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Proximal median (PMNE) and radial (RNE) nerve entrapment syndromes are uncommon. This article provides an evidenced-based treatment guideline for PMNE and RNE based on the available literature. Arriving at an accurate diagnosis must involve an electrodiagnostic (EDx) workup. EDx, including nerve conduction velocity studies and needle electromyography, should corroborate the clinical diagnosis and must be done before consideration of any surgical treatment. Conservative care includes rest, modified activities, splinting at wrist and elbow, physical therapy, antiinflammatory drug therapy, and corticosteroid injections. Conservative care should be required for at least 6 weeks before any operative interventions are considered.

Analgesics, including opioids, steroidal and nonsteroidal anti-inflammatory drugs, aspirin, acetaminophen, antiepileptics, and serotonin-norepinephrine reuptake inhibitors, are medications commonly used to treat many forms of pain. However, all of these agents may have significant adverse side effects. Adverse effects may occasionally be inseparable from desired effects. Side effects are often dose dependent and time dependent. It is critical that the prescribing practitioner and the dispensing pharmacist provide a thorough, understandable review of the potential side effects to all patients before these drugs are administered. Proper monitoring and follow-up during therapy are crucial.

A growing body of research indicates that chronic pain is a significant problem for many persons with chronic, slowly progressive neuromuscular disease (NMD).1–16 However, it is still not clear how much pain intensity factors in to the negative bio-psychosocial and physical consequences of chronic pain in the setting of slowly progressive NMD. Pain intensity is one of the most common dimensions assessed by clinicians and researchers who treat and study pain. Reduction in global pain intensity is also the standard by which most pain treatments are judged. However, although average pain intensity is an important pain domain, other pain domains are also potentially important (eg, pain frequency, duration, location, and quality) as factors that could contribute to patient dysfunction, especially in individuals with chronic pain.17 Unfortunately, research is lacking regarding the relative importance of these additional domains for understanding adjustment to pain. The research that has been conducted on this topic in other pain populations suggests that pain site may contribute to adjustment to chronic pain over and above the effects of global pain intensity. For example, Marshall and colleagues18 found that the intensity of back pain in patients with amputation explained a significant amount of variance in interference in daily activities beyond the pain associated with limb amputation. Similarly, there is some preliminary evidence that pain in the low back and arms is more strongly associated with patient functioning than pain in other body locations in a sample of patients with a variety of chronic pain problems (Tan G, Jensen MP, unpublished data, 2011). Nonetheless, research in this area is sparse, and it is not known whether these preliminary findings replicate in other samples of patients with chronic pain, including those with NMD. If these findings do replicate across different chronic pain populations, then clinicians should assess both pain intensity and its location(s) to better understand the potential impact that the pain might have on a specific patient. Moreover, if low back pain or pain in the extremities is more closely linked to a patient’s quality of life than pain at other sites, then treatments that address pain at these sites may be more important to patients with chronic pain than treatments that address pain at other sites (eg, the head or torso). Thus, research in this area could help inform the work of clinicians and scientists who are developing new pain treatments for individuals with specific pain conditions. However, the authors are not aware of any research that has studied the relative importance of pain site to patient functioning in individuals with slowly progressive NMD. Pain extent is a separate and distinct domain from intensity and refers to the overall number of body areas with pain. Research suggests that this pain domain may also be important to patient functioning. For example, Tait and colleagues19 found a significant association between pain extent and the tendency of patients to report greater complaints of weakness, fatigue, and depression. Similarly, Toomey and colleagues20 reported that patients with more pain sites were more likely to report pain as having a greater negative impact in their functioning. Turp and colleagues21 found that pain extent, along with pain intensity, was a significant predictor of pain-related disability in a sample of female patients with chronic facial pain. Patients with pain at multiple sites have shown a reduced level of health-related functioning, are more likely to have difficulties with mobility regardless of physical impairments than those with no pain or localized pain, and have worse prognosis for future work ability.22–24 In a series of studies, Kamaleri and colleagues25 reported significant associations between pain extent and functioning in patients with musculoskeletal pain. They found a strong and linear association between increasing number of pain sites and decreasing functional ability; a strong relationship with decreasing psychological health, sleep quality, and overall health; and future work disability after a 14-year period.25,26 As with research on the importance of specific pain sites to patient functioning, it is unclear if these findings regarding pain extent replicate in other populations of individuals with chronic pain, including persons with slowly progressive NMD. Most published studies on these issues have been conducted with low back patients receiving treatment at secondary and tertiary care facilities. Thus, these findings may not generalize to other populations of patients with pain.27 Further delineating the relative importance of pain site and extent in relation to patient functioning is particularly important in patients with NMD, because research indicates they typically experience pain in more than one location.5 Given what previous studies have found in other pain populations, the authors hypothesize that pain extent would be negatively associated with psychological functioning and positively associated with pain interference, whereas pain intensity in specific pain sites would show stronger associations with measures of patient functioning than pain at other sites. More specifically, one would expect that pain in the low back and arms might evidence stronger associations with pain interference and psychological functioning than pain at other sites.

