Functional neurological seizures and migraine: A systematic review and case series.

BACKGROUND: The seizure subtype of functional neurological disorder (FND-seizures) is a common neuropsychiatric condition manifesting with episodic epilepsy-like events. Despite the common belief that FND-seizures are precipitated by psychological stressors, neurological disorders may also be triggers. In 1890, Babinski described four cases of FND symptoms associated with migraine attacks. Despite the passing of more than 130 years since this first clinical observation, the relationship between FND-seizures and migraine is not fully elucidated. OBJECTIVES: (1) To complete a systematic review of the literature that investigated potential associations between FND-seizures and migraine and the response of FND seizures to treatment with migraine prophylactic medications (2). To undertake a retrospective study of patients with FND-seizures and migraine, including response to migraine prophylaxis. METHODS: (1) Using PRISMA methods, we completed a systematic review of EMBASE and Scopus databases from inception to March 31, 2021, for literature on FND-seizures and migraine. (2) Our multi-disciplinary team, including subspecialists in psychosomatic medicine, epilepsy, and headache disorders, reviewed consecutive patients diagnosed with FND-seizures and migraine to assess potential causal associations and responses to standard migraine prophylactic medications. RESULTS: (1) The search yielded seven studies from 126 screened manuscripts (N = 1,186 patients with FND-seizures; mean age 38.7 years; 72.6% female). They varied substantially in design, population, diagnostic measures, and outcomes. Nevertheless, all studies found associations between FND-seizures and migraine, which were stronger than those between epileptic seizures and migraine in comparative investigations, but provided limited information on treatment response. (2) In our case series, investigators reached unanimous consensus that migraine attacks triggered FND-seizures in 28/43 (65.1%) patients reviewed (mean age, 38.8 years; 74% female). In 19/26 (73%) patients with adequate follow-up data, treatment with migraine prophylactic medications alone (no behavioral interventions) concomitantly reduced FND-seizure and headache frequency by >50%. CONCLUSION: Our systematic review and case series indicate that migraine attacks may trigger FND-seizures, perhaps more often that currently understood, and suggest that migraine prophylaxis may reduce FND-seizure frequency in such cases. To validate these observations, fully powered prospective investigations are required.

Outcome in Pediatric Functional Tic Disorders Diagnosed During the COVID-19 Pandemic.

OBJECTIVES: The incidence of pediatric functional tics has surged during the COVID-19 pandemic with little known about prognosis. To address this knowledge gap, the investigators examined clinical courses of functional tics diagnosed during the pandemic and explored factors predicting prognosis. METHODS: Study personnel reviewed electronic medical records of 29 pediatric patients diagnosed as having functional tics between March 1, 2020, and December 31, 2021, and estimated Clinical Global Impression-Improvement (CGI-I) scores at follow-up encounters. Twenty patient-guardian dyads completed telephone interviews. Logistic regression models were used to identify possible predictors of clinical trajectories. RESULTS: Of the 29 patients, 21 (82%) reported at least some improvement since diagnosis at the time of the last follow-up, with a median CGI-I score of 2 (much improved). During the telephone interview, 11 of 20 patients noted ongoing interference from tics, and 16 of 20 agreed with the diagnosis of functional tics. Median time from symptom onset to diagnosis was 197 days, with most patients reporting at least a mild reduction of symptoms (CGI-I score <4) at a median of 21 days after diagnosis. At a median follow-up time of 198 days after diagnosis, patients reported significant but not complete improvement. Greater age and longer time to diagnosis decreased odds of improvement within 1 month of diagnosis. CONCLUSIONS: Most patients showed improvements in but not the resolution of functional tic symptoms after diagnosis. These data support the importance of early diagnosis for functional tics.

Pediatric Functional Neurologic Disorders.

Functional neurologic disorders are common in the pediatric population. Recently, there has been a renewed focus on functional neurologic disorders, leading to improvements in diagnosis and management. This review focuses on updates in clinical presentation, diagnosis, pathophysiology (including neuroimaging), and treatment of functional neurologic disorders in the pediatric population.

Use of virtual reality for targeted physical rehabilitation: Case report on managing functional motor disorder.

