Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 6 de 6
Mais filtros

Base de dados
Tipo de estudo
Intervalo de ano de publicação
Arthritis Res Ther ; 22(1): 5, 2020 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-31915059


OBJECTIVE: To describe successful therapeutic strategies in statin-induced anti-HMGCR myopathy. METHODS: Retrospective data from a cohort of 55 patients with statin-induced anti-HMGCR myopathy, sequentially stratified by the presence of proximal weakness, early remission, and corticosteroid and IVIG use at treatment induction, were analyzed for optimal successful induction and maintenance of remission strategies. RESULTS: A total of 14 patients achieved remission with a corticosteroid-free induction strategy (25%). In 41 patients treated with corticosteroids, only 4 patients (10%) failed an initial triple steroid/IVIG/steroid-sparing immunosuppressant (SSI) induction strategy. Delay in treatment initiation was independently associated with lower odds of successful maintenance with immunosuppressant monotherapy (OR 0.92, 95% CI 0.85 to 0.97, P = 0.015). While 22 patients (40%) presented with normal strength, only 9 had normal strength at initiation of treatment. CONCLUSION: While corticosteroid-free treatment of anti-HMGCR myopathy is now a safe option in selected cases, initial triple steroid/IVIG/SSI was very efficacious in induction. Delays in treatment initiation and, as a corollary, delays in achieving remission decrease the odds of achieving successful maintenance with an SSI alone. Avoiding such delays, most notably in patients with normal strength, may reset the natural history of anti-HMGCR myopathy from a refractory entity to a treatable disease.

Muscle Nerve ; 48(2): 301-5, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23424031


INTRODUCTION: Hypertrophy of the nerve roots of the cauda equina may occur with both acquired and inherited neuropathies. Although selective nerve root involvement of the sensory roots has been described and termed chronic inflammatory sensory polyradiculoneuropathy (CISP), selective involvement of the proximal motor roots has not been described. METHODS: Clinical, electrophysiological, MRI, and pathological findings are reported. RESULTS: Here, we report a patient with cauda equina hypertrophy presenting with a pure lower motor neuron syndrome without clinical or electrophysiological evidence of sensory fiber involvement. Bowel and bladder functions were spared. Nerve root biopsy demonstrated abundant onion bulb formations. The patient experienced improvement in motor function with immunomodulatory treatment. CONCLUSIONS: We suggest the term chronic immune demyelinating motor polyradiculopathy (CIMP) to describe this particular form of CIDP, thereby expanding the clinical spectrum of CIDP.

Cauda Equina/patologia , Doença dos Neurônios Motores/etiologia , Doença dos Neurônios Motores/patologia , Polirradiculopatia/complicações , Idoso , Humanos , Hipertrofia/patologia , Laminectomia , Imagem por Ressonância Magnética , Masculino , Neurônios Motores/patologia , Neurônios Motores/ultraestrutura
Muscle Nerve ; 42(4): 604-8, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20730877


High titers of anti-GM1 ganglioside antibodies have been associated with multifocal motor neuropathy, a chronic asymmetric and exclusively motor disorder. We describe a patient with a progressive selective motor but symmetric polyneuropathy, followed over 5 years, with markedly elevated titers of anti-GM1 antibodies. The electrophysiological changes suggestive of motor demyelination were widespread, beyond conduction block alone, and involved contiguous nerve segments with complete sparing of sensory conduction. Progressive, predominantly motor, symmetric, demyelinating polyneuropathy may be an unusual relative of multifocal motor neuropathy, associated with anti-GM1 antibodies.

Anticorpos Anti-Idiotípicos/sangue , Doenças Desmielinizantes/imunologia , Gangliosídeo G(M1)/imunologia , Polineuropatias/imunologia , Potenciais de Ação , Adulto , Axônios/patologia , Doenças Desmielinizantes/patologia , Doenças Desmielinizantes/fisiopatologia , Doenças Desmielinizantes/terapia , Pessoas com Deficiência , Progressão da Doença , Extremidades/inervação , Humanos , Masculino , Músculo Esquelético/fisiopatologia , Fibras Nervosas Mielinizadas/patologia , Condução Nervosa , Nervo Fibular/patologia , Troca Plasmática , Polineuropatias/patologia , Polineuropatias/fisiopatologia , Polineuropatias/terapia , Índice de Gravidade de Doença
Curr Opin Neurol ; 22(5): 543-53, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19745731


PURPOSE OF REVIEW: The purpose of this review is to discuss the role of muscle biopsy in the current age of genetic testing. RECENT FINDINGS: The diagnostic approach to patients with suspected genetically determined myopathies has been altered by recent advances in molecular diagnostic technologies and by the increased number of conditions for which the genetic alterations have been identified. Myopathological aspects can narrow down the differential diagnosis when the clinical phenotype is not informative enough and can help guide the molecular investigation. SUMMARY: Here, we review genetic and myopathological aspects of selected genetically determined myopathies.

Testes Genéticos/métodos , Músculo Esquelético/patologia , Doenças Musculares/genética , Doenças Musculares/patologia , Biópsia/métodos , Humanos
Ann Neurol ; 65(1): 7-11, 2009 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19194875


Sporadic inclusion body myositis is the commonest acquired disease of skeletal muscles after 50 years of age, and as such it has commanded a great deal of attention of investigators over the past 25 years. As a result, a large amount of information has accumulated concerning its clinical profile, myopathology, and immunopathology. In the myopathology and immunopathology, there is general agreement that the characteristic features could be divided into a degenerative and an inflammatory group. However, there has been controversy about the possible role of these changes in the pathogenesis of muscle fiber damage. In particular, there is no agreement whether a cause-and-effect relationship exists between these two groups of changes, and if so, which is the primary one. In this brief overview, we examine the validity of the various controversial observations and critically review the justification for the two major hypotheses for the primary role of inflammation versus degeneration.

Miosite de Corpos de Inclusão/etiologia , Miosite de Corpos de Inclusão/patologia , Fatores Etários , Progressão da Doença , Humanos , Inflamação , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Miosite de Corpos de Inclusão/complicações