11 research outputs found

    Image of the month. Delayed and isolated basal ganglia damage after methadone intoxication

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    peer reviewedPreferential cerebellar localization during a severe methadone intoxication is explained by the high µ-opioid's receptors concentration in the cerebellum. If basal ganglia are affected, that may result in unfavorable progressive neurological evolution. Here, we report the case of a patient who developed neurological deterioration with akinetic mutism, pyramidal and extrapyramidal syndrome with parkinsonism and dystonia, ten days after methadone intoxication and rapid CPR. Magnetic resonance shows lenticular nuclei damage extending in caudate nuclei. Our patient is the first case reported with isolated basal ganglia’s injury in this conditions. It asks the question of physiopathological mechanisms: direct toxic effect of methadone or secondary to hypoxia. Therapeutic and neuroprotective management are also discussed

    Image of the month. Delayed and isolated basal ganglia damage after methadone intoxication

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    peer reviewedLa localisation cérébelleuse préférentielle des lésions liées à une intoxication sévère à la méthadone s'explique par la concentration élevée des récepteurs opioïdes de type µ dans le cervelet. Si les ganglions de la base sont touchés, cela peut entraîner une évolution neurologique progressivement défavorable. Dans cet article, nous rapportons le cas d'un patient qui a développé une détérioration neurologique avec un mutisme akinétique, un syndrome pyramidal et extrapyramidal avec parkinsonisme et dystonie, dix jours après une intoxication à la méthadone et une rapide réanimation cardio-pulmonaire. La résonance magnétique montre des lésions au niveau des noyaux lenticulaires s'étendant aux noyaux caudés. Notre patient est le premier cas décrit évoluant vers une atteinte isolée et retardée des ganglions de la base dans ces conditions. Les mécanismes physiopathologiques peuvent être discutés: effet toxique direct de la méthadone ou secondaire à l'hypoxie. La prise en charge thérapeutique et les moyens de prévention sont également abordés

    Belgique

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    Human herpesvirus 6 (HHV-6) has been demonstrated to be a neurotropic virus and has been linked to several neurological complications associated with a wide range of clinical outcomes. Here, we present data from immunocompetent children with a central neurological condition and intrathecal DNA detection of HHV-6 virus. Through these cases, physiopathology, investigations, and outcome of the disease will be described. Finally, specific treatment will be discussed

    Newly diagnosed and growing subependymal giant cell astrocytoma in adults with tuberous sclerosis complex: Results from the international TOSCA study

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    International audienceThe onset and growth of subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis complex (TSC) typically occurs in childhood. There is minimal information on SEGA evolution in adults with TSC. Of 2,211 patients enrolled in TOSCA, 220 of the 803 adults (27.4%) ever had a SEGA. Of 186 patients with SEGA still ongoing in adulthood, 153 (82.3%) remained asymptomatic, and 33 (17.7%) were reported to ever have developed symptoms related to SEGA growth. SEGA growth since the previous scan was reported in 39 of the 186 adults (21%) with ongoing SEGA. All but one patient with growing SEGA had mutations in TSC2. Fourteen adults (2.4%) were newly diagnosed with SEGA during follow-up, and majority had mutations in TSC2. Our findings suggest that surveillance for new or growing SEGA is warranted also in adulthood, particularly in patients with mutations in TSC2

    Treatment patterns and use of resources in patients with tuberous sclerosis complex : insights from the TOSCA registry

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    Tuberous Sclerosis Complex (TSC) is a rare autosomal-dominant disorder caused by mutations in the TSC1 or TSC2 genes. Patients with TSC may suffer from a wide range of clinical manifestations; however, the burden of TSC and its impact on healthcare resources needed for its management remain unknown. Besides, the use of resources might vary across countries depending on the country-specific clinical practice. The aim of this paper is to describe the use of TSC-related resources and treatment patterns within the TOSCA registry. A total of 2,214 patients with TSC from 31 countries were enrolled and had a follow-up of up to 5 years. A search was conducted to identify the variables containing both medical and non-medical resource use information within TOSCA. This search was performed both at the level of the core project as well as at the level of the research projects on epilepsy, subependymal giant cell astrocytoma (SEGA), lymphangioleiomyomatosis (LAM), and renal angiomyolipoma (rAML) taking into account the timepoints of the study, age groups, and countries. Data from the quality of life (QoL) research project were analyzed by type of visit and age at enrollment. Treatments varied greatly depending on the clinical manifestation, timepoint in the study, and age groups. GAB Aergics were the most prescribed drugs for epilepsy, and mTOR inhibitors are dramatically replacing surgery in patients with SEGA, despite current recommendations proposing both treatment options. mTOR inhibitors are also becoming common treatments in rAML and LAM patients. Forty-two out of the 143 patients (29.4%) who participated in the QoL research project reported inpatient stays over the last year. Data from non-medical resource use showed the critical impact of TSC on job status and capacity. Disability allowances were more common in children than adults (51.1% vs 38.2%). Psychological counseling, social services and social worker services were needed by <15% of the patients, regardless of age. The long-term nature, together with the variability in its clinical manifestations, makes TSC a complex and resource-demanding disease. The present study shows a comprehensive picture of the resource use implications of TSC
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