scholarly journals Clinical, neuroimaging and developmental outcomes of West syndrome

2021 ◽  
Vol 17 (3) ◽  
pp. 5-11
Author(s):  
Kemal Karataş ◽  
Hüseyin Tan ◽  
Şenay Güven Baysal

Background. West syndrome is characterized by a hipsarrhytmia pattern on electroencephalography, spasm type seizures, and psychomotor regression triad. In this study, we aimed to document demographic characteristics, clinical and laboratory findings, treatment responses, neurodevelopmental outcomes, and risk factors developed during long-term follow-up with the diagnosis of West syndrome. Materials and methods. The study included patients who were diagnosed with West syndrome between July 2011 and December 2012 in the Department of Pediatric Neurology of Atatürk University. The following data were collected from each patient’s history. Systemic and neurological examinations, cerebral imaging, and electroencephalography were reviewed. Biochemical tests were performed from laboratory tests. The development of each child was assessed using the Denver 2 developmental screening test, the Ankara Developmental Screening Inventory test. Results. The ratio of male/female of our patients was found 2,28/1 and the mean age of the referral was 8.62 ± 7.20 months (median: 8.0). 59 (85.51 %) of the patients were in the symptomatic group and 10 (14.49 %) were in the idiopathic group. The most common factors in the symptomatic group were anomalies of the congenital central nervous system (45.7 %) and hypoxic-ischemic encephalopathy (28.8 %). There was a significant difference between the idiopathic and symptomatic groups in terms of relapse rates, radiological findings, and prognosis rates (p: 0.035/p < 0.001/p < 0.001). Relapse was detected in 43,5 % of the patients. While 83 % of patients treated with adrenocorticotropic hormone, treatment was responded to, 17 % had resistant seizures. There was no statistically significant difference between the treatment responses with adrenocorticotropic hormone and other treatments (p = 0.093). 46 of 55 children (83.6 %) were found to be a developmental delay. Conclusions. Early diagnosis and treatment, the provision of appropriate and convenient treatment in the West syndrome can make a positive contribution to prognosis according to etiology.

2012 ◽  
Vol 18 (2) ◽  
pp. 63-66 ◽  
Author(s):  
MA Dias de Sousa ◽  
RCF Bonatti ◽  
V Rodrigues Jr ◽  
DS Azevedo ◽  
MHA Santos ◽  
...  

In this study we aimed to determine the levels of tumor necrosis factor beta (TNF-b), interleukin (IL) 1-beta (IL-1b), IL-5, IL-10, and interferon gamma (IFN-g ) in CSF from children during the onset of West syndrome (WS). We observed elevated levels of IL-1b and IFN-g correlated to clinical, EEG, therapeutic response, and follow-up suggesting the involvement of immune response in WS. These results suggest that inflammatory and immunologic mediators may play a role in the pathophysiologic mechanisms of infantile spasms. Our findings may explain the perfusion and cognitive disfunctions and actions of adrenocorticotropic hormone (ACTH), corticosteroids, and intravenous immunoglobulin (IVIg) observed in WS. In conclusion, WS results from association of neurophysiological mechanisms and structural abnormalities with participation of cytokines mainly in symptomatic group.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cristhian Rene Vargas Estrada ◽  
Bruna Fernanda Firmo ◽  
Daniele Belchior Vela ◽  
Marjury Cristina Maronezi ◽  
Ricardo Andrés Ramirez Uscategui ◽  
...  

AbstractThe aim of this study was to evaluate renal hemodynamics, routine clinical and laboratory parameters used to estimate renal function, and clinical evolution during six months in bitches with mammary carcinomas that underwent mastectomy and were treated (TG) or not (CG) with carprofen for three months after surgery. Twenty-six bitches with mammary carcinoma were equally distributed into TG that received carprofen 4.4 mg/kg/day for 90 days and CG that did not receive anti-inflammatory medication. Renal artery Doppler flowmetry, contrast-enhanced ultrasound (CEUS) of renal parenchyma, haematological, biochemical and clinical analyses were obtained once a month. These data were compared between groups and time via analysis of variance (ANOVA) in a completely randomized design with repeated measures (P < 0.05). On B-mode ultrasound, the area of the renal artery was greater (P = 0.0003) in the TG. Regarding laboratory findings, haematocrit and haemoglobin were similar in both groups, showing a significant and gradual increase after three months of treatment; MCV, MHC, and MCHC were increased (P < 0.05) and lymphocyte and band counts decreased (P < 0.05) in the TG. Regarding biochemical tests, ALT was the only parameter with a significant difference, being higher (P = 0.0272) in the treated group. It can be concluded that the use of carprofen for 90 days causes minimal changes in renal perfusion, erythrocyte parameters and ALT activity, and reduces the proportion of blood inflammatory cells. Therefore, use of this medication can be carried out safely in patients who require auxiliary cancer treatment.


