Involvement of the cerebellum in the regulation of executive functions in children-Preliminary analysis based on a neuropsychological study of children after cerebellar tumour surgery.

Aim: Preliminary assessment of executive functions in children with cerebellar lesions, description of their emotional-social functioning and selection of sensitive neuropsychological tools to detect the cerebellar cognitive affective syndrome (CCAS). Materials and methods: The study group consisted of 10 children after cerebellar tumour surgery. The control group consisted of 10 healthy children, matched for age and sex: The IDS-2 executive functions battery, the Conners 3 ADHD questionnaire, the Autism Spectrum Rating Scales (ASRS) and the International Cooperative Ataxia Rating Scale (ICARS) were used. Results: Statistical analysis showed statistically significant differences between the experimental and control groups in terms of two dimensions of executive functioning. Children from experimental group was characterised by worse planning and divided attention than healthy controls. Moreover children with cerebellar lesions were characterised by significantly higher levels of some behaviours similar to that observed in autism spectrum disorders, namely difficulties in social relationships, self-regulation of emotions, attention, and greater behavioural rigidity. Test power analysis and estimation of the effect size by the Cohen's d coefficient indicated that with a slight increase in the size of the experimental group, the probability of detecting statistically significant difference in the executive functions total measure score as well as in several ASRS subscales increased, but not in Conners 3 subscales. Conclusions: Cerebellar damage may pose a risk for dysexecutive syndrome and social-emotional problems in children. The IDS-2 executive functions battery and the ASRS test are sufficiently sensitive tools to assess elements of the CCAS in children.

Cognitive characteristics of children treated for deformational plagiocephaly and parental perception of the therapy.

BACKGROUND: Deformational plagiocephaly (DP) is a common acquired skull deformity, with the highest incidence at the age of 4 months (19.7%). As it is considered benign, good cosmetic outcome is the primary aim of treatment, which includes either repositioning therapy (RT) with or without physiotherapy or molding helmet therapy (MHT). However, the issue of possible developmental delays in this group has been investigated. METHODS: Children aged 2-7 years previously treated with RT or MHT for DP were eligible for the study. Patients were examined by a psychologist using Stanford-Binet 5 Scale (SB5), while parents filled in a questionnaire, investigating their perception of the therapy and child's cognitive performance. RESULTS: Twenty-one patients were found to be eligible and agreed to participate in the study. Subgroups consisting of 11 RT (eight males; median age 4 years [range 2-7 years]) and 10 MHT (seven males; median age 4 years [range 2-6 years]) patients were analyzed. Mean and median SB5 scores in whole group were within normal range and no significant differences were found between them in RT and MHT groups. In MHT group, children with left-sided asymmetry scored significantly higher in working memory (WM). Overall parental satisfaction with therapy had no relation to SB5 scores in whole analyzed group, but in RT group a rate of 5 was more common among parents whose children scored better in visual-spatial processing (VSP, P=0.042). Therapy safety was more likely to be rated as 5 in patients who scored higher in QR (P=0.023) and WM (P=0.005); in RT group this relationship was found only in WM (P=0.011). CONCLUSIONS: Our results suggest that, in absence of medical conditions affecting cognitive performance, cognitive abilities of children aged 2-7 years treated for DP either by MHT or RT are undisturbed. Although majority of parents are satisfied with chosen therapy, those whose children have better cognitive performance are more likely to rate therapy higher.

The management of gynecological complications in long-term survivors after allogeneic hematopoietic cell transplantation-a single-center real-life experience.

In everyday gynecological practice, there is an unmet need to manage survivors after allogeneic hematopoietic cell transplantation (allo-HCT). The major gynecological complications include premature ovarian insufficiency (POI), chronic graft-versus-host disease (cGVHD) of the anogenital zone (cGVHDgyn), and secondary neoplasms. Aiming to assess a real-life scale of problems associated with HCT, we performed a detailed analysis of a consecutive series of females after allo-HCT who were referred for a routine gynecological evaluation. The study includes 38 females after allo-HCT in whom gynecological examination with cervical smear and USG were performed, followed by colposcopy according to NCCN guidelines. NIH scoring system was used to classify a grade of cGVHDgyn. The incidence of cGVHD was 71% whereas GVHDgyn was 29%, including 5 patients with score 3 at the time of diagnosis. The other manifestations (frequently noted) included the skin, mucosa, eyes, and liver. Menopause was diagnosed in 93% females, and in 81% of them, POI criteria were fulfilled. Ovarian function resumed in 2 cases. The rate of abnormal cytology was 26%: 4 ASCUS, 1 AGUS, 1 LSIL, 3 HSIL/ASC-H, and one cytological suspicion of cervical cancer. GVHDgyn was documented in 10 patients, and 6 of them had abnormal cervical cytology. Early topical estrogen therapy led to a significant reduction in vaginal dryness (p < 0.05), dyspareunia (p < 0.05), and less frequent cGVHDgyn (p < 0.05). GVHDgyn develops in about 30% of long-term allo-HCT survivors. Topical estrogens and hormonal replacement therapy alleviate symptoms and prevent the occurrence of severe consequences of menopause.

Schizencephaly-diagnostics and clinical dilemmas.

BACKGROUND: Schizencephaly is an uncommon congenital disorder of cerebral cortical development. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotropic, dysplastic gray matter. The causes of schizencephaly are heterogeneous and can include teratogens, prenatal infection, maternal trauma, or EMX2 mutations. METHOD: In the present paper, the authors described difficulties in employing diagnostic imaging in differentiating between type II (open-lip) schizencephaly and much more common intracranial fluid spaces of a different origin (arachnoid cysts and hydrocephalus). RESULT: In all the three cases, the treatment consisted in implantation of a shunt system; nevertheless, it should be emphasized that a surgical intervention in the third presented case (type II schizencephaly) aimed at relieving the symptoms of intracranial hypertension-a directly life-threatening condition-since shunting is not a method of treating schizencephaly itself. CONCLUSIONS: Although proper interpretation of the character of intracranial fluid spaces is of significance for further therapeutic management, yet, the key decision as to the surgical intervention is made based on clinical presentation, predominantly on symptoms of intracranial hypertension.

Recurrence of Brenner ovary borderline tumor in the abdominal wall postoperative scar--a case report and research of the literature.

We report a case of a 74-year-old female, who underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy due to a solid-cystic tumor of the right ovary. The histopathological diagnosis revealed a borderline Brenner tumor (BBT). After 25 disease-free months the patient was admitted to a hospital because of a tumor in the postoperative scar of the abdominal wall, later diagnosed as infiltrating Brenner tumor consistent with ovarian borderline lesion. The tumor in the postoperative scar was therefore diagnosed as BBT. The article presents results of literature search on BBT in order to find characteristic features of this very rare ovarian tumor. To the best of our knowledge, this is the first report of subcutaneous recurrence of Brenner ovary tumor of low malignant potential.