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1.
Epilepsy Res ; 167: 106453, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32927329

RESUMEN

In the wake of the pandemic COVID-19 and nationwide lockdowns gripping many countries globally, the national healthcare systems are either overwhelmed or preparing to combat this pandemic. Despite all the containment measures in place, experts opine that this novel coronavirus is here to stay as a pandemic or an endemic. Hence, it is apt to be prepared for the confrontation and its aftermath. From protecting the vulnerable individuals to providing quality care for all health conditions and maintaining essential drug supplies, it is going to be a grueling voyage. Preparedness to sustain optimal care for each health condition is a must. With a higher risk for severe COVID-19 disease in infants, need of high-dose hormonal therapy with a concern of consequent severe disease, presence of comorbidities, and a need for frequent investigations and follow-up; children with West syndrome constitute a distinctive group with special concerns. In this viewpoint, we discuss the important issues and concerns related to the management of West syndrome during COVID-19 pandemic in the South Asian context and provide potential solutions to these concerns based on the current evidence, adeptness, and consensus. Some plausible solutions include the continuation of containment and mitigation measures for COVID-19, therapeutic decision- making for West syndrome based on risk stratification, and tele-epileptology.


Asunto(s)
Corticoesteroides/uso terapéutico , Hormona Adrenocorticotrópica/uso terapéutico , Anticonvulsivantes/uso terapéutico , Infecciones por Coronavirus/tratamiento farmacológico , Neumonía Viral/tratamiento farmacológico , Espasmos Infantiles/tratamiento farmacológico , Telemedicina , Vigabatrin/uso terapéutico , Corticoesteroides/provisión & distribución , Hormona Adrenocorticotrópica/provisión & distribución , Anticonvulsivantes/provisión & distribución , Antivirales/administración & dosificación , Betacoronavirus , Toma de Decisiones Clínicas , Prestación de Atención de Salud , Interacciones Farmacológicas , Humanos , Lactante , Pandemias , Tiempo de Tratamiento , Vigabatrin/provisión & distribución
6.
Epilepsia ; 61(3): 509-518, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-32065676

RESUMEN

OBJECTIVE: To estimate the incidence of epilepsy in children with Zika-related microcephaly in the first 24 months of life; to characterize the associated clinical and electrographic findings; and to summarize the treatment responses. METHODS: We followed a cohort of children, born during the 2015-2016 Zika virus (ZIKV) epidemic in Brazil, with congenital microcephaly and evidence of congenital ZIKV infection on neuroimaging and/or laboratory testing. Neurological assessments were performed at ≤3, 6, 12, 15, 18, 21, and 24 months of life. Serial electroencephalograms were performed over the first 24 months. RESULTS: We evaluated 91 children, of whom 48 were female. In this study sample, the cumulative incidence of epilepsy was 71.4% in the first 24 months, and the main type of seizure was infantile spasms (83.1%). The highest incidence of seizures occurred between 3 and 9 months of age, and the risk remained high until 15 months of age. The incidence of infantile spasms peaked between 4 and 7 months and was followed by an increased incidence of focal epilepsy cases after 12 months of age. Neuroimaging results were available for all children, and 100% were abnormal. Cortical abnormalities were identified in 78.4% of the 74 children evaluated by computed tomography and 100% of the 53 children evaluated by magnetic resonance imaging. Overall, only 46.1% of the 65 children with epilepsy responded to treatment. The most commonly used medication was sodium valproate with or without benzodiazepines, levetiracetam, phenobarbital, and vigabatrin. SIGNIFICANCE: Zika-related microcephaly was associated with high risk of early epilepsy. Seizures typically began after the third month of life, usually as infantile spasms, with atypical electroencephalographic abnormalities. The seizure control rate was low. The onset of seizures in the second year was less frequent and, when it occurred, presented as focal epilepsy.


