Abstract:
:Myotonia is the phenomenon of decrease of muscular relaxation rate, after either a contraction or a mechanical or electrical stimulus. Congenital myotonias are hereditary affections and do not present muscular dystrophy. The current trend is to group them as ionic channels diseases, together with the periodic paralysis. The authors accompanied the cases of seven patients, six males and one female, with ages ranging from 16 to 48 years (average 27 years) and onset of symptoms between 1 and 10 years (average 5 years). These patients presented a myotonic phenomenon unleashed by intensive contraction and global muscular hypertrophy. Three patients were diagnosed as cases of Becker type generalized myotonia because they presented a recessive autosomic heredity and/or transient episodes of muscular weakness. Two patients fitted the description of Thomsen congenital myotonia, with a pattern of dominating autosomic heredity and/or absence of weakness episodes or worsening factors for their condition. Two patients presented fluctuating myotonia, which because worse in cold weather or at potassium intake. The clinical diagnosis was confirmed through complementary tests (electroneuromyography, muscle biopsy and DNA study). Each of the patients made use of different drugs, in the search of optimal lessening of their myotonia. There were five reports of amelioration with the use of diphenilhydantoine; one report with the use of carbamazepine; three reports with the use of acetazolamide; one report with the use of a calcium channel blocker; one report with the use of a beta-adrenergic; one report with the use of thiazide; and none with the use of quinidine/procainamide.
journal_name
Arq Neuropsiquiatrjournal_title
Arquivos de neuro-psiquiatriaauthors
Azevedo HC,Mendonça LI,Salum PN,Carvalho MS,Nagahashi-Marie SK,Siqueira-Carvalho AA,Cerqueira MA,Reed UC,Levy JAdoi
10.1590/s0004-282x1996000400008subject
Has Abstractpub_date
1996-12-01 00:00:00pages
595-600issue
4eissn
0004-282Xissn
1678-4227journal_volume
54pub_type
杂志文章abstract::Mortality due to epilepsy is of great concern worldwide. Individuals with epilepsy have a two- or three-fold risk of death when compared to the general population. Based on biographical data and Anna Grigoriévna Dostoevskaia's memories, the authors concluded that a prolonged episode of status epilepticus was the culpr...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 历史文章,杂志文章
doi:10.1590/0004-282X20160147
更新日期:2016-11-01 00:00:00
abstract::With the increase in life expectancy in Brazil, concerns have grown about the most prevalent diseases in elderly people. Among these diseases are neurodegenerative diseases, such as Alzheimer's and Parkinson's diseases. Protein deposits related to the development of these diseases can pre-date the symptomatic phases b...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/0004-282X20150066
更新日期:2015-07-01 00:00:00
abstract::The purpose of this paper is to report a case of false aneurysm of the middle meningeal artery. The authors emphasize the value of "common" carotid angiography in diagnosis and treatment of this rare lesion. ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1993000300021
更新日期:1993-09-01 00:00:00
abstract::With the objective to show the characteristics of neurocysticercosis (NCC) in Brazil, was performed a critical analysis of national literature which showed a frequency of 1.5% in autopsies and 3.0% in clinical studies, corresponding to 0.3% of all admissions in general hospitals. In seroepidemiological studies the pos...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,meta分析
doi:10.1590/s0004-282x2003000500022
更新日期:2003-09-01 00:00:00
abstract::The electrophysiological studies of 45 patients with Charcot-Marie-Tooth disease (CMT) are presented. The nerve conduction of the motor median and ulnar nerves permitted us to separate our patients in two types: type I (demyelinating) with motor nerve conduction (MNC) below 38 m/s (11 cases) and type II with MNC norma...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 临床试验,杂志文章
doi:10.1590/s0004-282x1995000400002
更新日期:1995-09-01 00:00:00
abstract::The pterional craniotomy is one of the most frequently surgical approaches used in neurosurgery and currently it has become a mainsteam. It allows excellent microsurgical exposure of anterior and posterior regions of the arterial circle of Willis, supra and paraselar regions, the superior orbital fissure of sphenoid b...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,评审
doi:10.1590/s0004-282x2010000300020
