Long‐term effects of zonisamide in adult patients with intellectual disability

Author(s):  
Kattrinna Eck ◽  
Christophe Rauch ◽  
Frank Kerling ◽  
Hajo Hamer ◽  
Martin Winterholler
2006 ◽  
Vol 8 (4) ◽  
pp. 750-755 ◽  
Author(s):  
Marja Äikiä ◽  
Leena Jutila ◽  
Tuuli Salmenperä ◽  
Esa Mervaala ◽  
Reetta Kälviäinen

2020 ◽  
Vol 9 (2) ◽  
pp. 463
Author(s):  
Attila Nemes ◽  
Gergely Rácz ◽  
Árpád Kormányos ◽  
Péter Domsik ◽  
Anita Kalapos ◽  
...  

Background: In complete or dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed. The present study was designed to examine dTGA-associated left atrial (LA) volumetric and functional abnormalities in adult patients late after repair and to compare their results to those of healthy controls. Methods: The present study consisted of 15 dTGA patients (30.3 ± 8.1 years, 9 males), the patients had Mustard (n = 8) or Senning (n = 7) procedure performed. Their results were compared to those of 36 age- and gender-matched healthy subjects (28.7 ± 1.5 years, 24 males). Results: Increased maximum LA volume and reduced LA emptying fractions respecting the cardiac cycle could be demonstrated in our dTGA patients. LA stroke volumes representing all LA functions were significantly reduced. Peak LA circumferential, longitudinal, and area strains and LA circumferential, longitudinal, and area strains measured at atrial contraction were reduced in our dTGA patients. Most LA strains were reduced in patients having Mustard surgery compared to controls and patients undergoing Senning operation. Conclusions: Significant LA volumetric and functional abnormalities could be demonstrated in adult patients with dTGA late after repair. Senning procedure seems to have more beneficial long-term effects on LA volumetric and functional features as compared to the Mustard procedure.


2000 ◽  
Vol 10 (2) ◽  
pp. 195-208 ◽  
Author(s):  
Brian Cooper ◽  
Cliff Picton

This article reports on the long-term effects of relocation on a sample of 45 people with an intellectual disability who moved from an institution to the community and to other institutions. Data were gathered for 3 years after relocation. Results revealed that a move to supported community residences and to refurbished units within other institutions was associated with increased quality of life/care and, for interinstitutional movers, decreased challenging behaviors. Relocation did not lead to increased performance of independent community living skills, despite increased opportunities in the community. Where significant changes were identified they were modest and occurred within 6 months of relocation. It is concluded that positive outcomes result from community placement and interinstitutional relocation. The implications and limitations of this evidence for social work research and practice are discussed.


2020 ◽  
Vol 46 (11) ◽  
pp. 738-742
Author(s):  
Maria Luisa Di Pietro ◽  
Drieda Zaçe

Prader-Willi syndrome (PWS) is one of the 25 syndromic forms of obesity, in which patients present—in addition to different degrees of obesity—intellectual disability, endocrine disturbs, hyperphagia and/or other signs of hypothalamic dysfunction. In front of a severe/extreme obesity and the failure of non-invasive treatments, bariatric surgery is proposed as a therapeutic option. The complexity of the clinical condition, which could affect the long-term effects of bariatric surgery, and the frequent association with a mild to severe intellectual disability raise some ethical concerns in the treatment of obese PWS adolescents. This article analyses these issues referring to the principles of healthcare ethics: beneficence/non-maleficence (proportionality of treatments; minimisation of risks); respect of autonomy; justice. Based on these principles, three hypothetical scenarios are defined: (1) obese PWS adolescent, capable of making an autonomous decision; (2) obese PWS adolescent with a severe intellectual disability, whose parents agree with bariatric surgery; (3) obese PWS adolescent with a life-threatening condition and a severe intellectual disability, whose parents do not agree with bariatric surgery. The currently available evidence on efficacy and safety of bariatric surgery in PWS adolescents with extreme or severe obesity and the lack of adequate long-term follow-up suggests great caution even in a very life-threatening condition. Clinicians must always obtain a full IQ assessment of patients by psychologists. A multidisciplinary team is needed to analyse the clinical, psychological, social and ethical aspects and organise support for patient and parents, involving also the hospital ethical committee or, if necessary, legal authorities.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Fiorenza Stagni ◽  
Beatrice Uguagliati ◽  
Marco Emili ◽  
Andrea Giacomini ◽  
Renata Bartesaghi ◽  
...  