Asunto(s)
Epilepsias Parciales/fisiopatología , Malformaciones del Desarrollo Cortical/fisiopatología , Microcefalia/fisiopatología , Espasmos Infantiles/fisiopatología , Infección por el Virus Zika/fisiopatología , Anticonvulsivantes/uso terapéutico , Brasil , Corteza Cerebral/diagnóstico por imagen , Preescolar , Electroencefalografía , Epilepsias Parciales/tratamiento farmacológico , Epilepsias Parciales/epidemiología , Epilepsia/tratamiento farmacológico , Epilepsia/epidemiología , Epilepsia/fisiopatología , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Malformaciones del Desarrollo Cortical/diagnóstico por imagen , Microcefalia/diagnóstico por imagen , Espasmos Infantiles/tratamiento farmacológico , Espasmos Infantiles/epidemiología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Infección por el Virus Zika/congénito , Infección por el Virus Zika/diagnóstico por imagen
7.
Epileptic Disord ; 22(1): 73-82, 2020 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-32020895

RESUMEN

This study aimed to investigate the general presentation of epileptic spasms without hypsarrhythmia (ESwoH) and retrospectively determine whether there are differences in treatment effects related to ACTH therapy, long-term seizure outcome, and evolution of EEG features according to pre-treatment EEG patterns. According to the pattern of background activity, we divided our cohort into two groups: Group 1: normal background activity or with localized intermittent slow waves; Group 2: intermittent slow waves appearing generalized or in two or more lobes. Subjects included 22 children (Group 1: n=10; Group 2: n=12) diagnosed with ESwoH who received treatment from 2007 to 2017. The median age at onset of epileptic spasms was 5.5 months and the follow-up period lasted for 40.5 months. ACTH therapy was performed for seven patients from Group 1 and eight patients from Group 2. Only one patient from Group 2 responded to ACTH. Patients receiving effective treatments at early stages had excellent seizure outcome. Refractory cases included six patients in Group 1 and eight patients in Group 2; subsequent follow-up EEGs indicated hypsarrhythmia in one patient in Group 1 (17%) and six patients (75%) in Group 2, including one patient whose EEG pattern indicated progression to Lennox-Gastaut syndrome. Overall, ACTH is ineffective for patients with epileptic spasms without hypsarrhythmia. The EEG may indicate possible future development of hypsarrhythmia if epileptic spasms are resistant to treatment, especially in patients with diffuse slow waves on pre-treatment EEG. The efficacy of treatment introduced at early stages from onset may predict long-term seizure outcome.


Asunto(s)
Hormona Adrenocorticotrópica/farmacología , Ondas Encefálicas/fisiología , Espasmos Infantiles/tratamiento farmacológico , Espasmos Infantiles/fisiopatología , Niño , Preescolar , Progresión de la Enfermedad , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
8.
Epileptic Disord ; 22(1): 33-38, 2020 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-32031535

RESUMEN

Hormonal therapy is the first-line treatment for infantile spasms and is sometimes used in combination with topiramate for better seizure control and potentially improved developmental outcomes. Retrospective review of pediatric patients with infantile spasms, with data compiled on patient sex, age at onset, etiology, electroencephalographic and imaging findings, topiramate use, spasm resolution (at one, six, and 12 months), and developmental outcome (at 12 months). Of 105 patients screened, 55 (28 female) met inclusion criteria (28 [51%] had spasms with known etiology and 27 [49%] had spasms with unknown etiology). Forty-six patients were followed for 12 months or longer to determine seizure outcome; a 12-month developmental assessment was documented for 49 patients. Thirty-seven patients (67%) received combination therapy; 18 (33%) received hormonal therapy alone. Resolution of spasms was comparable among treatment groups, with no difference relative to spasm etiology (p>0.18 for all). No difference was found in developmental outcomes with and without adjunct topiramate (p=0.38). Combination therapy was the most common treatment at our institution. However, combination therapy was not found to be beneficial for the treatment of spasms or developmental outcomes when compared to hormonal therapy alone.


