Abstract:
:Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) is a rare mitochondrial disorder. Diagnostic criteria for MELAS include typical manifestations of the disease: stroke-like episodes, encephalopathy, evidence of mitochondrial dysfunction (laboratorial or histological) and known mitochondrial DNA gene mutations. Clinical features of MELAS are not necessarily uniform in the early stages of the disease, and correlations between clinical manifestations and physiopathology have not been fully elucidated. It is estimated that point mutations in the tRNALeu(UUR) gene of the DNAmt, mainly A3243G, are responsible for more of 80% of MELAS cases. Morphological changes seen upon muscle biopsy in MELAS include a substantive proportion of ragged red fibers (RRF) and the presence of vessels with a strong reaction for succinate dehydrogenase. In this review, we discuss mainly diagnostic criterion, clinical and laboratory manifestations, brain images, histology and molecular findings as well as some differential diagnoses and current treatments.
journal_name
Arq Neuropsiquiatrjournal_title
Arquivos de neuro-psiquiatriaauthors
Lorenzoni PJ,Werneck LC,Kay CS,Silvado CE,Scola RHdoi
10.1590/0004-282X20150154subject
Has Abstractpub_date
2015-11-01 00:00:00pages
959-67issue
11eissn
0004-282Xissn
1678-4227pii
S0004-282X2015001100959journal_volume
73pub_type
杂志文章,评审abstract:OBJECTIVE:To establish preoperatively the localization of the cortical projection of the inferior choroidal point (ICP) and use it as a reliable landmark when approaching the temporal horn through a middle temporal gyrus access. To review relevant anatomical features regarding selective amigdalohippocampectomy (AH) for...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282x20140165
更新日期:2014-10-01 00:00:00
abstract::Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneury...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282X20180027
更新日期:2018-05-01 00:00:00
abstract::The Déjérine-Roussy syndrome was not revised for several years by neurologists, although it has been known since 1906. The authors describe a typical case involving thalamic ischemic and calcarine other side cortex lesions emphasizing its study by computed axial tomography and by using new drugs on a vasodilatation, i...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1984000200012
更新日期:1984-06-01 00:00:00
abstract:OBJECTIVE:To evaluate the comorbidity of depressive disorders in patients with refractory temporal lobe epilepsy (TLE). METHOD:We evaluated 25 consecutive patients with refractory TLE (16 women and 9 men), using semi-structured psychiatric interviews, according to the International Classification of Diseases (ICD-10),...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2011000600010
更新日期:2011-10-01 00:00:00
abstract::Therapeutic aspects of convulsive and non convulsive status epilepticus observed in fourteen patients with Lennox-Gastaut syndrome were studied. A temporal relationship between the status epilepticus onset and the beginning of treatment with chlonazepam and diphenylhydantoin was observed; these drugs withdrawal was fo...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1977000300006
更新日期:1977-09-01 00:00:00
abstract:OBJECTIVE:The objective of this study was to evaluate technical, clinical and angiographic results of a nonsurgical series of intracranial aneurysms treated by endovascular approach at Hospital das Clínicas of Medical School of Ribeirão Preto - University of São Paulo. METHOD:Between August 2005 and November 2008, 137...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2010000500017
更新日期:2010-10-01 00:00:00
abstract::Thirteen alcoholic male patients that developed delirium tremens (DT) after admission in a psychiatric hospital for treatment of alcoholism (group I) had their clinical and laboratorial records examined. The laboratory samples were taken during the phase previous at the DT. Data on this group were compared to those of...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1997000100008
更新日期:1997-03-01 00:00:00
abstract::Neurodegeneration with brain iron accumulation (NBIA) represents a heterogeneous and complex group of inherited neurodegenerative diseases, characterized by excessive iron accumulation, particularly in the basal ganglia. Common clinical features of NBIA include movement disorders, particularly parkinsonism and dystoni...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,评审
doi:10.1590/0004-282X20160080
更新日期:2016-07-01 00:00:00
