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Functional Neurological Disorder
... 5,6 Diagnosis of an FND is not made by exclusion, but rather is based on a set of diagnostic criteria that include certain signs and symptoms. 7 Functional cognitive disorder (FCD), a type of FND, has previously been known by other names, including depressive pseudodementia, hysteria, dissociative state, psychological stress, brain fog, and disordered personality, which attests to the great heterogeneity of these disorders. 2 As occurs with other FNDs, in the case of FCD, a targeted interview may help to reveal inconsistencies in patient-reported cognitive complaints, 8 as well as to rule out the presence of an underlying psychiatric or medical condition that explains the symptoms. ...
... 25 Objective signs of cognitive involvement on tests assessing language, visuospatial skills, praxias, or visual agnosia constitute a warning sign. 7 Less frequently, patients display short-term memory alterations compared to patients with suspected neurodegenerative disease, and inconsistencies in divided and selective attention tasks. 11,26 When assessing these results, we must bear in mind that many patients with FCD, at the time of cognitive assessment, may show high levels of stress due to the assessment (alterations in selfmonitoring and high expectations), which may in turn interfere with test performance. ...
Resumen Introducción: Muchos pacientes con síntomas cognitivos, como aquellos con quejas subjetivas de memoria, pueden ser mejor clasificados en un trastorno cognitivo funcional. Desarrollo: A lo largo de esta revisión se exploran diversos aspectos del trastorno cognitivo funcional. Se describen las características clínicas que apoyan el diagnóstico, la necesidad de no considerarlo como un diagnóstico de exclusión, sino como una posibilidad que se sustenta en hallazgos positivos como la inconsistencia interna de los síntomas cognitivos. Se exponen los mecanismos que podrían explicar esta condición, los cuales incluyen las fallas en la metacognición, automonitoreo excesivo, procesamiento emocional, entre otras. Se explica la frecuente comorbilidad con otras condiciones, en particular con patologías psiquiátricas. Se describen circunstancias en donde se requieren apoyos diagnósticos y la valoración neuropsicológica. Se enfatiza en el pronóstico de esta condición, en donde, a pesar de llegar a ser incapacitante y acompañarse de angustia, muy pocos de los pacientes progresan a demencia, por lo que su correcta identificación permite diferenciarlos de aquellos con deterioro cognitivo leve, lo cual puede ayudar a evitar exámenes innecesarios y disminuye la incertidumbre para los pacientes. Finalmente, se explica como el tratamiento inicia desde el momento en que se comunica el diagnóstico al paciente, así como se puede apoyar en diversos enfoques de psicoterapia y reentrenamiento de la metacognición. Conclusiones: Los pacientes con trastorno cognitivo funcional dan cuenta de un porcentaje importante de las consultas por quejas de memoria y tienen necesidades particulares que pueden ser correctamente abordadas mediante criterios clínicos que permiten su identificación temprana y la selección de estrategias terapéuticas apropiadas. Palabras clave: demencia; deterioro subjetivo de memoria; trastornos cognitivos; trastorno cognitivo funcional; trastorno neurológico funcional.
... Aun cuando los datos de la anamnesis pueden ayudar a sospechar el diagnóstico de TNF, estos no son necesarios ni suficientes, por lo que el diagnóstico se debe basar principalmente en demostrar la inconsistencia interna para evitar errores y sobrediagnóstico de TNF (se puede ver los errores frecuentes en la tabla 1). Además, se debe tener cuidado en el diagnóstico de TNF cuando haya sospecha de condiciones neurológicas que puedan tener presentaciones inusuales y con variabilidad temporal, tales como la epilepsia del lóbulo frontal, encefalitis autoinmune, síndrome de persona rígida, demencia frontotem-poral, apraxia de las extremidades, marcha distónica, trastornos vestibulares, disquinesia paroxística y parálisis periódica (17,35). ...
... Los trastornos sensitivos son una manifestación subjetiva, por lo que pueden conllevar a errores diagnósticos, especialmente cuando se presentan aisladamente. Incluso, en algunos casos será prudente descartar compromiso a través de neuroimágenes o pruebas de electrodiagnóstico (35). Finalmente, se puede facilitar el diagnóstico cuando se presentan en conjunto con otros síntomas funcionales motores o de la marcha. ...
Introducción: los trastornos neurológicos funcionales son un motivo de consulta frecuente en la práctica clínica y una causa importante de discapacidad y costos.
Materiales y métodos: artículo de revisión narrativa que sintetiza las últimas dos décadas en investigación clínica en esta área.
Resultados: se describe un diverso grupo de síntomas motores, sensitivos, cognitivos, visuales y eventos paroxísticos en los que no hay lesión estructural del sistema nervioso, así como tampoco una enfermedad neurológica conocida. En su génesis interactúan factores biológicos, neuronales y ambientales con los mecanismos cognitivos, emocionales y conductuales que se originan en experiencias de vida adversas o aprendizaje desadaptativo.
Discusión: el diagnóstico de estas condiciones no se hace por exclusión, sino que se sustenta en un abordaje clínico basado en la presencia de datos a la anamnesis y signos positivos al examen físico. El manejo de estos va desde la primera consulta, la comunicación del diagnóstico, el establecimiento de confianza con el paciente y el uso de recursos terapéuticos como la psicoterapia, la terapia física y la ocupacional.
Conclusiones: a lo largo de esta revisión, se brindan las herramientas para entender los trastornos neurológicos funcionales, desde su fisiopatología y hasta el diagnóstico y el tratamiento.
... 5,6 Diagnosis of an FND is not made by exclusion, but rather is based on a set of diagnostic criteria that include certain signs and symptoms. 7 Functional cognitive disorder (FCD), a type of FND, has previously been known by other names, including depressive pseudodementia, hysteria, dissociative state, psychological stress, brain fog, and disordered personality, which attests to the great heterogeneity of these disorders. 2 As occurs with other FNDs, in the case of FCD, a targeted interview may help to reveal inconsistencies in patient-reported cognitive complaints, 8 as well as to rule out the presence of an underlying psychiatric or medical condition that explains the symptoms. ...
... 25 Objective signs of cognitive involvement on tests assessing language, visuospatial skills, praxias, or visual agnosia constitute a warning sign. 7 Less frequently, patients display short-term memory alterations compared to patients with suspected neurodegenerative disease, and inconsistencies in divided and selective attention tasks. 11,26 When assessing these results, we must bear in mind that many patients with FCD, at the time of cognitive assessment, may show high levels of stress due to the assessment (alterations in selfmonitoring and high expectations), which may in turn interfere with test performance. ...
... Figure 1 shows a simplified breakdown of FND subtypes, while Table 1 provides a list of additional readings related to FNDs, FS, FCD, and FMD. The modern day diagnosis of FND subtypes involves the identification of positive signs, which are clinical features that provide evidence for a rule-in determination (Bennett et al., 2021;Finkelstein & Popkirov, 2023;Silverberg & Rush, 2023). For example, FCD is diagnosed based in large part on the presence of internal inconsistency, in which the individual shows observable deficits within a cognitive domain in some situations but not in others (not due to the attentional fluctuations seen in delirium or Lewy body disease; Ball et al., 2020;Cabreira, Frostholm, et al., 2023;McWhirter et al., 2020). ...
... It is likely that the disorder's semiology places many of these patients on a different care path than patients with FS or FCD, and cognitive assessment may not be as well integrated into these movement-specific settings. However, results reviewed above about the shared pathophysiology, mechanisms, and symptom expressions across FNDs (Finkelstein & Popkirov, 2023;Forejtová et al., 2023;Hallett et al., 2022;Hopp et al., 2012;Lidstone, Araújo, et al., 2020;Tinazzi et al., 2021), together with some specific evidence for cognitive problems in FMDs (Alluri et al., 2020;Butler et al., 2021;Věchetová et al., 2018), argue for increased involvement of neuropsychologists in this FND subtype. ...
... On one hand, there is accumulating evidence for shared pathophysiology and underlying cognitive deficits across FS, FCD, and FMD (Hallett et al., 2022;Teodoro et al., 2018), arguing for a so-called "lumping" approach. On the other hand, there is mixed evidence with respect to individual phenotypes, with some data suggesting transdiagnostic presentations (Finkelstein & Popkirov, 2023;Forejtová et al., 2023), and other literature highlighting unique symptom expressions (Kola & LaFaver, 2022;Matin et al., 2017), possibly supporting "splitting." Currently, many researchers strike a balance, with some attention spent on overlapping characteristics of all FNSD (e.g., functional connectivity abnormalities; Drane et al., 2021;Pick et al., 2019) and additional complementary investigations highlighting nonshared symptoms and treatment approaches (e.g., response to interventions; Goldstein et al., 2020;Poole et al., 2023). ...
Objective: Functional neurological symptom disorder (FNSD) is a neuropsychiatric condition characterized by signs/symptoms associated with brain network dysfunction. FNSDs are common and are associated with high healthcare costs. FNSDs are relevant to neuropsychologists, as they frequently present with chronic neuropsychiatric symptoms, subjective cognitive concerns, and/or low neuropsychological test scores, with associated disability and reduced quality of life. However, neuropsychologists in some settings are not involved in care of patients with FNSDs. This review summarizes relevant FNSD literature with a focus on the role of neuropsychologists.
Methods: A brief review of the literature is provided with respect to epidemiology, public health impact, symptomatology, pathophysiology, and treatment.
Results: Two primary areas of focus for this review are the following: (1) increasing neuropsychologists' training in FNSDs, and (2) increasing neuropsychologists' role in assessment and treatment of FNSD patients.
Conclusions: Patients with FNSD would benefit from increased involvement of neuropsychologists in their care.
... Only half of discharge diagnoses were based on in-person semiological assessment, half of which had ictal video-EEG recording, so an increased risk of misdiagnosis has to be considered in the remaining cases. 39 Regarding the specificity of semiological diagnosis without ictal EEG, a study of smartphone video assessment showed that the chance of misidentifying epileptic convulsions as a dissociative seizure was just 1.6%. 40 The risk of misdiagnosing dissociative seizures based on detailed history and interictal EEG alone was less than 5% in a cohort study of 190 patients. ...
Background
Dissociative seizures, also known as functional or psychogenic non-epileptic seizures, account for 11%–27% of all emergency seizure presentations. Misdiagnosis as epileptic seizures is common and leads to ineffective and potentially harmful treatment escalations. We assess the potential for diagnostic improvement at different stages of emergency workup and estimate the utility of benzodiazepines.
Methods
A retrospective study of all emergency presentations with a discharge diagnosis of acute dissociative seizures seen at a university hospital 2010–2022 was performed to assess clinical characteristics and emergency decision-making.
Results
Among 156 patients (73% female, median 29 years), 15% presented more than once for a total of 203 presentations. Half of seizures were ongoing at first medical contact; prolonged seizures and clusters were common (23% and 24%). Diagnostic accuracy differed between on-site emergency physicians and emergency department neurologists (12% vs 52%). Typical features such as eye closure, discontinuous course and asynchronous movements were common. Benzodiazepines were given in two-thirds of ongoing seizures, often in high doses and preferentially for major hyperkinetic semiology. Clinical response to benzodiazepines was mixed, with a minority of patients remaining either unaffected (16%) or becoming critically sedated (13%). A quarter of patients given benzodiazepines by emergency medical services were admitted to a monitoring unit, 9% were intubated.
Conclusions
Improved semiological assessment could reduce early misdiagnosis of dissociative seizures. Although some seizures seem to respond to benzodiazepines, critical sedation is common, and further studies are needed to assess the therapeutic ratio.
Purpose
Time pressure in the treatment of acute ischemic stroke patients generates difficult decisions for neurologists, sometimes resulting in stroke mimic patients receiving intravenous thrombolytic therapy. Proper diagnosis and appropriate treatment for these patients are crucial, yet incredibly challenging in cases of functional stroke mimics (FSM).
Views
Functional neurological disorders, including FSM, are increasingly diagnosed. However, their pathophysiology and underlying mechanisms are poorly understood, even though the diagnostic criteria for diagnosing FSM exist, and various clinical indicators support them. Functional neurological symptoms often result in serious disability for patients but the effective treatment is unknown. Neurologists need to examine the symptoms and the key clinical findings to distinguish between FSM and stroke. The intravenous thrombolytic therapy appears safe for FSM, but the potential harm of thrombolysis should be a concern. FSMdiagnosed patients require long-term treatment, starting with open and clear communication about their condition and followed by psychoeducation and physiotherapy.
Conclusions
FSM should be diagnosed using the diagnostic criteria. Electronic health information exchange among healthcare providers is necessary to avoid unnecessary thrombolytic treatments in this group of patients.
Background
As a group, individuals with functional neurological disorder (FND) report an approximately 3-fold increase in adverse life experiences (ALEs) compared to healthy controls. In patients with FND, studies have identified a positive correlation between symptom severity and the magnitude of ALEs. While not all individuals with FND report ALEs, such findings raise the possibility of a trauma-subtype of FND.
Objective
This study investigated if patients with FND, with or without probable post-traumatic stress disorder (PTSD) and/or significant childhood maltreatment, differed in their symptom severity and physical health.
Materials and methods
Seventy-eight patients with FND were recruited (functional seizures, n = 34; functional movement disorder, n = 56). Participants completed self-report measures of symptom severity [Somatoform Dissociation Questionniare-20 (SDQ-20), Screening for Somatoform Disorders: Conversion Disorder subscale (SOMS:CD), Patient Health Questionniare-15 (PHQ-15)], physical health [Short Form Health Survey-36 (SF36-physical health)], childhood maltreatment [Childhood Trauma Questionnaire (CTQ)], and PTSD [PTSD Checklist-5 (PCL-5)]; a psychometric battery of other common predisposing vulnerabilities was also completed. To adjust for multiple comparisons, a Bonferroni correction was applied to all univariate analyses.
Results
Patients with FND and probable PTSD (n = 33) vs. those without probable PTSD (n = 43) had statistically significant increased scores on all symptom severity measures – as well as decreased physical health scores. In secondary post-hoc regression analyses, these findings remained significant adjusting for age, sex, race, college education, and: pathological dissociation; alexithymia; attachment styles; personality characteristics; resilience scores; functional seizures subtype; or moderate-to-severe childhood abuse and neglect scores; SOMS:CD and SDQ-20 findings also held adjusting for depression and anxiety scores. In a separate set of analyses, patients with FND and moderate-to-severe childhood abuse (n = 46) vs. those without moderate-to-severe childhood abuse (n = 32) showed statistically significant increased SDQ-20 and PHQ-15 scores; in post-hoc regressions, these findings held adjusting for demographic and other variables. Stratification by childhood neglect did not relate to symptom severity or physical health scores.
Conclusion
This study provides support for a possible trauma-subtype of FND. Future research should investigate the neurobiological and treatment relevance of a FND trauma-subtype, as well as continuing to delineate clinical characteristics and mechanisms in individuals with FND that lack a history of ALEs.
Objective
To determine the epidemiology of prolonged psychogenic non-epileptic seizures (pPNES) misdiagnosed as status epilepticus, as well as the risks associated with non-indicated treatment.
Methods
We performed an individual patient data analysis from the Rapid Anticonvulsant Medication Prior to Arrival Trial (RAMPART) and the Established Status Epilepticus Treatment Trial (ESETT) to assess incidence, patient characteristics and clinical course of misdiagnosed pPNES.
Results
Among 980 patients aged 8 years or older diagnosed and treated for status epilepticus in RAMPART and ESETT, 79 (8.1%) were discharged with a final diagnosis of pPNES. The relative incidence was highest in adolescents and young adults (20.1%). The typical female preponderance seen in that age bracket was not evident in children and older adults. Adverse effects, including respiratory depression and intubation, were documented in 26% of patients with pPNES receiving benzodiazepines in RAMPART and 33% of patients receiving additional second-line medication in ESETT. In ESETT, patients who were treated with benzodiazepines before hospital admission had higher rates of unresponsiveness and severe adverse effects than those treated after admission, suggesting cumulative effects of accelerated treatment momentum. Across trials, one in five patients with pPNES were admitted to an intensive care unit.
Conclusions
Misdiagnosis and treatment of pPNES as status epilepticus are a common and widespread problem with deleterious consequences. Mitigating it will require training of emergency staff in semiological diagnosis. Status epilepticus response protocols should incorporate appropriate diagnostic re-evaluations at each step of treatment escalation, especially in clinical trials.
Significance
In this manuscript, we report a rare case of a patient with localized seizures originating from the right anterior and dorsal posteromedial cortex (PMC). We mapped the electrophysiological and neuroimaging connectivity of the ictal onset site and replicated seizure auras by stimulating the homotopical PMC site in the left hemisphere. Our findings provide a causal link between PMC and the sense of self and provide unique clues about the pathophysiology of self-dissociation in neuropsychiatric conditions.
Functional motor disorders (FMD) are common and disabling. They are known to affect predominantly women and to start at young or middle age but to date, large case series are lacking, and demographic and clinical characteristics of patients with FMD rely on data from small cohorts. The aim of the current study was to describe demographic and clinical characteristics of these patients.
Methods: We prospectively collected data from FMD patients who were referred to the Neurophysiology Department of the Pitie-Salpetriere University Hospital between 2008 and 2016 for treatment with repeated transcranial magnetic stimulation.
Results: 482 patients were included. There was a majority of women (73.7%) with a median age of 40 years old at TMS treatment. Median age at the onset of the symptoms was 35.5 years old and symptoms were mostly characterized by an acute (47.3%) or subacute (46%) onset. Only 23% of patients were active workers while 58.3% were unemployed for medical reasons. Half of the patients referred for TMS treatment suffered from functional motor weakness (n= 241) and the other half suffered from movement disorders (n=241), mainly represented by tremor (21.15%) and dystonia (20.5%). Regarding psychiatric comorbidities, 33.6% had no psychiatric comorbidity and 17.4% reported no history of trauma. No significant differences were found according to clinical phenotypes.
Conclusion: We present the largest cohort of patients with FMD published to date. This cohort will contribute to a better understanding of FMD risk factors.
Objective
1) to describe the clinical manifestations of functional motor disorders (FMDs) coexisting with other neurological diseases (“comorbid‐FMDs”); 2) to compare comorbid‐FMDs to FMDs not overlapping with other neurological diseases (“pure FMDs”).
Methods
For this multicenter observational study, we enrolled outpatients with a definite diagnosis of FMDs attending 25 tertiary movement disorders centers in Italy. Each subject with FMDs underwent a detailed clinical assessment including screening for other associated neurological conditions. Groups comparisons (comorbid‐FMDs versus pure‐FMDs) were performed in order to compare demographical and clinical variables. Logistic regression models were created to estimate adjusted odds ratio (OR; 95% confidence interval) of comorbid‐FMDs (dependent variable) in relation to sociodemographic and clinical characteristics (independent variables).
Results
Out of 410 FMDs, 21.7% (n=89) of patients had comorbid‐FMDs. The most frequent coexisting neurological diseases were migraine, cerebrovascular disease and parkinsonism. In the majority of cases (86.5%) FMDs appeared after the diagnosis of neurological disease. Patients with comorbid‐FMDs were older, had more frequent tremor, non‐neurological comorbidities, paroxysmal non‐epileptic seizures, major depressive disorders, and benzodiazepine intake. Multivariate regression analysis showed that diagnosis of comorbid‐FMDs was more likely associated with longer time lag to reach the final diagnosis of FMDs, presence of tremor and non‐neurological comorbidities.
Conclusions
Our findings highlight the need of a prompt diagnosis of FMDs, given their relatively high frequency of associated neurological and non‐neurological diseases.
Objective
Hyperkinetic epileptic seizures (HKS) are difficult to characterize and localize according to semiologic features. We propose a multicriteria scale to help visual analysis and report results of cerebral localization.
Methods
We assessed seizures from 37 patients with HKS, explored with stereoelectroencephalography during presurgical evaluation. We used a multicriteria scale (hyperkinetic seizure scale [HSS]) with 10 semiologic features, scored independently by two neurologists. The item scores were used to group seizures using the k‐means method. Semiologic features were correlated with the seizure onset zone (SOZ) localization (temporal, prefrontal dorsolateral, prefrontal ventromesial, parietal, insular).
Results
Fifty‐five seizures were analyzed, and each item of the HSS was compared between the two examiners with good interrater agreement (85.3%). Dystonia, integrated behavior, and bilateral or unilateral hyperkinetic movements were statistically significant according to localization. Three clusters were identified according to the HSS and correlated with different patterns of anatomic localization of SOZ. Cluster 1 was characterized clinically by asymmetric hyperkinetic movements associated with marked dystonia and vocalization. It mainly included parietal seizures. Cluster 2 was characterized by bilateral and symmetrical stereotyped hyperkinetic movements without dystonia. It represented half of temporal seizures and one‐third of prefrontal seizures (dorsolateral). Cluster 3 was characterized by seizures with strong emotionality and vocalization with bilateral and symmetrical hyperkinetic movements and integrated behavior. It involved half of temporal seizures and a majority of prefrontal (ventromesial) seizures.
Significance
We propose a first attempt to quantify clinical patterns of HKS. The HSS may help to predict SOZ localization according to three main groups of hyperkinetic seizures.
Functional gait disorders are common in clinical practice. They are also usually disabling for affected individuals. The diagnosis is challenging because no single walking pattern is pathognomonic for a functional gait disorder. Establishing a diagnosis is based not primarily on excluding organic gait disorders but instead predominantly on recognizing positive clinical features of functional gait disorders, such as an antalgic, a buckling, or a waddling gait. However, these features can resemble and overlap with organic gait disorders. It is therefore necessary to also look for inconsistency (variations in clinical presentation that cannot be reconciled with an organic lesion) and incongruity (combination of symptoms and signs that is not seen with organic lesions). Yet, these features also have potential pitfalls as inconsistency can occur in patients with dystonic gait or those with freezing of gait. Similarly, patients with dystonia or chorea can present with bizarre gait patterns that may falsely be interpreted as incongruity. A further complicating factor is that functional and organic gait disorders may coexist within the same patient. To improve the diagnostic process, we present a sign-based approach—supported by videos—that incorporates the diverse clinical spectrum of functional gait disorders. We identify 7 groups of supportive gait signs that can signal the presence of functional gait disorders. For each group of signs, we highlight how specific clinical tests can bring out the inconsistencies and incongruencies that further point to a functional gait disorder.
Importance
Misdiagnosis of epilepsy is common. Video electroencephalogram provides a definitive diagnosis but is impractical for many patients referred for evaluation of epilepsy.
Objective
To evaluate the accuracy of outpatient smartphone videos in epilepsy.
Design, Setting, and Participants
This prospective, masked, diagnostic accuracy study (the OSmartViE study) took place between August 31, 2015, and August 31, 2018, at 8 academic epilepsy centers in the United States and included a convenience sample of 44 nonconsecutive outpatients who volunteered a smartphone video during evaluation and subsequently underwent video electroencephalogram monitoring. Three epileptologists uploaded videos for physicians from the 8 epilepsy centers to review.
Main Outcomes and Measures
Measures of performance (accuracy, sensitivity, specificity, positive predictive value, and negative predictive value) for smartphone video–based diagnosis by experts and trainees (the index test) were compared with those for history and physical examination and video electroencephalogram monitoring (the reference standard).
Results
Forty-four eligible epilepsy clinic outpatients (31 women [70.5%]; mean [range] age, 45.1 [20-82] years) submitted smartphone videos (530 total physician reviews). Final video electroencephalogram diagnoses included 11 epileptic seizures, 30 psychogenic nonepileptic attacks, and 3 physiologic nonepileptic events. Expert interpretation of a smartphone video was accurate in predicting a video electroencephalogram monitoring diagnosis of epileptic seizures 89.1% (95% CI, 84.2%-92.9%) of the time, with a specificity of 93.3% (95% CI, 88.3%-96.6%). Resident responses were less accurate for all metrics involving epileptic seizures and psychogenic nonepileptic attacks, despite greater confidence. Motor signs during events increased accuracy. One-fourth of the smartphone videos were correctly diagnosed by 100% of the reviewing physicians, composed solely of psychogenic attacks. When histories and physical examination results were combined with smartphone videos, correct diagnoses rose from 78.6% to 95.2%. The odds of receiving a correct diagnosis were 5.45 times greater using smartphone video alongside patient history and physical examination results than with history and physical examination alone (95% CI, 1.01-54.3; P = .02).
Conclusions and Relevance
Outpatient smartphone video review by experts has predictive and additive value for diagnosing epileptic seizures. Smartphone videos may reliably aid psychogenic nonepileptic attacks diagnosis for some people.
Psychogenic non‐epileptic seizures (PNES), also known as dissociative seizures, are paroxysms of altered subjective experience, involuntary movements and reduced self‐control that can resemble epileptic seizures, but have distinct clinical characteristics and a complex neuropsychiatric aetiology. They are common, accounting for over 10% of seizure emergencies and around 30% of cases in tertiary epilepsy units, but the diagnosis is often missed or delayed. The recently proposed “integrative cognitive model” accommodates current research on experiential, psychological and biological risk factors for the development of PNES, but in view of the considerable heterogeneity of presentations and medical context, it is not certain that a universal model can capture the full range of PNES manifestations. This narrative review addresses key learning objectives of the ILAE curriculum by describing the demographic profile, common risk factors (such as trauma or acute stress) and comorbid disorders (such as other dissociative and functional disorders, post‐traumatic stress disorder, depressive and anxiety disorders, personality disorders, comorbid epilepsy, head injury, cognitive and sleep problems, migraine, pain, and asthma). The clinical implications of demographic and aetiological factors for diagnosis and treatment planning are addressed.
Objective:
Depersonalization refers to the sensation of being detached from one's body, often associated with feelings of loss of control over one's own body, actions, or thoughts. Derealization refers to the altered perception of one's surroundings that is experienced as unreal. Although usually reported by psychiatric patients suffering from depression or anxiety, single case reports and small case series have described depersonalization- and derealization-like symptoms in the context of epilepsy.
Methods:
We investigated the brain mechanisms of ictal depersonalization- and derealization like symptoms by analyzing clinical and neuropsychological data as well as the epileptogenic zone based on a multimodal approach in a group of patients reporting depersonalization- (n = 9) and derealization-like symptoms (n = 7), from a single presurgical epilepsy center with focal epilepsy. We compared them with a group of control patients with experiential phenomena due to temporal lobe epilepsy (n = 28).
Results:
We show that all patients with ictal depersonalization-like symptoms report altered self-identification with their body and mostly suffer from frontal lobe epilepsy with the epileptogenic zone in the dorsal premotor cortex, while patients with derealization-like symptoms suffer from temporal lobe epilepsy. This finding is supported by post-ictal neuropsychological deficits, showing that depersonalization-like symptoms were significantly more often associated with frontal lobe dysfunction as compared to the control patients and patients with derealization-like symptoms.
Conclusion:
We argue that depersonalization of epileptic origin constitutes a distinct disorder due to frontal lobe epilepsy. We discuss these findings with respect to earlier accounts of depersonalization and the recent concept of bodily self-consciousness.
Supplementary motor area, the posterior third of the medial aspect of superior frontal gyrus, is known to be a heterogeneous area in function. It is involved in self-initiated motor movements, planning and sequencing the motor action, response inhibition, and bimanual movements. Blood supply for supplementary motor area is mostly by callosomarginal branch of anterior cerebral artery. Stroke in anterior cerebral artery territory is relatively uncommon, moreover, isolated supplementary motor area stroke is a rare entity. Supplementary motor area stroke, as a syndrome, has variable symptoms consisting of impairment of volitional movements, hemineglect, dyspraxia of contralateral limbs, impaired muscle tone, mutism and contralateral weakness. As symptoms are sometimes ambivalent, patients may be misdiagnosed as functional disorder and lose the chance for immediate adequate treatments such as thrombolysis. We report a 59-year-old man with previous history for myocardial infarction, referred to emergency room with an acute dense right-side hemiplegia, positive Hoover sign, asymmetrical Babinski responses and intermittent ability to move his arm in some specific reflex actions despite plegia. Since brain computed tomography scan was unremarkable we could not be sure whether his symptoms were organic or functional until a diffusion weighted imaging of magnetic resonance imaging elucidated the situation. To our knowledge, there is only one case report in the literature prior to ours, presenting a supplementary motor area stroke patient, mimicking functional disorder. Therefore, we may claim our report to be the second reported case.
Background
Several studies have shown that when patients with functional neurological disorders are followed up, it is rare to find another neurological condition that better explains the initial symptoms in hindsight. No study has examined the reverse, studying patients with a range of neurological disease diagnoses with the aim of assessing how often a new diagnosis of functional disorder better explains the original symptoms.
Methods
A prospective multi-centre cohort study of 2637 new neurology outpatient referrals from primary care in Scotland. Neurologists provided initial diagnoses and a rating of the extent to which their symptoms were explained by an ‘organic’ neurological disease. Patients were followed up 19 months later with a questionnaire to their primary care physician asking about diagnostic change, and when indicated also by discussion with the original assessing neurologist and review of secondary care records.
Results
Valid responses were obtained for 2378 out of 2637 patients (90%) with symptoms ‘largely’ or ‘completely’ explained by organic disease at baseline. At follow-up, we found diagnostic errors in 48 patients. Of those, ten (0.4%) had a functional diagnosis and 38 patients (1.6%) had a different ‘organic’ diagnosis which better explained the original symptoms.
Conclusions
Patients diagnosed with neurological disease sometimes have a functional diagnosis at follow-up which, with hindsight, better explains the original symptoms. This occurs at a frequency similar to the misdiagnosis of ‘organic’ neurological disease as functional disorder. Misdiagnosis can harm patients in either direction, especially as we enter an era of evidence-based treatment for functional neurological disorders.
Importance
Functional neurological disorders (FND) are common sources of disability in medicine. Patients have often been misdiagnosed, correctly diagnosed after lengthy delays, and/or subjected to poorly delivered diagnoses that prevent diagnostic understanding and lead to inappropriate treatments, iatrogenic harm, unnecessary and costly evaluations, and poor outcomes.
Observations
Functional Neurological Symptom Disorder/Conversion Disorder was adopted by the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, replacing the term psychogenic with functional and removing the criterion of psychological stress as a prerequisite for FND. A diagnosis can now be made in an inclusionary manner by identifying neurological signs that are specific to FNDs without reliance on presence or absence of psychological stressors or suggestive historical clues. The new model highlights a wider range of past sensitizing events, such as physical trauma, medical illness, or physiological/psychophysiological events. In this model, strong ideas and expectations about these events correlate with abnormal predictions of sensory data and body-focused attention. Neurobiological abnormalities include hypoactivation of the supplementary motor area and relative disconnection with areas that select or inhibit movements and are associated with a sense of agency. Promising evidence has accumulated for the benefit of specific physical rehabilitation and psychological interventions alone or in combination, but clinical trial evidence remains limited.
Conclusions and Relevance
Functional neurological disorders are a neglected but potentially reversible source of disability. Further research is needed to determine the dose and duration of various interventions, the value of combination treatments and multidisciplinary therapy, and the therapeutic modality best suited for each patient.
Objective
To ascertain demographic and clinical features of Parkinson disease (PD) associated with functional neurological features.
Methods
A standardised form was used to extract data from electronic records of 53 PD patients with associated functional neurological disorders (PD-FND) across eight movement disorders centres in the USA, Canada and Europe. These subjects were matched for age, gender and disease duration to PD patients without functional features (PD-only). Logistic regression analysis was used to compare both groups after adjusting for clustering effect.
Results
Functional symptoms preceded or co-occurred with PD onset in 34% of cases, nearly always in the most affected body side. Compared with PD-only subjects, PD-FND were predominantly female (68%), had longer delay to PD diagnosis, greater prevalence of dyskinesia (42% vs 18%; P=0.023), worse depression and anxiety (P=0.033 and 0.025, respectively), higher levodopa-equivalent daily dose (972±701 vs 741±559 mg; P=0.029) and lower motor severity (P=0.019). These patients also exhibited greater healthcare resource utilisation, higher use of [(123)I]FP-CIT SPECT and were more likely to have had a pre-existing psychiatric disorder (P=0.008) and family history of PD (P=0.036).
Conclusions
A subtype of PD with functional neurological features is familial in one-fourth of cases and associated with more psychiatric than motor disability and greater use of diagnostic and healthcare resources than those without functional features. Functional manifestations may be prodromal to PD in one-third of patients.
Schizophrenia is a neurodevelopmental disorder and its course is said to have an onset much before the presentation with psychotic symptoms. Even though the concept of prodrome in schizophrenia has been accepted, there is still an existence of a diagnostic dilemma. Various imaging studies and biomarkers have also been studied for confirmation of this diagnosis. The critical period of intervention when identified clarifies the doubts about faster and better outcomes.
Objective:
Approximately 30% of individuals who initially present with stroke are found to be stroke mimics (SM), with functional/psychological SM (FSM) accounting for up to 6.4% of all stroke presentations. Middle Eastern countries may have higher rates of somatization of emotional distress. The aim of this study was to evaluate the incidence and characteristics of FSM at a large general hospital in the Middle East.
Methods:
All patients presenting with an initial diagnosis of stroke from June 2015 to September 2016 were eligible for this study. Clinical and sociodemographic data were obtained from the hospital's stroke database. All SM and strokes were diagnosed by Joint Commission International-certified stroke program neurologists. SM was defined as any discharge diagnosis (other than acute stroke) for symptoms that prompted initial admission for suspected stroke. FSM were compared with medical stroke mimics (MSM) and strokes (ischemic, hemorrhagic, and transient ischemic attacks).
Results:
A total of 1961 patients were identified; 161 FSM (8.2%), 390 MSM (19.9%), and 1410 strokes (71.9%) (985 ischemic strokes, 196 transient ischemic attacks, 229 intracerebral hemorrhages). Admission with FSM was related to patients' nationality, with the highest frequency in Arabic (15.6%) and African (16.8%) patients. FSM patients were younger, more often female, and had fewer cardiovascular risk factors except for smoking compared with the strokes. FSM patients presented with more left-sided weakness and had more magnetic resonance imagings than the stroke and MSM groups. A total of 9.9% of FSM patients received thrombolysis versus only 0.5% of the MSM and 16.4% of ischemic strokes.
Conclusions:
FSM frequencies varied by nationality, with Arab and African nationals being twice as prevalent. Stress, vulnerable status as expats, sociopolitical instability, and exposure to trauma are proposed as potential factors contributing to FSM.
The association between emotional stimuli and temporal lobe epilepsy (TLE) is largely unknown. Here, we report the case of a depressive, 50-year-old female complaining of episodes of a “spaced out” experience precipitated by emotional stimuli. Psychogenic non-epileptic seizures were suspected. However, video-EEG coupled with emotional stimuli-provoked procedures and MRI findings of amygdala enlargement, led to the diagnosis of left TLE. Accurate diagnosis and explanation improved her subjective depression and seizure frequency. This case demonstrated that emotional stimuli can provoke seizures in TLE and suggested the involvement of the enlarged amygdala and the modulation of emotion-related neural circuits.
Background
The long-term safety of deep brain stimulation (DBS) is an important issue because new applications are being investigated for a variety of disorders. Studying instances where DBS was inadvertently implanted in patients without a movement disorder may provide information about the safety of the therapy. We report the case of a patient with a psychogenic movement disorder treated with deep brain stimulation (DBS).
Case Description
The patient presented at our clinic after 5 years of chronic DBS of the subthalamic nucleus (STN) for presumed Parkinson's disease. A dopamine transporter (DAT) scan (DaTscan) showed normal DAT distribution in the striatum. A positron emission tomography (PET) scan showed no abnormal metabolic patterns. Further psychiatric and neurological evaluations revealed that the patient was suffering from a psychogenic movement disorder. The patient displayed no sign or symptom from the stimulation, and DBS did not lead to any benefits or side effects for this patient.
Conclusion
We argue that the absence of side effects, the normal DaTscan, and PET scan after 5 years of chronic stimulation illustrate the safety of DBS on neural tissue.
Purpose:
To determine the frequency of false positive diagnoses of epilepsy and to explore its imitators and consequences.
Method:
A systematic review of all published observational studies (to November 2015) was conducted to determine the proportion of false positive diagnoses of epilepsy. We included studies of people of all ages receiving a diagnosis of epilepsy. All observational study designs were included with the exception of case-reports and case series with fewer than 3 participants.
Results:
Data were available from 27 studies (31 reports), reporting considerably varied frequencies of false positive diagnoses. The frequency of false positive diagnosis range from 2% to 71%. The data also suggest that syncope and psychogenic non-epileptic paroxysmal events were the commonest imitators of epilepsy. Misdiagnosis led to mismanagement with anti-epileptic drugs (AEDs) and affected legal driving status and employment.
Conclusions:
False positive diagnosis of epilepsy is common, even though there is considerable heterogeneity across studies. All potential imitators should be considered and clinicians should be cautious introducing AEDs without a definite diagnosis given the risk of side effects, and the possible impact on legal driving status and employment.
Temporal lobe epilepsy (TLE) is the most common type of focal epilepsy. First descriptions of TLE date back in time and detailed portraits of epileptic seizures of temporal origin can be found in early medical reports as well as in the works of various artists and dramatists. Depending on the seizure onset zone, several subtypes of TLE have been identified, each one associated with peculiar ictal semiology. TLE can result from multiple etiological causes, ranging from genetic to lesional ones. While the diagnosis of TLE relies on detailed analysis of clinical as well as electroencephalographic (EEG) features, the lesions responsible for seizure generation can be highlighted by multiple brain imaging modalities or, in selected cases, by genetic investigations. TLE is the most common cause of refractory epilepsy and despite the great advances in diagnostic tools, no lesion is found in around one-third of patients. Surgical treatment is a safe and effective option, requiring presurgical investigations to accurately identify the seizure onset zone (SOZ). In selected cases, presurgical investigations need intracerebral investigations (such as stereoelectroencephalography) or dedicated metabolic imaging techniques (interictal PET and ictal SPECT) to correctly identify the brain structures to be removed.
Functional neurological disorder is common in neurological practice. A new approach to the positive diagnosis of this disorder focuses on recognisable patterns of genuinely experienced symptoms and signs that show variability within the same task and between different tasks over time. Psychological stressors are common risk factors for functional neurological disorder, but are often absent. Four entities—functional seizures, functional movement disorders, persistent perceptual postural dizziness, and functional cognitive disorder—show similarities in aetiology and pathophysiology and are variants of a disorder at the interface between neurology and psychiatry. All four entities have distinctive features and can be diagnosed with the support of clinical neurophysiological studies and other biomarkers. The pathophysiology of functional neurological disorder includes overactivity of the limbic system, the development of an internal symptom model as part of a predictive coding framework, and dysfunction of brain networks that gives movement the sense of voluntariness. Evidence supports tailored multidisciplinary treatment that can involve physical and psychological therapy approaches.
Functional gait disorder represents one of the commonly encountered subtypes of functional movement disorder, often seen by neurologists in general neurology and movement disorders clinics. This chapter will present a case vignette of a patient with a mixed functional neurological disorder (functional gait disorder plus functional speech disturbance) and then proceed to describe the main aspects of functional gait disorder, with specific emphasis on the diagnostic process, including history, clinical signs and differential diagnosis, with the aim of providing helpful clues to clinicians managing this condition. Moreover, the chapter will discuss classification schema for functional gait disorder, underlying pathophysiology, and management recommendations.KeywordsFunctional movement disorderFunctional neurological disorderFunctional gait
The clinical presentation of tics can be very variable, and the distinction between different etiologies, specifically how to diagnose a functional tic disorder, can be particularly challenging. Here we provide the current state of knowledge on functional tics, and present helpful clinical diagnostic aids. We then discuss the role of neurophysiology, risk factors, and comment on treatment approaches. We also provide an illustrative case accompanied by video documentation to further highlight some of the clinical characteristics that are compatible with the diagnosis of a functional tic disorder.KeywordsFunctional movement disorderFunctional neurological disorderTicsTreatment
Functional parkinsonism is defined by the presence of a combination of marked slowness of movement in the absence of progressive decrement and variable resistance against passive movement, that is not caused by neurodegeneration or specific dysfunction of the dopaminergic system. Functional tremor and functional gait impairment may also be present. Functional parkinsonism is estimated to represent between 3% and 6% of patients with functional movement disorder. Functional parkinsonism should be considered if a patient exhibits sudden onset of symptoms with maximal severity at onset, and if there is no improvement with dopaminergic therapy. Clinical signs suggesting a functional neurological origin include distractibility, tremor entrainment, oppositional stiffness without cogwheel rigidity, and bradykinesia without decreased amplitude. Complementary exams, such as a DaTscan, can be used as adjunctive tools in the evaluation of functional parkinsonism, and be useful in providing evidence either for or against a comorbid neurodegenerative parkinsonism (in the case of a positive or negative scan, respectively). Treatment of functional parkinsonism should involve a multidisciplinary team including neurologists and psychiatrists and may include TMS, physical therapy, and psychotherapy.KeywordsParkinson’s diseaseFunctional neurological disorderFunctional parkinsonismFunctional movement disorderFunctional overlay
Functional jerky movements (or functional myoclonus) are commonly seen in patients with functional movement disorder. Positive features both from history and clinical examination are important for the diagnosis. However, due to their heterogeneous and paroxysmal nature a home-made video-recording of the jerky movements can be essential to make the diagnose by a movement disorder specialist. Supportive clinical clues include abrupt symptom onset often triggered by a physical event, whereas entrainment and distractibility are supportive signs during physical examination. Localization of the jerky movements, especially proximal localization, is supportive, and can also be helpful in distinguishing functional jerks from tics and myoclonus, which are the most important differential diagnostic considerations. Additional neurophysiological tests include polymyography and electroencephalography-electromyography (EEG-EMG) co-registration in order to demonstrate a readiness potential (RP). Management includes disease education and specialized physical therapy.KeywordsFunctional movement disorderJerksMyoclonusTicsClinical neurophysiology
Functional neurological disorder (FND), previously regarded as a diagnosis of exclusion, is now a rule-in diagnosis with available treatments. This represents a major step toward destigmatizing the disorder, which was often doubted and deemed untreatable. FND is prevalent, generally affecting young and middle aged adults, and can cause severe disability in some individuals. An early diagnosis, with subsequent access to evidence based rehabilitative and/or psychological treatments, can promote recovery—albeit not all patients respond to currently available treatments. This review presents the latest advances in the use of validated rule-in examination signs to guide diagnosis, and the range of therapeutic approaches available to care for patients with FND. The article focuses on the two most frequently identified subtypes of FND: motor (weakness and/or movement disorders) and seizure type symptoms. Twenty two studies on motor and 27 studies on seizure type symptoms report high specificities of clinical signs (64-100%), and individual signs are reviewed. Rehabilitative interventions (physical and occupational therapy) are treatments of choice for functional motor symptoms, while psychotherapy is an emerging evidence based treatment across FND subtypes. The literature to date highlights heterogeneity in responses to treatment, underscoring that more research is needed to individualize treatments and develop novel interventions.
Background
Patients with functional neurological disorders (FND) often present with multiple motor, sensory, psychological and cognitive symptoms. In order to explore the relationship between these common symptoms, we performed a detailed clinical assessment of motor, non-motor symptoms, health-related quality of life (HRQoL) and disability in a large cohort of patients with motor FND. To understand the clinical heterogeneity, cluster analysis was used to search for subgroups within the cohort.
Methods
One hundred fifty-two patients with a clinically established diagnosis of motor FND were assessed for motor symptom severity using the Simplified Functional Movement Disorder Rating Scale (S-FMDRS), the number of different motor phenotypes (i.e. tremor, dystonia, gait disorder, myoclonus, and weakness), gait severity and postural instability. All patients then evaluated each motor symptom type severity on a Likert scale and completed questionnaires for depression, anxiety, pain, fatigue, cognitive complaints and HRQoL.
Results
Significant correlations were found among the self-reported and all objective motor symptoms severity measures. All self-reported measures including HRQoL correlated strongly with each other. S-FMDRS weakly correlated with HRQoL. Hierarchical cluster analysis supplemented with gap statistics revealed a homogenous patient sample which could not be separated into subgroups.
Conclusions
We interpret the lack of evidence of clusters along with a high degree of correlation between all self-reported and objective measures of motor or non-motor symptoms and HRQoL within current neurobiological models as evidence to support a unified pathophysiology of ‘functional’ symptoms. Our results support the unification of functional and somatic syndromes in classification schemes and for future mechanistic and therapeutic research.
The semiology of epileptic seizures reflects activation, or dysfunction, of areas of brain (often termed the symptomatogenic zone) as a seizure begins and evolves. Specific semiologies in focal epilepsies provide an insight into the location of the seizure onset zone, which is particularly important for presurgical epilepsy assessment. The correct diagnosis of paroxysmal events also depends on the clinician being familiar with the spectrum of semiologies. Here, we summarise the current literature on localisation in focal epilepsies using illustrative cases and discussing possible pitfalls in localisation.
Background:
Functional neurological disorder (FND) is common, and symptoms can be severe. There have been no international large-scale studies of patient experiences of FND.
Methods:
We created a patient questionnaire to assess FND patient characteristics, symptom comorbidities, and illness perceptions. Respondents were recruited internationally through an open access questionnaire via social media and patient groups over a month-long period.
Results:
In total, 1048 respondents from sixteen countries participated. Mean age was 42 years (86% female). Median FND symptom duration was five years, and median time from first symptom to diagnosis was two years. Mean number of current symptoms (core FND and associated) was 9.9. Many respondents had associated symptoms, for example fatigue (93%), memory difficulties (80%), and headache (70%). Self-reported psychiatric comorbidities were relatively common (depression (43%), anxiety (51%), panic (20%), and post-traumatic stress disorder (22%)). Most respondents reported that FND had multiple causes, including physical and psychological.
Conclusions:
This large survey adds further evidence that people with FND typically have high levels of multiple symptom comorbidity with resultant distress. It also supports the notion that associated physical symptoms are of particular clinical significance in FND patients. Dualistic ideas of FND were not supported by respondents, who generally preferred to conceptualise the disorder as one at the interface of mind and brain. We highlight the need for a broad approach to this poorly served patient group. Potential selection and response biases due to distribution of the survey online, mostly via FND patient groups, is a key limitation.
Background
Functional motor disorders (FMDs) are abnormal movements that are significantly altered by distractive maneuvers and are incongruent with movement disorders seeing in typical neurological diseases.
Objective
1) to describe the clinical manifestations of FMDs, including non‐motor symptoms and occurrence of other functional neurological disorders (FND);2) to report the frequency of isolated and combined FMDs and their relationship with demographic and clinical variables.
Methods
For this multicentre, observational study, we enrolled consecutive outpatients with a definite diagnosis of FMDs attending 25 tertiary movement disorders centres in Italy. Each subject underwent detailed clinical evaluation with definition of phenotype, number of FMD (isolated, combined) and assessment of associated neurological and psychiatric symptoms.
Results
Out of 410 FMDs (71% females; mean age 47±16.1 years) the most common phenotypes were weakness and tremor. People with FMDs had higher educational level than general population, and frequent non‐motor symptoms, especially anxiety, fatigue and pain. Almost half of FMDs patients had associated other FND, such as sensory symptoms, non‐epileptic seizures and visual symptoms. Subjects with combined FMDs showed a higher burden of non‐motor symptoms and more frequent other FND. Multivariate regression analysis showed that diagnosis of combined FMDs was more likely to be delivered by a movement disorders neurologist. Also, FMDs duration, pain, insomnia, a diagnosis of somatoform disease and treatment with antipsychotic were all significantly associated to combined FMDs.
Conclusions
Our findings highlight the need for multidimensional assessment in patients with FMDs, given the high frequency of non‐motor symptoms and other FND, especially in patients with combined FMDs.
Functional neurological (conversion) disorder (FND) is a prevalent and disabling condition at the intersection of neurology and psychiatry. Advances have been made in elucidating an emerging pathophysiology for motor FND, as well as in identifying evidenced-based physiotherapy and psychotherapy treatments. Despite these gains, important elements of the initial neuropsychiatric assessment of functional movement disorders (FND-movt) and functional limb weakness/paresis (FND-par) have yet to be established. This is an important gap from both diagnostic and treatment planning perspectives. In this article, the authors performed a narrative review to characterize clinically relevant variables across FND-movt and FND-par cohorts, including time course and symptom evolution, precipitating factors, medical and family histories, psychiatric comorbidities, psychosocial factors, physical examination signs, and adjunctive diagnostic tests. Thereafter, the authors propose a preliminary set of clinical content that should be assessed during early-phase patient encounters, in addition to identifying physical signs informing diagnosis and potential use of adjunctive tests for challenging cases. Although clinical history should not be used to make a FND diagnosis, characteristics such as acute onset, precipitating events (e.g., injury and surgery), and a waxing and waning course (including spontaneous remissions) are commonly reported. Active psychiatric symptoms (e.g., depression and anxiety) and ongoing psychosocial stressors also warrant evaluation. Positive physical examination signs (e.g., Hoover's sign and tremor entrainment) are key findings, as one of the DSM-5 diagnostic criteria. The neuropsychiatric assessment proposed emphasizes diagnosing FND by using "rule-in" physical signs while also considering psychiatric and psychosocial factors to aid in the development of a patient-centered treatment plan.
The American Neuropsychiatric Association's Committee on Research assigned the task of defining the most helpful clinical factors and tests in establishing the diagnosis of psychogenic nonepileptic seizures (PNES) during a neuropsychiatric assessment. A systematic review of the literature was conducted using three search engines and specified search terms for PNES and the predetermined clinical factors and diagnostic tests, followed by a selection process with specific criteria. Data extraction results from selected articles are presented for clinical factors (semiology, psychiatric comorbidities, medical comorbidities, psychological traits) and diagnostic tests (EEG, psychometric and neuropsychological measures, prolactin level, clinical neuroimaging, autonomic testing). Semiology with video EEG (vEEG) remains the most valuable tool to determine the diagnosis of PNES. With the exception of semiology, very few studies revealed the predictive value of a clinical factor for PNES, and such findings were isolated and not replicated in most cases. Induction techniques, especially when coupled with vEEG, can lead to a captured event, which then confirms the diagnosis. In the absence of a captured event, postevent prolactin level and personality assessment can support the diagnosis but need to be carefully contextualized with other clinical factors. A comprehensive clinical assessment in patients with suspected PNES can identify several clinical factors and may include a number of tests that can support the diagnosis of PNES. This is especially relevant when the gold standard of a captured event with typical semiology on vEEG cannot be obtained.
Functional neurologic disorder (FND), although neglected for much of the 20th century, is among the most common conditions encountered by neurologists across clinical settings. High prevalence rates and limited provider expertise in FND have created a considerable need to develop educational initiatives and practical suggestions to guide neurologists in training working with this population. To help avoid diagnostic errors, trainees should keep in mind that (1) marginally positive functional examination signs have low specificity; (2) FND can coexist with other neurologic comorbidities; and (3) bizarre, not previously encountered, neurologic presentations should not be mistakenly diagnosed as FND. Furthermore, trainees should be encouraged to longitudinally follow in their clinics a subset of patients with FND to develop the interview, diagnostic, and neuropsychiatric skills needed to effectively care for this population. As the landscape of neurologic care evolves, neurologists with expertise in FND should advise on shaping elements of the educational curriculum for neurology residents.
Objective:
To describe prevalence and relevance of Post-Traumatic Stress Disorder (PTSD) symptoms in Functional Neurological Symptom Disorder (FNSD) and explore differences in PTSD symptom scores between subgroups with Psychogenic Non-Epileptic Seizures (PNES) or other FNSD.
Methods:
This cross-sectional study evaluated data from 430 consecutive patients referred to a specialist psychotherapy service (69.3% female, 56% with PNES/44% with other FNSD). We analysed self-reported symptoms of Post-Traumatic Stress Disorder (PTSD Civilian Checklist, PCLC), depression (PHQ-9), anxiety (GAD-7), physical symptoms (PHQ-15), social functioning (WSAS), and health related quality of life (SF-36). Relationships between PTSD scores, diagnosis and other measures were examined. Independent associations of PTSD scores were identified using multilinear regression.
Results:
Symptom scores likely to indicate clinical PTSD were reported by 60.7% of patients with no difference between PNES and FNSD subgroups. Those potentially symptomatic of PTSD were less likely to be living with a partner OR 2.95 (95% CI 1.83-4.04), or to be in employment OR 2.23 (95% CI 1.46-3.41) than less symptomatic patients. There were higher levels of anxiety (r = 0.62), depression (r = 0.63) and somatic symptoms (r = 0.45) and lower quality of life scores (r = 0.48) in patients with high PTSD symptom scores (p < .0001 for all comparisons). Anxiety, depression and somatic symptoms made independent contributions to the variance of PTSD symptoms.
Conclusion:
There is a high prevalence of PTSD symptoms in patient with FNSD regardless of whether they have PNES. Trauma and PTSD symptoms are negatively correlated with quality of life. Self-report instruments for anxiety, depression and somatic symptoms may predict the presence of PTSD.
Objective:
Whereas functional symptoms are common in Parkinson's disease (PD), a parkinsonian syndrome may occasionally reflect a pure functional disorder (also named functional parkinsonism [FP]). This review aimed to decipher these entities to clarify the link between functional manifestations and PD.
Methods:
Following the PRISMA guidelines, the authors performed a systematic literature search of the PubMed and Science Direct databases for the period 1988 to December 2018 to identify studies of patients with either FP or PD associated with functional neurological symptoms.
Results:
From the 844 articles screened, 22 were retained, including 12 studies of functional neurological symptoms in PD and 16 studies of FP. The studies of functional symptoms in PD included 121 patients-57% were women, and the mean age was 61.3 years. Psychiatric history (mostly depression) and exposure to triggering stressors were frequent: 60% and 82.5%, respectively. The most common symptom was tremor (33.8%), most often located on the side most affected by PD (50%). Studies of FP included a total of 120 patients-62% were women, and the mean age was 50.7 years. The first FP symptoms appeared on average 5 years before diagnosis, with an abrupt onset in half the cases; 67.6% had a psychiatric history, and 46.8% were exposed to triggering stressors, such as physical injury, stress at work, or loss of family or friends.
Conclusions:
Findings suggest a possible relationship between PD and FP. Clinicians should keep in mind the possibility of functional symptoms in PD patients.
Comorbid epilepsy and psychogenic nonepileptic seizures (PNES) represent a serious challenge for the clinicians. However, the frequency, associations, and outcomes of dual diagnosis of epilepsy and PNES are unclear. The aim of the review was to determine the frequency, correlates, and outcomes of a dual diagnosis. A systematic review of all published observational studies (from inception to Dec. 2016) was conducted to determine the frequency, correlates, and outcomes of dual diagnosis. We included studies of individuals of any age reporting a dual diagnosis of epilepsy and PNES. All observational study designs were included with the exception of case reports and case series with fewer than 10 participants. The mean frequency of epilepsy in patients with PNES across all studies was 22% (95% confidence intervals [CI] 20 to 25%, range: 0% to 90%) while the mean frequency of PNES in patients with epilepsy was 12% (95% CI 10 to 14%, range: 1% to 62%). High heterogeneity means that these pooled estimates should be viewed with caution. A number of correlates of dual diagnosis were reported. Some studies delineated differences in semiology of seizures in patients with dual diagnosis vs. PNES or epilepsy only. However, most of the correlates were inconclusive. Only a few studies examined outcome in patients with dual diagnosis. Dual diagnosis is common in clinical practice, especially among patients referred to specialized services, and requires careful diagnosis and management.
Introduction:
Diagnosis of acute ischemic stroke is critical for acute intervention. Its diagnosis may be obscured in trauma patients due to confounding injuries. We report its incidence in trauma patients following their presentation at our institution.
Methods:
Electronic charts of all acute trauma patients presenting to a designated level 1 trauma center emergency department between September 2012-November 2015 were screened and included in the study if they had a discharge diagnosis of acute ischemic stroke. Patient data were reviewed to identify the presence of neurologic deficit on initial triage, imaging type obtained (intracranial or extracranial) and time to diagnosis of stroke.
Results:
Of 192 trauma patients screened, 11 were found to have acute ischemic stroke (5.7%). Patients were generally young (median age, 49 years) and predominantly males (n = 8). Presentation after vehicular crash was most frequent (n = 8 or 73%). Patients had predominantly skeletal injuries (n = 8 or 73%). Initial workup involved vascular imaging below the neck (n = 9), while only one had intracranial vascular imaging. When patients underwent cervicocranial vascular imaging, 64% (n = 7) had findings explaining the etiology of their stroke. None of the patients was diagnosed with acute ischemic stroke on admission. Its diagnosis was delayed by an average 1.8 days following presentation.
Conclusions:
Acute ischemic stroke in trauma patients was a frequent diagnosis albeit with delay. Routine craniocervical vascular imaging at the time of presentation could potentially facilitate early diagnosis. A prospective study with routine craniocervical vascular imaging in trauma patients will be needed to further explore this hypothesis.
Background:
Psychogenic nonepileptic seizures (PNESs) are episodes that resemble epileptic seizures but are of psychological origin. A few studies have attempted to describe different types of PNES as a combination of clinical signs but their validation and robustness have not yet been reached. The aim of this study was to assess the inter-rater reliability (IRR) of five existing clinical PNES classifications.
Methods:
A total of 107 PNESs from 54 patients were retrospectively analyzed independently by two trained epileptologists, who were blinded to each other's findings. The recorded events were grouped according to the five chosen classifications systems. The IRR was measured using a kappa (κ) coefficient for each PNES classification. We also report category-specific κ values.
Results:
Our study demonstrated a mild to moderate IRR (κ from 0.44-0.68) for classifying PNES using the 5 proposed classification schemes. Within these classifications, the most reproducible classes are the subjective ones followed by the dialeptic group. Classes based on motor signs are the least reproducible.
Conclusion:
The IRR for current clinical classifications of PNES was only moderate. The difficulty to analyze motor signs could explain this poor reliability. It is necessary to ensure the reliability of clinical classifications of PNES in order for them to be a relevant tool in clinical practice or to explore correlations in clinical research. Future research would benefit from increased precision of diagnostic criteria specific to each class.
Background:
The differential diagnosis of generalized tonic-clonic seizures (GTCS), psychogenic nonepileptic seizures (PNES), and syncope constitutes a major challenge. Misdiagnosis rates up to 20 to 30% are reported in the literature.
Purpose:
To assess the clinical utility of serum lactate levels for differentiation of GTCS, PNES, and syncope based on gender differences.
Methods:
Data from 270 patients were evaluated retrospectively. Only patients ≥18 years old with the final diagnosis of GTCS, PNES, or syncope in their chart were recruited. Serum lactate levels were measured in the first 2h of the index event.
Results:
Serum lactate levels in patients with GTCS (n=157) were significantly higher than in the patients with PNES (n=25) (p<0.001) and syncope (n=88) (p<0.001). When compared with the females, serum lactate levels in patients with GTCS were significantly higher in the male subgroup (p=0.004). In male patients the ROC analysis yielded a serum lactate value of 2.43mmol/l with a sensitivity of 0.85 and a specificity of 0.88 as the optimal cut-off value to distinguish GTCS from other events. The ROC analysis for the AUC yielded a high estimate of 0.94 (95% confidence interval: 0.91-0.98). When a cut-off value of 2.43mmol/l was chosen for the females, which was an optimal value for male patients, the specificity was 0.85, however, the sensitivity was 0.64.
Conclusion:
We propose that serum lactate level when measured in the first 2h after the index event has a high clinical utility in the differential diagnosis of GTCS, PNES, and syncope. With concomitant clinical signs and physical examination findings besides neuroimaging and EEG, elevated levels of lactate should be taken into account when evaluating a patient with impaired consciousness. On the other hand, the suggested cut-off value 2.43mmol/l might not have a discriminative effect between GTCS, PNES, and syncope in female patients. This finding should be verified in a prospectively designed study with a larger patient population.
Objective:
Adverse early-life events are predisposing factors for functional neurological disorder (FND) and post-traumatic stress disorder (PTSD). Cingulo-insular regions are implicated in the biology of both conditions and are sites of stress-mediated neuroplasticity. We hypothesised that functional neurological symptoms and the magnitude of childhood abuse would be associated with overlapping anterior cingulate cortex (ACC) and insular volumetric reductions, and that FND and PTSD symptoms would map onto distinct cingulo-insular areas.
Methods:
This within-group voxel-based morphometry study probes volumetric associations with self-report measures of functional neurological symptoms, adverse life events and PTSD symptoms in 23 mixed-gender FND patients. Separate secondary analyses were also performed in the subset of 18 women with FND to account for gender-specific effects.
Results:
Across the entire cohort, there were no statistically significant volumetric associations with self-report measures of functional neurological symptom severity or childhood abuse. In women with FND, however, parallel inverse associations were observed between left anterior insular volume and functional neurological symptoms as measured by the Patient Health Questionnaire-15 and the Screening for Somatoform Symptoms Conversion Disorder subscale. Similar inverse relationships were also appreciated between childhood abuse burden and left anterior insular volume. Across all subjects, PTSD symptom severity was inversely associated with dorsal ACC volume, and the magnitude of lifetime adverse events was inversely associated with left hippocampal volume.
Conclusions:
This study reveals distinct cingulo-insular alterations for FND and PTSD symptoms and may advance our understanding of FND. Potential biological convergence between stress-related neuroplasticity, functional neurological symptoms and reduced insular volume was identified.
Since it was originally described nearly 70 years ago, insular epilepsy has been increasingly recognized and may explain failures after apparently well-planned operations. We review the history of awareness of the phenomenon, techniques for its assessment, and its surgical management. Insular epilepsy can mimic features of frontal, parietal, or temporal seizures. It should be considered when a combination of somatosensory, visceral, and motor symptoms is observed early in a seizure. Extraoperative intracranial recordings are required to accurately diagnose insular seizures. Stereo–electroencephalography (EEG) or craniotomy with implantation of surface and depth electrodes have been used successfully to identify insular onset of seizures. Surgical resection of an insular focus may be performed with good success and acceptable risk.
We sought to investigate (1) differences in ictal duration between psychogenic nonepileptic seizures (PNES) and epileptic seizures (ES), (2) the odds of being PNES when seizures last ≥ 5 min, and (3) the value of ictal duration as a diagnostic test to differentiate PNES from ES. We retrospectively reviewed video-EEG recordings and tabulated ictal durations of all PNES and ES. We estimated the mean ictal durations of PNES and ES using linear mixed models. The odds of being PNES when seizures last ≥ 5 min were estimated using logistic regression. We used receiver operating characteristics (ROC) curves to study the overall diagnostic accuracy of ictal duration in differentiating PNES from ES. We studied 441 ES and 341 PNES recorded from 138 patients. The mean ictal duration of PNES (148.7 s, 95% CI: 115.2–191.8) was significantly longer (p < 0.001) than that of ES (47.7 s, 95% CI: 37.6–60.6). The odds of being PNES was about 24 times higher (Odds ratio: 23.8, 95% CI: 7.9–71.3) when the ictal duration was ≥ 5 min. The ROC curve yielded an area under the curve of 0.80 (95% CI 0.73–0.88). Youden's index identified 123.5 s as the optimal threshold to diagnose PNES with 65% sensitivity and 93% specificity. Our results indicate that ictal duration is a useful test to raise suspicion of PNES. When a seizure lasts ≥ 5 min, it is 24 times more likely to be PNES with the potential risk of misdiagnosis as status epilepticus.
Purpose:
The diagnostic classification of disorders of consciousness is often challenging, particularly the distinction between epileptic and non-epileptic seizures. The aim of the study was to examine serum lactate as a diagnostic marker of transient loss of consciousness.
Method:
Serum lactate levels in blood samples drawn within 2h of the event were compared retrospectively between patients with generalized tonic-clonic seizures (n=195) and patients with other seizures (syncopes [n=52], psychogenic non-epileptic seizures [n=17], and complex focal seizures [n=37]), respectively.
Results:
Serum lactate in patients with generalized tonic-clonic seizures was significantly (p<0.001, Mann-Whitney-U test) increased in comparison to other forms of seizure incidences. The area under the ROC-curve was 0.94 (95% CI 0.91-0.96). For a cut-off concentration of 2.45mmol/l, the sensitivity was 0.88 and the specificity 0.87.
Conclusions:
Serum lactate levels in the acute diagnosis were an excellent biomarker for the discrimination of generalized seizures from psychogenic non-epileptic and syncopal events, corroborating its importance for the standard work-up of acute disturbances of consciousness.
