RESUMEN
OBJECTIVE: We hypothesized that the extensive epileptic network in patients with tuberous sclerosis complex (TSC) manifests as clustered and scattered distributions of magnetoencephalography spike sources (MEGSS). METHODS: We retrospectively analyzed pre-surgical MEG in 15 patients with TSC. We performed single moving dipole analysis to localize and classify clustered and scattered MEGSS. We compared the number of electrodes within the resected area (RA) and the proportions of clustered and scattered MEGSS within RA with the seizure outcome. RESULTS: The number of electrodes within RA ranged from 29 to 83 (mean=51). The MEGSS were distributed over multiple lobes (3-8; mean=5.9) and bilaterally in 14 patients. Clusters of MEGSS ranged from 1 to 4 (mean=1.4). The number of MEGSS ranged in total from 28 to 139 (mean=70); in the clusters, 10-128 (mean=49); and in the scatters, 0-45 (mean=21). Four patients achieved an Engel class I surgical outcome, four, a class II outcome; five, a class III outcome; and two, a class IV outcome. The proportion of MEGSS ranged in total from 0 to 92% (mean=57%) within RA; 0-100% (mean=67%) in the resection hemisphere; 0-100% (mean=63%) in the clusters; and 0-81% (mean=28%) in the scatters. Univariate ordinal logistic regression analyses showed that the proportion of scattered MEGSS within RA (p=0.049) significantly correlated with seizure outcomes. Multivariate analyses using three covariates (number of electrodes, proportions of clustered and scattered MEGSS within RA) showed that only the proportion of scattered MEGSS within RA significantly correlated with seizure outcomes (p=0.016). SIGNIFICANCE: MEG data showed a wide distribution of multilobar MEGSS in patients with TSC. The seizure outcome was not related to the clustered MEGSS within RA, since the grids were essentially planned to cover and resect the clustered MEGSS surrounding tubers. The maximal possible resection of scattered MEGSS correlated with improved seizure outcome in TSC. Some parts of the epileptogenic zone disrupted by multiple tubers did not have a sufficiently large area to produce clustered MEGSS. Although the wide distribution of scattered MEGSS is not interpreted as epileptogenic, they might be interrelated with clustered MEGSS to project a complex epilepsy network and be part of the extensive epileptogenic zones found in TSC.
Asunto(s)
Encéfalo/fisiopatología , Epilepsia Refractaria/fisiopatología , Magnetoencefalografía , Esclerosis Tuberosa/fisiopatología , Adolescente , Encéfalo/cirugía , Niño , Preescolar , Epilepsia Refractaria/etiología , Epilepsia Refractaria/cirugía , Electrocorticografía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Análisis Multivariante , Cuidados Preoperatorios , Estudios Retrospectivos , Convulsiones/etiología , Convulsiones/fisiopatología , Convulsiones/cirugía , Resultado del Tratamiento , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/cirugíaRESUMEN
Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.
Asunto(s)
Síndrome Antifosfolípido/patología , Colangitis/patología , Glucocorticoides/uso terapéutico , Insuficiencia Multiorgánica/patología , Púrpura Trombocitopénica Trombótica/patología , Rituximab/uso terapéutico , Anciano , Síndrome Antifosfolípido/diagnóstico , Autopsia , Enfermedad Catastrófica , Colangitis/etiología , Resultado Fatal , Femenino , Humanos , Insuficiencia Multiorgánica/tratamiento farmacológico , Intercambio Plasmático , Púrpura Trombocitopénica Trombótica/tratamiento farmacológico , RecurrenciaRESUMEN
A 53-year-old man initially presented with costalgia and was diagnosed with MM, based on the pathological findings. IgE monoclonal protein was detected by Serum protein electrophoresis (SPEP) and, surprisingly, IgE was elevated to 7,950,000 IU/ml. Monitoring the disease response during treatment, we employed quantification of serum M protein at SPEP, because IgE levels were found to be inaccurate and erratic. The patient was treated with CyBorD. He found injection site reactions to be very burdensome, due to extreme skin changes. The diameter of the hyperpigmentation area was 8 cm. To reduce the severity of this reaction, we used an air sandwich technique, and succeeded in ameliorating the skin changes.