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Int J Neurosci ; : 1-5, 2021 Feb 17.
Artículo en Inglés | MEDLINE | ID: mdl-33554696


INTRODUCTION: Prior to illustration of the causative genetic mutation responsible for Sotos syndrome, diagnosis was based on clinical criteria. They include characteristic facial gestalt, developmental delay, and evidence of overgrowth, in addition to other minor features as cardiac &genitourinary congenital malformation, seizures, scoliosis, among other features. Non-convulsive status epilepticus (NCSE) was not previously reported among Sotos patients. CASE SUMMARY: An eleven-years old boy, with developmental delay, characteristic facial & skeletal features presented to the emergency department with a two-hour episode of lapse of consciousness. Electroencephalogram (EEG) showed fluctuating generalized spike-wave/poly-spike wave discharge <2.5 Hertz (Hz), lasting throughout the duration of recording. Intravenous (IV) levetiracetam was associated with clinical & EEG improvement & accordingly the patient was diagnosed as NCSE. The mother reported history of polyhydramnios, febrile seizure & developmental delay. Through clinical & radiological assessment revealed generalized hypotonia, low intelligence quotient (IQ), congenital ureteric stricture & pulmonary hypertension, prominent retro-cerebellar cistern, in addition to scoliosis & facial features suggestive of Sotos Syndrome. Six months after presentation, the patient remained seizure free on levetiracetam monotherapy. CONCLUSION: NCSE could occur in Sotos syndrome. In our case, the first reported case of NCSE in Sotos syndrome, the characteristic facial & skeletal findings initiated further work up with fulfillment of the criteria required for the clinical diagnosis of Sotos syndrome.

Ther Clin Risk Manag ; 16: 759-767, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32884277


The ongoing coronavirus (COVID-19) pandemic is a global health emergency of international concern and has affected management plans of many autoimmune disorders. Immunosuppressive and immunomodulatory therapies are pivotal in the management of neuromyelitis optica spectrum disorder (NMOSD), potentially placing patients at an increased risk of contracting infections such as COVID-19. The optimal management strategy of NMOSD during the COVID-19 era remains unclear. Here, however, we examined the evidence of NMOSD disease-modifying therapies (DMTs) use during the present period and highlighted different scenarios including treatment of relapses as well as initiation and maintenance of DMTs in order to optimize care of NMOSD patients in the COVID-19 era.

Seizure ; 79: 1-7, 2020 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-32371363


PURPOSE: To evaluate the concordance between the EEG diagnosis of non-convulsive status epilepticus (NCSE) made by the treating physician and the application of the Salzburg Consensus Criteria (SCC) on consecutive urgent EEG requested for acute alteration of consciousness not otherwise explained. METHODS: Retrospective evaluation of all urgent EEGs acquired between January 1st and March 31st 2018 in adolescents and adult patients (≥14 years old). The 'reference' was the neurologist who first evaluated the patient and reported the corresponding EEG diagnosis. All were blind to the SCC. All included EEG were evaluated and classified by three neurologists trained in the application of SCC blind to the clinical diagnosis. RESULTS: 181 consecutive EEGs entered the study. The treating clinician diagnosed a NCSE in 17 cases (9%). According to the SCC, 12 EEGs were classified as Definite NCSE (7%), 71 (39 %) as Possible NCSE, and 98 (54 %) as no NCSE. A perfect concordance was found for the Definite- and No-NCSE categories, while only 5 patients with a Possible-NCSE were diagnosed by the treating clinician as having NCSE. It is relevant to note that aamong the 66 patients with a Possible NCSE not diagnosed as SE by the treating clinician, only two presented an evolution toward a definite SE. CONCLUSION: SCC have optimal performances for the 'Definite NCSE', and to exclude the presence of a NCSE. Conversely, the Possible NCSE category is still a a grey land of debate that could potentially include many different conditions.

Epilepsy Behav ; 102: 106686, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31760201


PURPOSE: There is a lack of data concerning the performance of the outcome prediction scores in patients with status epilepticus (SE) in developing countries. The aim of this study was to compare the predictive performances of the status epilepticus severity score (STESS) and the epidemiology-based mortality score in status epilepticus (EMSE) and adaptation of such scoring system to be compatible with the nature of society. METHOD: This is a prospective study, conducted in Egypt from the period of January 2017 to June 2018. The main outcome measure was survival versus death, on hospital discharge. The cutoff point with the best sensitivity and specificity to predict mortality was determined through a receiver operating characteristic (ROC) curve. RESULTS: Among the 144 patients with SE with a mean age of 39.3 ±â€¯19.5 years recruited into the study, 38 patients (26.3%) died in the hospital with the survival of 99 patients while 7 patients (4.9%) were referred to other centers with an unknown outcome. Although EMSE had a bit larger area under the curve (AUC) (0.846) than STESS-3 (AUC 0.824), STESS-3 had the best performance as in-hospital death prediction score as it has a higher negative predictive value (94.6%) than that of EMSE (90.9%) in order not to miss high-risk patients. CONCLUSION: In the Egyptian population, STESS and EMSE are useful tools in predicting mortality outcome of SE. The STESS performed significantly better than EMSEE combinations as a mortality prediction score.

Alta del Paciente/normas , Índice de Severidad de la Enfermedad , Estado Epiléptico/diagnóstico , Estado Epiléptico/mortalidad , Adulto , Anciano , Estudios de Cohortes , Egipto/epidemiología , Femenino , Mortalidad Hospitalaria/tendencias , Humanos , Masculino , Persona de Mediana Edad , Alta del Paciente/tendencias , Pronóstico , Estudios Prospectivos , Reproducibilidad de los Resultados , Estado Epiléptico/terapia , Resultado del Tratamiento , Adulto Joven