual confounding cannot entirely be excluded. Given the benefits of BCG vaccination, these results need to be reproduced in other populations before firm conclusions can be drawn.


Subject(s)
BCG Vaccine , Hodgkin Disease/epidemiology , Lymphoma, Non-Hodgkin/epidemiology , Vaccination , Adult , Cohort Studies , Female , Hodgkin Disease/diagnosis , Humans , Lymphoma, Non-Hodgkin/diagnosis , Male , Quebec/epidemiology , Registries , Risk Factors , Young Adult
9.
Int J Dent Hyg ; 16(3): 397-403, 2018 Aug.
Article in English | MEDLINE | ID: mdl-29124887

ABSTRACT

BACKGROUND AND OBJECTIVE: Our previous work showed a positive association between metabolic syndrome (MetS) and gingival crevicular fluid (GCF) tumour necrosis factor-alpha (TNF-α) in a sample of obese and non-obese children. However, whether this association persists among obese children is unknown. We aim to investigate the extent to which MetS is associated with GCF TNF-α level among obese children. METHODOLOGY: We performed a cross-sectional analysis using data from visit 1 of the QUebec Adipose and Lifestyle InvesTigation in Youth cohort. A total of 219 obese children aged 8-10 years, for whom data were available for both MetS and TNF-α, were included in our analysis. The independent variable, MetS, was defined according to the International Diabetes Federation recommendations. GCF samples were collected from the gingival sulcus using a paper strip, and the concentration of TNF-α was determined by enzyme-linked immunosorbent assay. Analyses included descriptive statistics and sex-specific linear regression analyses adjusting for potential confounders. RESULTS: In this sample comprising only obese children, 24 (10.9%) had MetS. Among obese boys, those with MetS had 44.9% higher GCF TNF-α (95% confidence interval: 16.5%-73.3%) compared to those without MetS. No such association was detected in obese girls. CONCLUSION: MetS was positively associated with GCF TNF-α concentration in obese boys. These results suggest that obese boys with MetS may have a worse gingival health profile compared to their obese counterpart without MetS.


Subject(s)
Gingival Crevicular Fluid/chemistry , Gingivitis/complications , Metabolic Syndrome/complications , Pediatric Obesity/complications , Tumor Necrosis Factor-alpha/analysis , Child , Female , Humans , Linear Models , Longitudinal Studies , Male , Sex Factors
10.
Cancer Epidemiol ; 39(6): 1036-46, 2015 Dec.
Article in English | MEDLINE | ID: mdl-26372414

ABSTRACT

Human papillomaviruses (HPV) are associated with head and neck cancers (H&NC). Transmission of HPV to the upper aerodigestive tract occurs plausibly through sexual contact, although epidemiologic evidence on the role of sexual behaviours in H&NC aetiology is inconsistent. We conducted a meta-analysis of studies examining the association between four indicators of sexual behaviours (number of sexual partners and oral sex partners, oral sex practice, and age at first intercourse) and H&NC. Summary odds ratios (OR) and 95% confidence intervals (CI) were estimated using fixed and random effects models for each indicator, contrasting 'highest' to 'lowest', 'ever' to 'never', or 'youngest' to 'oldest' categories. Twenty case-control studies were included out of 3838 identified publications. Using random effects models, summary ORs suggested an increased risk of H&NC for number of sexual partners [OR=1.29, 95% CI: 1.02-1.63] (19 studies) and number of oral sex partners [OR=1.69, 95% CI: 1.00-2.84] (5 studies), whereas no effect was observed with oral sex practice [OR=1.09, 95% CI: 0.88-1.35] (17 studies) and age at first intercourse [OR=1.40, 95% CI: 0.71-2.79] (6 studies). For number of sexual partners and oral sex practice, which were assessed in more studies, we further excluded studies contributing to heterogeneity and those not adjusted for age, sex, smoking, and alcohol consumption. The summary ORs were 0.95 (95% CI: 0.75-1.20) for number of sexual partners and 1.03 (95% CI: 0.84-1.26) for oral sex practice. Our findings highlight that observed associations might be partly attributed to confounding effects of sociodemographic and behavioural factors.


