Long-Term Outcomes of Deep Brain Stimulation for Pediatric Dystonia.

BACKGROUND: Deep brain stimulation (DBS) has been utilized for over two decades to treat medication-refractory dystonia in children. Short-term benefit has been demonstrated for inherited, isolated, and idiopathic cases, with less efficacy in heredodegenerative and acquired dystonia. The ongoing publication of long-term outcomes warrants a critical assessment of available information as pediatric patients are expected to live most of their lives with these implants. SUMMARY: We performed a review of the literature for data describing motor and neuropsychiatric outcomes, in addition to complications, 5 or more years after DBS placement in patients undergoing DBS surgery for dystonia at an age younger than 21. We identified 20 articles including individual data on long-term motor outcomes after DBS for a total of 78 patients. In addition, we found five articles reporting long-term outcomes after DBS in 9 patients with status dystonicus. Most patients were implanted within the globus pallidus internus, with only a few cases targeting the subthalamic nucleus and ventrolateral posterior nucleus of the thalamus. The average follow-up was 8.5 years, with a range of up to 22 years. Long-term outcomes showed a sustained motor benefit, with median Burke-Fahn-Marsden dystonia rating score improvement ranging from 2.5% to 93.2% in different dystonia subtypes. Patients with inherited, isolated, and idiopathic dystonias had greater improvement than those with heredodegenerative and acquired dystonias. Sustained improvements in quality of life were also reported, without the development of significant cognitive or psychiatric comorbidities. Late adverse events tended to be hardware-related, with minimal stimulation-induced effects. KEY MESSAGES: While data regarding long-term outcomes is somewhat limited, particularly with regards to neuropsychiatric outcomes and adverse events, improvement in motor outcomes appears to be preserved more than 5 years after DBS placement.

The role of the locus coeruleus/norepinephrine system in the pathogenesis of neurodegenerative disorders: An update.

PURPOSE OF REVIEW: The aim of this review was to provide an update on current and emerging knowledge of the neuropathological processes affecting the locus coeruleus/norepinephrine (LC/NE) system, their effect on Alzheimer's disease and Parkinson's disease symptomatology, including efforts to translate these notions into therapeutic actions targeting the noradrenergic system. RECENT FINDINGS: Over the past 2 years, work from multiple groups has contributed to support an early role of locus coeruleus degeneration and/or hyperactivation in the neurodegenerative process, including a trigger of neuroinflammation. Imaging advances are allowing the quantification of locus coeruleus structural features in vivo, which is critical in the early stages of disease. Nonmotor and noncognitive symptoms, often secondary to the involvement of the LC/NE system, are becoming more important in the definition of these diseases and their treatment. SUMMARY: The diverse symptomatology of Parkinson's disease and Alzheimer's disease, which is not limited to cardinal motor and cognitive abnormalities, strongly suggests a multisystem neurodegenerative process. In this context, it is increasingly clear how the LC/NE system plays a key role in the initiation and maintenance of the neurodegenerative process.

Skeletal and Brain Abnormalities in Fucosidosis, a Rare Lysosomal Storage Disorder.

Fucosidosis is a rare genetic lysosomal storage disorder caused by a deficiency in alpha- L-fucosidase. We present a case of a 4-year, 11-month-old girl with developmental delay, as well as skeletal and brain abnormalities as shown on X-ray and MRI. Her spinal X- rays demonstrated lumbar kyphosis and anterior beaking of lumbar vertebral bodies. Lower iliac segment constriction, increased angulation of the acetabular roof, and widening of the ribs were apparent on abdominal X-ray. Her brain MRI illustrated symmetric T1 hyperintensity and T2 hypointensity of the bilateral globi pallidi. The case report highlights clinical and imaging findings of this rare disease.

Head computed tomography in the emergency department: a collection of easily missed findings that are life-threatening or life-changing.

BACKGROUND: The use of noncontrast head computed tomography (CT) has become commonplace in the emergency department (ED) as a means of screening for a wide variety of pathologies. Approximately 1 in 14 ED patients receives a head CT scan, and analyzing and interpreting this high volume of images in a timely manner is a daily challenge. OBJECTIVES: Minimizing interpretation error is of paramount importance in the context of life-threatening and time-sensitive diagnoses. Therefore, it is prudent for the physician to recognize particular pitfalls in head CT interpretation and establish search patterns and practices that minimize such errors. In this article, we discuss a collection of common ED cases with easily missed findings, and identify time-effective practices and patterns to minimize interpretation error. DISCUSSION: There are numerous reasons for false-negative interpretations, including, but not limited to, incomplete or misleading clinical history, failure to review prior studies, suboptimal windowing and leveling, and failure to utilize multiple anatomic views via multi-planar reconstructions and scout views. We illustrate this in four specific clinical scenarios: stroke, trauma, headache, and altered mental status. CONCLUSION: Accurate and timely interpretation in the emergent setting is a daily challenge for emergency physicians. Knowledge of easily overlooked yet critical findings is a first step in minimizing interpretation error.

Making the hospital safer for older adult patients: a focus on the indwelling urinary catheter.

The needs of hospitalized geriatric patients differ from the needs of hospitalized younger adults. In an attempt to improve systems of care for the older adult, the Centers for Medicare and Medicaid Services classified urinary tract infections related to the use of indwelling urinary catheters (IUC) as one of eight "never events." The insertion of an IUC is a commonly performed procedure that can cause an array of iatrogenic complications. In addition, the placement of an IUC without medical indication is a risk factor for prolonged hospitalization and inpatient mortality. Foley catheterization has been documented as a culprit in urosepsis and as being associated with geriatric syndromes such as delirium and functional impairment. This article will discuss the indications for the IUC, the complications that can occur because of the IUC, and comment on the Kaiser Permanente Southern California Region's efforts to minimize the unnecessary use of the IUC. Thoughtful and judicious use of the IUC, such as minimizing the use of urinary catheterization, either by not inserting an IUC or by removing it as soon as it is no longer needed, will most likely reduce inpatient morbidity and improve the health of the hospitalized older adult.