RESUMEN
In Japan, 156 cases of dura mater-transplanted Creutzfeldt-Jakob disease(dCJD)with a history of Lyodura transplantation have been confirmed until February 2022, with only a few new cases still being identified. The history of Lyodura transplantation is one involving a neurosurgical procedure. The cumulative global number of cases of bovine spongiform encephalopathy-related variant CJD(BSE-related vCJD), which has shaken societies around the world, is 232 as of 2019. Thus, the impact of dCJD on the society in Japan needs no explanation. Thanks to the world's concerted efforts in research and countermeasures, medically induced prion diseases are finally becoming a thing of the past. However, due to the extremely long incubation period of CJD and the difficulty of tracing the source of infection, immediate action in the event of an outbreak is not possible, and efforts must focus on preventing disease outbreaks. Independent of this, approximately 200 cases of solitary and hereditary prion diseases occur annually in Japan. If neurosurgery must be performed on such patients, secondary transmission of prion disease by neurosurgical instruments must be prevented. Therefore, sterilization methods for neurosurgical instruments are critical, and various measures including sterilization methods have been determined and published by a research group designated by the Japanese Ministry of Health, Labour and Welfare. The sterilization of neurosurgical instruments should comply with the latest guidelines that are published by this study group.
Asunto(s)
Síndrome de Creutzfeldt-Jakob , Neurocirugia , Enfermedades por Prión , Priones , Animales , Bovinos , Colágeno , Síndrome de Creutzfeldt-Jakob/prevención & control , Síndrome de Creutzfeldt-Jakob/cirugía , Humanos , Procedimientos Neuroquirúrgicos , Enfermedades por Prión/epidemiología , Enfermedades por Prión/prevención & control , Enfermedades por Prión/cirugíaRESUMEN
Patients with prion diseases can live for long periods of time in a state of akinetic mutism given appropriate management of their symptoms. To study symptom support in these cases, we performed gastrostomies on 3 patients with V180I genetic Creutzfeldt-Jakob disease (CJD) who had become akinetic and mute, and compared them to 14 other similar patients being fed by tube. In the 3 gastrostomy cases, there were no direct complications due to the gastrostomy or tube feeding, nor were there episodes of discontinuation of tube feeding or initiation of continuous drip infusion due to severe complications. Antibiotics were administered for mild infections, a complication of CJD, with 0.2% and 8.8% of the total time after gastrostomy being used for intravenous or transluminal administration, respectively. We compared the present patient series with that of our previous report statistically, and found that patients undergoing gastrostomy required significantly fewer discontinuations of tube feeding than those who did not. No significant difference in antibiotic administration was found between groups, however. It is our conclusion that gastrostomy should be allowed for symptom support in akinetic patients with prion disease, but adequate informed consent must be provided to the patient's family.
Asunto(s)
Síndrome de Creutzfeldt-Jakob/cirugía , Nutrición Enteral/métodos , Gastrostomía/métodos , Enfermedades por Prión/cirugía , Anciano , Mutismo Acinético/tratamiento farmacológico , Mutismo Acinético/etiología , Antibacterianos/administración & dosificación , Síndrome de Creutzfeldt-Jakob/complicaciones , Femenino , Humanos , Infusiones Intravenosas , Enfermedades por Prión/complicacionesRESUMEN
Hyponatremia is the most frequent electrolyte disorder in critically ill neurological patients. The major differential diagnoses in this situation are the syndrome of inappropriate antidiuretic hormone secretion, marked by inappropriate retention of free water, and cerebral salt wasting, characterized by excessive urinary loss of sodium and resulting in polyuria and extracellular volume contraction. Cerebral salt wasting is a syndrome of hyponatremia due to increased urine output and excessive natriuresis described in patients with central nervous system disease. Although cerebral salt wasting has been well described in neurosurgical patients, data regarding pediatric patients is sparse. We present a 34-month-old boy with lissencephaly who developed cerebral salt wasting after brain biopsy. The patient was treated with hypertonic saline and multiple antiepileptic drugs. Fludrocortisone supplementation effectively treated cerebral salt wasting.
