RESUMO
The appearance of tremors in patients with childhood, chronic spinal muscular atrophy has been known for years. We were struck by the presence of a "muscle tremor" artifact in the ECGs of all our patients with this diagnosis. This observation has not been noted previously. The consistency of this finding in patients with this disease is the basis for this report.
Assuntos
Eletrocardiografia , Atrofia Muscular/diagnóstico , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Coluna VertebralRESUMO
One hundred patients with motor unit disease were examined to determine the diagnostc reliability of several clinical signs of large motor units. These signs were high-intensity, low-pitched rumbling on skeletal muscle auscultation, voluntary contraction fasciculations, contraction fasciculation trembling, and palpable contraction fasciculations. Among 22 cases of spinal muscular atrophy, contration fasciculation trembling was noted in 86 percent, abnormal muscle auscultation in 68 percent, palpable contraction fasciculations in 64 percent, and voluntary contraction fasciculations in 50 percent.
Assuntos
Neurônios Motores/fisiologia , Atrofia Muscular/fisiopatologia , Doenças Neuromusculares/fisiopatologia , Adolescente , Adulto , Auscultação , Criança , Pré-Escolar , Humanos , Métodos , Pessoa de Meia-Idade , Contração MuscularRESUMO
A motor polyneuropathy developed in a woman with subcorneal pustular dermatosis of 16 years' duration, who had received at least 300 gm of dapsone over a five-year period and 80 gm during the four-month period of progression of her neurologic symptoms. Although the patient believed her muscle power had returned to normal four months after the drug was stopped, a slight peripheral neuropathy remained. Electrodiagnostic and clinical features during the period of greatest weakness and the subsequent 16 months were consistent with a polyneuropathy of the axonal type. The neurotoxicity of dapsone appears to be dose-dependent, but the mechanism by which it occurs is unknown.
Assuntos
Dapsona/efeitos adversos , Neurônios Motores , Doenças Neuromusculares/induzido quimicamente , Dermatopatias/tratamento farmacológico , Adulto , Dapsona/administração & dosagem , Relação Dose-Resposta a Droga , Feminino , Humanos , Degeneração Neural , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Prednisona/uso terapêutico , Dermatopatias/patologia , Sulfapiridina/uso terapêuticoRESUMO
We report an unusual case of massive intraventricular spread of B-cell lymphoma of the breast, presenting with rapidly progressive ataxia and impaired cognition with need for ventriculostomy. Rapid resolution followed intravenous dexamethasone and radiation therapy.