Asunto(s)
Hormona Adrenocorticotrópica/farmacología , Anticonvulsivantes/farmacología , Evaluación de Resultado en la Atención de Salud , Espasmos Infantiles/tratamiento farmacológico , Topiramato/farmacología , Hormona Adrenocorticotrópica/administración & dosificación , Anticonvulsivantes/administración & dosificación , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios Retrospectivos , Topiramato/administración & dosificación
9.
Dev Med Child Neurol ; 62(5): 575-580, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-31903560

RESUMEN

AIM: To compare the efficacy and safety of prednisolone/prednisone and adrenocorticotropic hormone (ACTH) in the treatment of infantile spasms using a meta-analysis of randomized controlled trials (RCTs). METHOD: In a systematic literature search of electronic databases (MEDLINE, Embase, the Cochrane Library), we identified RCTs that assessed prednisolone/prednisone compared with ACTH/tetracosactide in patients with infantile spasms. The electroclinical response and adverse events were evaluated. RESULTS: Six RCTs (616 participants) were included in the meta-analysis. Compared with prednisolone/prednisone, ACTH/tetracosactide was not superior in terms of cessation of spasms at day 14 (relative risk 1.19, 95% confidence interval [CI] 0.74-1.92), day 42 (relative risk 1.02, 95% CI 0.63-1.65), and resolution of hypsarrhythmia on electroencephalogram (relative risk 1.14, 95% CI 0.71-1.81); the incidences of common adverse reactions caused by ACTH/tetracosactide were not lower than that of prednisolone/prednisone for irritability (relative risk 0.79, 95% CI 0.57-1.10), increased appetite (relative risk 0.78, 95% CI 0.57-1.08), weight gain (relative risk 0.86, 95% CI 0.56-1.32), and gastrointestinal upset (relative risk 0.60, 95% CI 0.35-1.02), though it seemed less frequent. INTERPRETATION: Prednisolone/prednisone elicits a similar electroclinical response as ACTH for infantile spasms, which indicates that it can be an alternative to ACTH for treating infantile spasms. What this paper adds Prednisolone/prednisone is as effective as adrenocorticotropic hormone (ACTH) in electroclinical response of infantile spasms. Prednisolone/prednisone and ACTH cause similar and tolerable adverse effects, whose incidences are comparable. High-dose prednisone/prednisolone might be preferable to low dose for achieving freedom from spasms.


Asunto(s)
Hormona Adrenocorticotrópica/uso terapéutico , Parasimpatolíticos/uso terapéutico , Prednisolona/uso terapéutico , Prednisona/uso terapéutico , Espasmos Infantiles/tratamiento farmacológico , Humanos , Lactante , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del Tratamiento
11.
Eur J Paediatr Neurol ; 25: 77-81, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31926846

RESUMEN

Epilepsy of Infancy with Migrating Focal Seizures (EIMFS) is a rare, developmental and epileptic encephalopathy most commonly associated with mutations in KCNT1, a potassium channel. Polymorphous migrating focal seizures begin within 6 months of life and are pharmacoresistant to standard anticonvulsants. Additional therapies are needed to decrease seizure frequency and subsequent developmental deterioration associated with EIMFS. Cannabidiol (CBD) has recently arisen in public interest due to its potential in treatment-resistant epilepsies as demonstrated in randomized controlled trials for Dravet Syndrome and Lennox-Gastaut Syndrome. Here we evaluate the response of three patients, all diagnosed with EIMFS secondary to KCNT1 mutations, to pharmaceutical grade CBD. Two patients showed no benefit and have since voluntarily stopped CBD. One patient showed no overall reduction in seizure frequency, however showed a notable reduction in seizure intensity with possible developmental progression. Further studies are needed to assess the potential benefit of CBD in treatment-resistant epilepsies such as EIMFS, with a focus on early identification and intervention.


