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Functional Neurological Disorders in Children and Adolescents – A Long-Overdue Renaissance

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We present a practical overview of functional neurological disorder (FND), its epidemiology, assessment and diagnosis, diagnostic pitfalls, treatment, aetiology and mechanism. We present an update on functional limb weakness, tremor, dystonia and other abnormal movements, dissociative seizures, functional cognitive symptoms and urinary retention, and 'scan-negative' cauda equina syndrome. The diagnosis of FND should rest on clear positive evidence, typically from a combination of physical signs on examination or the nature of seizures. In treatment of FND, clear communication of the diagnosis and the involvement of the multidisciplinary team is beneficial. We recommend that patients with FND are referred to specialists with expertise in neurological diagnosis. FND is a common presentation in emergency and acute medical settings and there are many practical elements to making a positive diagnosis and communication which are useful for all physicians to be familiar with.
Article
Importance There is limited information about health care use and costs in patients with functional neurological disorders (FNDs). Objective To assess US emergency department (ED) and inpatient use and charges for FNDs. Design, Setting, and Participants This economic evaluation used Healthcare Cost and Utilization Project data to assess all-payer (1) adult (age, ≥18 years) hospitalizations (2008-2017), (2) pediatric (age, 5-17 years) hospitalizations (2003, 2006, 2009, 2012, and 2016), and (3) adult and pediatric ED evaluations (2008-2017). International Classification of Diseases, Ninth Revision, Clinical Modification code 300.11 (conversion disorder) or 306.0 (musculoskeletal malfunction arising from mental factors) and International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Clinical Modification codes for conversion disorder/functional neurological symptom disorder (F44.4 to F44.7) were used to conservatively define FNDs and to compare them with other neurological disorders that are associated with high levels of health care use. Analysis was performed between January 2019 and July 2020. Main Outcomes and Measures Admission traits (eg, demographic characteristics of patients, length of stay, and discharge disposition) and hospital charges. Results Compared with other neurological disorders in 2017, emergency FND evaluations of 36 359 adults (25 807 women [71.0%] and 3800 children (2733 girls [71.9%]) more frequently resulted in inpatient admissions (22 895 adult admissions [69.2% female] and 1264 pediatric admissions [73.4% ]). These FND admissions had a shorter mean (SEM) hospital length of stay (5.21 [0.15] days vs 6.03 [0.03] days, P < .001) but higher workup rates than admissions for comparable neurological diagnoses. Admissions for FNDs had low rates of inpatient physical therapy, occupational therapy, speech and language pathology, and psychiatric consultation. The total annual costs (a proxy for total costs in 2017 US dollars) were $1066 million (95% CI, $971-$1160 million) for adult FND inpatient charges in 2017 compared with $1241 million (95% CI, $1132-$1351 million) for anterior horn cell disease; $75 million (95% CI, $57-$92 million) for pediatric FND inpatient charges in 2012 compared with $86 million (95% CI, $63-$108 million) for demyelinating diseases; and $163 million (95% CI, $144-$182 million) for adult and pediatric ED visits in 2017 compared with $135 million (95% CI $111-$159 million) for refractory epilepsy. Total charges per admission for ED care of FNDs were higher than the other comparison groups in adults. Total costs and costs per admission for FNDs increased from 2008 to 2017 at a higher rate than that of other neurological disorders. Conclusions and Relevance This economic evaluation found that the more than $1.2 billion and increasing annual costs for ED and inpatient care of FNDs were similar to other investigation-intensive and pharmacologically demanding neurological disorders. Unnecessary investigations and iatrogenic harm inflate costs at the expense of necessary but neglected psychiatric and rehabilitative treatments.
Article
One of the considerations when a child presents with paroxysmal events is psychogenic nonepileptic seizures (PNES). PNES are discernible changes in behavior or consciousness that resemble epileptic seizures but are not accompanied by electrophysiologic changes. They are usually understood as the manifestation of a conversion disorder that reflects underlying psychological distress. There is a lack of population-based data on the prevalence or incidence of PNES in pediatric populations. The prevalence of PNES in children would appear to be lower than that in the adult population, but the prevalence of PNES seems to increase with age, and nonepileptic paroxysmal events are more likely to be PNES in adolescence than earlier in childhood. In terms of manifestation, PNES in childhood have been described using various categorizations and terminology, making comparisons across studies difficult. There is some evidence that events are more likely to involve unresponsiveness in younger children and prominent motor symptoms in older children. The most common precipitating factors would appear to be school-related difficulties and interpersonal conflict within the child's family. In terms of psychopathology, children with PNES are at high risk for symptoms of depression and anxiety. Accurate diagnosis of PNES in children is likely to involve taking a comprehensive description of the episodes, garnering the child's medical/developmental history, video-electroencephalography (video-EEG) to rule out epileptic seizures, and an evaluation of family functioning. The importance of effective and sensitive communication of the diagnosis of PNES has been emphasized and management approaches will typically involve multidisciplinary efforts to safely manage the events at home and at school. Interventions to reduce the effect of precipitating psychosocial stressors and the involvement of a mental health professional to treat comorbid psychopathology will also form part of an effective management plan. Outcome at follow-up is reported to be largely positive, although studies have not been able to follow all children, and few studies have focused on predictors of a good outcome. Future controlled intervention studies using a range of outcome measures are needed to identify efficacious approaches and predictors of best outcome.
Article
Information on the nature and relative frequency of diagnoses made in referrals to neurology outpatient clinics is an important guide to priorities in services, teaching and research. Previous studies of this topic have been limited by being of only single centres or lacking in detail. We aimed to describe the neurological diagnoses made in a large series of referrals to neurology outpatient clinics. Newly referred outpatients attending neurology clinics in all the NHS neurological centres in Scotland, UK were recruited over a period of 15 months. The assessing neurologists recorded the initial diagnosis they made. An additional rating of the degree to which the neurologist considered the patient's symptoms to be explained by disease was used to categorise those diagnoses that simply described a symptom such as 'fatigue'. Three thousand seven hundred and eighty-one patients participated (91% of those eligible). The commonest categories of diagnosis made were: headache (19%), functional and psychological symptoms (16%), epilepsy (14%), peripheral nerve disorders (11%), miscellaneous neurological disorders (10%), demyelination (7%), spinal disorders (6%), Parkinson's disease/movement disorders (6%), and syncope (4%). Detailed breakdowns of each category are provided. Headache, functional/psychological disorders and epilepsy are the most common diagnoses in new patient referral to neurological services. This information should be used to shape priorities for services, teaching and research.