Foreign accent syndrome as a developmental motor speech disorder

Introduction: Foreign Accent Syndrome (FAS) is a relatively rare motor speech disorder in which the pronunciation of a patient is perceived by listeners of the same language community as distinctly foreign. FAS has been well documented in adult patients with etiologically heterogeneous, though mostly vascular brain lesions affecting the motor speech network of the language dominant hemisphere. In addition, reports exist of adult patients in whom FAS was due to a psychiatric illness. Although FAS has been reported in children, such accounts are rare and have remained largely anecdotal in that there have been no formally documented cases of FAS as a developmental motor speech disorder. Methods and results: For the first time, we describe the clinical, cognitive and neurolinguistic findings in two patients who in the absence of a history of psychiatric illness or acquired brain damage already presented with FAS at an early stage of speech and language development. In the first patient “developmental FAS” was associated with a dysharmonic distribution of neurocognitive test results indicating slight underdevelopment of visuo-spatial skills and visual memory. The second patient presented with “developmental FAS” associated with specific language impairment (SLI). Independent support for a diagnosis of FAS in both patients was obtained in an accent attribution experiment in which groups of native speakers of (Belgian) Dutch assessed the type of foreign accent of a sample of the patients’ conversational speech. Both patients were judged as non-native speakers of Dutch by the majority of participants who predominantly identified the accent as French. Conclusion: This paper for the first time documents two patients who presented with FAS on a developmental basis. The finding that FAS does not only occur in the context of acquired brain damage or psychogenic illness but also exists as developmental motor speech impairment requires a re-definition of FAS as a clinical syndrome

Developmental Coordination Disorder: Disruption of the Cerebello-Cerebral Network evidenced by SPECT

Little is known about the neurobiological substrate of developmental coordination disorder (DCD), a neuro-developmental syndrome with significant, negative impact on the motor, cognitive and affective level throughout lifespan. This paper reports the clinical, neurocognitive and neuroradiological findings of a 19-year-old patient with typical DCD. As demonstrated by mild ataxia and a close semiological correspondence with the recently acknowledged ‘cerebellar cognitive affective syndrome’, clinical and neurocognitive investigations unambiguously indicated functional disruption of the cerebellum. Structural MRI of the brain confirmed cerebellar involvement revealing a slight anterior/superior asymmetry of vermal fissures consistent with rostral vermisdysplasia. Although this abnormality of vermal fissuration is generally considered an incidental neuroradiological finding without any clinical relevance, a potentially subtle impact on the developmental level has never been formally excluded. In addition to a generally decreased perfusion of the cerebellum, a quantified Tc-99m-ECD SPECT disclosed functional suppression of the anatomoclinically suspected supratentorial regions involved in the execution of planned actions, visuo-spatial processing and affective regulation. Based on these findings, it is hypothesised that the cerebellum is crucially implicated in the pathophysiologcial mechanisms of DCD, reflecting disruption of the cerebello-cerebral network involved in the execution of planned actions, visuo-spatial cognition and affective regulation

Paradoxical reaction in non HIV-tuberculosis, a rare case with pancreatic involvement

A 24-year-old young man presents a sudden hepatic cytolysis and cholestasis after two months of isoniazid, rifampin and pyrazinamide therapy for severe lung tuberculosis. Initial data included heroin consumption history, HIV-negative serology, drug sensitivity and a normal abdominal CT

European Lung Cancer Working Party Clinical Practice Guidelines. Small Cell Lung Cnacer: IV. Limited disease

The present guidelines on the management of limited disease small cell lung cancer (SCLC) were formulated by the ELCWP in April 2007. They are designed to answer the following seven questions: 1) What is the definition of limited disease? 2) Should chest radiotherapy be provided and what are the benefits? 3) What is the optimal timing and mode of administration of chest irradiation? 4) Which are the optimal radiotherapy parameters: dose, fractionation, target volume? 5) What is the optimal chemotherapy regimen for limited disease SCLC? 6) Should prophylactic cranial irradiation be provided, when and for which patients? 7) What is the additional role of thoracic surgery in early SCLC

Risk analysis for inland vessels in estuary service

At present, less than 2 % of the hinterland traffic to the harbour of Zeebrugge is carried by inland navigation, due to the insufficient capacity of inland waterway connections. An alternative could be offered by estuary vessels, i.e. inland vessels strengthened and equipped for safe operation between the Belgian coastal harbours and the West Scheldt in favourable weather and wave conditions. However, the window determined by the present regulations of the Belgian Shipping Inspectorate is too restrictive, traffic being impossible for 60 days a year on average.Accordingly, the Belgian federal authorities have recently prepared new regulations, based on probabilistic design procedures, including a risk analysis with respect to criteria which take due account of the limitations inherent to the design of inland vessels.Measures have been proposed by the Flemish authorities to stimulate the operation of estuary inland vessels for container transport from and to the coastal harbours, implying financial support for construction and exploitation