Subject(s)
Head and Neck Neoplasms/epidemiology , Papillomaviridae/isolation & purification , Sexual Behavior/statistics & numerical data , Female , Humans , Male , Odds Ratio , Risk , Sexual Partners
11.
Rev Epidemiol Sante Publique ; 63(4): 247-52, 2015 Aug.
Article in French | MEDLINE | ID: mdl-26143087

ABSTRACT

BACKGROUND: Long-term intratracheal ventilated patients need continuous artificial ventilation support. After the acute periods, these patients may benefit from dedicated follow-up in rehabilitation care centers. In this paper, we aimed to study the validity of the data provided by a French diagnosis-related group (DRG) information system. METHODS: For a sample of intratracheal ventilated patients in two rehabilitation units, we compared the data provided in the DRG information system with the data available in the medical charts. Furthermore, we asked the medical, nursing and allied health staff to assess the data provided by the French DRG information system. RESULTS: The diagnosis was found accurate for 86% of hospital stays. In the DRG information system, 77% of the medical care, and 39% of the nursing and allied health care were mentioned correctly. Overall, 55% of the nursing and allied health care procedures in the DRG information system were not reported in the medical charts. The healthcare providers estimated that the frequency of the care provided was underestimated in the DRG information system for 30% of the nursing and allied health care. CONCLUSION: The patients' main characteristics were found correctly reported in the DRG information system. However, the diversity and the frequency of the care provided were underestimated. These underestimates were mainly related to care frequently provided in these patients (for example, urinary catheterization, massages, counseling for relatives).


Subject(s)
Diagnosis-Related Groups , Medical Records , Adult , Female , France , Health Personnel , Humans , Intubation, Intratracheal/statistics & numerical data , Length of Stay , Male , Medical Records/standards , Reproducibility of Results
12.
Brain Inj ; 29(7-8): 837-42, 2015.
Article in English | MEDLINE | ID: mdl-25950262

ABSTRACT

AIM: This study concerns the aetiologies, comorbidities and places and causes of death of a population of persons with severe polyhandicap (PLH). METHODS: Based on the medical files of all deceased PLH patients, who were cared for between 2006-2012. Data collected were aetiological diagnosis of the polyhandicap, duration and type of hospitalization, age, place and cause of death, comorbidities: chronic respiratory insufficiency, recurrent attacks of pulmonary infections, urinary infections, active epilepsy, scoliosis, chronic digestive disorders and behavioural problems. RESULTS: One hundred and thirty-three patients died, 70 children and 63 adults. The sex ratio was 84 men to 49 women. The average stay in these institutions was 10 years 4 months. The average age at the time of death was 21 years, in 60% of cases the place of death was in the specialist rehabilitation centres. The causes of death in decreasing order were: pulmonary infections (63.2%), sudden death (18%) and status epilepticus (6.8%); 79.7% of patients suffered from chronic respiratory insufficiency, 60.2% suffered serious scoliosis, 66.9% drug-resistant epilepsy and 78.9% had digestive disorders. The main aetiologies of the polyhandicap were: pre- and perinatal encephalopathies (31.6%), metabolic encephalopathies (18%) and convulsive encephalopathies (11.3%). CONCLUSION: The main comorbidity and main cause of death in patients with severe PLH is respiratory failure.


Subject(s)
Chronic Disease/mortality , Disabled Persons , Rehabilitation Centers , Adolescent , Adult , Cause of Death , Child , Child, Preschool , Comorbidity , Disabled Persons/rehabilitation , Epilepsy/mortality , Female , Follow-Up Studies , France/epidemiology , Hospitalization/statistics & numerical data , Humans , Intellectual Disability/mortality , Length of Stay , Lung Diseases/mortality , Male , Mental Disorders/mortality , Mental Health Services , Middle Aged , Patient Admission , Rehabilitation Centers/statistics & numerical data , Scoliosis/mortality , Severity of Illness Index , Surveys and Questionnaires , Urinary Tract Infections/mortality
13.
Prev Med ; 66: 87-94, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24945693