Virtual reality (VR) technology has seen increasing use in physical rehabilitation and in the management of acute and chronic pain. Functional movement disorders (FMDs) are a source of disability with no known association to neurologic pathology, and patients are generally offered multidisciplinary treatment approaches to improve functional movement. However, patients who are not compliant with rehabilitation may have persistent FMD and long-term disability. Given VR's use in physical rehabilitation, it may serve as a useful adjunct for the management of FMD. Utilizing an application called MovementTM to create a playlist of targeted applications for the restoration of motor function and balance, this case study presents the application of VR as a tool to engage patients in physical therapy for the management of FMD. The VR games were selected to encourage movement while customization of levels within the games facilitated achievement of physical therapy goals. Physical rehabilitation aided by VR, when used in collaboration with a multidisciplinary care team, may be used to facilitate recovery from FMD.

Functional tremor.

Functional tremor is the most common functional movement disorder. It can be diagnosed with clinically definite certainty at the bedside by ascertaining its inconsistent (distractibility, frequency variability) and incongruent features (entrainment, ballistic suppression), requiring no additional neurological investigations except, in selected cases, those serving to elevate the diagnostic category to laboratory supported using accelerometry and surface electromyography. In the background of excessive attention to the affected body part and abnormal beliefs and expectations, functional correlates include the impairment of emotion processing, sense of agency, and abnormal connectivity between limbic and motor regions. While the treatment options remain under-studied, promising interventions in physiotherapy, cognitive behavioral therapy, and other psychotherapies are under evaluation to assessing their efficacy in attenuating this important source of neurological disability. This article is part of the Special Issue "Tremor" edited by Daniel D. Truong, Mark Hallett, and Aasef Shaikh.

Functional movement disorder and functional seizures: What have we learned from different subtypes of functional neurological disorders?

The objective of this paper is to compare and contrast FMD and FS, and highlight important differences in etiology and the clinical approach towards these two entities. While patients with FMD often experience abnormal movements on a daily basis, FS is characterized by paroxysmal events. Both patient populations share psychiatric and environmental comorbidities, but patients with FS may have increased anxiety and neuroticism and a higher percentage of childhood trauma. Functional MRI scans have demonstrated impaired executive control over motor behavior in both groups. FMD responds well to multidisciplinary rehabilitation-oriented treatment, while psychotherapy remains the mainstay of treatment for FS. For practicing clinicians, recognizing commonalities and differences in patients with FMD and FS is important to develop the most appropriate treatment plan.

Alpha-delta sleep pattern in an acute functional neurological patient with no perception of sleep.

Functional neurological disorders are characterized by neurologic symptoms not consistent with a primary neurologic pathology. Although neurological disorders are commonly associated with poor sleep, alpha intrusion of slow-wave sleep is not described in cases of functional neurological disorder. We describe a case demonstrating an alpha-delta sleep pattern in a patient presenting with a functional neurological disorder and no perception of sleep. Although alpha-delta sleep is more commonly associated with fibromyalgia, this pattern may be a potential biomarker for the physiology of sleep misperception and potentially functional neurologic symptoms disorder. It is important to recognize this pattern via close sleep electroencephalogram or spectral analysis for patients with concerning clinical histories. CITATION: Christian F, Pollak A, Sullivan L. Alpha-delta sleep pattern in an acute functional neurological patient with no perception of sleep. J Clin Sleep Med. 2022;18(6):1711-1715.

[Augmented psychotherapy with rTMS in functional neurological disorder]. / Psychothérapie augmentée par rTMS pour les troubles neurologiques fonctionnels.

BACKGROUND: We report the observation of a 47-year-old woman with functional neurological disorder (tetraparesis, mixed tremors and non-epileptic seizures) treated with a protocol of augmented psychotherapy in combination with repeated transcranial magnetic stimulation (rTMS). INTERVENTION: We carried out a biofeedback psychotherapy protocol with rTMS (twenty sessions, two sessions per day for ten days; 1Hz, 150% of the motor threshold, twenty minute sessions, 300 pulses per session) in which the patient visualized the motor activity of her upper limbs during stimulation of the primary motor area (PMA). The evolution of neurological symptoms was assessed using the Medical Research Council Scale for Muscle Strength (MRC). RESULTS: Symptoms were improved between the 4th and 6th days of treatment (8th and 12th sessions) with first a relief of paresis, then a secondary cascade improvement of other functional symptoms. At two months the patient no longer presented any functional neurological symptoms. DISCUSSION & CONCLUSION: We propose several hypotheses concerning the effectiveness of this type of biofeedback protocol using rTMS. We also suggest that this type of protocol should be systematically associated with psychotherapeutic support on biographical elements for holistic management. This observation underlines the interest of potentiating cognitive-behavioral therapies using the principle of operant conditioning with the aid of brain stimulation in functional neurological disorders, and motivates the realization of future studies.

