ABSTRACT
Motor neuron disease (MND) is a systemic disease with a broad clinical spectrum. It is characterized by primary involvement of the lower or upper motor neuron (UMN), or both, simultaneously, represented by the most common form, amyotrophic lateral sclerosis (ALS). ALS is rapidly progressive and fatal disease that evolve to death due to respiratory failure, on average, in three to five years since the onset of symptoms. This fact attends to the early and correct diagnosis of the disease. OBJECTIVE: To evaluate clinical, epidemiological and electrophysiological variables for the early diagnosis of ALS. METHODS: This is an observational, descriptive and retrospective study, conducted from the collect of the database, in which the variables were submitted to statistical analysis: Mann-Whitney test and Fisher's exact test. RESULTS: When correlating clinical, epidemiological and electrophysiological findings of patients with ALS and other forms of MND, the variables: age of onset of symptoms (P=0,02) hyperreflexia (P=0,001), presence of bulbar symptoms/signs (P<0,001), pathological reflexes (P=0.001), and presence of fasciculation in electromyography (P=0,001) presented statistical significance for the diagnosis of ALS. CONCLUSION: Despite the small sample size, the findings reinforce the importance of well- done neurological examination, to search for signs of involvement of the UMN, in the first evaluation of patients with suspected MND. And that more research is needed to better understand the different phenotypes of the disease in order to obtain an increasingly early diagnosis to offer improvements in the quality of life of these patients
A doença do neurônio motor (DNM) é uma doença sistêmica com amplo espectro clínico. É caracterizada pelo envolvimento primário do neurônio motor inferior ou superior (NMS), ou ambos, simultaneamente, representados pela forma mais comum de esclerose lateral amiotrófica (ELA). A ELA é uma doença rapidamente progressiva e fatal que evolui para óbito devido à insuficiência respiratória, em média, em três a cinco anos desde o início dos sintomas. Esse fato atenta ao diagnóstico precoce e correto da doença. OBJETIVO: Avaliar variáveis clínicas, epidemiológicas e eletrofisiológicas para o diagnóstico precoce de ELA. MÉTODOS: Estudo observacional, descritivo e retrospectivo, realizado a partir da coleta do banco de dados, no qual as variáveis foram submetidas a análises estatísticas: teste de Mann-Whitney e teste exato de Fisher. RESULTADOS: Ao correlacionar achados clínicos, epidemiológicos e eletrofisiológicos de pacientes com ELA e outras formas de DNM, as variáveis: idade de início dos sintomas (P=0,02) hiperreflexia (P=0,001), presença de sintomas/sinais bulbares (P<0,001), reflexos patológicos (P=0,001) e presença de fasciculação na eletromiografia (P=0,001) apresentaram significância estatística para o diagnóstico de ELA. CONCLUSÃO: Apesar do pequeno tamanho da amostra, os achados reforçam a importância do exame neurológico bem feito, na busca de sinais de envolvimento da NMS, na primeira avaliação de pacientes com suspeita de DMN. E que são necessárias mais pesquisas para melhor entendimento dos diferentes fenótipos da doença, a fim de obter um diagnóstico cada vez mais precoce para oferecer melhorias na qualidade de vida desses pacientes
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Amyotrophic Lateral Sclerosis/diagnosis , Amyotrophic Lateral Sclerosis/epidemiology , Neuromuscular Diseases/diagnosis , Brazil/epidemiology , Prevalence , Follow-Up Studies , Sensitivity and Specificity , Statistics, Nonparametric , Early Diagnosis , Electromyography , Observational Studies as Topic , Neurologic Examination/methodsABSTRACT
