Emerging Subspecialties: Pediatric Movement Disorders Neurology.

Pediatric movement disorders (PMD) neurologists care for infants, children, and adolescents with conditions that disrupt typical movement; serving as important subspecialist child neurologists in both academic and private practice settings. In contrast to adult movement disorders neurologists whose "bread and butter" is hypokinetic Parkinson disease, PMD subspecialty practice is often dominated by hyperkinetic movement disorders including tics, dystonia, chorea, tremor, and myoclonus. PMD neurology practice intersects with a variety of subspecialties, including neonatology, developmental pediatrics, rehabilitation medicine, epilepsy, child & adolescent psychiatry, psychology, orthopedics, genetics & metabolism, and neurosurgery. Over the past several decades, significant advancements in the PMD field have included operationalizing definitions for distinct movement disorders, recognizing the spectrum of clinical phenotypes, expanding research on genetic and neuroimmunologic causes of movement disorders, and advancing available treatments. Subspecialty training in PMD provides trainees with advanced clinical, diagnostic, procedural, and management skills that reflect the complexities of contemporary practice. The child neurologist who is fascinated by the intricacies of child motor development, appreciates the power of observation skills coupled with a thoughtful physical examination, and is excited by the challenge of the unknown may be well-suited to a career as a PMD specialist.

Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) and COVID-19: is there a connection?

OBJECTIVES: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) is a chronic systemic disease that leads to neurological, immunological, autonomic, and energy metabolism dysfunction. COVID-19 has been reported to cause similar symptoms to ME/CFS. The study aims to investigate the prevalence of myalgic encephalomyelitis in patients post-COVID-19 infection by assessing acute and long-term COVID-19 symptoms. METHODS: A cross-sectional questionnaire was developed based on the ME/CFS diagnostic criteria, as specified by the IOM clinical diagnostic criteria, and administered to participants with confirmed COVID-19 who are more than 18 years old and have BMI below 40 Kg/m2. Data from 437 participants were completed. RESULTS: The current study results revealed that 8.1% of the study participants met the ME/CFS diagnostic criteria. Interestingly, 2.8 of the study participants were classified to have COVID-19 related to ME/CFS. While 4.6% of participants were determined to have disease-related fatigue, 0.7% of participants showed ME/CFS that was not related to COVID-19, and 3.7% of participants were considered to have long COVID-19. Almost one-fourth of the study participants had a family history of ME/CFS. The current study demonstrated that the prevalence of ME/CFS is similar to slightly higher than reported in the literature. CONCLUSION: The presence of a relationship between ME/CFS and COVID-19 has been supported by the results of our study. Follow-up of COVID-19 patients is strongly recommended to ensure proper management of ME/CFS symptoms.

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.

Considerations in the Management of Functional Neurological Disorders in Patients with Hearing Loss.

CASE: Adam is a 14-year-old adolescent boy with hearing loss who presented to the pediatric neurology clinic accompanied by his father for evaluation of new-onset left hand tremor for a duration of 1 month. An American Sign Language interpreter was present and used throughout the visit.Adam has bilateral sensorineural hearing loss related to premature birth at 28 weeks' gestation. He uses sign language and attends a school for the hearing impaired. He has been diagnosed with attention-deficit/hyperactivity disorder (ADHD) and a nonspecific learning disorder. His ADHD symptoms are well controlled with a stimulant medication. He is independent in activities of daily living, and there is no concern for intellectual disability. His father is concerned that Adam may have anxiety, but this has not been evaluated.After careful history, it is found that the tremor was first noted the day after burglars broke into his home and stole precious belongings. Current stressors include difficulties with schoolwork and a strained relationship with an extended family member. There is no family history of tremor.The tremor was intermittent initially, with episodes lasting around 30 minutes. Over time, the tremor became more persistent. Adam is left-handed, and the tremor is now interfering with handwriting, eating, and other fine motor skills. The tremor worsens when Adam is tired or stressed and improves with relaxation. No tremor has been noted in other body parts. Adam denies any other neurological symptoms, including headache, vision changes, or gait abnormalities.On examination, Adam seemed anxious but showed no significant distress and had normal vital signs. His general examination was unremarkable. His neurological examination showed intact cranial nerves, apart from the hearing impairment. He had normal muscle tone, intact strength and coordination, and a normal casual gait. Rhythmic shaking of the left upper extremity was present with action, while maintaining posture. Using specific examination techniques, the examiner was able to alter the rhythm of the tremor, and the tremor was noted to subside when the patient was engaged with the examiner. These findings in addition to signs of suggestibility and variable frequency/direction were consistent with a functional etiology.The diagnosis of a functional neurological disorder manifesting in the form of functional tremor was discussed with the patient and his father with assistance from the sign language interpreter. Counseling regarding management consisting mainly of cognitive behavioral therapy and evaluation of possible coexisting conditions, such as anxiety, was discussed.What factors would you consider in diagnosis and management of functional neurological disorder in a hearing-impaired child/adolescent?

Deep Brain Stimulation for Pediatric Dystonia: A Review of the Literature and Suggested Programming Algorithm.

Deep brain stimulation (DBS) is an established intervention for use in pediatric movement disorders, especially dystonia. Although multiple publications have provided guidelines for deep brain stimulation patient selection and programming in adults, there are no evidence-based or consensus statements published for pediatrics. The result is lack of standardized care and underutilization of this effective treatment. To this end, we assembled a focus group of 13 pediatric movement disorder specialists and 1 neurosurgeon experienced in pediatric deep brain stimulation to review recent literature and current practices and propose a standardized approach to candidate selection, implantation target site selection, and programming algorithms. For pediatric dystonia, we provide algorithms for (1) programming for initial session and follow-up sessions, and (2) troubleshooting side effects encountered during programming. We discuss common side effects, how they present, and recommendations for management. This topical review serves as a resource for movement disorders specialists interested in using deep brain stimulation for pediatric dystonia.

Experience with Pediatric Neurology e-Consults from a Specialist Perspective at an Academic Center.

INTRODUCTION: E-consults improve access to specialty expertise and increase satisfaction for patients and Primary Care Providers (PCPs). Pediatric neurology may be perceived as less amenable to e-consults compared to other specialties. METHODS: We performed a retrospective analysis of e-consults performed by pediatric neurologists at the University of Virginia. We reviewed the electronic medical records and analyzed the content of e-consults, recommendations from specialists, and subsequent actions by PCPs. RESULTS: Between April 2019 and April 2021, 58 e-consults were performed by pediatric neurologists. Most common indications included spells, headache, and tremor. In 56.9% of cases, the answer was provided without recommending a face-to-face (FTF) visit. For patients seen in clinic, e-consults reduced wait time and facilitated the clinic visit. DISCUSSION: E-consults are a suitable option for pediatric neurology, and they help with the workflow. Further education is needed to improve utility of e-consults in pediatric neurology.

Acute Disseminated Encephalomyelitis: A Gray Distinction.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system. METHODS: We describe a three-year-old boy whose clinical presentation was suspicious for ADEM but whose initial imaging abnormalities were confined to the deep gray matter (without evidence of white matter involvement). His clinical course was fluctuating and repeat imaging one week after presentation demonstrated interval development of characteristic white matter lesions. RESULTS: Treatment with adjunctive intravenous immunoglobulin and high-dose corticosteroids resulted in significant clinical improvement. CONCLUSIONS: Isolated deep gray matter involvement can precede the appearance of white matter abnormalities of ADEM, suggesting that repeat imaging is indicated in individuals whose findings are clinically suspicious for ADEM but who lack characteristic imaging findings.