Giseli Quintanilha

North America and South America (NA-SA) neuropathy project.

Peripheral neuropathy is a common neurological disorder. There may be important differences and similarities in the diagnosis of peripheral neuropathy between North America (NA) and South America (SA). Neuromuscular databases were searched for neuropathy diagnosis at two North American sites, University of Kansas Medical Center and University of Texas Southwestern Medical Center, and one South American site, Federal Fluminense University in Brazil. All patients were included into one of the six major categories: immune-mediated, diabetic, hereditary, infectious/inflammatory, systemic/metabolic/toxic (not diabetic) and cryptogenic. A comparison of the number of patients in each category was made between North America and South America databases. Total number of cases in North America was 1090 and in South America was 1034 [immune-mediated: NA 215 (19.7%), SA 191 (18%); diabetic: NA 148 (13.5%), SA 236 (23%); hereditary: NA 292 (26.7%), SA 103 (10%); infectious/inflammatory: NA 53 (4.8%), SA 141 (14%); systemic/metabolic/toxic: NA 71 (6.5%), SA 124 (12%); cryptogenic: NA 311 (28.5%), SA 239 (23%)]. Some specific neuropathy comparisons were hereditary neuropathies [Charcot–Marie–Tooth (CMT) cases] in NA 246/292 (84.2%) and SA 60/103 (58%); familial amyloid neuropathy in SA 31/103 (30%) and none in NA. Among infectious neuropathies, cases of human T-lymphotropic virus type 1 (HTLV-1) neuropathy in SA were 36/141(25%), Chagas disease in SA were 13/141(9%) and none for either in NA; cases of neuropathy due to leprosy in NA were 26/53 (49%) and in SA were 39/141(28%). South American tertiary care centers are more likely to see patients with infectious, diabetic and hereditary disorders such as familial amyloid neuropathies. North American tertiary centers are more likely to see patients with CMT. Immune neuropathies and cryptogenic neuropathies were seen equally in North America and South America.

Perfil da dor Neuropática: a propósito do exame neurológico mínimo de 33 pacientes

JUSTIFICATIVA E OBJETIVOS: Ha poucos textos na literatura a lidar com o exame neurologico do paciente com dor neuropatica (DN). O objetivo deste estudo foi avaliar o perfil de pacientes com DN atraves de exame clinico neurologico. METODO: Em estudo observacional, uma serie de casos de pacientes com DN foi acompanhada no periodo de um ano. A avaliacao do exame neurologico foi efetuada durante visita ao ambulatorio e atraves de analise prospectiva. Foram incluidos pacientes cuja intensidade da dor era igual ou maior que seis, segundo a Escala Analogica Visual. RESULTADOS: A dor em queimacao predominou como descritor em 54,5% dos pacientes. A polineuropatia foi o padrao clinico-topografico predominante (48%) com padrao distal e simetrico, em oposicao a quadros de neuropatia multifocal (15,15%). As modalidades termoalgesica e tatil do exame de sensibilidade foram as mais comprometidas, logo acompanhadas por alteracoes motoras e reflexos profundos, enquanto modalidades de sensibilidade proprioceptiva vieram a seguir. Apesar de nenhum sinal ou sintoma ser especifico de DN, a queimacao como sintoma costuma ser atribuida ao acometimento de fibras finas, assim como o padrao tipico destas e a alteracao termico-dolorosa. CONCLUSOES: A historia e os achados do exame fisico sao a chave para o diagnostico de DN. O registro das alteracoes encontradas ao exame deve ressaltar o comprometimento observado e assim nortear a abordagem diagnostica e terapeutica, se curativa ou paliativa.

Mitochondrial neurogastrointestinal encephalomyopathy mimicking chronic inflammatory demyelinating polyradiculoneuropathy.

Departamento de Neurologia e Nucleo de Pesquisa Clinica em Neurologia/Neurociencias da Universidade Federal Fluminense (NeuroUPC-UFF), Rio de Janeiro, Brazil.Correspondence: Osvaldo J. M. Nascimento; Rua Siqueira Campos 53/1204; 22031-070 Rio de Janeiro RJ - Brasil; Email: osvaldo_nascimento@hotmail.com Conflict of interest: There is no conflict of interest to declare.Received 08 September 2011; Received in final form 14 October 2011; Accepted 21 October 2011

Anestesia venosa total (AVT) em lactente com doença de Werdnig-Hoffmann: relato de caso