Objetivos: Evaluar las características clínicas neruofisiológicas e histológicas de los casos con neuropatía posterior a completar el tratamiento para la lepra, donde las biopsias revelaron cambios inflamatorios. Pacientes y métodos: Siete pacientes fueron evaluados por un solo neurólogo. En todos los pacientes se obtuvo una electro neuromiografía y biopsia del nervio periférico. Resultados: la edad media era de 50.6 años. El intervalo de tiempo desde la liberación del tratamiento e inicio de síntomas variaba entre 1 y 12 años (media 6.6 años). Los síntomas sensitivos constituyen la principal queja de los afectados, incluyendo dolor (71%) y parestesia (71%). El 51% de los casos presentó debilidad muscular y el 43% de los individuos atrofia muscular. El engrosamiento neural periférico estaba presente en todos los pacientes. Los estudios neurofisiológicos revelaron procesos inflamatorios con fibrosis del endoneuro, perineuro y epineuro y pérdida total o parcial de fibras. La tinción de Wade no detectó ningún bacilo. El tratamiento con corticoesteroides proporcionó algún alivio. Conclusión: Después de finalizar el tratamiento, los pacientes de lepra pueden desarrollar de forma insidiosa síntomas progresivos en los nervios periféricos que no constituyen totalmente el criterio para una recidiva o leprorreacción. Los síntomas sensoriales predominan y el engrosamiento neural periférico constituye un hallazgo importante. Nosotros sospechamos que estos síntomas de presentación tardía son secundarios a procesos crónicos inmunológicamente mediados en respuesta a antígenos de M. leprae (AU)
Objectives: To evaluate the clinical, neurophysiological and histological features of cases of neuropathy developing after completion of antileprosy treatment, where-biopsy showed inflammatory changes. Patients and Methods: Seven patients were evaluated by a single neurologist. Electroneuro-myography and peripheral nerve biopsy were performed in all patients. Results: Median age was 50-6years. Time form release from treatment and onset of symptoms ranged from 1 to 12 years (median of 6-6 years). Sensory symptoms were the most common complaint, incluiding pain (71%) and paresthesiae (71%) Muscle weakness was found in 51% and muscle atrophy in 43% of the subjects. Peripheral sensory-motor polyneuropathy and multiple mono-neuropath. Nerve bipsy showed inflammatory processes with fibrosis of endoneurium, perineurium and epineurium and total or partiar loss of fibres. No bacilli were detected with Wade staning. Patients treated with corticosteroids had some relief of symptoms. Conclusion: After release from treatment, leprosy patients may insidiously develop progressive peripheral nerve symptoms not fulfilling criteria for relapse or leprosy reactions. Sensory symptoms predominate and peripheral nerve thickening is an inmportant finding. We speculate that these late onset symptoms are secondary to chronic immune-mediated processes in response to antigens of M. Leprae (AU)
Subject(s)
Humans , Leprosy/complications , Mycobacterium leprae/pathogenicity , Central Nervous System Infections/epidemiology , Leprostatic Agents/therapeutic useABSTRACT
OBJECTIVES: To evaluate the clinical, neurophysiological and histological features of cases of neuropathy developing after completion of anti-leprosy treatment, where biopsy showed inflammatory changes. PATIENTS AND METHODS: Seven patients were evaluated by a single neurologist. Electro-neuro-myography and peripheral nerve biopsy were performed in all patients. RESULTS: Median age was 50-6 years. Time from release from treatment and onset of symptoms ranged from 1 to 12 years (median of 6.6 years). Sensory symptoms were the most common complaint, including pain (71%) and paresthesiae (71%). Muscle weakness was found in 51% and muscle atrophy in 43% of the subjects. Peripheral nerve thickening was present in all patients. Neurophysiological studies suggested sensory-motor polyneuropathy and multiple mono-neuropathy. Nerve biopsy showed inflammatory processes with fibrosis of endoneurium, perineurium and epineurium and total or partial loss of fibres. No bacilli were detected with Wade staining. Patients treated with corticosteroids had some relief of symptoms. CONCLUSION: After release from treatment, leprosy patients may insidiously develop progressive peripheral nerve symptoms not fulfilling criteria for relapse or leprosy reactions. Sensory symptoms predominate and peripheral nerve thickening is an important finding. We speculate that these late onset symptoms are secondary to chronic immune-mediated processes in response to antigens of M. leprae.