ABSTRACT

OBJECTIVES: To identify determinants of Bacillus Calmette-Guérin (BCG) vaccination among children born in Québec, Canada, in 1974, the last year of the systematic vaccination campaign. METHOD: A retrospective birth cohort was assembled in 2011 through probabilistic linkage of administrative databases (n=81,496). Potential determinants were documented from administrative databases and by interviewing a subset of subjects (n=1643) in 2012. Analyses were conducted among subjects with complete data, 71,658 (88%) birth cohort subjects and 1154 (70%) interviewed subjects, then redone using multiple imputation. Determinants of BCG vaccination during the organized vaccination program (in 1974), and after the program (1975 onwards) were assessed separately. Logistic regression with backward elimination was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs). RESULTS: Overall, 46% of subjects were BCG vaccinated, 43% during the program and 4% after it ended. BCG vaccination during the program was associated with parents' birthplace and urban or rural residence. BCG vaccination after the organized program was only related to ethnocultural origin of the child's grandparents. CONCLUSION: Different factors were related to vaccination within and after the organized program. Determinants of BCG vaccination in Québec, Canada, have never been studied and will be useful for future research and vaccination campaigns.


Subject(s)
BCG Vaccine , Vaccination/statistics & numerical data , Adolescent , Adult , Child , Emigrants and Immigrants , Female , Humans , Immunization Programs , Logistic Models , Male , Odds Ratio , Quebec , Retrospective Studies , Socioeconomic Factors , Young Adult
14.
Neurology ; 72(15): 1301-9, 2009 Apr 14.
Article in English | MEDLINE | ID: mdl-19365051

ABSTRACT

OBJECTIVE: The literature on propriospinal myoclonus (PSM) is poor and there are no systematic reviews of the subject. We sought to clarify the spectrum of PSM. METHODS: We first prospectively investigated all patients seen in our movement disorders clinic with a firm diagnosis of PSM between 2002 and 2007. All had a standardized interview, detailed clinical examination, laboratory investigations, comprehensive neurophysiologic examination, and spinal cord MRI, including diffusion tensor imaging with fiber tracking (DTI-FT). We also collected drug responses. Finally, we conducted a systematic review of the literature. RESULTS: We enrolled 10 patients meeting the strict criteria for PSM, and also analyzed data on 50 patients from 26 previous reports. PSM occurred predominantly in male and middle-aged patients. The typical clinical picture consisted of myoclonic jerks consistently involving abdominal wall muscles, which worsen in the lying position. A premonitory sensation preceding the jerks and wake-sleep transition phase worsening were frequent. Most patients had a myoclonic generator at the thoracic level, with a myoclonus duration between 200 msec and 2 s. An underlying cause was infrequently found. DTI-FT detected cord abnormalities all of our patients. CONCLUSION: The clinico-physiologic spectrum of propriospinal myoclonus (PSM) is homogenous. Involvement of the abdominal wall muscles, worsening in the lying position, premonitory sensation, and wake-sleep transition phase worsening are helpful clinical clues. Diffusion tensor imaging with fiber tracking appears more sensitive than conventional MRI for detecting associated microstructural abnormalities of the spinal cord. Symptomatic treatment of PSM is not straightforward, and clonazepam is reported to be the most effective drug. Zonisamide may be an interesting option.


Subject(s)
Myoclonus/diagnosis , Myoclonus/therapy , Adolescent , Adult , Aged , Diffusion Magnetic Resonance Imaging , Electrodiagnosis , Electroencephalography , Electromyography , Evoked Potentials, Somatosensory/physiology , Female , Hematologic Tests , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Myoclonus/pathology , Neural Pathways/physiopathology , Prospective Studies , Spinal Cord/pathology , Spinal Cord/physiopathology , Tomography, X-Ray Computed , Young Adult
15.
Rev Neurol (Paris) ; 165(11): 924-32, 2009 Nov.
Article in French | MEDLINE | ID: mdl-19285698