This article discusses the role of palliative care in the treatment pathway of patients with progressive neuromuscular disease (NDM), including amyotrophic lateral sclerosis and Duchenne muscular dystrophy (DMD). People with severe NMDs like DMD are now living much longer, well in to adulthood. This makes them suitable for the medical model of palliative care. Yet palliative medicine is a new area, especially for "adults" with DMD. Strategies for identifying the most effective modalities to alleviate suffering in patients with an NMD receiving palliative services and creating best practice standards in pain and symptom management for this patient population are discussed.

Assessing burden of disease is a complex process involving identifying the physical, psychological, and socioeconomic aspects that make up the totality of disease burden on patients, families, and caregivers, and society as a whole, with chronic pain affecting all of these aspects. It is the job of the physiatrist to identify disability and promote interventions to minimize it, including facilitating access to appropriate treatment for chronic pain, and ultimately easing disease burden. Chronic pain causes significant psychosocial dysfunction for patients with neuromuscular disease and contributes substantially to the overall disease burden. There are many psychosocial factors closely associated with pain and dysfunction in patients with neuromuscular disease, most notably a perception of inadequate psychosocial support. The assessment of children with neuromuscular disorders must include the parents or guardians as well, given that chronic pain in a child will affect the entire family.

This article reviews the current knowledge regarding the benefits and contraindications of exercise on individuals with neuromuscular diseases (NMDs). Specific exercise prescriptions for individuals with NMDs do not exist because the evidence base is limited. Understanding the effect of exercise on individuals with NMDs requires the implementation of a series of multicenter, randomized controlled trials that are sufficiently powered and use reliable and valid outcome measures to assess the effect of exercise interventions-a major effort for each NMD. In addition to traditional measures of exercise efficacy, outcome variables should include measures of functional status and health-related quality of life.

Amyotrophic lateral sclerosis (ALS) is a devastating, progressive motor neuron disorder that poses a myriad of clinical problems. Patients who have ALS are best cared for in a multidisciplinary fashion, with involvement of clinicians from various specialties, including neurology, physical medicine and rehabilitation, pulmonary medicine, clinical nurse specialists or nurse practitioners, physical and occupational therapists, speech language pathologists, dietitians, psychologists, social workers, and case managers. This article provides a summary of the current research into the rehabilitation of ALS, including the role of exercise, spasticity management, mood disorders, pain, and palliative care.

Amyotrophic lateral sclerosis (ALS) is a devastating condition characterized by progressive muscle wasting, inanition, respiratory failure, and death within approximately 2 to 5 years of onset. ALS is among the most common neuromuscular conditions, with an overall prevalence in the world of approximately 5 to 7 cases/100,000 population. Epidemiologic studies have identified some potential risk factors for developing ALS, including a high-fat, low-fiber diet; cigarette smoking; slimness and athleticism; and living in urban areas. Between 5% and 10% of ALS is genetic, with up to 11 genetic loci identified. Although understanding of the pathophysiology of this disease has advanced over the past 60 years, scant progress has been made regarding effective treatment. The authors review the current understanding of the pathogenic mechanisms of ALS and approaches to treating the disease.

Marijuana contains over 60 different types of cannabinoids, which are its medicinally active ingredients. Cannabinoids have the capacity for neuromodulation--through direct, receptor-based mechanisms--at many levels within the nervous system, providing therapeutic properties that may be applicable to the treatment of neurologic disorders. These include antioxidation, neuroprotection, analgesia, anti-inflammation, immunomodulation, modulation of glial cells, and tumor growth regulation. This article reviews the current and emerging research on the physiologic mechanisms of endogenous and exogenous cannabinoids and their applications in the management of neurologic disease.

Much progress has been made in the assessment and management of neuropathic pain over the past 5 years. Assessment has improved with the Neuropathic Pain Scale, a new, easily administered, diagnostic tool. Mechanistically, recent studies indicate that peripheral neuropathic pain is generated through a focal inflammatory process rather than axonal destruction. This process also appears to involve mRNA regulation of fast sodium channels, which produce ectopic discharges and are presumably responsible for pain generation. In addition the entire neuraxis undergoes neuroplastic changes as a result of peripheral nerve injury. The available clinical trial data indicate that newer antiepileptic drugs (AEDs), most notably gabapentin, are better alternatives to older medications such as carbamazepine or phenytoin in the treatment of neuropathic pain. Gabapentin is at least as good with respect to actual pain relief as the antidepressants, including amitriptyline, but has a much better safety profile with minimal drug-drug interactions and side effects. Mexiletine is a reasonable alternative agent in patients who have not had a satisfactory response to, or cannot tolerate, the AEDs or antidepressants. Long-acting opioids should be considered in patients refractory to these adjunctive agents. With the advent of the topical lidocaine patch, the first drug with an FDA-approved indication for postherpetic neuralgia, a revolutionary new agent is now available for the treatment of neuropathic pain that does not have any systemic side effects.