更新日期:2010-06-01 00:00:00
abstract:OBJECTIVE:To evaluate the possible association between celiac disease (CD) and/or gluten sensitivity (GS) and autism spectrum disorder (ASD). METHODS:Occurrences of CD were determined in a group of children and adolescents affected by ASD and, conversely, occurrences of ASD were assessed in a group of biopsy-proven ce...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2012000100007
更新日期:2012-01-01 00:00:00
abstract:OBJECTIVE:To describe the etiologies of West syndrome (WS) among children followed in a rehabilitation center. METHOD:Retrospective study with emphasis in the following items: gender, age at the diagnosis of WS and its etiology. The etiologies were divided into three categories: symptomatic, cryptogenic and idiopathic...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2007000400022
更新日期:2007-09-01 00:00:00
abstract::The idiopathic facial paralysis or Bell's palsy installs abruptly or within a few hours, without any apparent cause. It corresponds to approximately 75% of all peripheral facial palsies. Three theories try to explain its pathogenecity: vascular-ischemic, viral and auto-immune. We reviewed the records of the EMG Sector...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1997000500007
更新日期:1997-12-01 00:00:00
abstract::The results obtained in a casuistic of 170 patients with mental diseases and behavior disturbances treated by neurosurgical procedures are reported. The authors call attention to the advantages of the atraumatic stereotaxic psychosurgery over the major surgical procedures on the frontal lobes for the treatment of psyc...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1977000300004
更新日期:1977-09-01 00:00:00
abstract::Chédiak-Higashi syndrome is a rare hematological disease characterized by increased fusion of cytoplasmatic granules. Neurological symptoms occur in approximately half of the patients. We describe the clinical, eletrophysiological, hematological and radiological findings in a girl who had Chédiak-Higashi syndrome and ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1999000300024
更新日期:1999-06-01 00:00:00
abstract::We report the case of a 41-year-old man with iatrogenic Creutzfeldt-Jakob disease (CJD) acquired after the use of growth hormone (GH) obtained from a number of pituitary glands sourced from autopsy material. The incubation period of the disease (from the midpoint of treatment to the onset of clinical symptoms) was rat...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2002000300022
更新日期:2002-06-01 00:00:00
abstract::The authors report a case of chronic bulbar muscular dysfunction in a thyrotoxic man, with complete remission of the symptoms after the use of propranolol, with and without concomitant use of propylthiouracil. Some aspects of this unique complication of hyperthyroidism are discussed. ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1988000400010
更新日期:1988-12-01 00:00:00
abstract::A 42 year-old woman developed paraplegia that resolved in six months, followed by sudden right hemiparesis and dysphasia two years later. The clinical work-up, including CT and MR scans, visual evoked potential, CSF examination and cerebral biopsy suggested the possibility of either multiple sclerosis or multiphasic d...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1996000300024
更新日期:1996-09-01 00:00:00
abstract::We studied 1086 AIDS patients in the last six years. Of these 389 (35.82%) had neurological manifestation and 7 (1.8%) male patients had abnormal involuntary movements (parkinsonism in 3, hemichorea-hemiballism in 2, spinal myoclonus in 1 and rubral tremor in another). All patients were men, 5 white and 2 black. Four ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1993000400011
更新日期:1993-12-01 00:00:00
abstract::[This corrects the article doi: 10.1590/0004-282x20180071]. ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章,已发布勘误
doi:10.1590/0004-282x20180071err
更新日期:2018-12-01 00:00:00
abstract:OBJECTIVE:the aim of this report is to warn that sodium valproate used during pregnancy can produce craniosynostosis in the newborn, particularly trigonocephaly. METHOD:we describe a case of trigonocephaly in a six month-old girl, daughter of a young non-smoker couple, whose mother had used phenobarbital 100 mg daily ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:
更新日期:2001-06-01 00:00:00
