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?

Diagnosis and Management of Functional Tic-Like Phenomena.

Over the past 3 years, a global phenomenon has emerged characterized by the sudden onset and frequently rapid escalation of tics and tic-like movements and phonations. These symptoms have occurred not only in youth known to have tics or Tourette syndrome (TS), but also, and more notably, in youth with no prior history of tics. The Tourette Association of America (TAA) convened an international, multidisciplinary working group to better understand this apparent presentation of functional neurological disorder (FND) and its relationship to TS. Here, we review and summarize the literature relevant to distinguish the two, with recommendations to clinicians for diagnosis and management. Finally, we highlight areas for future emphasis and research.

Lipomatous Infiltration in Tuberous Sclerosis Complex - A Case Series and Literature Review.

Diffuse Lipomatosis is a dermatological lesion consisting of a poorly circumscribed, infiltrative overgrowth of mature adipose tissue that usually affects the trunk and the extremities. The lesions in the Tuberous Sclerosis Complex (TSC) are usually hamartomatous in nature, but lesions arising from adipocytes are rare. There are only three previous reports of association of TSC with diffuse lipomatosis. Herein we present a case series of diffuse lipomatosis in three subjects with TSC and proceed to review the literature for any other reported cases. On the basis of the three index cases and identification of three more cases in the literature, we believe that there is an association of diffuse lipomatosis with TSC that has not been appreciated until now. We believe that this association in some selected cases will serve to improve diagnosis, surveillance, and management..

Hypnosis in the Treatment of Pediatric Functional Neurological Disorder: The Magic Glove Technique.

The treatment of functional neurological disorder (FND), previously referred to as conversion disorder, can be challenging. Despite these challenges and with increased understanding of this condition, new treatment options are emerging. One such treatment is hypnosis, which has a long history of use in the treatment of FND. Jean-Martin Charcot, considered by many to be the father of modern neurology, used therapeutic hypnosis as early as the 19th century. In this report, we discuss the novel use of a hypno-anesthetic technique (the magic glove) for treatment of FND presenting as diplegia. We illustrate the use of the technique with the case of a 9-year-old girl who suffered from chronic pain and lower extremity weakness secondary to FND. With the magic glove technique, she improved rapidly and was free of symptoms by her sixth month follow-up visit. We review the current literature on FND interventions and emphasize the need for continued research in this field.

Current Approaches and New Developments in the Pharmacological Management of Tourette Syndrome.

Tourette syndrome (TS) is a neurodevelopmental disorder of unknown etiology characterized by spontaneous, involuntary movements and vocalizations called tics. Once thought to be rare, TS affects 0.3-1% of the population. Tics can cause physical discomfort, emotional distress, social difficulties, and can interfere with education and desired activities. The pharmacologic treatment of TS is particularly challenging, as currently the genetics, neurophysiology, and neuropathology of this disorder are still largely unknown. However, clinical experience gained from treating TS has helped us better understand its pathogenesis and, as a result, derive treatment options. The strongest data exist for the antipsychotic agents, both typical and atypical, although their use is often limited in children and adolescents due to their side-effect profiles. There are agents in a variety of other pharmacologic categories that have evidence for the treatment of TS and whose side-effect profiles are more tolerable than the antipsychotics; these include clonidine, guanfacine, baclofen, topiramate, botulinum toxin A, tetrabenazine, and deutetrabenazine. A number of new agents are being developed and tested as potential treatments for TS. These include valbenazine, delta-9-tetrahydrocannabidiol, and ecopipam. Additionally, there are agents with insufficient data for efficacy, as well as agents that have been shown to be ineffective. Those without sufficient data for efficacy include clonazepam, ningdong granule, 5-ling granule, omega-3 fatty acids, and n-acetylcysteine. The agents that have been shown to be ineffective include pramipexole and metoclopramide. We will review all of the established pharmacologic treatments, and discuss those presently in development.

A model for evaluating the activities of a coalition-based policy action group: the case of Hermosa Vida.

Scholars and clinicians are increasingly recognizing the complexity of social contexts of health and the need for multifunctioning approaches to health care problems including community- and policy-level strategies. Barriers to change in health care policy can sometimes be attributed to the actions of advocacy coalitions who operate from a limited view of "policy change." Advocates have a tendency to pressure stakeholders to mandate laws as a final resolution of a movement, often leading to failure or, worse, stigmatizing of issues. A more inclusive focus on health policy change as an ongoing process increases the efficacy of advocacy and outcomes measurement. This article presents a tool for policy action that coalition members developed through the implementation of a 3-year grant to improve the safety net for preventing childhood obesity. Scholars and policy makers developed the Policy Coalition Evaluation Tool with the intent to create a model to guide and measure efforts and outcomes of a local community-based policy coalition. The authors suggest using community-based participatory research approaches for developing a coalition-specific Policy Coalition Evaluation Tool to increase the effectiveness of advocacy groups and the documentation of coalition activities over time.

A call for further research on the impact of state-level immigration policies on public health.

Arizona's Support Our Law Enforcement and Safe Neighborhoods Act, signed into law in April 2010, is already adversely affecting public health in the state. Our findings from a study on childhood obesity in Flagstaff suggest that the law changed health-seeking behaviors of residents of a predominantly Latino neighborhood by increasing fear, limiting residents' mobility, and diminishing trust of officials. These changes could exacerbate barriers to healthy living, limit access to care, and affect the overall safety of the neighborhood. Documentation of the on-the-ground impact of Arizona's law and similar state-level immigration policies is urgently needed. To inform effective policymaking, such research must be community engaged and include safety measures beyond the usual protocols.

Cryptococcal osteomyelitis of the skull.

An otherwise healthy 65-year-old male from a rural area presented with a 1-month old non-tender scalp mass. He had a history of being stuck with a stone in the parietal region a year earlier but hadn't developed any complications. Needle aspiration of the mass revealed numerous yeast cells, which were confirmed to be Cryptoccus neoformans. This case describes a rare presentation of C. neoformans infection in a human immunodeficiency virus (HIV)-negative patient. Moreover, while osteomyelitis due to Cryptococcus is generally preceded by fungemia, in the present case it was caused by direct inoculation of the etiologic agent. We review 11 similar cases published since 1983 in which most of the patients developed an insidious mass in their scalps with osteolytic lesions as seen on X-ray and all were HIV-negative. Nine of the eleven patients in these cases had good recovery after surgical debridement and treatment with amphotericin B, flucytocise and/or fluconazole. The remaining two patients died during hospitalization. Cryptococcal osteomyelitis should be part of a differential diagnosis when confronted with an insidious growing mass or abscess of unclear origin in the scalp.