The purpose of this article is to show the value of assessing the health-related quality of life for individuals with peripheral neuropathies. The authors will briefly describe methods used to assess the quality of life and examine the results from studies that have been used to evaluate the health-related issues for those individuals with peripheral neuropathies.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive motor neuron disease that poses a myriad of clinical problems. Patients with ALS are best treated in a multidisciplinary setting involving physicians, clinical nursing specialists, and physical, occupational, speech, and respiratory therapists, as well as psychologists and social workers. Palliative and rehabilitative strategies may ease suffering, while new treatments provide hope for effective treatment of this disease.

Patients with neuromuscular disease (NMD) are complex and pose a myriad of clinical problems. Management is best carried out by a team consisting of physicians, physical, occupational, and speech therapists, social workers, vocational counselors, and psychologists, among others. Physiatrists are well-suited to this type of multi-disciplinary care and can play a significant role in the care of these patients.

The importance of pain extent (ie, number of body areas with pain) and pain site as factors contributing to dysfunction in persons with chronic, slowly progressive neuromuscular disease (NMD), remains poorly understood. This article discusses the importance of assessing pain site in addition to global pain intensity in patients with chronic, slowly progressive NMD. The importance of addressing pain at multiple sites will have a major impact on future studies assessing interventions to treat pain in this patient population.

Muscle degeneration in muscular dystrophies often includes a period of edema before fatty replacement of muscle tissue. Magnetic resonance imaging (MRI) has been used successfully to characterize muscle and fat patterns in several types of muscular dystrophies. Recent MRI techniques enable characterization of edema in tissues. This article reviews the advantages of using MRI assessment of edema and fat in muscle tissue to evaluate disease progression, and discusses inflammation and sarcolemma compromise as sources of edema in muscular dystrophy. Lastly, refining current techniques and adapting other MRI capabilities may enhance detection and assessment of edema for better evaluation of disease progression and treatment outcomes.

As someonewhohas spent the vastmajority of his career in thePacific Northwest, when we decided to do an updated issue on spinal cord injury (SCI) rehabilitation, I immediately thought of my friend and colleague, Dr Diana Cardenas. When Dr Cardenas was here at the University of Washington, she developed and directed a nationally recognized SCI Center of Excellence. This was the “go to” place for all of us to refer our SCI patients for their annual complete evaluation and checkup. For many years now, Dr Cardenas has served as Professor and Chair, Department of Rehabilitation Medicine and Chief of Service andMedical Director, Department of RehabilitationMedicine at the University of Miami Miller School of Medicine. She is also part of the Miami Project to Cure Paralysis (the Buoniconti Fund). As I suspected she would, Dr Cardenas recruited a list of “who’s who” in spinal cord medicine to author the articles within this issue. This issue starts with an excellent article entitled, “Updates for the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI),” by Drs Kirshblum and Waring. This is a great overview and provides an excellent treatise on how to use these standards to improve care. Drs Goetz and Klausner authored, “Strategies for Prevention of Urinary Tract Infections in Neurogenic Bladder Dysfunction,” which provides up-to-date strategies on how to approach and treat this significant problem seen in the vast majority of patients of SCI and neurogenic bladder. Moving into a more experimental topic, Drs Sherman andWang present, “Hypothermia as a Clinical Neuroprotectant.” As they point out, there is now a very robust body of experimental evidence that hypothermia is beneficial in the treatment of acute SCI. The potential benefits of hypothermia in preventing or limiting SCI following trauma are discussed in detail along with an overview of the potential use of hypothermia after SCI.

Major advances in the fields of medical science and physiology, molecular genetics, biomedical engineering, and computer science have provided individuals with muscular dystrophy (MD) with more functional equipment, allowing better strategies for improvement of quality of life. These advances have also allowed a significant number of these patients to live much longer. As progress continues to change management, it also changes patients' expectations. A comprehensive medical and rehabilitative approach to management of aging MD patients can often fulfill expectations and help them enjoy an enhanced quality of life.

Electrophysiologic evaluation is a critical component of the diagnosis and management of patients with myopathic disorders. These studies can help guide further diagnostic studies, including muscle biopsy and imaging, as well as help monitor rehabilitation. This article will examine the role of electrophysiologic testing in diagnosing and managing the rehabilitation of patients with myopathic disorders. It is assumed that the reader has a basic understanding of muscle anatomy, physiology, and electrophysiology.