AbstractNeurogenesis impairment is a key determinant of intellectual disability in Down syndrome (DS), a genetic pathology due to triplication of chromosome 21. Since neurogenesis ceases after birth, apart in the hippocampus and olfactory bulb, the only means to tackle the problem of neurogenesis impairment in DS at its root is to intervene during gestation. A few studies in DS mouse models show that this is possible, although the drugs used may raise caveats in terms of safety. We previously found that neonatal treatment with 7,8-dihydroxyflavone (7,8-DHF), a flavonoid present in plants, restores hippocampal neurogenesis in the Ts65Dn model of DS. The goal of the current study was to establish whether prenatal treatment with 7,8-DHF improves/restores overall brain proliferation potency. Pregnant Ts65Dn females received 7,8-DHF from embryonic day 10 until delivery. On postnatal day 2 (P2) the pups were injected with BrdU and were killed after either 2 h or 52–60 days (P52–60). Evaluation of the number of proliferating (BrdU+) cells in various forebrain neurogenic niches of P2 mice showed that in treated Ts65Dn mice proliferation potency was improved or even restored in most of the examined regions, including the hippocampus. Quantification of the surviving BrdU+ cells in the dentate gyrus of P52–60 mice showed no difference between treated and untreated Ts65Dn mice. At P52–60, however, treated Ts65Dn mice exhibited a larger number of granule cells in comparison with their untreated counterparts, although their number did not reach that of euploid mice. Results show that 7,8-DHF has a widespread impact on prenatal proliferation potency in Ts65Dn mice and exerts mild long-term effects. It remains to be established whether treatment extending into the neonatal period can lead to an improvement in brain development that is retained in adulthood.


2018 ◽  
Vol 13 (3-4) ◽  
pp. 28-37
Author(s):  
M.B. Dzhus ◽  
T.V. Marushko ◽  
H.V. Mostbauer ◽  
О.І. Ivashkivsky ◽  
I.V. Kurylchyk ◽  
...  

Relevance. It is known that the poly-articular variant of JIA is associated with significant articular and extra-articular damages with predominant lesions of small and medium joints with limitation of their function and the development of ankylosis, requiring surgical intervention in these patients. Objective. The aim of the study was to evaluate the risk factors of articular and extra-articular damages in adults with the poly-articular variant of the JIA and to work out a prognostic model for their development. Materials and methods. The study included 45 adult patients with poly-articular (RF+ and RF-) variant of the JIA. Retrospective analysis of medical records of patients with poly-articular variant of JIA was made, taking into account clinical manifestations in the onset of the disease, laboratory parameters and response to therapy. In adulthood, the integral index of articular (JADI-A) and extra-articular (JADI-E) damages was used to assess the long-term effects of JIA. Results. Unfavorable prognostic factors were revealed by method of binary logistic regression. Mathematical model for predicting the probability of long-term negative articular and extra-articular damages of poly-articular JIA was developed. Conclusions 1. The risk factors for the development of articular and extra-articular damages in adulthood in patients with a poly-articular variant of the JIA are female sex, the presence of lymphadenopathy and/or splenomegaly in childhood, the level of ESR during treatment in childhood, the activity of JADAS in childhood, the presence of symmetric arthritis in childhood, the RF positivity in the debut of the disease, the treatment with glucocorticoids, the cumulative dose of the DMARDs, and lack of therapy by DMARDs. 2. A prognostic model for the development of articular and extra-articular damages in adult patients with a poly-articular variant of the JIA is developed. This model is effective and allows determine the adverse course of the disease and can be the basis for personalized treatment for the prevention of the development of significant articular and extra-articular damages of JIA.


2021 ◽  
Vol 7 (2) ◽  
pp. 21
Author(s):  
Kenji Yamada ◽  
Seiji Yamaguchi ◽  
Kazunori Yokoyama ◽  
Kikumaro Aoki ◽  
Takeshi Taketani

Japanese newborn screening (NBS) for phenylketonuria (PKU) was initiated in 1977. We surveyed the neurological outcomes of Japanese adult patients with PKU to investigate the long-term effects and of and issues with NBS. Eighty-five patients with PKU aged over 19 years who continued to be treated with a phenylalanine-free amino acid formula were investigated by administering questionnaires regarding clinical characteristics, such as mental ability, education status, and therapeutic condition. Of the 85 subjects, 68 patients were detected by NBS (NBS group), while the other 17 were clinically diagnosed before the initiation of NBS (pre-NBS group). Further, 10 of the 68 NBS patients presented intellectual and/or psychiatric disabilities, 5 of whom had a history of treatment discontinuation; in contrast, 12 of the 17 pre-NBS patients presented with neuropsychiatric symptoms. Regarding social outcomes, almost all patients in the NBS group could live an independent life, while over half of the patients in the pre-NBS group were not employed or lived in nursing-care facilities. Neurological outcomes are obviously improved by NBS in Japan. However, some patients, even those detected by NBS, developed neuropsychiatric symptoms due to treatment disruption. Lifelong and strict management is essential to maintain good neurological and social prognoses for patients with PKU.


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