abstract::HTLV-1 antibodies were investigated in serum and in CSF of 150 patients with neurologic disorders mainly myelopathies. The patients were considered into three groups according to the possible relationship of their disease to the presence of HTLV-1 antibodies: no relationship risk (control group), occasional risk group...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1990000400007
更新日期:1990-12-01 00:00:00
abstract:OBJECTIVE:Investigate the auditory pathway in the brainstem of children with acute lymphoblastic leukemia submitted to chemotherapy (by intravenous or intrathecal infusion). METHODS:Fourteen children aged between 2 and 12 years with diagnosis of acute lymphoid leukemia were evaluated. The following procedures were use...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282X20190139
更新日期:2020-02-01 00:00:00
abstract::Stroke mortality rates are higher in Brazil when compared to other countries. The city of São Paulo has a good system of mortality surveillance that allow us to describe the epidemiology of the stroke in the city. Our aim was to describe the stroke mortality pattern by gender and age characterizing the ischemic/ hemor...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2004000600014
更新日期:2004-12-01 00:00:00
abstract::In order to evaluate the cellular component of the inflammatory chronic response, 357 cerebrospinal fluid (CSF) samples from 40 patients with neurocysticercosis (NC) were studied. All patients were treated with usual doses of praziquantel (50 mg/kg/day during 21 days) associated with dexamethasone (12 mg/day). NC diag...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:
更新日期:1987-12-01 00:00:00
abstract::The main objective of this manuscript is to describe some personal and professional aspects of two outstanding researchers and pioneers in neuro-otology. Among other achievements, Margaret Ruth Dix, and Charles Skinner Hallpike, designed the most-used provocative test for positional nystagmus for the diagnosis of lith...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 传,历史文章,杂志文章
doi:10.1590/0004-282X20180075
更新日期:2018-08-01 00:00:00
abstract::Patients with epilepsy face innumerable obstacles in daily life, related to work, permission to drive and interpersonal relationships, which require medical guidance. This paper reports a literature review based on scientific articles and civil and traffic system, as a way to resolve doubts about medical obligations i...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,评审
doi:10.1590/0004-282X20190133
更新日期:2019-10-24 00:00:00
abstract::The purpose was to describe the main features of headache incidence in a hospital community, its frequency and the most requested medical investigation. Due to the stressful work environment, hospital is considered to hold a high-risk population. Interviews and questionnaires were utilized. Of a 1006 files, which were...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1998000400007
更新日期:1998-09-01 00:00:00
abstract::We studied the clinical and evolution characteristics of multiple sclerosis (MS) patients followed since the onset of HUCFF/UFRJ in 1978. The diagnosis of MS was based on Poser's et al. and MC Donald's et al. criteria. From 188 patients, 122 were included. Eighty-five were females. The mean age onset was 32.2 years-ol...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2008000500012
更新日期:2008-09-01 00:00:00
abstract::Alzheimer disease (AD) is usually associated with cognitive, language and behavioral impairments, which can get more and more serious as the disease progresses. The aim of this study is to verify language disorders in the early stages of this disease. Twelve patients meeting criteria for problable AD were evaluated by...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2005000200020
更新日期:2005-06-01 00:00:00
abstract::True neurogenic thoracic outlet syndrome (TOS) is an uncommon disorder despite of be a frequent reason for referral to the EMG laboratories. We describe the second case in the literature of true TOS in a competitive swimmer with progressive weakness and severe atrophy of the left thenar eminence. EMG showed lower trun...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2007000700031
更新日期:2007-12-01 00:00:00
abstract:BACKGROUND:Motor functional neurological disorder (mFND) is a common and disabling condition. There are no evidence-based guidelines for treatment. Long-term outcome is often poor. This study describes the epidemiological profile, symptom pattern and outcome of patients admitted to the Belo Horizonte unit of the SARAH ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282X20200009
更新日期:2020-06-01 00:00:00
