Divisha Raheja

Pain in amyotrophic lateral sclerosis: Patient and physician perspectives and practices

Abstract Our objective was to better understand the experience and impact of pain on ALS patients in the U.S., and to survey ALS physicians on their pain assessment and management practices. Individuals with ALS were invited to complete an online survey of pain in ALS. ALS specialist physicians were sent an e-mail survey about their experiences in evaluating and managing patients’ pain. Nearly 75% of patients with ALS reported significant pain, and most thought that ALS was the source of at least some of this pain. Pain intensity scores (mean 3.9/10) and pain interference scores (mean 4.3/10) were moderate on average, but nearly 80% of participants were using pain medication, including 22% using opioids. Nearly 25% of patients thought they needed stronger pain medication than they were receiving. Physicians generally assess and manage pain in ALS patients, but few use standardized assessment tools. Nearly two-thirds felt that there is a need for better pain management practices and more than one-third felt better training was needed. In conclusion, pain in patients with ALS is not always well controlled. Improvement in care may be facilitated by a more standardized approach to evaluation, and by additional education and training of ALS health care professionals.

Patient-reported problematic symptoms in an ALS treatment trial.

Abstract This study was undertaken to determine which symptoms are perceived to be most problematic for patients with ALS and how their severity changes over time. A retrospective study was performed of data from a randomized, double-blind, placebo-controlled trial of ceftriaxone in ALS. Participants completed the ALS Specific Quality of Life Instrument (ALSSQoL) at baseline and at intervals up to 96 weeks. Ten ALSSQoL items ask participants to rate how problematic symptoms are (the subjective feeling of burden of these symptoms), ranging from 0 (no problem) to 10 (tremendous problem). Six are non-bulbar (pain, fatigue, breathing, strength and ability to move, sleep, and bowel and bladder) and four are bulbar (eating, speaking, excessive saliva, and mucus). Results revealed that there were 82 subjects (56% males, mean age 53 ± 10.3 years) with ALSSQoL data for weeks 0 and 96. All 10 symptoms became more problematic over time. For non-bulbar symptoms, strength/ability to move and fatigue were the most problematic. Speaking was the most problematic bulbar symptom. In conclusion, although all the symptoms in the ALSSQoL were acknowledged as problematic, some had greater impact than others. All became more problematic over time. This should help prioritize research into symptom management, and assist individual clinicians in their approach to patient care.

Central neurotoxicity of immunomodulatory drugs in multiple myeloma

Immunomodulatory drugs (IMiDs) currently used in the treatment of multiple myeloma, are thalidomide, lenalidomide and pomalidomide. One of the most common side effects of thalidomide is neurotoxicity, predominantly in the form of peripheral neuropathy. We report 6 cases of significant central neurotoxicity associated with IMiD therapy. Treatment with thalidomide (1 patient), lenalidomide (4 patients), and pomalidomide (1 patient) was associated with various clinical manifestations of central neurotoxicity, including reversible coma, amnesia, expressive aphasia, and dysarthria. Central neurotoxicity should be recognized as an important side effect of IMiD therapy.

The role of mental health and self-efficacy in the pain experience of patients with amyotrophic lateral sclerosis

Abstract Complex interactions between pain, depression, and anxiety impact quality of life in patients with ALS. Psychological approaches to pain control may be useful. This study explored the role of self-efficacy in mitigating pain. Individuals registered with the Agency for Toxic Substances and Disease Registry National ALS Registry and who experienced pain were invited to participate in an online survey. Subjects completed the Brief Pain Inventory-Short Form, Hospital Anxiety and Depression Scale, and Chronic Pain Self-Efficacy Scale. Correlations between variables were determined. Multiple linear regression models assessed relationships between depression, anxiety and self-efficacy predictions, and pain severity, interference, and relief. Results recorded that there were 197 participants (58% males, mean age 59 ± 10 years). Cases or borderline cases of depression or anxiety were common. Mean levels of pain were moderate. Higher pain self-efficacy scores predicted lower pain severity, lower pain interference, and higher pain relief with treatment. As depression scores increased, pain interference with daily life was higher. In conclusion, anxiety and depression are common in patients with ALS and pain. Self-efficacy appears to mitigate pain. A multifactorial approach to pain management should be considered in these patients, addressing mental health and self-efficacy to augment pharmacologic pain treatments.

Implementing Motor Unit Number Index (MUNIX) in a large clinical trial: Real world experience from 27 centres

OBJECTIVE Motor Unit Number Index (MUNIX) is a quantitative neurophysiological method that reflects loss of motor neurons in Amyotrophic Lateral Sclerosis (ALS) in longitudinal studies. It has been utilized in one natural history ALS study and one drug trial (Biogen USA) after training and qualification of raters. METHODS Prior to testing patients, evaluators had to submit test-retest data of 4 healthy volunteers. Twenty-seven centres with 36 raters measured MUNIX in 4 sets of 6 different muscles twice. Coefficient of variation of all measurements had to be <20% to pass the qualification process. MUNIX COV of the first attempt, number of repeated measurements and muscle specific COV were evaluated. RESULTS COV varied considerably between raters. Mean COV of all raters at the first measurements was 12.9% ± 13.5 (median 8.7%). Need of repetitions ranged from 0 to 43 (mean 10.7 ± 9.1, median 8). Biceps and first dorsal interosseus muscles showed highest repetition rates. MUNIX variability correlated considerably with variability of compound muscle action potential. CONCLUSION MUNIX revealed generally good reliability, but was rater dependent and ongoing support for raters was needed. SIGNIFICANCE MUNIX can be implemented in large clinical trials as an outcome measure after training and a qualification process.

Muscle & nerve joins the “App World”

Muscle & Nerve has joined a growing list of leading healthcare journals that have apps to facilitate access through mobile devices (tablets and smartphones). To familiarize ourselves and readers of the journal with the app, we tried it out, realizing that one of us (Z.S.) is considerably older, and much more a product of the “print” generation, than the other (D.R.). Strikingly, we had very similar, positive experiences. The app can be downloaded from the Apple App Store onto an iPad or an iPhone. Upon doing so, Muscle & Nerve will appear on the Newsstand with the latest version of the cover, just as it would look in print. Tap on the journal icon, and a screen with several choices presents itself. Touching Early View reveals a list of recent articles, accepted but not yet in print, in chronological order. These are formatted in the same manner as the journal table of contents, but organized by date. Touching the Issues icon brings up icons of the past 12 issues. Touching any of these icons reveals a Table of

A case of encephalomyeloradiculopathy in a non-carcinomatous patient associated with P/Q type voltage gated calcium channel antibodies

The presence of P/Q type voltage gated calcium channel (VGCC) antibodies has been strongly correlated with Lambert Eaton Syndrome (LES), present in 90% of non-immunocompromised patients with LES. However, there have been case reports which have shown its association between paraneoplastic syndrome affecting both central nervous system and the peripheral nervous system causing encephalomyelitis and sensory neuronopathy/neuropathy. We present a case of a young man, who presented with encephalomyelitis, and was further noted to have superimposed cervical polyradiculopathy associated with P/Q type VGCC antibodies.