Search

Tanycytic ependymoma is an unusual morphological variant of WHO grade II ependymoma, typically arising from the cervical or thoracic spinal cord. Although the literature deals extensively with pathological features of this tumour entity, imaging features have not been well characterised. The purpose of this study was to review magnetic resonance imaging (MRI) features of spinal tanycytic ependymomas reported in the literature to date, exemplified by a case of a patient with tanycytic ependymoma of the conus medullaris presenting to our hospital.A Medline search of the English literature for all previously published cases of spinal tanycytic ependymoma was carried out and the reported MRI features reviewed. The tumours were found to be typically well-demarcated masses, predominantly showing isointensity on T1-weighted signal, and T2-weighted hyperintensity, with variable patterns of contrast enhancement. A cystic component was seen in half of the cases, and in a minority a mural nodule was present within the cyst wall. Associated syrinx formation was observed in one-third of the cases and haemorrhage was rare, which may be helpful pointers in differentiating the lesion from other ependymoma subtypes.In conclusion, MRI characteristics of spinal tanycytic ependymoma are variable and non-specific, and radiological diagnosis thus remains challenging, although certain predominant features are identified in this report. Knowledge of these is important in the diagnostic differentiation from other intramedullary and extramedullary spinal tumours in order to guide appropriate surgical management. [ABSTRACT FROM AUTHOR]

Epilepsy is associated with a significant burden of false beliefs and social stigma in the setting of Sub-Saharan Africa. To assess the impacts of epilepsy on child education in Sierra Leone (SL), we carried out a cross-sectional descriptive study examining its effects on school attendance, participation in physical activities, and social acceptance among classmates. We also assessed the knowledge, beliefs, and attitudes regarding epilepsy of both the children's caregivers and teachers. The data were collected at various epilepsy clinics and schools in Freetown, SL. A total of 50 patients were interviewed and questionnaires administered to their caregivers and teachers, making a total of 150 respondents. Fifty-one percent of the children were absent from school for > 5 days per month. Ninety percent did not participate in games and sports, with the commonest reason being fear of occurrence of seizures. Thirty-six percent claimed having experienced negative attitude from their classmates. Regarding the caregivers, 48% believed that epilepsy was a medical illness, while 34% considered it a demonic manifestation. Forty-eight percent were apprehensive about sending their children to school, with 83% of these caregivers stating fear of seizures and potential injuries. Only 8% of the caregivers did not prevent their children from taking part in any physical activity at school. Regarding the teachers, 16% believed that epilepsy was a demonic manifestation, and 10% thought that it was contagious. Fourteen percent did not think that children with epilepsy should go to school, and 80% would prevent children with epilepsy from participating in games and sports. When faced with a child having a seizure, 48% would hold the child down, 12% would place a hard object in the child's mouth, and 12% would avoid any physical contact. In total, 20% of the children ceased attending school permanently; daily occurrence of seizures (p < 0.05), negative attitude of classmates (p < 0.001), and having an illiterate caregiver (p < 0.02) all showed a significant association with permanent cessation of schooling. The study demonstrates significant negative impacts of epilepsy on child education. Notably, the reasons for permanent exclusion from school appear to be as much related to attitudes as to the medical aspects of the disease itself. The data thus highlight the need for educational programs to address the widely prevalent misconceptions among both caregivers and teachers. [ABSTRACT FROM AUTHOR]

Background: The importance of connected speech analysis in the diagnosis and further classification of primary progressive aphasia (PPA) is becoming apparent. However, methodological inconsistencies in elicitation and analysis of speech samples hinder comparison of different laboratories' results. The recent recommendations for further classification of PPA have characterised three main variants, but we have demonstrated that a considerable proportion of patients do not conform to any of these, as their language deficits extend beyond those of a single syndromic variant (mixed PPA). This report is an attempt to characterise the language profiles of different non-semantic variants of PPA using a combination of connected speech analysis and standard neuropsychological tests. Aims: Our aims were (a) to assess the relative efficacy of semi-structured interviews and picture description tasks in revealing the important aspects of language deficit in our patient groups, (b) to document the language profile of a mixed PPA group for the first time, (c) to compare the patterns of speech abnormality in non-fluent variant and mixed PPA, relative to each other and to normal control participants, and (d) to identify those features of spontaneous speech decline that are frequent and tangible enough to be detected during clinical consultations. Methods & Procedures: Connected speech samples obtained from picture description and semi-structured interviews were analysed quantitatively and the outcomes were contrasted between the elicitation methods and among the participant groups. Due to a very small number of cases fulfilling criteria for logopaenic PPA, the speech of these patients was scrutinised individually. Outcomes & Results: Pair-wise comparisons of the two speech elicitation methods at a group level revealed only a partial concordance and greater sensitivity of semi-structured interviews in detecting different aspects of speech abnormality. At a group level there was a significant impairment in the morpho-syntactic aspects of connected speech in both non-fluent and mixed PPA. A statistically significant difference from controls was observed in the semantic measures of discourse only in the mixed PPA group. Clinically useful features that differentiate both non-fluent and mixed PPA groups from healthy volunteers were decreased speech rate, shorter utterances, fewer complex grammatical structures, lack of spontaneity, more non-sentential speech units (elliptical and abandoned units), more hesitation markers, and, to a lesser extent, more phonological errors and editing breaks. Conclusions: The combination of the present results with those from our previous report on semantic dementia that used the same methodology offers a quantitative detailed assessment of connected speech across the spectrum of PPA. [ABSTRACT FROM AUTHOR]

Background: Neurodegenerative syndromes are associated with varying degrees of language impairment. Connected speech analysis provides the most realistic measure of language function but its use has been restricted by operational constraints. Aims: In this prospective study we assessed the relative utility of a picture description task and a semi-structured interview in exposing the language decline in semantic dementia (SD) and typical mild Alzheimer's disease (AD), compared to each other and to healthy volunteers. Methods & Procedures: Our cohort comprised 16 patients with a clinical diagnosis of SD, 20 with mild typical AD, and 30 healthy participants. All speech samples were recorded during a face-to-face interview and were subjected to a well-controlled quantitative analysis. Outcomes & Results: Our results suggest that (a) the picture description task is more sensitive to abnormalities in the semantic measures, whereas the interviews are better at exposing morpho-syntactic deficits; (b) circumlocution is not, as has sometimes been claimed, a salient feature of speech in SD; (c) increases in the frequency of hesitation markers and of phonological and syntactic errors are prominent features of language decline in mild AD; and (d) a 150-word interview sample is adequate to provide a realistic reflection of language impairment. Conclusions: This study compared, for the first time, the two most commonly used methods of eliciting connected speech and documented many similarities in results from the two but also some salient differences in their sensitivity to specific aspects of language deficit. In addition, although replicating some aspects such as anomia, of what is already known about the language impairments in these two conditions, the study provides novel findings for both that could help clinicians in syndromic classification of anomic speech. [ABSTRACT FROM AUTHOR]