Over the past decade, MRI has become a widely used technique for imaging the brain and spinal cord. MRI has also become an important tool for evaluating the spine and musculoskeletal system. Ongoing research and clinical experience with MRI is further defining the role of this technique in evaluating disorders of the neuromuscular system. There have been numerous case series and reports documenting the usefulness of MRI in this area [1–10]. Although still evolving, MRI is becoming an increasingly useful method of depicting disorders of muscle and nerve. Clinical evaluation of neuromuscular disorders typically consists of obtaining a detailed clinical history, physical examination, and electrophysiologic examinations. Electrodiagnostic examinations significantly aid in distinguishing between myopathy, neuropathy, and neuromuscular disorders. Electrodiagnostic examinations also assist in determining the severity and extent of disease. Progress can also be monitored on follow-up testing. The benefit of MRI in neuromuscular disease evaluation lies primarily in identifying a specific underlying gross pathologic cause and its anatomic location [10]. MRI is also helpful in identifying associated secondary findings. In some cases, MRI may be particularly helpful when a solitary, small, deep muscle is affected. Imaging can be useful in assessing clinical progress in some cases. Causes of muscle denervation include mass lesions and trauma as well as infectious, autoimmune, and idiopathic causes. In cases in which

Chronic Pain in Persons with Neuromuscular Disease Amy J. Hoffman, MPH, Mark P. Jensen, PhD, R. Ted Abresch, MS, Gregory T. Carter, MD* Department of Rehabilitation Medicine, Box 356490, University of Washington School of Medicine, Seattle, WA 98195-6490, USA Multidisciplinary Pain Center, University of Washington Medical Center–Roosevelt, 4245 Roosevelt Way Northeast, Seattle, WA 98105-6920, USA Department of Physical Medicine and Rehabilitation, PM&R TB 191, University of California, Davis, CA 95616, USA

Electrodiagnosis can classify hereditary motor and sensory neuropathies (HMSN) into two basic types: primarily demyelinating with secondary axonal loss and primarily axonal. For the most part, the various forms of HMSN show uniform symmetric nerve conduction slowing, in contrast to acquired neuropathies, which may be multifocal with nonuniform conduction velocity slowing and temporal dispersion. Nevertheless, there are exceptions. This article reviews the available literature and describes the electrodiagnostic approach to HMSN, detailing potential sources of error that can lead to misinterpretation of data.

Much progress has been made in the assessment and management of neuropathic pain over the past 5 years. Assessment has improved with the Neuropathic Pain Scale, a new, easily administered, diagnostic tool. Mechanistically, recent studies indicate that peripheral neuropathic pain is generated through a focal inflammatory process rather than axonal destruction. This process also appears to involve mRNA regulation of fast sodium channels, which produce ectopic discharges and are presumably responsible for pain generation. In addition the entire neuraxis undergoes neuroplastic changes as a result of peripheral nerve injury. The available clinical trial data indicate that newer antiepileptic drugs (AEDs), most notably gabapentin, are better alternatives to older medications such as carbamazepine or phenytoin in the treatment of neuropathic pain. Gabapentin is at least as good with respect to actual pain relief as the antidepressants, including amitriptyline, but has a much better safety profile with minimal drug-drug interactions and side effects. Mexiletine is a reasonable alternative agent in patients who have not had a satisfactory response to, or cannot tolerate, the AEDs or antidepressants. Long-acting opioids should be considered in patients refractory to these adjunctive agents. With the advent of the topical lidocaine patch, the first drug with an FDA-approved indication for postherpetic neuralgia, a revolutionary new agent is now available for the treatment of neuropathic pain that does not have any systemic side effects.

It is now possible for the clinician to use multidimensional measures to assess the quality of life of their patients. Some of the more widely used instruments to measure HRQOL are the Medical Outcomes Study Short Form 36, the Nottingham Health Profile, the Sickness Impact Profile, and the World Health Organization Quality of Life instrument. Potential uses of quality of life assessment tools include: (1) monitoring the health and social status of a given population, (2) evaluating health care policy, (3) conducting clinical trials, (4) assessing the effectiveness of rehabilitation services, (5) justifying the allocation of limited social and health care resources, and (6) tailoring management to the needs of the patient.

Patients with neuromuscular disease (NMD) are complex and pose a myriad of clinical problems. Management is best carried out by a team consisting of physicians, physical, occupational, and speech therapists, social workers, vocational counselors, and psychologists, among others. Physiatrists are well-suited to this type of multi-disciplinary care and can play a significant role in the care of these patients.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive motor neuron disease that poses a myriad of clinical problems. Patients with ALS are best treated in a multidisciplinary setting involving physicians, clinical nursing specialists, and physical, occupational, speech, and respiratory therapists, as well as psychologists and social workers. Palliative and rehabilitative strategies may ease suffering, while new treatments provide hope for effective treatment of this disease.