25 documents found, page 1 of 3
Derek Denny-Brown: the man behind the ganglia
Martinez,Alberto R. M.; Faber,Ingrid; Martins Jr,Carlos Roberto; Casseb,Raphael F.; Nucci,Anamarli; França Jr,Marcondes C.; Teive,Hélio A. G.ABSTRACT The authors present an historical review about the main contributions of Professor Derek Denny-Brown to neurology. Some of his achievements include the first description of sensory neuronopathies, and some of the essential textbooks on the function and anatomy of the basal ganglia. In 2016, on the 35th anniversary of his death, modern neurologists are still strongly influenced by his legacy.
Brazilian consensus on Duchenne muscular dystrophy. Part 1: diagnosis, steroid ...
Araujo,Alexandra P. Q. C.; Carvalho,Alzira A. S. de; Cavalcanti,Eduardo B. U.; Saute,Jonas Alex M.; Carvalho,Elmano; França Junior,Marcondes C.ABSTRACT Significant advances in the understanding and management of Duchenne muscular dystrophy (DMD) took place since international guidelines were published in 2010. Our objective was to provide an evidence-based national consensus statement for multidisciplinary care of DMD in Brazil. A combination of the Delphi technique with a systematic review of studies from 2010 to 2016 was employed to classify evidenc...
Myotonic dystrophy type 1: frequency of ophthalmologic findings
Ikeda,Karin Suzete; Iwabe-Marchese,Cristina; França Jr,Marcondes Cavalcante; Nucci,Anamarli; Carvalho,Keila Monteiro deABSTRACT The purpose of the study was to evaluate the frequency of ophthalmologic abnormalities in a cohort of myotonic dystrophy type 1 (DM1) patients and to correlate them with motor function. We reviewed the pathophysiology of cataract and low intraocular pressure (IOP). Method Patients were included after clinical and laboratory diagnosis and after signed informed consent. They were evaluated by Motor Funct...
Ulnar sensory-motor amplitude ratio: a new tool to differentiate ganglionopathy...
Garcia,Raphael Ubirajara; Ricardo,João Adilson Gama; Horta,Cassiana Abreu; Garibaldi,Solange Garcia; Nucci,Anamarli; França Junior,Marcondes CavalcanteThe objective of this study was to evaluate if the ratio of ulnar sensory nerve action potential (SNAP) over compound muscle action potential (CMAP) amplitudes (USMAR) would help in the distinction between ganglionopathy (GNP) and polyneuropathy (PNP).MethodsWe reviewed the nerve conductions studies and electromyography (EMG) of 18 GNP patients, 33 diabetic PNP patients and 56 controls. GNP was defined by simul...
Evento com aparente risco de morte recorrente como manifestação inicial de sínd...
Romaneli,Mariana Tresoldi das N.; Castro,Carla Cristina T. de S.; Fraga,Andrea de Melo A.; Lomazi,Elizete Aparecida; Nucci,AnamarliOBJETIVO: Alertar os pediatras sobre a necessidade de investigar criteriosamente a etiologia de eventos com aparente risco de morte recorrente. Não foram encontrados relatos associando tais eventos à miastenia congênita. DESCRIÇÃO DO CASO: Lactente de sete meses apresentando história de eventos com aparente risco de morte recorrente foi internado para investigação. Durante a internação, apresentou cianose e dis...
Clinical attention and assistance profile of patients with amyotrophic lateral ...
Lima,Núbia Maria Freire Vieira; Nucci,AnamarliOBJECTIVE: To evaluate the functional status of amyotrophic lateral sclerosis (ALS) patients diagnosed at this institution; to analyze hospital and palliative care; to identify patients' knowledge about home care and supportive resources. METHOD: Twenty-nine patients were evaluated on the ALSFRS-R scale and two semi-structured questionnaires, at the start of the study and every four months thereafter for 1 year...
Cerebellar atrophy is frequently associated with non-paraneoplastic sensory neu...
Damasceno,Alfredo; França Jr,Marcondes C.; Cendes,Fernando; Nucci,AnamarliSensory neuronopathies (SN) are peripheral nervous system disorders associated with degeneration of dorsal root ganglion neurons. Despite the evidence of a defective proprioceptive sensory input in SN,the prominent gait and truncal ataxia raises the question of a concomitant involvement of the cerebellum. OBJECTIVE: To evaluate cerebellar atrophy in SN. METHOD: We analyzed MRI-based volumetry of anterior lobe (...
Amyotrophic lateral sclerosis: prospective study on respiratory parameters
Almeida,Sara Regina Meira; Silva,Luciano Bruno C.; Guerreiro,Carlos Alberto M.; Nucci,AnamarliOBJECTIVE: To verify how efficient respiratory parameters are in the follow-up of subjects with amyotrophic lateral sclerosis (ALS) and to observe possible correlations between respiratory and nutritional functions. METHOD: Sixteen patients with probable or defined ALS were selected and evaluated over eight months using the following respiratory parameters: spirometry, maximum inspiratory pressure (MIP), maximu...
Effect of nutritional supplementation with milk whey proteins in amyotrophic la...
Silva,Luciano Bruno de Carvalho; Mourão,Lucia Figueiredo; Silva,Ariovaldo Armando; Lima,Núbia Maria Freire Vieira; Almeida,Sara ReginaOBJECTIVE: We evaluated the efficacy of oral supplementation with milk whey proteins and modified starch (70%WPI:30%MS), on nutritional and functional parameters of patients with ALS. METHOD: A prospective randomized double-blind study was performed with 16 ALS patients, divided in two groups, the treatment group received (70%WPI:30%MS) and the control group received (maltodextrin). They underwent prospective n...
Fatores associados à maior mortalidade e tempo de internação prolongado em uma ...
Oliveira,Ana Beatriz Francioso de; Dias,Olivia Meira; Mello,Marcos Moreira; Araújo,Sebastião; Dragosavac,Desanka; Nucci,AnamarliOBJETIVO: A unidade de terapia intensiva é sinônimo de gravidade e apresenta taxa de mortalidade entre 5,4% e 33%. Com o aperfeiçoamento de novas tecnologias, o paciente pode ser mantido por longo período nessa unidade, ocasionando altos custos financeiros, morais e psicológicos para todos os envolvidos. O objetivo do presente estudo foi avaliar os fatores associados à maior mortalidade e tempo de internação pr...