RESUMEN
Wernicke's encephalopathy (WE) is most commonly associated with alcoholism, although other causes have also been implicated. In the years 1994-1997, 9 patients with no history of alcohol abuse presented with acute signs of ophthalmoplegia or nystagmus and ataxia which resolved within 48 h after intravenous thiamine. There were 7 women and 2 men aged 17-57 (7 below the age of 30). Precipitating events included vomiting 2, drastic weight-reducing diet 2, renal colic in a postpartum woman 1, colonic surgery 2 and chronic hemodialysis 1. In 2 patients there was no obvious precipitating event but their history was suggestive of a genetic predisposition. Mental changes were slight or absent in all patients and all of them made good functional recovery. These cases suggest that the diagnosis of WE should be considered more often in nonalcoholics in various clinical settings.
Asunto(s)
Alcoholismo/fisiopatología , Tiamina/administración & dosificación , Encefalopatía de Wernicke/fisiopatología , Enfermedad Aguda , Adolescente , Adulto , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
We describe four patients who developed an encephalopathic syndrome characterized by obtundation or stupor, myoclonic jerks, and asterixis in association with cefuroxime therapy. Three patients had renal failure. These cases suggest that cefuroxime in overdose or in conventional doses in patients with renal failure can cause a reversible encephalopathy. This syndrome may have been unrecognized because it usually occurs in severely ill patients with additional causes for encephalopathy.
Asunto(s)
Encefalopatías/inducido químicamente , Cefuroxima/efectos adversos , Cefalosporinas/efectos adversos , Encefalopatías/fisiopatología , Cefuroxima/administración & dosificación , Cefalosporinas/administración & dosificación , Sobredosis de Droga , Discinesia Inducida por Medicamentos/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mioclonía/inducido químicamente , Fases del Sueño/fisiologíaRESUMEN
Ocular bobbing, dipping and "reversed" ocular dipping were observed and recorded in two patients presenting a severe neurological symptomatology: quadruplegia and coma in one and locked-in syndrome in the other. CT scan showed a pontine infarction in both these patients. This is the first time that the ocular dipping and "reversed" ocular dipping are related to such an anatomic structure.
Asunto(s)
Infarto Cerebral , Movimientos Oculares , Puente , Anciano , Infarto Cerebral/diagnóstico , Infarto Cerebral/diagnóstico por imagen , Electrooculografía , Femenino , Humanos , Persona de Mediana Edad , Puente/diagnóstico por imagen , Cuadriplejía , Tomografía Computarizada por Rayos XRESUMEN
Five cases of motor neuron disease occurred during the period 1983-1987 in 4 small and medium-sized textile factories. These cases were 45% of 11 motor neuron disease patients diagnosed in this period in a population of about 300,000 persons. This observations may be relevant to toxic occupational hazards in the pathogenesis of motor neuron disease.
Asunto(s)
Neuronas Motoras/patología , Enfermedades Neuromusculares/fisiopatología , Enfermedades Profesionales/fisiopatología , Industria Textil , Anciano , Humanos , Masculino , Persona de Mediana EdadRESUMEN
A 52-year-old woman developed symmetric sensorimotor polyneuropathy and cerebellar syndrome after 24 months of amiodarone treatment. Sural nerve biopsy revealed mild demyelination and many inclusion bodies in the axoplasm and in Schwann cell cytoplasm. Improvement in her condition was evident 6 months after discontinuing amiodarone.
Asunto(s)
Amiodarona/efectos adversos , Enfermedades Cerebelosas/inducido químicamente , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Amiodarona/uso terapéutico , Biopsia , Complejos Cardíacos Prematuros/tratamiento farmacológico , Femenino , Humanos , Persona de Mediana Edad , Enfermedades del Sistema Nervioso Periférico/patología , Nervio Sural/patología , Síndrome , Factores de TiempoRESUMEN
A 59-year-old man had proximal weakness and wasting that started in early childhood. EMG was "myopathic," serum CK activity was increased, and muscle biopsy showed accumulations of glycogen. Biochemical studies revealed elevated glycogen concentration and absence of myophosphorylase activity. This unusual presentation of a long-standing, painless, and quite static weakness due to myophosphorylase deficiency represents another example of clinical heterogeneity.
Asunto(s)
Atrofia Muscular/enzimología , Fosforilasas/deficiencia , Humanos , Masculino , Persona de Mediana Edad , Músculos/enzimología , Músculos/patología , Atrofia Muscular/congénito , Atrofia Muscular/patologíaRESUMEN
A 40-year-old woman developed neuropathy and myelopathy with vitamin B12 deficiency without anemia. Motor and sensory nerve conduction velocities were moderately decreased. Sural nerve biopsy showed the coexistence of demyelination and axonal degeneration.
Asunto(s)
Neuritis/diagnóstico , Deficiencia de Vitamina B 12/diagnóstico , Adulto , Biopsia , Femenino , Humanos , Neuritis/etiología , Neuritis/patología , Nervio Sural/ultraestructura , Deficiencia de Vitamina B 12/complicacionesRESUMEN
A 51-year-old man had primary amyloidosis, with typical amyloid neuropathy and signs of motor neuron disease, including widespread fasciculation in limb muscles, tongue atrophy and fasciculation, swallowing and chewing difficulty, symmetric hyperreflexia, and bilateral Hoffmann's signs. Fasciculations, fibrillations, and positive sharp waves were found in electromyography of all muscles tested. Motor nerve conduction velocities were moderately slow. Lambda chains were detected in serum and CSF. Amyloid was found in sural nerve biopsy. This combination of amyloid neuropathy and features of amyotrophic lateral sclerosis is related to the motor neuron disease of plasma cell dyscrasias.
Asunto(s)
Amiloidosis/complicaciones , Neuronas Motoras , Enfermedades Neuromusculares/complicaciones , Enfermedades del Sistema Nervioso Periférico/complicaciones , Humanos , Masculino , Persona de Mediana EdadRESUMEN
A single an-encephalus neonate kidney graft was transplanted into the portal system of a 6-year-old recipient who had previously undergone removal of the right kidney and inferior vena cava because of Wilms tumor. The left kidney ceased to function shortly thereafter. The child was supported very poorly on hemodialysis, and showed repeated very high levels of cytotoxic antibodies in her serum. The first cross-negative kidney graft that was available harbored two main arteries and duplicate collecting system with two very thin ureters. These vascular anatomic and pathologic variations of both donor graft and recipient necessitated the use of the portal system for renal graft venous drainage and the aorta for the graft revascularization. The ureters that had pinpoint-like lumen were inserted together into the lumen of the native ureter stump and fixated. One year after the transplantation the serum creatinine level is 1.8 mg/dL.
Asunto(s)
Neoplasias Renales/cirugía , Trasplante de Riñón , Sistema Porta/cirugía , Tumor de Wilms/cirugía , Aorta/cirugía , Niño , Femenino , Humanos , Recién Nacido , Vena Esplénica/cirugíaRESUMEN
Three anoxic comatose children had EEG alpha-like activity and in two of them mu rhythm was recorded. The paradoxical appearance of these electrical activities in comatose children seems to indicate a grave prognosis. A possible role for barbiturate treatment in this phenomenon is not excluded.