Functional vision disorders in adults: a paradigm and nomenclature shift for ophthalmology.

Vision loss with clinical findings that are incompatible with the symptoms and recognized neurological or ophthalmic conditions is a common presentation of patients to neurologists, ophthalmologists, and neuro-ophthalmologists. The accepted terminology to describe such patients has evolved over time, including functional visual disorder (FVD), non-organic vision loss, non-physiologic vision loss, functional vision loss, psychogenic, psychosomatic, and medically unexplained visual loss. Likewise, attitudes and recommended management options have changed over the years in the fields of psychiatry and neurology. FVD is a diagnosis of inclusion, and it is critical that the diagnosis be made and delivered efficiently and effectively to reduce patient and physician duress. We review the current Diagnostic and Statistical Manual (DSM V) terminology and the prior literature on FVD and describe how the approaches to diagnosis and management have changed. We provide recommendations on the appropriate techniques and diagnostic approach for patients with FVD. We also propose a protocol for consistent and standardized discussion with the patient of the diagnosis of FVD. We believe that the adoption of FVD as both a paradigm and nomenclature shift in ophthalmology will improve patient care.

Functional neurological disorder: Engaging patients in treatment.

Patients with a functional neurological disorder can be difficult to engage in treatment. The reasons for this are complex and may be related to physician, patient and health care system issues. Providers contribute to difficulties in treatment engagement by giving confusing explanations for the patient symptoms, stigmatizing patients, and not allowing patients time to voice their questions and concerns. Patient factors include a lack of engagement after an explanation of the diagnosis, resistance to treatment, family/work dynamics and prior negative experiences with the health care system. The scarcity of providers skilled in the treatment of functional neurological disorder is yet another hurdle. This article will define these barriers and discuss good clinical practices to help improve outcomes by tackling those challenges and discuss why for many patients an integrated care team approach is needed.

Increased Amygdala Activity Associated With Cognitive Reappraisal Strategy in Functional Neurologic Disorder.

Cognitive reappraisal is an emotion regulation strategy to reduce the impact of affective stimuli. This regulation could be incomplete in patients with functional neurologic disorder (FND) resulting in an overflowing emotional stimulation perpetuating symptoms in FND patients. Here we employed functional MRI to study cognitive reappraisal in FND. A total of 24 FND patients and 24 healthy controls employed cognitive reappraisal while seeing emotional visual stimuli in the scanner. The Symptom Checklist-90-R (SCL-90-R) was used to evaluate concomitant psychopathologies of the patients. During cognitive reappraisal of negative IAPS images FND patients show an increased activation of the right amygdala compared to normal controls. We found no evidence of downregulation in the amygdala during reappraisal neither in the patients nor in the control group. The valence and arousal ratings of the IAPS images were similar across groups. However, a subgroup of patients showed a significant higher account of extreme low ratings for arousal for negative images. These low ratings correlated inversely with the item "anxiety" of the SCL-90-R. The increased activation of the amygdala during cognitive reappraisal suggests altered processing of emotional stimuli in this region in FND patients.

Bickerstaff's Brainstem Encephalitis Suspected as Functional Neurologic Disorders.

Functional neurologic disorders feature nervous system symptoms that cannot be explained by a neurological disease or other medical condition. The patient described here was a 21-year-old Japanese woman who was initially diagnosed with a functional neurologic disorder based on numbness and weakness of the limbs with no abnormalities in routine examinations. Further detailed examinations revealed monocytes in cerebrospinal fluid (CSF), and electroencephalography revealed widespread, low-voltage, slow waves with concentrated spindle waves. Thus, encephalitis was suspected, and steroid pulse therapy was initiated. Her symptoms subsequently improved. Afterward, CSF analysis was positive for serum anti-GQ1b IgG antibodies. We made a final diagnosis of Bickerstaff's brainstem encephalitis (BBE). Our report describes the difficult differentiation of functional neurologic disorders from BBE. Physicians and psychiatrists should be aware of BBE.

