Autologous hematopoietic stem cell transplantation (aHSCT) is a promising therapy for multiple sclerosis (MS), which has mainly been used in adults. The purpose of this study was to investigate efficacy and adverse events of aHSCT in the treatment of children with MS using data from the European Society for Blood and Marrow Transplantation registry. Twenty-one patients with a median follow-up time of 2.8 years could be identified. PFS at 3 years was 100%, 16 patients improved in expanded disability status scale score and only 2 patients experienced a clinical relapse. The procedure was generally well tolerated and only two instances of severe transplant-related toxicity were recorded. There was no treatment-related mortality, although one patient needed intensive care. aHSCT may be a therapeutic option for children with disease that does not respond to standard care.
IntroductionOpsoclonus myoclonus syndrome (OMS) in childhood still remains unexplored from the perspective of clinical psychology.Objectives and aimsThe research was aimed at defining types of psychological development in the group of children with OMS. The study included 18 children between ages 2 years 4 months and 9 years with OMS (8 boys, 10 girls).MethodsThe following methods were used: analysis of patient's development and medical record, psychological interview with parents, neurological state assessment and pathopsychological assessment.ResultsPatients were divided into three groups according to cognitive development level:– group with normal intelligence level (n = 4);– group with developmental delay (n = 11);– group with intellectual disability in different forms (n = 3).Several children in group 1 (n = 3) and in group 2 (n = 4) expressed neurotic behavior with increased levels of anxiety and fear of trying unusual actions during psychological assessment. Children with delay in psychoverbal development (n = 7) and with developmental delay (n = 1) expressed psychopathy-like states with verbal aggression and had difficulties controlling their emotions. In some cases (n = 2), the psychological state of the patient was defined as borderline state with emotional instability, especially in mother–child interaction.ConclusionPsychological features of children with OMS indicate heterogeneity of their development types that implies different prognosis and developmental dynamics for each type. The results point out the necessity of detailed psychological examination aimed at correlation of psychological help to children with OMS and their families with their psychological state.Disclosure of interestThe authors have not supplied their declaration of competing interest.
Cortexin as add-on to AEDs reduced for more than two times the number of seizures, along with improvement of motor function, in 31 (36.9%) patients. The improvement of motor function, but without a significant decrease in epileptic seizures, was achieved in 15 (17.8%) of the patients. Reduction of epileptic seizures frequency (>2 times), but without a significant effect on motor function, was observed in 14 cases (16.7%). Twenty-three patients (27.4%) did not respond the therapy. The aggravation of epileptic seizures during cortexin therapy was observed in only 1 girl with West syndrome (1.2%), and this was significantly lower than the probability of seizures aggravation on AED. Polypeptide nootropic medication cortexin demonstrated efficacy and safety as adjunctive therapy in children with CP combined with epilepsy.
The aim of the study was to determine the clinical features of anti-NMDAR encephalitis in children. Materials and methods of research: 11 patients were selected from the group of children with autoimmune encephalitis, who met the diagnostic criteria for reliable anti-NMDAR encephalitis. A retro-prospective analysis of clinical symptoms, laboratory, neurophysiological and neuroimaging data, treatment, duration of primary hospitalization and long-term results of treatment was carried out, neuropsychological testing of patients was performed in the follow-up. Results: the age of the patients was 8.5±4.4 years, the gender composition of boys/girls was 1/10 (9.1%/90.9%). The average follow-up period was 17±12 months. In 73% of cases, there was an acute onset of the disease without a prodromal phase and with rapidly growing signs of neurological dysfunction: behavior change (11/100%), epileptic seizures (11/100%), speech impairment (10/90.9%), movement disorders (10/90.9%), disturbed sleep/wakefulness rhythm (9/81.8%), hallucinations (5/45.5%), autonomic disorders (6/54.5%), sensory disturbance (1/9,1%). In 82% of cases, therapy was required in the intensive care unit. When analyzing EEG monitoring in children, the extreme delta brush pattern was revealed in only one patient. Neuroimaging revealed no specific changes in the substance of the brain. The paraneoplastic nature of the disease could not be established in any case. 4 patients (36.36%) had relapses of the disease after 1.5–27 months. from the onset of the disease. All children showed a favorable outcome without a gross neurological deficit, but complaints of increased fatigue, headaches, poor memory, a decrease in vocabulary, and impulsive behavior persisted.
Pediatric-onset multiple sclerosis (MS) can lead to cognitive impairment (CI). In general, in early-onset MS, there are disturbances in cognitive processes, such as information processing speed, attention, and controlling functions. Also, unlike adults with MS, children show a failure in various spheres of speech activity. The age of onset in MS, its duration and recurrence rate can affect not only the accumulation of a stable neurological deficit, but also the state of the cognitive sphere.Objective: to study of the features of CI in children and adolescents with MS; to assess the relationship of CI to clinical characteristics, such as age at onset in the disease and its duration.Patients and methods. The study involved 45 pediatric and adolescent patients with an established diagnosis of MS, who underwent a general neuropsychological examination of the cognitive sphere (Luria’s tests) with transfer to a point system; in addition, psychometric techniques were used to assess attention, controlling functions, memory, verbal fluency, and various types of thinking. Clinical characteristics, such as age at onset in MS and its duration at the time of the examination, were also taken into account.Results and discussion. The leading factors that combine certain symptom complexes of CI in children and adolescents with MS were established. These factors include attention, controlling functions, auditory-verbal and visuospatial memories, various spheres of speech activity. Early-onset MS (at age of 5–8 years) was ascertained to have a greater impact on the formation of speech and controlling functions than adolescence- onset (at age of 13–16-years).Conclusion. The risk of cognitive deficit and subsequent disability was found to be highest in early-onset MS.
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