Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 14 de 14
Filtrar
1.
Rev Neurol (Paris) ; 177(10): 1276-1282, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34272066

RESUMO

OBJECT: Adult brainstem gliomas are a rare group of heterogeneous brain tumors. Classical clinical presentation includes progressive impairment of cranial nerves associated with long tract signs. The prognosis and response to treatment are poor; nevertheless, some patients do have a long survival. The objective of this study was to describe a series of patients with an isolated persistent hemifacial spasm and/or facial nerve palsy as the presenting symptom of a brainstem glioma. METHODS: Fourteen patients from 3 French hospitals (Paris, Caen, Lille) were included. Clinical and radiological features and overall survival were retrospectively analyzed. A review of the literature of similar cases was performed. RESULTS: Mean age at diagnosis was 35 years (range 19-57 years). Mean duration of facial nerve involvement before diagnosis was 17 months (range 1-48 months). Tumors were characterized on MRI by a lateralized location in the pons, a T1-weighted hyposignal, a T2-weighted hypersignal and no contrast enhancement after Gadolinium injection except for 2 cases. Biopsies were performed in 10 cases and showed 8 low-grade and 2 high-grade gliomas. All the patients were initially treated with radiotherapy and 6 patients with chemotherapy after progression. Eleven patients died from tumor progression. Median survival time was 90 months. CONCLUSIONS: Adult brainstem gliomas revealed by a progressive isolated involvement of the facial nerve seem to have particular clinico-radiological features of slow progressive tumors and may be associated with long patient survival.


Assuntos
Glioma , Espasmo Hemifacial , Adulto , Nervo Facial , Glioma/diagnóstico , Glioma/diagnóstico por imagem , Espasmo Hemifacial/diagnóstico por imagem , Espasmo Hemifacial/etiologia , Humanos , Pessoa de Meia-Idade , Paralisia , Ponte , Estudos Retrospectivos , Adulto Jovem
2.
EJNMMI Res ; 7(1): 21, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28266002

RESUMO

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease of the nervous system involving both upper and lower motor neurons. The patterns of structural and metabolic brain alterations are still unclear. Several studies using anatomical MRI yielded a number of discrepancies in their results, and a few PET studies investigated the effect of ALS on cerebral glucose metabolism. The aim of this study was threefold: to highlight the patterns of grey matter (GM) atrophy, hypometabolism and hypermetabolism in patients with ALS, then to understand the neurobehavioral significance of hypermetabolism and, finally, to investigate the regional differences between the morphologic and functional changes in ALS patients, using a specially designed voxel-based method. Thirty-seven patients with ALS and 37 age- and sex-matched healthy individuals underwent both structural MRI and 18[F]-fluorodeoxyglucose (FDG) PET examinations. PET data were corrected for partial volume effects. Structural and metabolic abnormalities were examined in ALS patients compared with control subjects using two-sample t tests in statistical parametric mapping (SPM). Then, we extracted the metabolic values of clusters presenting hypermetabolism to correlate with selected cognitive scores. Finally, GM atrophy and hypometabolism patterns were directly compared with a one-paired t test in SPM. RESULTS: We found GM atrophy as well as hypometabolism in motor and extra motor regions and hypermetabolism in medial temporal lobe and cerebellum. We observed negative correlations between the metabolism of the right and left parahippocampal gyri and episodic memory and between the metabolism of right temporal pole and cognitive theory of mind. GM atrophy predominated in the temporal pole, left hippocampus and right thalamus, while hypometabolism predominated in a single cluster in the left frontal superior medial cortex. CONCLUSIONS: Our findings provide direct evidence of regional variations in the hierarchy and relationships between GM atrophy and hypometabolism in ALS. Moreover, the 18FDG-PET investigation suggests that cerebral hypermetabolism is deleterious to cognitive function in ALS.

3.
Transpl Infect Dis ; 14(6): E147-9, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23025483

RESUMO

Microsporidiosis is an opportunistic infection in organ transplant recipients and patients with other cellular immunodeficiency. Fumagillin is an effective treatment against Enterocytozoon bieneusi, one of the two main species causing the microsporidiosis involved in human diseases. We report the first case, to our knowledge, of a probable drug-induced aseptic meningoencephalitis, after administration of fumagillin in a kidney transplant recipient with microsporidiosis.