Asunto(s)
Fludrocortisona/uso terapéutico , Hiponatremia/etiología , Lisencefalia/cirugía , Enfermedades por Prión/cirugía , Antiinflamatorios/uso terapéutico , Anticonvulsivantes/uso terapéutico , Preescolar , Humanos , Hiponatremia/tratamiento farmacológico , Lisencefalia/diagnóstico , Lisencefalia/patología , Imagen por Resonancia Magnética , Masculino , Poliuria/tratamiento farmacológico , Poliuria/etiología , Enfermedades por Prión/diagnóstico , Convulsiones/tratamiento farmacológico , Convulsiones/etiología , Resultado del TratamientoRESUMEN
Prion diseases are progressive neurological disorders due to abnormal prion protein (PrP(Sc)) deposition in the central nervous system. At present, there is no effective treatment available for any form of prion disease. Pentosan polysulfate (PPS) has been shown to prolong significantly the incubation period in mice with PrP(Sc) infection when administered to the cerebral ventricles in preclinical trials. In human studies conducted in European countries and Japan, intraventricular PPS was administered to patients with different forms of prion disease and was well tolerated. We report 11 patients with prion disease treated with intraventricular PPS at Fukuoka University from 2004. Cases included three familial CJD (two with V180I mutation, one GSS with P102L mutation), two iatrogenic CJD, and six sporadic CJD cases. At present, average survival period after treatment was 24.2 months (range, 4-49). Seven cases died of sepsis and pneumonia. Subdural effusion with various degrees was seen on CT scan in most cases. Except for these, adverse effects did not occur in the treatment period. Although our preliminary study of the new treatment with PPS by continuous intraventricular infusion showed no apparent improvement of clinical features in patients with prion disease, the possibility of extended survival in some patients receiving long-term PPS was suggested.
Asunto(s)
Antiinfecciosos/uso terapéutico , Poliéster Pentosan Sulfúrico/uso terapéutico , Enfermedades por Prión/tratamiento farmacológico , Adulto , Anciano , Antiinfecciosos/administración & dosificación , Encéfalo/diagnóstico por imagen , Encéfalo/cirugía , Cateterismo , Femenino , Estudios de Seguimiento , Humanos , Bombas de Infusión Implantables/efectos adversos , Infusiones Parenterales/efectos adversos , Masculino , Persona de Mediana Edad , Poliéster Pentosan Sulfúrico/administración & dosificación , Neumonía/etiología , Neumonía/mortalidad , Enfermedades por Prión/diagnóstico por imagen , Enfermedades por Prión/cirugía , Estudios Prospectivos , Sepsis/etiología , Sepsis/mortalidad , Factores de Tiempo , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
Bone marrow-derived mesenchymal stem cells (MSCs) have been reported to migrate to brain lesions in experimental models of ischemia, tumors, and neurodegenerative diseases and to ameliorate functional deficits. In this study, we attempted to evaluate the therapeutic potential of MSCs for treating prion diseases. Immortalized human MSCs (hMSCs) that express the LacZ gene were transplanted into the unilateral hippocampi or thalami of mice, and their distributions were monitored by the expression of beta-galactosidase. In mice infected with prions, hMSCs transplanted at 120 days postinoculation (dpi) were detected on the contralateral side at 2 days after transplantation and existed there even at 3 weeks after transplantation. In contrast, few hMSCs were detected on the contralateral side for mock-infected mice. Interestingly, the migration of hMSCs appeared to correlate with the severity of neuropathological lesions, including disease-specific prion protein deposition. The hMSCs also migrated to a prion-specific lesion in the brain, even when intravenously injected. Although the effects were modest, intrahippocampal and intravenous transplantation of hMSCs prolonged the survival of mice infected with prions. A subpopulation of hMSCs in the brains of prion-infected mice produced various trophic factors and differentiated into cells of neuronal and glial lineages. These results suggest that MSCs have promise as a cellular vehicle for the delivery of therapeutic genes to brain lesions associated with prion diseases and, furthermore, that they may help to regenerate neuronal tissues damaged by prion propagation.
