scholarly journals West syndrome: ethiology, management, long-term outcomes

2009 ◽  
Vol 8 (4(2)) ◽  
pp. 137-140
Author(s):  
L. S. Krayeva ◽  
V. M. Alifirova ◽  
O. V. Grebenyuk

The article is based on long-term observation of 27 patients with West syndrome, age of onset, etiology of symptomatic forms, neurological status, efficiency of hormone therapy and antiepileptic drugs.

2021 ◽  
Author(s):  
Shimpei Baba ◽  
Tohru Okanishi ◽  
Yoichiro Homma ◽  
Takeshi Yoshida ◽  
Tomohide Goto ◽  
...  

2021 ◽  
Vol 19 (2) ◽  
pp. 221-228
Author(s):  
Roza M. Shaimardanova ◽  
Rimma G. Gamirova

AIM: To conduct a retrospective comparative analysis of the efficacy and safety of epilepsy therapy with antiepileptic drugs. MATERIALS AND METHODS: The analysis of the treatment of 428 patients with epilepsy at the Childrens City Hospital No. 8 in Kazan, receiving antiepileptic drugs. RESULTS: It was found that valproic acid is more effective in the treatment of idiopathic generalized epilepsies compared to focal epilepsies (p = 0.0006). Valproate and carbamazepine were the most effective in the treatment of focal epilepsy with short- and long-term follow-up. Valproic acid is more effective than topiramate (p = 0.02), oxcarbazepine (p = 0.003), and levetiracetam (p = 0.003) in the treatment of focal epilepsy in short- and long-term follow-up. Carbamazepine is more effective than topiramate (p = 0.01), oxcarbazepine (p = 0.02), and levetiracetam (p = 0.001) in the treatment of focal epilepsy in long-term follow-up. It was revealed that more often they complained about side effects when using carbamazepine (63.2%). Levetiracetam was found to be better tolerated compared to valproate (p = 0.0006) and carbamazepine (p = 0.0006). Topiramate is better tolerated than carbamazepine (p = 0.02) and valproate (p = 0.03). Oxcarbazepine is better tolerated than carbamazepine in women (p = 0.04). CONCLUSIONS: When choosing an antiepileptic drug, it is necessary to be guided by the principle: first the basic, and then the drugs of the next generations, in the future, rely on information about the tolerability of the drug. It is necessary to evaluate the therapeutic effect of antiepileptic drugs with long-term observation, and use the criterion of complete absence of seizures as an indicator of the effectiveness of drugs.


2020 ◽  
Vol 4 (1) ◽  
pp. e000683
Author(s):  
Yang He ◽  
Jun Tang ◽  
Meng Zhang ◽  
Tao Xiong ◽  
Shalini Ojha ◽  
...  

IntroductionSeizures are one of the most common neurological disorders of neonates, which is also an emergency in the neonatal intensive care unit. For neonates, the recommended first-line antiepileptic drugs (AEDs) include phenobarbitone, which may be effective in only 50% of seizures. Some new AEDs, such as levetiracetam, have been shown to be effective in adults and older children. However, their efficacy for neonatal seizures remains uncertain. The aim of this investigation is to conduct a systematic review to evaluate the efficacy of all AEDs in neonates. Additionally, the long-term outcomes following neonatal seizures, in relation to the development of cerebral palsy and epilepsy, will be studied.MethodWe will perform a systematic review including randomised controlled studies (RCTs), cohort studies, case-controlled studies and case series studies which evaluated the efficacy of AEDs and short-term and long-term outcomes in neonatal seizures. PubMed, Embase, Web of Science, Cochrane Library and Clinical trial.gov will be searched. There will be no language restriction. Risk bias in RCTs will be evaluated by the Cochrane risk-of-bias tool, while cohort and case-control studies will be evaluated by the Newcastle-Ottawa Scale. A network meta-analysis will be performed by the Bayesian model using WinBUGS V.1.4.3 and R software if there is a high degree of homogeneity among studies. Otherwise, we will perform a narrative review without pooling. Subgroup analyses will be performed in different AEDs and dosage groups.OutcomeThe primary outcomes will be seizure cessation confirmed by electroencephalogram and long-term neurodevelopmental outcome. Secondary outcomes will be neonatal mortality during hospitalisation and suspected drug toxicity.Ethics and disseminationFormal ethical approval is not required as no primary data are collected. This systematic review will be disseminated through a peer-reviewed publication.