Asunto(s)
Anticonvulsivantes/uso terapéutico , Cannabidiol/uso terapéutico , Síndromes Epilépticos/tratamiento farmacológico , Espasmos Infantiles/tratamiento farmacológico , Adolescente , Preescolar , Síndromes Epilépticos/genética , Femenino , Humanos , Lactante , Masculino , Mutación , Proteínas del Tejido Nervioso/genética , Canales de potasio activados por Sodio/genética , Estudios Prospectivos , Convulsiones/tratamiento farmacológico , Convulsiones/genética , Espasmos Infantiles/genética
12.
Eur J Paediatr Neurol ; 24: 142-147, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31928905

RESUMEN

BACKGROUND: PCDH19 Girls clustering epilepsy (GCE) has a phenotypic spectrum that includes developmental and epileptic encephalopathy. PCDH19-GCE presents with clusters of seizures in the first years of life. Although patients typically outgrow their seizures, many are left with intellectual disability. Here we retrospectively assess the effect of levetiracetam in two independent cohorts of females with PCDH19-GCE. METHODS: Cohort A was identified by searching our epilepsy genetics research database for girls with PCDH19-GCE who had trialled levetiracetam. Cohort B consisted of girls aged 2 years or older, including women, participating in an international online questionnaire. Information regarding seizure frequency and levetiracetam use was obtained by in-person patient interview and review of clinical records for cohort A, and by patient report for cohort B. RESULTS: Cohort A consisted of 17 females, aged 3-37 years, who had a trial of levetiracetam at an average age of 10.7 years. 13/17 females became seizure free for >12 months; while 10/17 remained seizure free for >24 months. Cohort B comprised 62 females, aged 1.5-41 years. 26/62 became seizure free for >12 months, and 19/62 for >24 months on levetiracetam therapy. DISCUSSION: Levetiracetam was effective in two cohorts of females with PCDH19-GCE where 42% and 76% of females became seizure free for >12 months, respectively. Levetiracetam is an effective therapy for females with PCDH19-GCE and should be considered early in the management of the highly refractory clusters of seizures that characterise this genetic disease.


Asunto(s)
Anticonvulsivantes/uso terapéutico , Levetiracetam/uso terapéutico , Espasmos Infantiles/tratamiento farmacológico , Adolescente , Adulto , Cadherinas/genética , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Estudios Retrospectivos , Espasmos Infantiles/genética , Adulto Joven
13.
Dev Med Child Neurol ; 62(5): 581-586, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-31850517

RESUMEN

AIM: To analyse the effects of felbamate in refractory infantile spasms/West syndrome. METHOD: We conducted a 10-year retrospective study of infants (including all infants younger than 18mo) treated with felbamate for electroencephalography-recorded epileptic spasms persisting after first-line treatment. RESULTS: In total, 29 infants (17 males, 12 females) were included in the study. Felbamate was initiated at a mean age of 13.8 months (range 4.5-66mo) after sequential administration or combination of vigabatrin and oral steroids; a ketogenic diet was implemented in 23 infants. Eight infants became spasm-free at a mean dose of 34.6mg/kg/day felbamate (range 26-45mg/kg/day). Mean duration of felbamate use was 19 months (range 1-67mo) for the 19 infants whose treatment was terminated. No severe side effects were observed. Reversible neutropenia led to withdrawal of felbamate in six patients. One spasm-free patient demonstrated recurrence when felbamate was withdrawn. INTERPRETATION: N-methyl-d-aspartate receptors with felbamate controlled epileptic spasms in eight infants resistant to first-line treatment should be targeted.


Asunto(s)
Anticonvulsivantes/uso terapéutico , Felbamato/uso terapéutico , Espasmos Infantiles/tratamiento farmacológico , Resistencia a Medicamentos , Electroencefalografía , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Espasmos Infantiles/fisiopatología , Resultado del Tratamiento
14.
Epilepsy Behav ; 102: 106826, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31816477