2021 ◽  
Author(s):  
Cristhian Vargas Estrada ◽  
Bruna Fernanda Firmo ◽  
Daniele Belchior Vela ◽  
Marjury Cristina Maronezi ◽  
Ricardo Andrés Ramirez Uscategui ◽  
...  

Abstract Objective. The aim of this study was to evaluate renal hemodynamics, routine clinical and laboratory parameters used to estimate renal function, and clinical evolution during six months in bitches with mammary carcinomas that underwent mastectomy and were treated (TG) or not (CG) with carprofen for three months after surgery. Methods. Twenty-six bitches with mammary neoplasia were equally distributed into TG that received carprofen 4.4 mg/kg/day for 90 days and CG that did not receive anti-inflammatory medication. Renal artery Doppler flowmetry, contrast-enhanced ultrasound (CEUS) of renal parenchyma, haematological, biochemical and clinical analyses were obtained once a month. These data were compared between groups and moments via analysis of variance (ANOVA) in a completely randomized design with repeated measures (P<0.05). Results. On B-mode ultrasound, the area of the ​​renal artery was greater (P=0.0003) in the TG. Regarding laboratory findings, haematocrit and haemoglobin were similar in both groups, showing a significant and gradual increase after three months of treatment; MCV, MHC, and MCHC were increased (P<0.05) and lymphocyte and band counts decreased (P<0.05) in the TG. Regarding biochemical tests, ALT was the only parameter with a significant difference, being higher (P=0.0272) in the treated group.Conclusion. It can be concluded that the use of carprofen for 90 days causes minimal changes in renal perfusion, erythrocyte parameters and ALT activity, and reduces the proportion of blood inflammatory cells. Therefore, use of this medication can be carried out safely in patients who require auxiliary cancer treatment.


Author(s):  
Ruka Nakasone ◽  
Kazumichi Fujioka ◽  
Yuki Kyono ◽  
Asumi Yoshida ◽  
Takumi Kido ◽  
...  

To date, the difference in neurodevelopmental outcomes between late preterm infants (LPI) born at 34 and 35 gestational weeks (LPI-34 and LPI-35, respectively) has not been elucidated. This retrospective study aimed to evaluate neurodevelopmental outcomes at 18 months of corrected age for LPI-34 and LPI-35, and to elucidate factors predicting neurodevelopmental impairment (NDI). Records of all LPI-34 (n = 93) and LPI-35 (n = 121) admitted to our facility from 2013 to 2017 were reviewed. Patients with congenital or chromosomal anomalies, severe neonatal asphyxia, and without developmental quotient (DQ) data were excluded. Psychomotor development was assessed as a DQ using the Kyoto Scale of Psychological Development at 18 months of corrected age. NDI was defined as DQ < 80 or when severe neurodevelopmental problems made neurodevelopmental assessment impossible. We compared the clinical characteristics and DQ values between LPI-34 (n = 62) and LPI-35 (n = 73). To elucidate the factors predicting NDI at 18 months of corrected age, we compared clinical factors between the NDI (n = 17) and non-NDI (n = 118) groups. No significant difference was observed in DQ values at 18 months of corrected age between the groups in each area and overall. Among clinical factors, male sex, intraventricular hemorrhage (IVH), hyperbilirubinemia, and severe hyperbilirubinemia had a higher prevalence in the NDI group than in the non-NDI group, and IVH and/or severe hyperbilirubinemia showed the highest Youden Index values for predicting NDI. Based on the results of this study, we can conclude that no significant difference in neurodevelopmental outcomes at 18 months of corrected age was observed between LPI-34 and LPI-35. Patients with severe hyperbilirubinemia and/or IVH should be considered to be at high risk for developing NDI.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Lingcheng Zeng ◽  
Qi Mei ◽  
Hua Li ◽  
Changshu Ke ◽  
Jiasheng Yu ◽  
...  