SUMMARY Resende MAC, Silva EV, Nascimento OJM, Gemal AE, Quintanilha G, Vasconcelos EM – Total Intravenous Anesthesia (TIVA) in an In-fant with Werdnig-Hoffmann Disease. Case Report. BACKGROUND AND OBJECTIVES: Werdnig-Hoffmann disease is the most common cause of hypotonia in infants and its prognosis is worse if it is present shortly after delivery. Symmetrical muscular weakness, areflexia, and fasciculations of the tongue are character-istic. The majority of the infants die before two years of age as a con-sequence of respiratory failure. The present report presents a case in which total intravenous anesthesia was used. CASE REPORT: This is a 1 year old white female weighing 10 kg, physical status ASA III, with Werdnig-Hoffmann disease diagnosed at two months of age. The patient was a candidate for open gas-trostomy, fundus gastroplication, and tracheostomy. After venoclysis, the patient was monitored with cardioscope, non-invasive blood pres-sure, pulse oximeter, precordial stethoscope, and rectal temperature. She was oxygenated and, after bolus administration of atropine (0.3 mg), boluses of remifentanil (20 µg) and propofol (30 mg) were ad-ministered for anesthetic induction. After tracheal intubation, she was ventilated with manual controlled system without CO

Total intravenous anesthesia (TIVA) in an infant with Werdnig-Hoffmann disease. Case report.

BACKGROUND AND OBJECTIVES Werdnig-Hoffmann disease is the most common cause of hypotonia in infants and its prognosis is worse if it is present shortly after delivery. Symmetrical muscular weakness, areflexia, and fasciculations of the tongue are characteristic. The majority of the infants die before two years of age as a consequence of respiratory failure. The present report presents a case in which total intravenous anesthesia was used. CASE REPORT This is a 1 year old white female weighing 10 kg, physical status ASA III, with Werdnig-Hoffmann disease diagnosed at two months of age. The patient was a candidate for open gastrostomy, fundus gastroplication, and tracheostomy. After venoclysis, the patient was monitored with cardioscope, non-invasive blood pressure, pulse oximeter, precordial stethoscope, and rectal temperature. She was oxygenated and, after bolus administration of atropine (0.3 mg), boluses of remifentanil (20 microg) and propofol (30 mg) were administered for anesthetic induction. After tracheal intubation, she was ventilated with manual controlled system without CO(2) absorber, Baraka (Mapleson D system), FGF of 4 L.min(-1), and FiO(2) 0.5 (O(2)/N(2)O). Anesthesia was maintained with continuous manual infusion of propofol, 250 microg x kg(-1) x min(-1), and remifentanil, 0.3 microg x kg(-1) x min(-1). The surgery lasted 150 minutes. The patient regained consciousness 8 minutes after the end of the infusion, ventilating spontaneously. Two hours later, she was transferred to the pediatric unit, being discharged from the hospital on the fourth postoperative day. CONCLUSIONS The choice of anesthetic technique gives priority to the safety associated with the familiarity of handling available drugs. In children with neuromuscular diseases, due to the extremely short duration, total intravenous anesthesia with remifentanil and propofol in infusion systems can have a favorable influence on disease evolution.

Neuropathic pain profile: the basic neurological exam of 33 patients

BACKGROUND AND OBJECTIVES Very few texts in the literature approach the neurologic exam of patients with neuropathic pain (NP). The objective of this study was to evaluate the profile of patients with NP through the neurological exam. METHODS This is an observational study that followed-up patients with NP for one year. The neurologic exam was evaluated at the outpatient clinic and through prospective analysis. Patients whose pain severity was equal or greater than six on the Visual Analogue Scale were included in this study. RESULTS Burning pain predominated, affecting 54.5% of the patients. Unlike multifocal neuropathy (15.15%), distal and symmetrical polyneuropathy was the predominant clinical-topographic pattern (48%). The thermoalgic and tactile modalities of the sensorial exam were affected the most, followed by changes in motor function and deep tendon reflexes, and proprioception. Although NP does not have specific signs and symptoms, burning pain is attributed to the involvement of thin nerve fibers and thermoalgic pain is typical of those changes. CONCLUSIONS History and physical exam findings are key factors in the diagnosis of NP. The log of changes in the physical exam should emphasize the involvement observed, guiding the diagnostic and therapeutic approach, curative or palliative.

Severity and Functional Ability Scale for Amyotrophic Lateral Sclerosis patients:preliminary results

Recebido em: 01/08/08 Revisado em: 02/08/08 a 03/09/08 Aceito em: 04/09/08 Conflito de interesses: nao Neuromuscular Disease Outpatient Division, Federal Fluminense University – UFF, Antonio Pedro University Hospital – HUAP. Physical Therapist, Chair of the Neurological Rehabilitation, Escola Superior de Ensino Helena Antipoff (ESEHA) and Neuroscience Researcher at UFF. Neurologist, Chair of the Neurology Department and Head of Clinical Neurology Staff, UFF. Neurologist, MD, PhD, Chair of the Neurology Department, UFF. Physical therapist, Department of Neuroscience, UFF. Neuropsychiatry Institute, Instituto de Psiquiatria da Universidade do Brasil, UFRJ. Neurolgist, Neurology Department, Deolindo Couto Neurology Institute, UFRJ. MD, PhD, Neurologist, Department of Neurology, Unifesp. Neurologist, Neurology Department, UFF. Medical Student, UFF. PhD, Psychologist, Neuro-Sono, Department of Neurology, Universidade Federal de Sao Paulo. 1.