RESUMEN

PURPOSE: Limited data suggest that cannabidiol (CBD) may be effective for treatment of refractory infantile spasms (IS). This study was designed to more rigorously evaluate the efficacy and safety of synthetic CBD in the treatment of IS. METHODS: Children six to 36 months of age with IS that failed treatment with both adrenocorticotropic hormone (ACTH) and vigabatrin (VGB) were eligible for enrollment. Children receiving clobazam were excluded. After baseline overnight video-electroencephalography (vEEG) to confirm diagnosis and ascertain hypsarrhythmia, patients were treated with synthetic CBD oral solution (20 mg/kg/day). Overnight video-EEG was repeated after 14 days, and both baseline and repeat video-EEGs were completely de-identified and reviewed in a pairwise fashion by an independent, blinded pediatric electroencephalographer. The primary efficacy endpoint was freedom from spasms and hypsarrhythmia on day 14. RESULTS: Nine patients were enrolled, comprising an older (median age = 23 months) cohort with long-standing IS (median duration = 13 months) and numerous prior treatment failures (median = 6). One patient responded to therapy and eight patients exhibited neither clinical nor electrographic response. CONCLUSIONS: The immediate but temporary response in a single patient suggests that CBD oral solution is not particularly effective in highly refractory cases, but may, nevertheless, be effective in younger patients with shorter durations of IS. Further study, examining both short- and long-term outcomes, is warranted to further evaluate the efficacy and safety of CBD oral solution in the treatment of IS.


Asunto(s)
Anticonvulsivantes/farmacología , Cannabidiol/farmacología , Epilepsia Refractaria/tratamiento farmacológico , Evaluación de Resultado en la Atención de Salud , Espasmos Infantiles/tratamiento farmacológico , Anticonvulsivantes/administración & dosificación , Cannabidiol/administración & dosificación , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino
15.
Neurology ; 94(6): e575-e582, 2020 02 11.
Artículo en Inglés | MEDLINE | ID: mdl-31792095

RESUMEN

OBJECTIVE: To determine the prevalence of retinal defect in children with infantile spasms (IS) unrelated to treatment with vigabatrin and clarify if specific primary etiologies for IS are associated with retinal defect more than others. METHODS: This was an observational cohort study including 312 patients (176 male, 136 female) with IS who were vigabatrin-naive. Participants ranged from 1.7 to 34.7 months of age (mean 8.8 months). Electroretinograms (ERGs) were performed according to the International Society for Clinical Electrophysiology of Vision. Retinal defect was identified as abnormal if the 30-Hz flicker ERG amplitude was lower than the age-corrected normal 95% prediction interval. The primary etiology for IS, as determined by the treating pediatric neurologist(s), was obtained from patient health records and classified into 1 of 9 etiologic subgroups: (1) genetic disorders alone, (2) genetic-structural disorders, (3) structural-congenital, (4) structural-acquired (perinatal), (5) structural-acquired (postnatal), (6) metabolic disorders, (7) immunologic disorders, (8) infectious, and (9) unknown causes. RESULTS: Fifty-nine of the 312 vigabatrin-naive children (18.9%) showed retinal defect and the prevalence of retinal defect was highest (24.4%) in the structural-acquired (perinatal) subgroup, which included hypoxic-ischemic defect. Retinal function compared across subgroups showed no significant difference. CONCLUSIONS: Care is required in diagnosing retinal toxicity, which would be enhanced by baseline flicker ERG in children with IS prior to starting vigabatrin.


Asunto(s)
Enfermedades de la Retina/epidemiología , Espasmos Infantiles/epidemiología , Anticonvulsivantes/efectos adversos , Anticonvulsivantes/uso terapéutico , Preescolar , Electrorretinografía , Femenino , Enfermedades Genéticas Congénitas/complicaciones , Humanos , Hipoxia-Isquemia Encefálica/complicaciones , Lactante , Infecciones/complicaciones , Masculino , Enfermedades Metabólicas/complicaciones , Prevalencia , Enfermedades de la Retina/fisiopatología , Espasmos Infantiles/tratamiento farmacológico , Espasmos Infantiles/etiología , Espasmos Infantiles/fisiopatología , Vigabatrin/uso terapéutico
16.
Indian J Pediatr ; 87(1): 6-11, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31721072

