IgM anti-GM1 antibody titers in patients with monomelic amyotrophy.

BACKGROUND: Monomelic amyotrophy (MMA) is a benign motor neuron disorder, which particularly affects young people and the etiology is still unknown. Gangliosides are located on the outer surface of motor neurons. Anti-GM1 antibodies have been found to be elevated in multi-focal motor neuropathy with conduction block and other neurological diseases, which may have therapeutic implication. AIM: To evaluate IgM anti-GM1 antibody titers in patients of monomelic amyotrophy. SETTING AND DESIGN: prospective controlled study. MATERIALS AND METHODS: Forty-six clinically and electrophysiologically diagnosed cases of MMA were assessed for IgM anti-GM1 antibody titers by enzyme-linked immunosorbent assay (ELISA) method and compared with titers in healthy controls, cases of amyotrophic lateral sclerosis (ALS) and acute inflammatory demyelinating polyneuropathy (AIDP). Titer of 800 units was taken as upper limit of normal (Buhlmann Laboratories AG, Switzerland). STATISTICAL ANALYSIS USED: one-way ANOVA. RESULTS: The mean age of 46 patients with MMA was 24.5 (+/- 7.3) years, with male female ratio of 44:2. The mean age of 19 healthy controls was 24.1 (+/- 3) years with male: female ratio of 18:1. Five (26%) individuals in the healthy control group, 22 (48%) patients of MMA, four (30%) of ALS and five (50%) of AIDP had high titers of IgM anti-GM1 antibody (P> 0.05). CONCLUSIONS: Although larger number of patients with MMA had higher IgM anti-GM1 antibody titers, the difference was not statistically significant from titers of healthy individuals and of patients in the ALS and AIDP group.

Are there causal relationships between the development of the inflammatory diseases amyotrophic lateral sclerosis and asthma?

Amyotrophic lateral sclerosis (ALS) and asthma are inflammatory diseases. ALS is a fatal progressive, neurodegenerative disease with inflammation around the upper and lower motor neurons leading to their degeneration, muscle atrophy, paralysis, and death. Asthma is a chronic inflammatory disease with reversible airway obstruction and nonspecific airway hyper-reactivity. The local release of sensory neuropeptides from capsaicin-sensitive primary afferents causes motor neuron pathophysiology and airway inflammation and hyper-reactivity. While there is no cure for ALS, asthma is managed according to its symptoms and severity, to decrease the symptoms, improve pulmonary function, and reduce morbidity. To determine whether understanding asthma may provide insights into how to clinically deal with ALS, the authors examined the etiologies of ALS and asthma, and the factors that exacerbate the symptoms. Although no direct correlations were found, the similar multifactorial triggers, and the critical roles of neuronal inflammation, suggest that one or more exists.

Evolutive levels of NGF in neurodegenerative disorders

The two-site enzyme immunoassasy (EIA) using the monoclonal antibody (MAb) 27/21 is a valuable method capable of detecting mouse and human NGF quantitatively (Soderstrom et al., 1990). The presence of NGF in serum has been controversial, since t6he previous assay methods failed to detect circulating NGF. Recently, we described the immunological detection of low levels of NGF in human serum samples and introduced a blocking test validating the specificity of the immunoreactivity for NGF in human serum (Lorigados et al., 1982). In the present work, we applied this two-site EIA using monoclonal NGF antibody 27/21 in the study of NGF serum levels from diverse neurodegenerative disorders. We also studied evolutive samples of Parkinson's patients that received neural transplant

Expression of HLA-DR in pheripheral nerve of amyotrophic lateral sclerosis

Para investigar a possibilidade de comprometimento auto imune no nervo perifárico de pacientes com esclerose lateral amiotrófica (ELA) 83 biópsias do nervo periférico de 79 pacientes (51 sexo masculino, 28 sexo feminino) com média de idade de 62 anos (variaçäo de 19 a 82) foram congelada e coradas para demonstraçäo de HLA-DR usando anticorpo monoclonal. Quarenta amostras (48 por cento) mostraram expressäo nitidamente aumentada de HLA-DR na regiäo endoneural. Usando proteínas S-100 como marcador, demonstramos que HLA-DR se expressava principalmente nas células de Schwann. Näo encontramos co-expressäo com HLA-DR usando anticorpos antineurofilamento ou antimielina, mas detectamos co-expressäo de HLA-DR e antireceptor de fator de crescimento nervoso na maioria das células HLA-DR positivas. Células inflamatórias foram encontradas ocasionalmente, sendo detectadas em somente 11 casos, predominantemente ao redor de vasos sanguíneos epineurais. Biopsias de nervo sensitivo e motor feitas simultaneamente mostraram maior expressäo de HLA-DR em nervos motores de 2 dos 4 pacientes. A significância desses achados ainda näo é clara. A presença de células endoneurais expressando HLA-DR sugere que mecanismos auto imunes podem estar envolvidos na ELA tendo a célula de Schwann como um dos principais alvos

Perspectivas em doenças neuromusculares: 2. Esclerose lateral amiotrófica / Perspectives in neuromuscular diseases: 2. Amyotrophic lateral sclerosis

Desde a criaçåo clássica de Chacort em 1874, pouco veio å ser acrescentado à esclerose lateral amiotrófica (ELA) sob o ponto de vista clínico, patológico e terapêutico eficaz. Na última década, alguns estudos epidemiológicos, imunológicos e novas tendências terapêuticas levantaram novas hipóteses sobre a patogenia dessa doença. Sob o ponto de vista clínico, melhores conhecimentos foram se acumulando através de estudos da história natural da doença, que permitirá avaliar com mais segurança novas medidas terapêuticas. Abaixo vamos relacionar os principais pontos e que representam linhas de pesquisa, que permitem abrir algumas perspectivas na elucidaçåo do mecanismo básico da ELA