The feasibility of a multidisciplinary group therapy clinic for the treatment of nonepileptic seizures.

A high percentage of patients presenting to epilepsy centers have a functional neurological disorder with apparent seizures, ultimately diagnosed as nonepileptic seizures (NES). Meta-analyses suggest that psychological treatment is required, but this treatment is not reliably available, resulting in reentry of these patients to neurology clinics and urgent care settings, reducing access for these services to patients with epilepsy and resulting in inadequate psychological care for patients with NES. A sustainable, group therapy-focused treatment clinic for patients with NES was developed as a combined effort between the departments of neurology and psychiatry at the University of Colorado Hospital, consisting of a full psychiatric evaluation, a five-week psychoeducational group, a 12-week psychodynamic therapy group, individual therapy, medication management, and family assessment. One hundred and six patients were treated in this clinic between July 2016 and October 2018. Patient retention after referral for treatment was 89/136 (65.4%), and group therapy adherence was 89/106 (84.0%). Healthcare utilization, used as a proxy to demonstrate worth, decreased during and after treatment. Analysis of the 106 treated patients elucidates other clinical characteristics of this population, including psychiatric comorbidities and specific medication classes at time of NES diagnosis. We conclude that this clinic model is feasible for recruiting, retaining, and engaging patients in appropriate treatment for their NES.

Upper extremity conversion disorder in children.

BACKGROUND: Conversion disorder in children presents a challenge to orthopedic surgeons. The condition is frequently associated with unnecessary diagnostic tests, treatments, and cost. The purpose of this study was to report a series of children with upper extremity conversion disorder to raise awareness for this uncommon condition and to assist with its diagnosis and management. METHODS: A retrospective review was conducted of 4 pediatric patients with upper extremity conversion disorder at a tertiary pediatric hospital from 2015 to 2017. Medical records were reviewed for patient demographics, including psychiatric history, clinical findings, diagnostic studies, treatment, and cost of care. RESULTS: Patients presented with upper extremity muscle stiffness, unremitting dysmorphic muscle spasms, weakness, pain, very limited shoulder range of motion, and complaints of recurrent shoulder dislocations. All patients had been evaluated by multiple specialists and had an extensive prior diagnostic workup that was inconclusive. Two patients had a history of prior psychiatric illness and suicidal ideation, and all patients expressed despair and depression. All patients had normal physical examination findings under anesthesia. Two patients with muscle stiffness were treated with botulism injections and improved their shoulder range of motion. The average total charge for care since presentation was $42,729. CONCLUSIONS: Conversion disorder should be considered in patients with an extensive prior diagnostic workup, deficits inconsistent with anatomic patterns or imaging findings, and a history of prior psychiatric illness. Examination under anesthesia is a successful diagnostic approach in children with suspected conversion disorder.

Pseudo Status Cataplecticus in Narcolepsy Type 1.

ABSTRACT: The rare constellation of multiple episodes of cataplexy that are refractory to therapy is called status cataplecticus. We describe a young adult with known narcolepsy type 1 who reported a marked exacerbation of her symptoms that was initially suspicious for status cataplecticus. Neurological evaluation, polysomnography, and additional history revealed that the spells were not consistent with cataplexy, but rather with a somatoform disorder. The case highlights the importance of considering psychosomatic factors in narcolepsy type 1 when presumed cataplexy remains suboptimally controlled despite adequate dosing with appropriate medications.

Pediatric Psychogenic Nonepileptic Seizures: A Concise Review.

Psychogenic nonepileptic seizures is a complicated biopsychosocial disorder with significant morbidity and high cost in children's social, emotional, family, and academic functioning as well as health care service utilization. Misdiagnosis and diagnostic delay, resulting from both lack of access to approved standards for diagnosing and service providers comfortable with diagnosing and treating this disorder, impact prognosis. Treatment in close proximity to symptom onset is thought to provide the best chance for remission.

Assessment of patients with functional neurologic disorders.