Subject(s)
Leprosy/pathology , Peripheral Nervous System Diseases/microbiology , Adult , Anti-Inflammatory Agents/therapeutic use , Female , Histocytochemistry , Humans , Leprostatic Agents/therapeutic use , Leprosy/drug therapy , Male , Middle Aged , Peripheral Nerves/microbiology , Peripheral Nerves/pathology , Peripheral Nervous System Diseases/pathology , Prednisone/therapeutic useABSTRACT
The Guilllain-Barré syndrome (GBS) is an acute predominantly demyelinating polyneuropathy. In many cases GBS is preceding by infection, immunization, surgery or trauma. Although there are a few reports of GBS after head trauma, there is no report of this syndrome after brachial plexus injury. We report on a 51 years-old man who presented GBS fifteen days after a brachial plexus trauma. The polineuropathy resolved completely in a few weeks. We believe that GBS was triggered by the trauma that evoked an immune mediated disorder producing inflammation and demyelination of the peripheral nerves.
A síndrome de Guillain-Barré (SGB) é uma polineuropatia predominantemente desmielinizante, que ocorre na maioria das vezes após uma infecção, vacinação, cirurgia ou traumatismo. Embora tenham sido descritos alguns casos após traumatismo crânio encefálico, ainda não foi referido caso de SGB após traumatismo do plexo braquial. Relatamos o caso de um homem de 51 anos que 15 dias após ter apresentado paralisia traumática do plexo braquial, desenvolveu SGB. Recuperou-se inteiramente em algumas semanas. Achamos que em nosso caso a SGB foi desencadeada pelo traumatismo, que provocou distúrbios imunológicos com conseqüente acometimento dos nervos periféricos.
Subject(s)
Humans , Male , Middle Aged , Brachial Plexus/injuries , Guillain-Barre Syndrome/etiology , Guillain-Barre Syndrome/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Treatment OutcomeABSTRACT
The Guilllain-Barré syndrome (GBS) is an acute predominantly demyelinating polyneuropathy. In many cases GBS is preceding by infection, immunization, surgery or trauma. Although there are a few reports of GBS after head trauma, there is no report of this syndrome after brachial plexus injury. We report on a 51 years-old man who presented GBS fifteen days after a brachial plexus trauma. The polineuropathy resolved completely in a few weeks. We believe that GBS was triggered by the trauma that evoked an immune mediated disorder producing inflammation and demyelination of the peripheral nerves.
Subject(s)
Brachial Plexus/injuries , Guillain-Barre Syndrome/etiology , Guillain-Barre Syndrome/drug therapy , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Middle Aged , Treatment OutcomeABSTRACT
O estado atual dos conhecimentos sobre as alteraçöes musculares na desnutriçäo protéica experimental é apresentado. Apontam os autores para os aspectos bioquímicos e histológicos ressaltando a importância dos estudos histoquímicos. Consideram os primeiros relatos sobre o dano muscular em pacientes desnutridos. Chamam a atençäo para os poucos trabalhos encontrados quanto ao efeito da desnutriçäo sobre o músculo, na literatura, comentando os principais trabalhos realizados em nosso meio
Subject(s)
Rats , Animals , Muscles/metabolism , Protein-Energy Malnutrition , Muscles/pathology , PhotomicrographyABSTRACT
O estado atual dos conhecimentos sobre as alteracoes musculares na desnutricao proteica experimental e apresentado. Apontam os autores para os aspectos bioquimicos e histologicos ressaltando a importancia dos estudos histoquimicos. Consideram os primeiros relatos sobre o dano muscular em pacientes desnutridos. Chamam a atencao para os poucos trabalhos encontrados quanto ao efeito da desnutricao sobre o musculo, na literatura, comentando os principais trabalhos realizados em nosso meio.