RESUMEN

OBJECTIVE: To evaluate the safety, tolerability, and effectiveness of oral topiramate therapy in children with West syndrome. METHODS: The present study was designed as a prospective, observational study and was performed from July 2016 through June 2018 at a tertiary care pediatrics centre in North India. The study was approved by Institute Ethics Committee. RESULTS: Data on 39 children with West syndrome were analyzed. Topiramate was used as an adjunct in 38 children who failed to hormonal therapy and/or vigabatrin and as initial monotherapy in one case. The study participants had a long treatment lag to hormonal therapy (median 2 mo, IQR 1-8), a preponderance of male sex (67%) and structural etiology (87%). Nine (23%) children had a cessation of epileptic spasms at a median dose of 3.8 mg/kg/d. However, seven children with initial response had relapses. There were no significant group differences between responders and non-responders. Overall, topiramate was well tolerated. Somnolence and lethargy with decreased oral intake were commonly observed adverse effects. CONCLUSIONS: The study observed poor effectiveness of topiramate therapy, which is partially due to a long treatment lag and a high proportion of structural etiology.


Asunto(s)
Espasmos Infantiles/tratamiento farmacológico , Topiramato/administración & dosificación , Topiramato/efectos adversos , Topiramato/uso terapéutico , Anticonvulsivantes/uso terapéutico , Tolerancia a Medicamentos , Femenino , Humanos , India , Lactante , Masculino , Estudios Prospectivos , Resultado del Tratamiento , Vigabatrin/uso terapéutico
17.
Neurosciences (Riyadh) ; 24(4): 311-314, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31872811

RESUMEN

Several studies have reported a variable benefit of valproic acid for the treatment of infantile spasm. However, valproic acid can also worsen spasms, as occurred with this child who presented with post-traumatic seizure which evolved to spasms. The child was started on antiepileptic medications, including valproic acid, despite that spasms persisting. For this reason, she was admitted for adrenocorticotropic hormone therapy. The baseline electroencephalogram showed modified hypsarrhythmia, and the laboratory workup showed thrombocytopenia, which was attributed to the valproic acid. After the valproic acid cessation, the spasms and the hypsarrhythmic pattern resolved dramatically next day, and the intended adrenocorticotropic hormone therapy was not started. Eight months later, she was still free of spasms. In conclusion, though valproic acid might have a beneficial effect in some patients with infantile spasm, it might have a negative impact on spasms in some patients which warrants its discontinuation sooner than later during spasms treatment.


Asunto(s)
Anticonvulsivantes/efectos adversos , Espasmos Infantiles/tratamiento farmacológico , Trombocitopenia/etiología , Ácido Valproico/efectos adversos , Anticonvulsivantes/administración & dosificación , Anticonvulsivantes/uso terapéutico , Esquema de Medicación , Femenino , Humanos , Lactante , Espasmos Infantiles/patología , Ácido Valproico/administración & dosificación , Ácido Valproico/uso terapéutico
18.
Medicine (Baltimore) ; 98(44): e17749, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31689829

RESUMEN

RATIONALE: Early infantile epileptic encephalopathy (EIEE) 65 was recently shown to be caused by the cytoplasmic FMRP interacting protein 2 (CYFIP2) mutation. To date, only 5 cases have been reported in two articles, and all the outcomes in all cases were poor. PATIENT CONCERNS: In this study, we reported an 8-month-old girl with a 1 month-long history of seizures and developmental delay. Over 1 month later, she developed epileptic spasms in clusters with hypsarrhythmia on electroencephalography. DIAGNOSIS: The patient was diagnosed with EIEE 65 and trio-based whole-exome sequencing revealed a causative de novo CYFIP2 mutation c.260G >T (p.Arg87Leu). INTERVENTIONS: The proband was successively treated with multiple antiepileptic drugs, including levetiracetam, phenobarbital, VitB6, topiramate, methylprednisolone, prednisone, valproic acid and vigabatrin. OUTCOMES: After resistance to multiple anti-epileptic drugs over 2 months of treatment, she finally achieved seizure-free several days after vigabatrin administration and her developmental delay steadily improved. LESSONS: OUR: case confirmed that CYFIP2 was the pathogenic gene of EIEE 65. We also first demonstrated vigabatrin might be effective for control of seizures and helpful for the improved outcomes of these patients.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/genética , Mutación/genética , Espasmos Infantiles/genética , Anticonvulsivantes/uso terapéutico , Diagnóstico Precoz , Femenino , Humanos , Lactante , Espasmos Infantiles/diagnóstico , Espasmos Infantiles/tratamiento farmacológico , Resultado del Tratamiento
19.
Ann Clin Transl Neurol ; 6(11): 2270-2281, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31657133