We describe an overall approach and structure to the clinical assessment of the patient with a functional neurologic disorder. Whilst the primary purpose of the assessment is to make a diagnosis and develop a treatment plan, we believe the assessment also plays a key role in treatment in its own right, as it sets a tone and context for future clinical interactions. We aim to set up an atmosphere of collaboration based on taking the patient's problems seriously, and emphasizing that all facets of the patient's presentation - physical, psychologic, and social - are of importance. Patients with functional disorders can be perceived as difficult to help and yet with the correct approaches we believe the consultation can be much more satisfying for both patient and doctor. Finally, we discuss and list some of the common diagnostic pitfalls in the assessment of functional neurologic disorders, looking at features that lead to erroneous diagnosis of neurologic disease (such as old age, la belle indifférence, and lack of psychiatric comorbidity) and an erroneous diagnosis of a functional disorder (such as "bizarre" gait in stiff-person syndrome).

Functional coma.

Functional coma - here defined as a prolonged motionless dissociative attack with absent or reduced response to external stimuli - is a relatively rare presentation. In this chapter we examine a wide range of terms used to describe states of unresponsiveness in which psychologic factors are relevant to etiology, such as depressive stupor, catatonia, nonepileptic "pseudostatus," and factitious disorders, and discuss the place of functional or psychogenic coma among these. Historically, diagnosis of functional coma has sometimes been reached after prolonged investigation and exclusion of other diagnoses. However, as is the case with other functional disorders, diagnosis should preferably be made on the basis of positive findings that provide evidence of inconsistency between an apparent comatose state and normal waking nervous system functioning. In our review of physical signs, we find some evidence for the presence of firm resistance to eye opening as reasonably sensitive and specific for functional coma, as well as the eye gaze sign, in which patients tend to look to the ground when turned on to one side. Noxious stimuli such as Harvey's sign (application of high-frequency vibrating tuning fork to the nasal mucosa) can also be helpful, although patients with this disorder are often remarkably unresponsive to usually painful stimuli, particularly as more commonly applied using sternal or nail bed pressure. The use of repeated painful stimuli is therefore not recommended. We also discuss the role of general anesthesia and other physiologic triggers to functional coma.

Functional voice disorders: Clinical presentations and differential diagnosis.

In this chapter, an overview of the heterogeneous group of functional voice disorders is given, including the psychogenic voice disorder (PVD) and hyperfunctional or muscle tension voice disorder (MTVD) subgroups. Reference is made to prevalence and demographic data, with empiric evidence for psychosocial factors commonly associated with the onset and maintenance of these disorders. Clinical features that distinguish between the different presentations of PVD and MTVD are described. While there are some shared characteristics, key differences between these two subgroups indicate that PVD more closely resembles the psychogenic movement disorders and a range of other functional neurologic disorders. Assessment procedures and auditory-perceptual features of the voice that distinguish these disorders from the neurologically based voice disorders are discussed, with case examples highlighting ambiguous features that may influence differential diagnosis. The clinical profiles of PVD and MTVD affirm approaches to clinical management by speech-language pathologists that integrate symptomatic behavioral voice therapy with "top-down" models of counseling or psychotherapy. They also support the proposition that PVD may be construed as a subtype of functional neurologic disorders.

Prognosis of functional neurologic disorders.

The prognosis of functional (psychogenic) neurologic disorders is important in being able to help answer patients' and carers' questions, determine whether treatment is worthwhile, and to find out which factors predict outcome. We reviewed data on prognosis of functional neurologic disorders from two systematic reviews on functional motor disorders and dissociative (nonepileptic) seizures as well as additional studies on functional visual and sensory symptoms. Methodologic problems include heterogeneity in studied samples and outcome measures, diagnostic suspicion and referral bias, small size and retrospective design of available studies, possible treatments during follow-up, and literature review bias. With these caveats, the prognosis of functional neurologic disorders does appear to be generally unfavorable. In most studies, functional motor symptoms and psychogenic nonepileptic attacks remain the same or are worse in the majority of patients at follow-up. Measures of quality of life and working status were often poor at follow-up. Frequency of misdiagnosis at follow-up was as low as other neurologic and psychiatric disorders. Long duration of symptoms was the most distinct negative predictor. Early diagnosis and young age seem to predict good outcome. Emotional disorders and personality disorders were inconsistent predictors. Litigation and state benefits were found to be negative predictors in some studies, but others found they did not influence outcome.