Assuntos
Antifúngicos/efeitos adversos , Cicloexanos/efeitos adversos , Ácidos Graxos Insaturados/efeitos adversos , Transplante de Rim/efeitos adversos , Meningoencefalite/etiologia , Microsporidiose/tratamento farmacológico , Cicloexanos/uso terapêutico , Ácidos Graxos Insaturados/uso terapêutico , Feminino , Humanos , Hospedeiro Imunocomprometido , Pessoa de Meia-Idade , Sesquiterpenos/efeitos adversos , Sesquiterpenos/uso terapêutico
4.
Rev Neurol (Paris) ; 166(8-9): 683-98, 2010.
Artigo em Francês | MEDLINE | ID: mdl-20472259

RESUMO

This paper, written by French amyotrophic lateral sclerosis (ALS) center experts, presents an update of recent advances in fundamental, epidemiological and clinical research in ALS based on a review of the literature between September 2008 and November 2009. Among other pathophysiological mechanisms, the role of stress of the endoplasmic reticulum and the importance of energetic metabolic disturbances have been underscored. In the field of genetics, research has been advanced through the identification of mutations of the gene FUsed in Sarcoma/Translated in LipoSarcoma (FUS/TLS) in individuals with familial and sporadic ALS. This gene is involved in the regulation of transcription, splicing and RNA transport, and has functional homology to another ALS gene, TARDBP, which suggests that a common mechanism may underlie motor neuron degeneration. A report showed that mice expressing a mutant form of human TDP-43 develop a progressive and fatal neurodegenerative disease reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates (FTLD-U), providing a new animal model that may help to better understand the pathophysiology and test new therapeutics. Beside genetic studies, several epidemiologic studies have investigated the role of environmental factors. A recent study suggests that smoking is a risk factor for developing ALS and it is hypothesized that this could occur through lipid peroxidation via formaldehyde exposure. From a neuroprotective perspective, trials with IGF-1, sodium valproate, coenzyme Q or glatiramer acetate have failed to demonstrate any beneficial effect. A study published in 2008 argued that lithium may have a neuroprotective effect in ALS mice and also in patients. However, two preclinical studies failed to replicate the neuroprotective effect of lithium in ALS mice. Therapeutic trials have been performed or are currently ongoing in Europe and North America. Their results have not yet been published.


Assuntos
Esclerose Lateral Amiotrófica , Esclerose Lateral Amiotrófica/tratamento farmacológico , Esclerose Lateral Amiotrófica/epidemiologia , Esclerose Lateral Amiotrófica/genética , Esclerose Lateral Amiotrófica/fisiopatologia , Esclerose Lateral Amiotrófica/psicologia , Animais , Biomarcadores , Ensaios Clínicos como Assunto , Proteínas de Ligação a DNA/deficiência , Proteínas de Ligação a DNA/genética , Proteínas de Ligação a DNA/fisiologia , Modelos Animais de Doenças , Avaliação Pré-Clínica de Medicamentos , Exposição Ambiental , Humanos , Desnutrição/etiologia , Desnutrição/terapia , Camundongos , Camundongos Transgênicos , Músculo Esquelético/metabolismo , Fármacos Neuroprotetores/uso terapêutico , Proteína FUS de Ligação a RNA/deficiência , Proteína FUS de Ligação a RNA/genética , Proteína FUS de Ligação a RNA/fisiologia , Fatores de Risco , Superóxido Dismutase/deficiência , Superóxido Dismutase/genética , Superóxido Dismutase/fisiologia , Superóxido Dismutase-1
5.
Rev Neurol (Paris) ; 165(6-7): 532-41, 2009.
Artigo em Francês | MEDLINE | ID: mdl-19419744

RESUMO

This paper from a group of French experts in amyotrophic lateral sclerosis (ALS) presents an update of recent advances in fundamental, epidemiological and clinical research in ALS. Recent development in the pathogenesis of ALS suggests that motor neuron degeneration is a multifactorial and noncell autonomous process. Research has been advanced through the identification of the TAR-DNA-binding protein (TDP-43) as a common neuropathological marker of ALS and frontotemporal lobar degeneration with ubiquitin-positive inclusions. Recently, mutations in the TDP-43 gene have been described in individuals with familial and sporadic ALS. Fundamental research in ALS is expected to lead to the disclosure of new diagnostic markers and therapeutic targets. A small trial has suggested that lithium carbonate may slow ALS progression but larger trials will be needed to confirm these results.


Assuntos
Esclerose Lateral Amiotrófica/terapia , Esclerose Lateral Amiotrófica/epidemiologia , Esclerose Lateral Amiotrófica/genética , Esclerose Lateral Amiotrófica/fisiopatologia , Eletrofisiologia , Humanos , Fármacos Neuroprotetores/uso terapêutico , Apoio Nutricional , Mecânica Respiratória/fisiologia
6.
Neurology ; 72(15): 1301-9, 2009 Apr 14.
Artigo em Inglês | MEDLINE | ID: mdl-19365051

RESUMO

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.