Asunto(s)
Diferenciación Celular/inmunología , Trasplante de Células Madre Mesenquimatosas , Células Madre Mesenquimatosas/citología , Células Madre Mesenquimatosas/inmunología , Enfermedades por Prión/inmunología , Animales , Movimiento Celular/inmunología , Proliferación Celular , Femenino , Humanos , Ratones , Enfermedades por Prión/patología , Enfermedades por Prión/cirugía , Tasa de SupervivenciaRESUMEN
BACKGROUND: Epidemiologic evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains controversial. METHODS: From Danish and Swedish registries we selected 167 definite and probable sCJD cases (with onset between 1987 and 2003) and 3,059 controls (835 age-, sex-, and residence-matched, and 2,224 unmatched). Independent of case/control status, surgical histories were obtained from National Hospital Discharge Registries. Surgical procedures were categorized by body system group and lag time to onset of sCJD. Exposure frequencies were compared using logistic regression. RESULTS: A history of any major surgery, conducted >/=20 years before sCJD onset, was more common in cases than both matched (OR = 2.44, 95% CI = 1.46-4.07) and unmatched controls (OR = 2.25, 95% CI = 1.48-3.44). This observation was corroborated by a linear increase in risk per surgical discharge (OR = 1.57, 95% CI = 1.13-2.18; OR = 1.50, 95% CI = 1.18-1.91). Surgery of various body systems, including peripheral vessels, digestive system and spleen, and female genital organs, was significantly associated with increased sCJD risk. CONCLUSIONS: A variety of major surgical procedures constitute a risk factor for sCJD following an incubation period of many years. A considerable number of sCJD cases may originate from health care-related accidental transmission.
Asunto(s)
Síndrome de Creutzfeldt-Jakob/epidemiología , Síndrome de Creutzfeldt-Jakob/cirugía , Procedimientos Quirúrgicos Operativos/efectos adversos , Estudios de Casos y Controles , Síndrome de Creutzfeldt-Jakob/mortalidad , Síndrome de Creutzfeldt-Jakob/transmisión , Dinamarca/epidemiología , Femenino , Humanos , Masculino , Enfermedades por Prión/epidemiología , Enfermedades por Prión/cirugía , Valores de Referencia , Sistema de Registros , Procedimientos Quirúrgicos Operativos/estadística & datos numéricos , Análisis de Supervivencia , Suecia/epidemiología , Factores de Tiempo , Reacción a la TransfusiónRESUMEN
UNLABELLED: BACKGROUND, PURPOSE AND METHODS: This observational study assessed the effect of continuous intraventricular infusion of pentosan polysulphate (PPS) in seven patients at different clinical centres in the UK. RESULTS: Complications of intraventricular catheterization were frequent. PPS was well-tolerated over a wide dose range (11-110 microg/kg/day) during the 6-month study. Four patients were assessed for the entire study period: one remained stable, two showed minimal deterioration and one progressed significantly. CONCLUSION: Mean survival of all patients was longer than reported values for natural history of specific prion disorders. Possible reasons for these findings are explored.
Asunto(s)
Anticoagulantes/administración & dosificación , Poliéster Pentosan Sulfúrico/administración & dosificación , Enfermedades por Prión/tratamiento farmacológico , Adulto , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Intraventriculares/métodos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Examen Neurológico/métodos , Observación , Enfermedades por Prión/mortalidad , Enfermedades por Prión/patología , Enfermedades por Prión/cirugía , Resultado del Tratamiento , Reino UnidoRESUMEN
No disponible
Asunto(s)
Priones/efectos adversos , Enfermedades por Prión/inducido químicamente , Enfermedades por Prión/complicaciones , Enfermedades por Prión/diagnóstico , Síndrome de Creutzfeldt-Jakob/complicaciones , Síndrome de Creutzfeldt-Jakob/cirugía , Biopsia/métodos , Western Blotting/métodos , Inmunohistoquímica/métodos , Enfermedades por Prión/cirugía , Enfermedad Iatrogénica/epidemiología , Duramadre/trasplanteRESUMEN
Prions are a novel class of infectious agents that cause subacute encephalopathy in man and animals as human Creutzfeldt-Jakob disease (CJD), sheep scrapie and bovine spongiform encephalopathy (BSE). Previously, prions were shown to be transmitted by neuro- and ophthalmosurgical measures and by application of brain-derived therapeutic hormones. Recently, prions have been detected in blood specimens of experimentally infected monkeys indicating a principal threat to transfusion medicine, furthermore in human or bovine materials used in reconstructive surgery. In this article the risk of prion transmission from the surgeon to the patient or vice versa during (orthopedic) surgery is reevaluated including the issues of blood transfusion. This is accomplished based on recent epidemiologic findings and biometric calculations on the spread of prions in animals and humans as well as in terms of experimental data on artificially contaminated medical materials and devices. The overall risk of prion transmission in orthopedic surgery is considered very low if adequately prepared and sterilized materials and devices are used.