AbstractTo evaluate the surgical effect on survival in patients with incidental low-grade glioma (LGG) through comparison between asymptomatic and symptomatic patients. The medical records of surgically treated adult cerebral incidental LGG (iLGG) patients in our department between January 2008 and December 2015 were retrospectively reviewed. The survival of patients was calculated starting from the initial imaging diagnosis. Factors related to progression-free survival (PFS), overall survival (OS) and malignant progression-free survival (MPFS) were statistically analyzed. Seventy-five iLGG patients underwent surgery: 49 in the asymptomatic group, who underwent surgery in the asymptomatic period, and 26 in the symptomatic group, who underwent surgery after the tumor had grown and the patients had developed tumor-related symptoms. Significantly more tumors were initially located adjacent to the functional area in the symptomatic group than in the asymptomatic group (P < 0.05), but there was no significant difference in the total resection rate between the two groups. The incidence of postoperative complications (15.4%) and postoperative epilepsy (23.1%) was higher in the symptomatic group than in the asymptomatic group (4.1% and 10.2%, respectively). Multivariate analysis showed that surgical timing, namely, surgery performed before or after symptom occurrence, had no significant effect on PFS, OS or MPFS, while total resection significantly prolonged PFS, OS and MPFS, and the pathology of oligodendroglioma was positively correlated with PFS and OS (P < 0.05). Surgical timing for iLGGs should facilitate total resection. If total resection can be achieved, even after symptom occurrence, patients can achieve comparable survival benefits to those treated with surgery in the asymptomatic phase.


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

2021 ◽  
Vol 10 (6) ◽  
pp. 1255
Author(s):  
Hiroyuki Ishiyama ◽  
Masayuki Hideshima ◽  
Shusuke Inukai ◽  
Meiyo Tamaoka ◽  
Akira Nishiyama ◽  
...  

The aim of this study was to determine the utility of respiratory resistance as a predictor of oral appliance (OA) response in obstructive sleep apnea (OSA). Twenty-seven patients with OSA (mean respiratory event index (REI): 17.5 ± 6.5 events/h) were recruited. At baseline, the respiratory resistance (R20) was measured by impulse oscillometry (IOS) with a fitted nasal mask in the supine position, and cephalometric radiographs were obtained to analyze the pharyngeal airway space (SPAS: superior posterior airway space, MAS: middle airway space, IAS: inferior airway space). The R20 and radiographs after the OA treatment were evaluated, and the changes from the baseline were analyzed. A sleep test with OA was carried out using a portable device. The subjects were divided into Responders and Non-responders based on an REI improvement ≥ 50% from the baseline, or REI < 5 after treatment, and the R20 reduction rate between the two groups were compared. The subjects comprised 20 responders and 7 non-responders. The R20 reduction rate with OA in responders was significantly greater than it was in non-responders (14.4 ± 7.9 % versus 2.4 ± 9.8 %, p < 0.05). In responders, SPAS, MAS, and IAS were significantly widened and R20 was significantly decreased with OA (p < 0.05). There was no significant difference in non-responders (p > 0.05). A logistic multiple regression analysis showed that the R20 reduction rate was predictive for OA treatment responses (2% incremental odds ratio (OR), 24.5; 95% CI, 21.5–28.0; p = 0.018). This pilot study confirmed that respiratory resistance may have significant clinical utility in predicting OA treatment responses.


2021 ◽  
Author(s):  
Shima Salehi ◽  
Rozita Hosseini Shamsabadi ◽  
Hassan Otukesh ◽  
Reza Shiari ◽  
Monir Sharafi

Abstract Background: Lupus is an inflammatory and autoimmune disease that involves various tissues and organs of the body. Identification of diagnostic elements to rapid identification of seronegative lupus cases is very important in order to prevent morbidity and progression of disease. This study aimed to compare clinical and laboratory findings of seropositive cases with seronegative lupus patients. Methods: This cross-sectional analytic study was performed on 43 children (17 seronegative and 26 seropositive) with lupus who were admitted to Ali Asghar Hospital during 2007-2017. Seropositive patients had anti-nuclear antibody (ANA) titration >1/80, while seronegative patients had ANA titration <1/80 (at the time of disease diagnosis). Clinical and laboratory findings were compared between two groups.Results: Serositis in patients with ANA- was significantly higher than ANA+ (41.17% vs. 23.07%; p = 0.042). ANA- group had higher autoimmune disease history than ANA+ group (42.85% vs. 15.0%; p = 0.041). The family history of the disease in the ANA- group was greater than ANA+ group (50% vs. 23.52%). The percentage of hypertensive patients in ANA- group was higher than ANA+ group (52.94% vs. 26.92%; p = 0.037). Neurologic symptoms in ANA+ and ANA- groups were 38.46% and 17.64%, respectively (p = 0.043). The frequency of patients with thrombocytopenia in ANA+ group was significantly greater than ANA- group (32% vs. 12.5%; p=0.041). There was no significant difference in other clinical and laboratory findings between two groups. Conclusion: Seronegative lupus patients had higher percentage of musculoskeletal symptoms, autoimmune disease history, familial history of disease, and hypertension, while neurological and thrombocytopenia symptoms were higher in seropositive patients compared to seronegative cases. Therefore, evaluation of these factors can be helpful to diagnosis of seronegative patients.