ABSTRACT

INTRODUCTION: Long-term follow-up studies indicate a low remission rate in idiopathic generalised epilepsies (IGE) (Martinez-Juarez et al., 2006), suggesting they may persist to an advanced age. However there are few estimates of IGE frequency in the elderly. METHODS: EEGs of 700 patients aged over 70 years, recorded between January 2006 and March 2007, were reviewed for anomalies consistent with IGE. We then examined the clinical history of patients with these anomalies. RESULTS: A persistent IGE was identified in four female patients (mean age: 79 years); in two cases it was a juvenile myoclonic epilepsy (JME) and in two an epilepsy with grand mal seizures. Seizures in three patients had begun in childhood or adolescence and in one at 40 years. Before hospitalization, few or no seizures were reported and IGE had not been diagnosed. IGE was revealed in each patient by a relatively severe event: an absence status (AS), subcontinuous myoclonic seizures or repeated convulsive generalised seizures (CGS). These events were not situation-related but in one patient the relapse of simple convulsive seizures, may have been related to the withdrawal of anti-epileptic drugs (AED) several months previously. EEG records showed generalised spikes or polyspikes and waves organised in a status epilepticus or in interictal rhythmic discharges. In one case they were evident only from a 24 hours recording. Clonazepam injection was used to suppress the AS episode and the subintrant myoclonia. After the AS, interictal generalised epileptic discharges persisted. Two of the four patients had familial history of epilepsy or febrile seizures but in no case was an epileptogenic lesion evident in brain CT scan or MRI. Clinical exams and biologic parameters were normal. All of the patients had worked and were married with children. Appropriate therapies were followed after the diagnosis of IGE. One patient with JME had been treated by Valproate which was discontinued by the general practitioner because of lethargy and replaced by Carbamazepine; seizures were aggravated under both Carbamazepine and then Lamotrigine and until the patient became seizure-free on Levetiracetam. The antiepiletic treatment was also modified in a second patient, while the two others responded well to Valproate. CONCLUSIONS: IGE can exacerbate in the elderly, as different types of seizures including AS, subintrant myoclonia or repeated CGS. Our data suggest persistent IGE are quite frequent in an aged population and may be underestimated due to difficulties in diagnosis. Correctly diagnosed, adjustment of AED may offer substantial clinical improvements in IGE of the elderly.


Subject(s)
Epilepsy, Generalized/epidemiology , Aged , Aged, 80 and over , Anticonvulsants/therapeutic use , Clonazepam/therapeutic use , Electroencephalography , Epilepsy, Generalized/complications , Epilepsy, Generalized/drug therapy , Epilepsy, Generalized/physiopathology , Female , Humans , Seizures/physiopathology , Status Epilepticus/physiopathology
16.
Brain Inj ; 22(2): 123-34, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18240041

ABSTRACT

BACKGROUND: The persistent vegetative state (PVS) is a devastating medical condition characterized by preserved wakefulness contrasting with absent voluntary interaction with the environment. However, very little is known about the actual degree of perception in these patients and the extent of progressive brain injury induced by very prolonged unawareness. METHODS: The authors have conducted a 2-year longitudinal study using a multimodal MRI-MRSI-fMRI protocol in four patients in long-lasting PVS (over 3 years at inclusion) characterized by various brain injuries. RESULTS: Although one subject showed initially preserved local brain metabolism and brain activity related to primary perception suggesting the presence of potential residual brain plasticity even in this critical stage, none of the four patients recovered to consciousness during the 2 years of the protocol. Moreover, significant deterioration of parameters related to brain atrophy, metabolism and functional excitability of primary cortices was observed in all patients during the follow-up. CONCLUSIONS: Heterogeneity of brain injury, consequences of long term minimal brain activity and potential factors that prevent recovery to consciousness are discussed.


Subject(s)
Brain Injuries/complications , Coma/complications , Evoked Potentials/physiology , Magnetic Resonance Imaging/methods , Persistent Vegetative State/complications , Adolescent , Adult , Brain Injuries/metabolism , Decision Making/ethics , Female , Humans , Longitudinal Studies , Male , Somatosensory Cortex/metabolism
17.
Am J Epidemiol ; 166(9): 1005-14, 2007 Nov 01.
Article in English | MEDLINE | ID: mdl-17690218

ABSTRACT

The International Agency for Research on Cancer recently classified inorganic lead as a probable carcinogen, while organic lead remained unclassifiable. Uncertainty persists because of limited epidemiologic evidence. The authors addressed the relation between occupational exposure to lead and the risk of 11 types of cancer among men in a case-control study conducted in Montreal, Quebec, Canada, in the 1980s. Incident cases (n = 3,730) and general population controls (n = 533) were interviewed to elicit information on job history and potential confounders. Expert chemists translated each job into a list of substances to which the subject had potentially been exposed. Exposure to lead was classified into three categories: organic lead (3% of subjects ever exposed), inorganic lead (17%), and lead in gasoline emissions (39%). Odds ratios and 95% confidence intervals were estimated by logistic regression using two control groups: general population controls and cancer controls. Stomach cancer was associated with organic lead when the authors used population controls (odds ratio (OR) = 3.0, 95% confidence interval (CI): 1.2, 7.3) and cancer controls (OR = 2.0, 95% CI: 1.1, 3.8) and with substantial exposure to lead in gasoline emissions when they used cancer controls (OR = 2.9, 95% CI: 1.4, 5.9). There was no association with inorganic lead and little evidence for associations with other cancer types.