2019 ◽  
Vol 104 (10) ◽  
pp. 4264-4272 ◽  
Author(s):  
Friedhelm Raue ◽  
Thomas Bruckner ◽  
Karin Frank-Raue

Abstract Context Recent data on long-term outcomes and aggressiveness of medullary thyroid carcinoma (MTC) are lacking for patients with multiple endocrine neoplasia type 2 (MEN2). Objectives To analyze the long-term outcomes in MEN2 and compare MTC aggressiveness in three defined RET mutation-risk categories: moderate risk (MOD), high risk (H), and highest risk (HST). Design, Setting Retrospective study of 263 operated patients with MEN2 from one German tertiary referral center from 1979 to 2017 comparing demographic, biochemical, genetic, and outcome parameters Intervention None (observational study) Main Outcome Measure Long-term survival and outcomes in three risk groups Results Surgery was performed at a mean age of 35.3 ± 18.8 (MOD, n = 122), 23.0 ± 15.7 years (H, n = 120), and 14.9 ± 9.3 (HST, n = 21) years (P < 0.05). The mean follow-up was 12.9 ± 9.8 years. Age and tumor stage at diagnosis differed among the three risk groups (P < 0.0001). Multivariate analysis of disease-specific survival (DSS) showed that increasing age [hazard ratio (HR), 1.06; 95% CI, 1.02 to 1.09], stage III/IV at diagnosis (HR, 7.39; 95% CI, 2.39 to 22.8), and HST group (HR, 14.4; 95% CI, 3.32 to 62.6) were significantly associated with worse DSS; the H group was not (P = 0.175). The DSS rates and outcomes were not different between the MOD and H groups (P = 0.179 and P = 0.893, respectively) but were significantly inferior in the HST group (P < 0.0008 and P < 0.0001, respectively). Conclusion MTC in patients with MEN2 showed a clearly different age of onset in the different risk groups. DSS and outcomes after MTC diagnosis were similar in the MOD and H groups, suggesting similar tumor behavior. The HST group had inferior outcomes and survival vs the MOD and or H groups.


2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Yasushi Oshima ◽  
Kota Miyoshi ◽  
Yoji Mikami ◽  
Hideki Nakamoto ◽  
Sakae Tanaka

Incidences of cervical laminoplasty in the elderly are increasing; the influence of other age-related complications and neurological status must be considered for justifying surgery. This study identified the aforementioned influence on long-term outcomes of cervical laminoplasty in patients aged ≥75 years. Thirty-seven of 38 consecutive patients aged ≥75 years who underwent cervical laminoplasty were retrospectively evaluated. Minimum 5-year follow-up was acceptable if patients were complication-free. Follow-up was terminated when neurological evaluation was not possible, owing to death or other serious complications affecting activities of daily living (ADL). Postoperative neurological changes and newly developed severe complications were investigated. Postoperatively, one patient died of acute pneumonia, one remained nonambulatory owing to cerebral infarction, and 35 were ambulatory and were discharged. At a mean follow-up of 78 months, three patients died and nine developed serious complications severely affecting ADL. Of the 25 remaining patients, 23 remained ambulatory at mean follow-up of 105 months. Cox proportional hazard analysis revealed that postoperative motor upper and lower extremities JOA scores of ≤2 and ≤1, respectively, were risk factors for mortality or other severe complications. Postoperative neurological status can be maintained in the elderly if they remain complication-free. Poorer neurological status significantly affected their ADL and mortality.


2017 ◽  
Vol 32 (10) ◽  
pp. 861-866 ◽  
Author(s):  
Adam Wallace ◽  
Victoria Allen ◽  
Kristen Park ◽  
Kelly Knupp

The association of infantile spasms and periventricular leukomalacia and/or intraventricular hemorrhage is well documented. Data regarding early treatment-based and long-term outcomes are limited. A retrospective chart review identified children with infantile spasms born prematurely (<37 weeks) with diagnoses of periventricular leukomalacia and/or intraventricular hemorrhage. Thirteen children were included. Median gestational age was 30 weeks and age of onset of infantile spasms was 8 months. Nine children had intraventricular hemorrhage, 10 had periventricular leukomalacia, and 6 children had both. Twelve of 13 children had resolution of spasms. In responders, the successful medication was adrenocorticotropic hormone (ACTH) in 7, topiramate in 3, and vigabatrin in 2. Follow-up after a median of 7.1 years found that all patients had developmental delay but only 1 had refractory epilepsy. Standard therapies (ACTH and vigabatrin) appeared to be more effective than other treatments. Developmental delay is common in children with periventricular leukomalacia / intraventricular hemorrhage and infantile spasms, but refractory epilepsy might be less frequent.


2009 ◽  
Vol 5 (5) ◽  
pp. 485-496 ◽  
Author(s):  
Cheryl Bushnell

Aims: To determine differences in stroke severity and outcomes in women using hormone therapy (HT) versus nonusers at baseline, and to investigate whether there is a ‘healthy-user effect’ of HT in women with stroke. Materials and methods: A total of 133 women over the age of 18 years with acute ischemic stroke were enrolled and categorized based on their use of HT at the time of stroke. Initial stroke severity was assessed at admission, and disability and activities of daily living were assessed at 6-month intervals for 2 years. Results: A total of 30% of the cohort were HT users. HT users were less likely to have hypertension and were leaner than nonusers. There were no differences in initial stroke severity, mortality or any of the functional status outcomes based on HT use at baseline. Conclusion: There did appear to be a healthy-user effect for HT users at baseline, but following stroke, there were no significant differences in long-term outcomes.


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