RESUMO
INTRODUCTION: Multifocal motor neuropathy is a severe chronic demyelinating, disimmune, crippling and potentially treatable disease. The clinical features are characterized by a syndrome of progressive motor deficiency with muscle atrophies, fasciculations, areflexia, myokimias and cramps, that sometimes we misdiagnosed as motor neuron disease. Electrophysiologically it is characterized by the occurrence of multifocal conduction blocks of motor fibres. CLINICAL CASES: Three patients with asymmetric multifocal motor neuropathy are reported in whom diagnosis was made by nerve conduction studies. All the patients had developed an asymmetric chronic progressive motor deficit that involved mostly upper limbs. One patient exhibited mild tactile sensory disturbances and a sural nerve biopsy showed moderated involvement of myelinated fibers. All the patients showed electrophysiologically, multifocal conduction blocks of motor fibers with spared conduction through sensory fibers at the same segments where motor conduction was blocked. The response to high doses intravenous cyclophosphamide therapy followed by 100 mg orally for six months, was satisfactory in all three patients. There was no response to previous trials with prednisone, azathioprine, and intravenous immunoglobulin. One patient was also submitted to several sessions of plasma exchange without benefit. The patient had been previously diagnosed as having motor neuron disease. CONCLUSION: The importance of early diagnosis of this disorder and the possibility of successful treatment with high doses of intravenous cyclophosphamide is stressed.
Assuntos
Doenças Desmielinizantes/diagnóstico , Condução Nervosa/fisiologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Nervo Sural/fisiopatologia , Adulto , Antineoplásicos Alquilantes/uso terapêutico , Doença Crônica , Ciclofosfamida/uso terapêutico , Doenças Desmielinizantes/tratamento farmacológico , Diagnóstico Diferencial , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnóstico , Fibras Nervosas/fisiologia , Doenças do Sistema Nervoso Periférico/diagnósticoRESUMO
Se estudian 23 pacientes con polineuritis (PRN) aguda tipo Landry-Guillain-Barré-Strohl (LGBS) ingresados sucesivamente en el Instituto de Neurología y Neurocirugía. Se realizaron extensiones de sangre periférica obtenidas por punción venosa en el codo, coloreadas con MG. Giemsa. Se contaron 100 células mononucleares, clasificándolas en linfocitos grandes, linfocitos pequeños y monocitos. Se consideraron como linfocitos grandes aquellas células de más de 11 micras de diámetro, con escaso citoplasma intensamente azul claro y con un núcleo grande, redondo o ligeramente escotado con cromatina en granos finos y con problables nucléolos. Se clasificaron como monocitos de células grandes -casi siempre superiores a 16 micras- con una relación núcleo-citoplasma menor que las anteriores y con un núcleo arriñonado o en herradura con cromatina más laxa(AU)