abstract::We report on four cases of primary orthostatic tremor. The purpose of this study is the rarity of this type of tremor and the differential diagnosis with other tremors. The electrophysiological study showed a 15-20 Hz tremor frequency in our cases. There are clinical, electrophysiological and therapeutic differences o...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2000000100022
更新日期:2000-03-01 00:00:00
abstract::We describe five patients with Schwartz-Jampel syndrome (SJS) examined at the outpatient service for neuromuscular disorders at our Institution from 1996 to 1999 with the objective of emphasizing the characteristic dysmorphic phenotype of SJS and its different clinical forms. Two cases presented SJS-type 1A, two had S...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:
更新日期:2002-09-01 00:00:00
abstract:OBJECTIVE:3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, or statins, have been associated with improved clinical outcomes after ischemic stroke and subarachnoid hemorrhage, but with an increased risk of incidental spontaneous intracerebral hemorrhage (ICH). We investigated whether the statin use ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2011000400008
更新日期:2011-06-01 00:00:00
abstract::Sebum secretion was measured on the forehead of 47 patients with Parkinson's disease before and after treatment with anticholinergic (biperiden), levodopa + AAID and bromocriptine, by the osmic acid technique. Another 100 patients under biperiden, levodopa + AAID or association of both, for at least one year, were als...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1989000100005
更新日期:1989-03-01 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::Encephalitozoonosis is an increasingly important opportunistic protozoan infection in immunocompromised individuals. This study aims to examine the development of an experimental Encephalitozoon cuniculi infection in the central nervous system of immunosuppressed mice. Adult Balb-C mice were inoculated intraperitoneal...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2005000200010
更新日期:2005-06-01 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:OBJECTIVE:To investigate the correlation between facial sensory abnormalities and lesional topography in eight patients with lateral medullary infarcts (LMIs). METHOD:We reviewed eight sequential cases of LMIs admitted to the Neurology Division of Hospital das Clínicas/São Paulo University between July, 2001 and Augus...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2005000600008
更新日期:2005-12-01 00:00:00
abstract:PURPOSE:To assess the outcome of patients with epilepsy treated at primary care health units under the framework of the demonstration project on epilepsy in Brazil, part of the WHO/ILAE/IBE Global Campaign Against Epilepsy. METHOD:We assessed the outcome of patients treated at four primary health units. The staff of t...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2007001000010
更新日期:2007-06-01 00:00:00
abstract::Considering the genetical peculiarity of Charcot-Marie-Tooth's muscular atrophy, a family with three affected brothers was investigated, in which beginning date was discovered to be progressively antedated. Afterwards, this possible variant of anticipation is discussed, because such condition is quite ununsual, not on...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1977000200011
更新日期:1977-06-01 00:00:00
abstract::After brief comments about the angiographic findings on 298 consecutive cases of isquemic or hemorrhagic cerebral vascular strokes, cerebral angiography is emphasized as the main diagnostic method, owing to the variety of physiopathogenic and ethiologic factors involved in the genesis of this syndromes. The results of...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x1977000100002
更新日期:1977-03-01 00:00:00
abstract:OBJECTIVE:The study has been carried out through anthropometric measures of Bahian newborns, to define measures according with the reality of this region. METHOD:Study type transversal cut in term babies with the measures of cephalic perimeter, biauricular and anteroposterior distances, fontanel and cephalic index. R...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2004000400011
更新日期:2004-09-01 00:00:00
abstract::Hallervorden-Spatz syndrome is a neurodegenerative disease, autosomic recessive with two clinical features: early and late onset. Psychiatric, pyramidal and extrapyramidal signs are present in the late subtype. We report the case of a 41-old woman with extrapyramidal signs. Magnetic resonance imaging (MRI) showed the ...
journal_title:Arquivos de neuro-psiquiatria
pub_type: 杂志文章
doi:10.1590/s0004-282x2004000400031
更新日期:2004-09-01 00:00:00