RESUMEN

OBJECTIVE: To compare the therapeutic effectiveness of oral corticosteroids with that of adrenocorticotrophic hormone for infantile spasms. METHODS: PubMed, Embase, Scopus, and the Cochrane library were searched to retrieve studies published before December 2018 to identify pediatric patients with a diagnosis of infantile spasms. The interventions of oral corticosteroids and adrenocorticotrophic hormone were compared. We included only randomized controlled trials that reported the cessation of spasms as treatment response. The primary outcome was clinical spasm cessation on day 13 or 14. The secondary outcomes were the resolution of hypsarrhythmia, side effects, continued spasm control, spasm relapse rate, and subsequent epilepsy rate. Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, the study-level quality assessment was conducted using the Cochrane risk-of-bias tool. RESULTS: After extensive review, 39 articles were included for meticulous evaluation. Five randomized controlled trials with a total of 239 individuals were eligible for further analysis. No significant difference was detected between the corticosteroids and adrenocorticotrophic hormone in the cessation of clinical spasms (odds ratio [OR]: 0.54; 95% confidence interval [CI]: 0.16 to 1.81; P = 0.32). The subgroups of high-dose prednisolone versus adrenocorticotrophic hormone and low-dose prednisone versus adrenocorticotrophic hormone also exhibited no significant difference. Furthermore, the two subgroups did not differ in terms of hypsarrhythmia resolution, side effects, relapse rate, or subsequent epilepsy rate. INTERPRETATION: This meta-analysis suggests that high-dose prednisolone is not inferior to adrenocorticotrophic hormone and that it be considered a safe and effective alternative treatment.


Asunto(s)
Corticoesteroides/uso terapéutico , Hormona Adrenocorticotrópica/uso terapéutico , Espasmos Infantiles/tratamiento farmacológico , Antiinflamatorios/uso terapéutico , Humanos , Lactante , Prednisolona/uso terapéutico , Prednisona/uso terapéutico
20.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(9): 845-850, 2019 Sep.
Artículo en Chino | MEDLINE | ID: mdl-31506140

RESUMEN

OBJECTIVE: To investigate the factors in first-time adrenocorticotropic hormone (ACTH) therapy and their influence on spasm control time in infants with infantile spasms. METHODS: A total of 72 infants with infantile spasms who were admitted from January 2008 to October 2013 were enrolled. Their clinical data were collected, and the exposure factors for infantile spasms were selected. A Cox proportional-hazards regression model analysis was performed for these factors to analyze their influence on spasm control time. RESULTS: Clarification of the etiology (known or unexplained etiology), frequency of spasms before treatment, and presence or absence of combination therapy (ACTH used alone or in combination with magnesium sulfate) had a significant influence on spasm control time in infants with infantile spasms. The infants with a known etiology had a significantly shorter spasm control time than those with unexplained etiology, and the infants with a low frequency of spasms before treatment and receiving ACTH combined with magnesium sulfate early had a significantly longer spasm control time than their counterparts (P<0.05). CONCLUSIONS: For infants with infantile spasms at initial diagnosis, etiology should be clarified, which may helpful for evaluating prognosis. A combination of ACTH and magnesium sulfate should be given as soon as possible, which may improve their prognosis.


Asunto(s)
Hormona Adrenocorticotrópica/uso terapéutico , Espasmos Infantiles , Anticonvulsivantes , Humanos , Lactante , Modelos de Riesgos Proporcionales , Espasmo , Espasmos Infantiles/tratamiento farmacológico
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