abstract:Methods:Review of hospital records and structured telephone interviews of 100 consecutive stroke patients. Forward stepwise logistic regression was used for the statistical analysis. Results:Seventy patients (75%) arrived at the hospital 4.5 hours after stroke symptoms onset. The use of EMS did not improve arrival tim...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282X20160142
更新日期:2016-11-01 00:00:00
abstract:INTRODUCTION:Hormonal fluctuation is responsible for worsening of epileptic seizures during the menstrual cycle. OBJECTIVE:To identify irregularities in the menstrual cycles of women with mesial temporal lobe epilepsy (MTLE) and extratemporal focal epilepsy (ETFE) and correlate the frequency of seizures during the men...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2005000500006
更新日期:2005-09-01 00:00:00
abstract::Cachexia, ageing and disuse and their effects on the human and animals neuromuscular system are reviewed. Disuse induces reduction of muscle fibers (mainly II) diameter with peripheral myofibrils lost; there is no core-targetoid or even reduction on myophosphorilase activity, both typical of denervation; the acetylcho...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,评审
doi:10.1590/s0004-282x1993000300001
更新日期:1993-09-01 00:00:00
abstract:OBJECTIVE:To report a case of ganglioneuroblastoma of cerebellum, with emphasis to the neuroimaging and pathological findings. CASE REPORT:A one year and eight-month-old girl presented with a two-month history of hypoactivity and tremor in the legs. The MRI showed an enhancing cerebellar mass hypointense on T1 and hyp...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2007000200029
更新日期:2007-06-01 00:00:00
abstract::Ketogenic diet (KD) is a high fat and low carbohydrate diet, which controls refractory epilepsy. We analyzed the KD effects on 54 children of the Children's Institute of the University of São Paulo. Efficacy, tolerability, and adverse effects were studied. Response to KD was effective (E) if seizure control was >75%, ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2007000300003
更新日期:2007-06-01 00:00:00
abstract::Functional imaging of brain electrical activity was performed in 25 chronic medicated schizophrenics and 40 controls, analyzing the classical frequency bands (delta, theta, alpha, and beta) of 19-channel EEG during resting state to identify brain regions with deviant activity of different functional significances, usi...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2003000500002
更新日期:2003-09-01 00:00:00
abstract::The best clinical treatment for spinal metastases requires an integrated approach with input from an interdisciplinary cancer team. The principle goals of treatment are maintenance or improvement in neurologic function and ambulation, spinal stability, durable tumor control, and pain relief. The past decade has witnes...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,评审
doi:10.1590/0004-282X20150099
更新日期:2015-09-01 00:00:00
abstract::Twenty-five non-Hodgkin's adult patients of a cohort studied for detection of neurologic involvement were evaluated on the cerebrospinal fluid (CSF) protein profile. CSF and serum were collected in the same occasion. Blood-brain barrier and local synthesis of IgG were studied. There was an incidence of neurologic sign...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1998000500011
更新日期:1998-12-01 00:00:00
abstract:UNLABELLED:This study was thought to characterized clinical and laboratory findings of a narcoleptic patients in an out patients unit at São Paulo, Brazil. METHOD:28 patients underwent polysomnographic recordings (PSG) and Multiple Sleep Latency Test (MSLT) were analyzed according to standard criteria. The analysis of...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2010000500007
更新日期:2010-10-01 00:00:00
abstract::Four hundred thirty one epileptic patients, aged 10 years or more old, have been studied in the neurology clinic of Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da U.S.P., from February to July, 1981. The authors selected 119 patients aged between 13 to 45 years, that presented periodic menstrual b...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1983000200004
更新日期:1983-06-01 00:00:00
abstract::A case of central nervous system histoplasmosis located at the pons is reported. The authors point out difficulties for the diagnosis of the pathology during life and some aspects of the treatment and their results. The rarity of the process is emphasized. ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:
更新日期:1984-12-01 00:00:00