Assuntos
Mioclonia/diagnóstico , Mioclonia/terapia , Adolescente , Adulto , Idoso , Imagem de Difusão por Ressonância Magnética , Eletrodiagnóstico , Eletroencefalografia , Eletromiografia , Potenciais Somatossensoriais Evocados/fisiologia , Feminino , Testes Hematológicos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mioclonia/patologia , Vias Neurais/fisiopatologia , Estudos Prospectivos , Medula Espinal/patologia , Medula Espinal/fisiopatologia , Tomografia Computadorizada por Raios X , Adulto Jovem
8.
Eur J Neurol ; 15(11): 1245-51, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18973614

RESUMO

BACKGROUND AND PURPOSE: To prospectively investigate causes of death and the circumstances surrounding death in 302 patients with amyotrophic lateral sclerosis (ALS). The functional status of patients immediately before death was also determined. METHODS: Information was obtained from neurologists at ALS centres, patients' files, and, when deaths occurred outside a medical facility, attending physicians. RESULTS: Most patients (63%) died in a medical facility. The most frequently reported cause of death was respiratory failure (77%), including terminal respiratory insufficiency (58%), pneumonia (14%), asphyxia due to a foreign body (3%) and pulmonary embolism (2%). Ten per cent of patients died from other causes: post-surgical or traumatic conditions (5%), cardiac causes (3.4%), suicide (1.3%) and sudden death of unknown origin (0.7%). The cause of death could not be determined in 13% of cases (6% inside a medical facility and 25% outside). At the time of death, only 55% of patients were receiving riluzole, 33% were undergoing non-invasive ventilation, 3% had a tracheotomy and 37% a gastrostomy. CONCLUSION: The information provided by this study helps to improve our understanding of the natural history of the disease and may help optimize the quality of care we can offer patients at the end of life.


Assuntos
Esclerose Lateral Amiotrófica/mortalidade , Insuficiência Respiratória/mortalidade , Idoso , Esclerose Lateral Amiotrófica/fisiopatologia , Asfixia/mortalidade , Comorbidade , Feminino , França/epidemiologia , Cardiopatias/mortalidade , Cuidados Paliativos na Terminalidade da Vida/normas , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia/mortalidade , Estudos Prospectivos , Embolia Pulmonar/mortalidade , Qualidade de Vida , Insuficiência Respiratória/fisiopatologia , Paralisia Respiratória/mortalidade , Paralisia Respiratória/fisiopatologia
9.
Ann Chir Plast Esthet ; 53(1): 59-62, 2008 Feb.
Artigo em Francês | MEDLINE | ID: mdl-17600609

RESUMO

We are reporting the use of a xylocain test before the injection of botulinal toxin within the "facial paralysis and botulinal toxin" multidisciplinary consultation of the Caen regional hospital. Our assessment is about 16 patients tested. This test is based on a digital film and a questionnaire. It is often proposed when toxin is first used. It influences the patient's choice. It permits a better understanding of the toxin action by the patient, it reassures. It is reliable for the practitioner even though we do not have a mathematical correlation between the xylocain volume and the botulinal toxin volume. Simple and far, it is a help for the patient and the practitioner before a delicate injection of botulinal toxin.


Assuntos
Anestésicos Locais , Toxinas Botulínicas Tipo A/uso terapêutico , Paralisia Facial/tratamento farmacológico , Lidocaína , Fármacos Neuromusculares/uso terapêutico , Adolescente , Adulto , Idoso , Anestésicos Locais/administração & dosagem , Toxinas Botulínicas Tipo A/administração & dosagem , Paralisia Facial/etiologia , Feminino , Humanos , Injeções Intramusculares , Lidocaína/administração & dosagem , Masculino , Pessoa de Meia-Idade , Fármacos Neuromusculares/administração & dosagem , Seleção de Pacientes , Inquéritos e Questionários
12.
Ann Chir Plast Esthet ; 47(5): 390-401, 2002 Oct.
Artigo em Francês | MEDLINE | ID: mdl-12449866

RESUMO

There are many lips kinetic disorders whose clinical appearances greatly differ. An acute examination directs toward the diagnosis on a neurologic, neurogenous, muscular, myogenous, medicamentous, dermatological or psychiatric etiology. The authors review the different etiologies that have to be discussed, in order to choose the appropriate further investigations and specialized examinations, before a possible surgical treatment.


Assuntos
Doenças Labiais/fisiopatologia , Doenças Labiais/cirurgia , Lábio/anormalidades , Lábio/fisiologia , Transtornos dos Movimentos/fisiopatologia , Transtornos dos Movimentos/cirurgia , Paralisia Facial/complicações , Humanos , Cinética , Doenças Labiais/psicologia , Transtornos dos Movimentos/psicologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA