Preventing the risk of iatrogenic harm when assessing and diagnosing functional neurological disorders and other functional somatic symptoms.

The present commentary raises some concerns about the risk of iatrogenic harm arising out of the diagnosis of functional neurologic and somatic disorders. These concerns are supported by evidence from the history of hysteria and findings from contemporary brain imaging. We discuss their implications for practice.

A window into the mind-brain-body interplay: Development of diagnostic, prognostic biomarkers, and rehabilitation strategies in functional motor disorders.

BACKGROUND AND AIMS: Functional motor disorders (FMD) present a prevalent, yet misunderstood spectrum of neurological conditions characterized by abnormal movements (i.e., functional limb weakness, tremor, dystonia, gait impairments), leading to substantial disability and diminished quality of life. Despite their high prevalence, FMD often face delayed diagnosis and inadequate treatment, resulting in significant social and economic burdens. The old concept of psychological factors as the primary cause (conversion disorder) has been abandoned due to the need for more evidence about their causal role. According to a predictive coding account, the emerging idea is that symptoms and disability may depend on dysfunctions of a specific neural system integrating interoception, exteroception, and motor control. Consequently, symptoms are construed as perceptions of the body's state. Besides the main pathophysiological features (abnormal attentional focus, beliefs/expectations, and sense of agency), the lived experience of symptoms and their resulting disability may depend on an altered integration at the neural level of interoception, exteroception, and motor control. METHODS AND MATERIALS: Our proposal aims to elucidate the pathophysiological mechanisms of FMD through a three-stage research approach. Initially, a large cohort study will collect behavioral, neurophysiological, and MRI biomarkers from patients with FMD and healthy controls, employing eXplainable Artificial Intelligence (XAI) to develop a diagnostic algorithm. Subsequently, validation will occur using patients with organic motor disorders. Finally, the algorithm's prognostic value will be explored post-rehabilitation in one subgroup of patients with FMD. RESULTS: Data collection for the present study started in May 2023, and by May 2025, data collection will conclude. DISCUSSION: Our approach seeks to enhance early diagnosis and prognostication, improve FMD management, and reduce associated disability and socio-economic costs by identifying disease-specific biomarkers. TRIAL REGISTRATION: This trial was registered in clinicaltrials.gov (NCT06328790).

[Separation/Conversion Disorders in Functional Coma With Pseudocataplexy: Report of One Case and Literature Review].

Separation/conversion disorders in functional coma with pseudocataplexy are rare.On December 9,2021,a young female patient with separation/conversion disorders was treated in the Department of Neurology in the First Affiliated Hospital of Shandong First Medical University.The main symptoms were episodic consciousness disorders,sudden fainting,and urinary incontinence.Complete laboratory tests and cranial magnetic resonance imaging showed no obvious abnormalities.Standard multi-channel sleep monitoring and multiple sleep latency tests were performed.The patient was unable to wake up during nap and underwent stimulation tests.There was no response to orbital pressure,loud calls,or tapping,while the α rhythm in all electroencephalogram leads and the increased muscular tone in the mandibular electromyography indicated a period of wakefulness.The results of 24-hour sleep monitoring suggested that the patient had sufficient sleep at night and thus was easy to wake up in the morning.The results of daytime unrestricted sleep and wake-up test showed that the patient took one nap in the morning and one nap in the afternoon.When the lead indicated the transition from N3 to N2 sleep,a wake-up test was performed on the patient.At this time,the patient reacted to the surrounding environment and answered questions correctly.Because the level of orexin in the cerebrospinal fluid was over 110 pg/mL,episodic sleep disorder was excluded and the case was diagnosed as functional coma accompanied by pseudocataplexy.The patient did not present obvious symptom remission after taking oral medication,and thus medication withdrawl was recommended.Meanwhile,the patient was introduced to adjust the daily routine and mood.The follow-up was conducted six months later,and the patient reported that she did not experience similar symptoms after adjusting lifestyle.Up to now,no similar symptoms have appeared in multiple follow-up visits for three years.Functional coma with pseudocataplexy is prone to misdiagnosis and needs to be distinguished from true coma and episodic sleep disorders.

'Positive' inter-ictal clinical signs of functional neurological disorders are found in patients with functional dissociative seizures.

BACKGROUND AND PURPOSE: Prior studies highlighted the high diagnostic specificity (ranging from 92% to 100%) of clinical signs observed in functional neurological disorders (FNDs). However, these signs are rarely looked for by epileptologists when trying to distinguish between functional dissociative seizure (FDS) and epileptic seizure. The aim of this study was to determine the prevalence of inter-ictal clinical signs of FND in a cohort of patients with probable FDS. The secondary objective was to compare the prevalence of inter-ictal FND clinical signs in FDS patients with age- and gender-matched epileptic patients without FDS. METHODS: Patients diagnosed with FDS seen at two tertiary care centres and epileptic outpatients were included in the study. Each patient underwent a physical examination, searching for inter-ictal clinical signs of FND. RESULTS: In the FDS group, 79% of patients presented at least one sign of FND, compared to 16.6% of patients with epilepsy (p < 0.001). Moreover, 66.6% of FDS patients presented three or more FND signs, whereas only 4.1% of epileptic patients did (p < 0.001). The median number of FND clinical signs in the FDS group was four (SD 1.7; 5.5). Using the threshold of three signs or more, the specificity of detecting three or more FND signs was 83.3%, with a sensitivity of 79.2%. CONCLUSION: Inter-ictal clinical signs of FND are present in patients with FDS and should be looked for during neurological examination.

Young people diagnosed with psychogenic nonepileptic seizures (PNES) years ago - How are they now?

BACKGROUND: Up to 30% of patients referred to epilepsy centres for drug-resistant epilepsy turn out to have psychogenic nonepileptic seizures (PNES). Patients with PNES are a very heterogeneous population with large differences in regard to underlying causes, seizures severity, and impact on quality of life. There is limited knowledge regarding the long-term seizure prognosis of youth with PNES and its influential factors. METHODS: We have performed a retrospective study on adolescents diagnosed with PNES who were receiving inpatient care at our hospital for 2-4 weeks in the period of 2012-2020. They all attended psychoeducational courses to educate them about PNES, coping with the seizures, and possible contributors to seizure susceptibility. There were 258 patients who fulfilled the inclusion criteria. We contacted them by text messages, through which they received brief information about the study and an invitation to participate. There were 62 patients (24 %) who agreed and participated in structured telephone interviews. We excluded 10 patients due to concomitant epilepsy. The mean age of the remaining 52 participants was 20.9 years (16-28 years), and 45 (87 %) were women. RESULTS: After a mean of 4.7 years (2-9 years) since discharge from our hospital, 28 patients (54 %) had been free of seizures in the last 6 months. There were 16 patients (31 %) who had better situations in regard to seizures but were not completely seizure free, while 8 patients (15 %) were either unchanged (3 patients) or worse (5 patients). There were 39 patients (75 %) who had received conversation therapy, and 37 patients (71 %) had been treated by a psychologist or psychiatrist. There were 10 patients (19 %) who had dropped out of school or work, and the percentage increased with age. There were 42 patients (80 %) who perceived their health as good or very good. CONCLUSION: Patients had a relatively favourable seizure prognosis as 54% were free of seizures and 31% had a better seizure situation, at the time of this study. However, the fact that 19% had dropped out of school or work was worrying. Young age and satisfaction with treatment were associated with being employed or receiving education. Satisfaction with perceived treatment was significantly associated with personal experience of good health. This emphasizes the importance of early diagnosis, adapted interventional measures, and long-term follow-up by healthcare for young people with PNES.

Design and validation of a diagnostic suspicion checklist to differentiate epileptic from psychogenic nonepileptic seizures (PNES-DSC).

OBJECTIVE: Psychogenic non-epileptic seizures (PNES) are complex clinical manifestations and misdiagnosis as status epilepticus remains high, entailing deleterious consequences for patients. Video-electroencephalography (vEEG) remains the gold-standard method for diagnosing PNES. However, time and economic constraints limit access to vEEG, and clinicians lack fast and reliable screening tools to assist in the differential diagnosis with epileptic seizures (ES). This study aimed to design and validate the PNES-DSC, a clinically based PNES diagnostic suspicion checklist with adequate sensitivity (Se) and specificity (Sp) to discriminate PNES from ES. METHODS: A cross-sectional study with 125 patients (n = 104 drug-resistant epilepsy; n = 21 PNES) admitted for a vEEG protocolised study of seizures. A preliminary PNES-DSC (16-item) was designed and used by expert raters blinded to the definitive diagnosis to evaluate the seizure video recordings for each patient. Cohen's kappa coefficient, leave-one-out cross-validation (LOOCV) and balance accuracy (BAC) comprised the main validation analysis. RESULTS: The final PNES-DSC is a 6-item checklist that requires only two to be present to confirm the suspicion of PNES. The LOOCV showed 71.4% BAC (Se = 45.2%; Sp = 97.6%) when the expert rater watched one seizure video recording and 83.4% BAC (Se = 69.6%; Sp = 97.2%) when the expert rater watched two seizure video recordings. CONCLUSION: The PNES-DSC is a straightforward checklist with adequate psychometric properties. With an integrative approach and appropriate patient history, the PNES-DSC can assist clinicians in expediting the final diagnosis of PNES when vEEG is limited. The PNES-DSC can also be used in the absence of patients, allowing clinicians to assess seizure recordings from smartphones.

Understanding Functional Neurological Disorder: Recent Insights and Diagnostic Challenges.

Functional neurological disorder (FND), formerly called conversion disorder, is a condition characterized by neurological symptoms that lack an identifiable organic purpose. These signs, which can consist of motor, sensory, or cognitive disturbances, are not deliberately produced and often vary in severity. Its diagnosis is predicated on clinical evaluation and the exclusion of other medical or psychiatric situations. Its treatment typically involves a multidisciplinary technique addressing each of the neurological symptoms and underlying psychological factors via a mixture of medical management, psychotherapy, and supportive interventions. Recent advances in neuroimaging and a deeper exploration of its epidemiology, pathophysiology, and clinical presentation have shed new light on this disorder. This paper synthesizes the current knowledge on FND, focusing on its epidemiology and underlying mechanisms, neuroimaging insights, and the differentiation of FND from feigning or malingering. This review highlights the phenotypic heterogeneity of FND and the diagnostic challenges it presents. It also discusses the significant role of neuroimaging in unraveling the complex neural underpinnings of FND and its potential in predicting treatment response. This paper underscores the importance of a nuanced understanding of FND in informing clinical practice and guiding future research. With advancements in neuroimaging techniques and growing recognition of the disorder's multifaceted nature, the paper suggests a promising trajectory toward more effective, personalized treatment strategies and a better overall understanding of the disorder.

Misdiagnosis of functional neurological symptom disorders in paediatrics: Narrative review and relevant case report.

Functional neurological symptom disorders (FNSD) pose a common challenge in clinical practice, particularly in pediatric cases where the clinical phenotypes can be intricate and easily confused with structural disturbances. The frequent coexistence of FNSDs with other medical disorders often results in misdiagnosis. In this review, we highlight the distinctions between FNSD and various psychiatric and neurological conditions. Contrary to the misconception that FNSD is a diagnosis of exclusion, we underscore its nature as a diagnosis of inclusion, contingent upon recognizing specific clinical features. However, our focus is on a critical learning point illustrated by the case of a 14-year-old male initially diagnosed with FNSD, but subsequently found to have a rare primary monogenic movement disorder (paroxysmal kinesigenic dyskinesia, PKD). The crucial takeaway from this case is the importance of avoiding an FNSD diagnosis based solely on psychiatric comorbidity and suppressible symptoms. Instead, clinicians should diligently assess for specific features indicative of FNSD, which were absent in this case. This emphasizes the importance of making a diagnosis of inclusion. Extended follow-up and clinical-oriented genetic testing might help identify comorbidities, prevent misdiagnosis, and guide interventions in complex cases, which cannot be simply classified as "functional" solely because other conditions can be excluded.

Understanding and Avoiding Mistakes in Diagnosing Children with Functional Neurological Symptom Disorders: A Review and Case Report: This article discusses Functional Neurological Symptom Disorders (FNSDs), focusing on misdiagnosis, differential diagnosis, and other diagnostic challenges, particularly in pediatric cases. FNSDs involve motor or sensory symptoms that are inconsistent over time and unexplained by neurological disease, often associated with psychosocial factors. The article highlights the complexity of distinguishing FNSDs from other neurological and psychiatric conditions, emphasizing the importance of careful evaluation. The authors review various conditions that can mimic FNSDs, such as epileptic seizures, syncope, and different motor disorders. They emphasize the need to consider psychiatric conditions in the differential diagnosis, including factitious disorders, and malingering. The article presents a case study of a 14-year-old with involuntary movements, initially diagnosed as having a Functional Movement Disorder. After careful evaluation, the patient was diagnosed with a genetic dystonia (PRRT2 mutation). The case shows the importance of not rely solely on psychological problems, bizarre presentations or suppressible symptoms when diagnosing FNSDs.

Which psychogenic nonepileptic seizure (PNES) patients are more likely to be treated with anti-seizure medications?

BACKGROUND: The average time for psychogenic nonepileptic seizures (PNES) diagnosis is about 7.5 years. Many patients receive inadequate treatment and sometimes even life-threatening treatments such as tracheal intubation during this time. PURPOSE: To determine the risk factors for misdiagnosis of PNES as Epilepsy. METHODS: The medical records of patients who underwent video-electroencephalogram (EEG) monitoring were reviewed retrospectively. Patients who had PNES without epileptic seizures (ES) were included in this study. Baseline personal and monitoring characteristics were collected. The patients were then divided into two groups based on their therapeutic status. Patients in the treatment group were again divided into two groups based on the number of anti-seizure medications (ASM) they were treated with. RESULTS: Fifty-seven patients diagnosed with PNES were included in this study. Thirty-seven patients were under treatment, and 20 patients were not under treatment at the time of monitoring. Motor seizures, abnormal interictal EEG patterns, and pathological brain imaging findings were more frequent among patients in the treatment group (p<0.05). Patients with motor seizures were more likely to be treated with multiple ASM than patients with only dialeptic nonmotor seizures (p<0.05). Lastly, patients in the treatment group were monitored longer and had fewer seizures during monitoring (p<0.05). CONCLUSION: PNES patients with abnormal EEG patterns and pathological brain imaging findings are more likely to be treated with ASM. The pure dialeptic nature of seizures is less likely to be misdiagnosed as ES. In addition, patients with such seizures are less likely to be treated with multiple treatment lines.

[Transdisciplinary treatment of functional movement disorders: integration instead of dissociation]. / Die transdisziplinäre Behandlung funktioneller Bewegungsstörungen: Integration statt Dissoziation.

Successful treatment of patients with functional motor disorders is integrative in several ways: the primary treatment goal is the (re)integration of sensorimotor, cognitive and social functioning. The prerequisites for this are an integrated biopsychosocial model of everyone involved as well as close transdisciplinary cooperation. Instead of a simple addition of treatment components, all care providers and patients act in concert.

[Conclusion instead of exclusion-The clinical diagnosis of functional movement disorders]. / Abschluss statt Ausschluss ­ die klinische Diagnosesicherung funktioneller Bewegungsstörungen.

Functional neurological movement disorders are common in neurological practice and lead to a high degree of impairment and chronification. Affected patients usually receive a diagnosis with considerable delay and often do not get disease-specific treatment. The reasons for this delay are related to extensive diagnostic measures to exclude other nonfunctional neurological diseases. As a consequence, functional movement disorders are typically communicated as diagnoses of exclusion, which makes it difficult for patients to understand and accept the diagnosis. This is particularly unfortunate, because in the majority of patients the diagnosis can be made with confidence based on clinical features, i.e., inconsistency and incongruence. The clarification of the symptoms and the resulting treatment options should be supplemented by patient-friendly explanations of the pathophysiological basis of the disease. In this way, patients are enabled to understand and accept the diagnosis. Moreover, it can put an end to the search for a diagnosis, which can sometimes take decades, and paves the way for treatment. Thus, the diagnosis by exclusion itself becomes the starting point for treatment and can itself have a therapeutic effect.

[Understanding and explaining functional movement disorders]. / Funktionelle Bewegungsstörungen verstehen und verständlich machen.

Functional movement disorders are not uncommon in neurological consultations, hospitals and emergency departments. Although the disorder can usually be recognized clinically, the communication of the diagnosis is often unsatisfactory. Those affected are indirectly accused of a lack of insight or openness but it is often the doctors who fail to formulate a coherent and comprehensible explanation of the underlying disorder. In this review an integrative model for the development of functional movement disorders is presented, which places the motor (and nonmotor) symptoms in a neuroscientific light. In addition, explanations and metaphors are presented that have proven helpful in conveying an understanding of the disorder.

[Conversion disorder, caring for body, mind]. / Le trouble de conversion, prendre soin du corps et de l'esprit.

Conversion disorder is a psychiatric disorder whose clinic and management lie at the crossroads between body and mind. It challenges healthcare professionals in terms of diagnosis, further investigation, referral and care. A number of questions arise, such as how caregivers perceive the relationship between body and mind, the place of the psychiatric hypothesis among the initial diagnostic hypotheses, and the temporality of care.

[The historical diagnosis of 'hysterical scoliosis']. / Die historische Differenzialdiagnose der 'hysterischen Skoliose'.

BACKGROUND: Until the beginning of the 20th century, 'hysterical scoliosis' could be considered an established diagnosis in Middle Europe's German-speaking countries. The responsible physicians claimed that certain cases of scoliosis were caused solely by psychological factors, and they did not distinguish between real scoliosis (e.g. with neuromuscular cause) and abnormal posture caused by psychological reasons. Instead, 'hysterical scoliosis' was obviously believed to be a real scoliosis caused by psychologically induced contractures. OBJECTIVE: This study critically discusses the historical diagnosis 'hysterical scoliosis' from today's point of view. MATERIALS AND METHODS: This work is based on historical research (including journals and literature) approximately from the time around 1900. Furthermore, it is also based on recently discovered original material. RESULTS: The original material is a correspondence between H. Schlesinger and G. Muskat, who published an article about 'hysterical scoliosis' in Schlesinger's journal. CONCLUSIONS: Obviously, the underlying medical concepts of 'hysterical scoliosis' were wrong and led to clear diagnostic problems and inaccuracies. 'Hysterical scoliosis' can be considered one of adolescent psychiatry's early fashionable illnesses around the year 1900.

Functional neurological disorder: A common reason for a neurology inpatient referral.

BACKGROUND AND PURPOSE: In 2021, the European Academy of Neurology's training requirements were updated to include functional neurological disorder (FND) as a core topic for the first time. To reinforce these changes, we aimed to understand the proportion of inpatients (in non-neurology settings) who are diagnosed with FND. METHODS: We prospectively collected data on diagnoses made after inpatient ward reviews from neurology trainees at three tertiary neurology centres in Scotland from April to September 2021. We assessed healthcare utilization data for patients with a diagnosis of FND, epilepsy and epileptic seizures, or a neuroinflammatory disorder over the preceding 12 months. RESULTS: There were 437 inpatient reviews for 424 patients by 13 trainees. The largest single diagnosis was FND (n = 80, 18%), followed by epilepsy (n = 64, 14%), primary headache disorder (n = 40, 9%) and neuroinflammatory disorders (n = 28, 6%). There was an uncertain diagnosis for 48 reviews (11%). Compared to patients with epilepsy or neuroinflammatory disorders, patients with FND had a similar number of admissions (2 vs. 2 vs. 1) and brain/spine imaging studies (2 vs. 1 vs. 2). CONCLUSIONS: In Scotland, FND was the most common diagnosis made after a request for an inpatient review by a neurologist from another department in the hospital. Patients with FND have similar health resource needs to those with other common neurological disorders when they present to hospitals with tertiary neurology centres. This data supports the inclusion of FND as a core curriculum topic in neurology training.

Consultation-Liaison Case Conference: A Case of Factitious Disorder Imposed on Self.

We present the case of a young woman with an extensive medical history that most notably includes over 60 emergency-room visits for unfounded respiratory distress that often prompted intubations. Each presentation displays elements of deceitfulness or inappropriate demands that align with factitious disorder imposed on self. Top experts in the Consultation-Liaison field provide guidance for this commonly encountered clinical case based on their experience and review of available literature. Key teaching topics include a review of risk factors for development of deceptive syndromes, distinguishing factitious disorder from malingering and conversion disorder, and the role of a consulting psychiatrist in such cases. Patients with factitious disorder often show signs of pathologic lying, obstinance, and erratic behavior. Such attributes frequently arouse negative countertransference in providers, causing frustration and dread with continuing care, rendering psychiatric involvement. We address the unique challenges in managing factitious disorder and how to effectively collaborate with an interdisciplinary inpatient team with these cases.

[Validated care programs for patients with functional neurological disorders]. / Modèles de soins validés pour les patients souffrant de troubles neurologiques fonctionnels.

Functional neurological disorder (FND) is a common cause of persistent and disabling neurological symptoms. Diagnostic delay may lead to no treatment, inappropriate treatment or even iatrogenic symptoms. Yet, several treatments significantly reduce physical symptoms and improve functioning in FND patients even though not all patients respond to the currently available treatments. This review aims to describe the range of evidence-based rehabilitative and/or psychological therapeutic approaches available for FND patients. The most effective treatments are multidisciplinary and coordinated; using an outpatient or inpatient setting. Building a network of FND-trained healthcare professionals around the patient is an essential aspect of optimal patient management. Indeed, a supportive environment coupled with a collaborative therapeutic relationship improves understanding of FND and appears to help patients engage in appropriate treatments. Patients need to be invested in their own care and have to understand that recovery may depend on their commitment. The conventional treatment combines psychoeducation, physical rehabilitation and psychotherapy (cognitive and behavioral therapy, hypnosis, psychodynamic interpersonal therapy). Early referral of patients to physical therapy is recommended; however, the optimal parameters of treatment, duration and intensity are unknown and seem to vary with the severity and chronicity of symptoms. The goal is to minimize self-awareness by diverting attention or by stimulating automatically generated movements with non-specific and gradual exercises. The use of compensatory technical aids should be avoided as much as possible. Psychotherapeutic management should encourage self-evaluation of cognitive distortions, emotional reactions and maladaptive behaviors while empowering the patient in managing symptoms. Symptom management can use anchoring strategies to fight against dissociation. The aim is to connect to the immediate environment and to enrich one's sensoriality. The psychological interventions should then be adapted to the individual psychopathology, cognitive style and personality functioning of each patient. There is currently no known curative pharmacological treatment for FND. The pharmacological approach rather consists of progressively discontinuing medication that was introduced by default and that could lead to undesirable side effects. Finally, neurostimulation (transcranial magnetic stimulation, transcranial direct current stimulation) can be effective on motor FND.