Subject(s)
Lead/adverse effects , Neoplasms/chemically induced , Neoplasms/epidemiology , Occupational Diseases/chemically induced , Occupational Diseases/epidemiology , Occupational Exposure/adverse effects , Adult , Aged , Case-Control Studies , Confidence Intervals , Data Collection , Humans , Incidence , Male , Middle Aged , Odds Ratio , Quebec/epidemiology , Regression Analysis , Risk Assessment , Risk Factors , Surveys and Questionnaires
18.
Eur J Cancer Prev ; 14(5): 431-8, 2005 Oct.
Article in English | MEDLINE | ID: mdl-16175048

ABSTRACT

Among men there is epidemiological evidence for an association between obesity and increased risk of renal cell carcinoma, colon cancer and adenocarcinoma of the oesophagus. The evidence for other cancer sites remains inconsistent. We conducted a large population-based, multi-site, case-control study of environmental causes of cancer among males in Montreal, Canada. Among the many questionnaire items collected by interview were height and usual weight. We compared height, weight and body mass index (BMI) among individuals with 11 different cancer types (combined N=3016) and population-based controls (N=509). Linear regression was used to model the relationship of the disease status with each of three dependent continuous variables (height, weight and BMI), while adjusting for covariates. For most cancer groups, weight and BMI were lower than among population controls. Because of potential information bias and reverse causality bias, we focused on the comparisons among cancer types. The lowest BMI values were observed among men with squamous cell carcinoma of the oesophagus, lung and stomach cancers. The highest BMIs were reported by men with prostate and kidney cancers, and oesophageal adenocarcinoma. Inconsistencies in the epidemiological literature on obesity and cancer risk could be related to the difficulties in obtaining unbiased reports of pre-disease weight and to publication bias.


Subject(s)
Body Height , Body Weight , Neoplasms/epidemiology , Adult , Aged , Body Mass Index , Case-Control Studies , Humans , Linear Models , Male , Middle Aged , Neoplasms/classification , Neoplasms/physiopathology , Quebec/epidemiology , Smoking/epidemiology
19.
Brain Inj ; 17(10): 883-7, 2003 Oct.
Article in English | MEDLINE | ID: mdl-12963554

ABSTRACT

OBJECTIVES: To evaluate the frequency of pulmonary and digestive infections in a population of disabilitated patients receiving gastric acid-lowering medications for a long duration. METHODS: Cases underwent gastric acid-lowering medications for at least 4 years and controls were matched on type of handicap, aetiologies of handicap, sex and age and didn't receive gastric acid-lowering medications. For each patient, the following data were collected: age, sex, aetiology of disabilitation, type of gastric acid-lowering medications (histamine receptor H2 antagonists or proton pump inhibitors), occurrence of gastric or oesophageal pathology, nasogastric tube or gastrostomy device, motor handicap, number of digestive infections and number of pulmonary infections during the last 5 years. RESULTS: Total rate of infections was significantly more frequent in patients receiving gastric acid lowering medications than in patients who did not receive gastric acid-lowering medications (p = 0.035). Pulmonary infections were significantly more frequent in patients receiving gastric acid-lowering medications than in those who did not receive gastric acid-lowering medications (p < 0.05). The rate of digestive infections was not influenced by acid-suppressive treatments. CONCLUSION: Oesophagitis may lead to complications and requires gastric acid-lowering medications; in fact these drugs promote bacterial development and subsequently increase the risk of pulmonary infections.


Subject(s)
Antacids/adverse effects , Bacterial Infections/chemically induced , Digestive System Diseases/chemically induced , Lung Diseases/chemically induced , Adolescent , Adult , Bacterial Infections/metabolism , Digestive System Diseases/metabolism , Female , Gastric Acid/metabolism , Humans , Lung Diseases/metabolism , Male , Mental Disorders/complications , Respiratory Tract Infections/chemically induced , Respiratory Tract Infections/metabolism , Retrospective Studies
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