Author(s):  
Anne R Synnes ◽  
Julie Petrie ◽  
Ruth E Grunau ◽  
Paige Church ◽  
Edmond Kelly ◽  
...  

ObjectiveTo examine whether the family integrated care (FICare) programme, a multifaceted approach which enables parents to be engaged as primary caregivers in the neonatal intensive care unit, impacts infant neurodevelopment and growth at 18 months’ corrected age.Design/MethodsProspective cohort study of infants born <29 weeks’ gestational age (GA) who participated in the FICare cluster randomised control trial (cRCT) and were assessed in the Canadian Neonatal Follow-Up Network (CNFUN). The primary outcome measure, Cognitive or Language composite score <85 on the Bayley-III, was compared between FICare exposed and routine care children using logistic regression, adjusted for potential confounders and employing generalised estimation equations to account for clustering of infants within sites.ResultsOf 756 infants <29 weeks’ GA in the FICare cRCT, 505 were enrolled in CNFUN and 455 were assessed (238 FICare, 217 control). Compared with controls, FICare infants had significantly higher incidence of intraventricular haemorrhage (IVH) (19.5% vs 11.7%, p=0.024) and higher proportion of employed mothers (76.6% vs 73.6%, p=0.043). There was no significant difference in the odds of the primary outcome (adjusted OR: 0.92 (0.59 to 1.42) FiCare vs Control) on multivariable analyses adjusted for GA, IVH and maternal employment. However, Bayley-III Motor scores (adjusted difference in mean (95% CI) 3.87 (1.22 to 6.53) and body mass index 0.67 (0.36 to 0.99) were higher in the FICare group.ConclusionsVery preterm infants exposed to FICare had no significant difference in incidence of cognitive or language delay but had better motor development.Trial registration numberParticipants in this cohort study were previously enrolled in a registered trial: NCT01852695


2021 ◽  
pp. 088506662110388
Author(s):  
Divya Birudaraju ◽  
Sajad Hamal ◽  
John A. Tayek

Purpose To test the benefits of Solumedrol treatment in sepsis patients with a blunted adrenocorticotropic hormone (ACTH)-cortisol response (delta <13 µg/dL) with regard to the number of days on ventilator, days on intravenous blood pressure support, length of time in an intensive care unit (ICU), 14-day mortality, and 28-day mortality. The trial was prospective, randomized, and double-blind. As part of a larger sepsis trial, 54 patients with sepsis had an intravenous ACTH stimulation test using 250 µg of ACTH, and serum cortisol was measured at times 0, 30, and 60 min. Eleven patients failed to increase their cortisol concentration above 19.9 µg/dL and were excluded from the clinical trial as they were considered to have adrenal insufficiency. The remaining 43 patients had a baseline cortisol of 32 ± 1 µg/dL increased to 38 ± 3 µg/dL at 30 min and 40 ± 3 at 60 min. All cortisol responses were <12.9 µg/dL between time 0 and time 60, which is defined as a blunted cortisol response to intravenous ACTH administration. Twenty-one were randomized to receive 20 mg of intravenous Solumedrol and 22 were randomized to receive a matching placebo every 8 h for 7-days. There was no significant difference between the two randomized groups. Data analysis was carried out bya two-tailed test and P < .05 as significant. Results Results: The mean age was 51 ± 2 (mean ± SEM) with 61% female. Groups were well matched with regard to APACHE III score in Solumedrol versus placebo (59 ± 6 vs 59 ± 6), white blood cell count (18.8 ± 2.2 vs 18.6 ± 2.6), and incidence of bacteremia (29 vs 39%). The 28-day mortality rate was reduced in the Solumedrol treated arm (43 ± 11 vs 73 ± 10%; P < .05). There was no change in days in ICU, days on blood pressure agents, or days on ventilator. Seven days of high-dose intravenous Solumedrol treatment (20 mg every 8 h) in patients with a blunted cortisol response to ACTH was associated with an improved 28-day survival. This small study suggests that an inability to increase endogenous cortisol production in patients with sepsis who are then provided steroid treatment could improve survival.


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