Progressive multifocal leukoencephalopathy in dimethyl fumarate-treated multiple sclerosis patients.

Dimethyl fumarate (DMF), a fumaric acid with antioxidant and immunomodulatory properties, is among the most commonly used oral therapies for relapsing multiple sclerosis (MS). Progressive multifocal leukoencephalopathy (PML) has been associated with several disease-modifying therapies (DMTs), including DMF in treating MS. We present detailed clinical characteristics of nine PML cases and show that the PML incidence in DMF-treated patients is 0.02 per 1000 patients. In addition to persistent severe lymphopenia, older age appears to be a potential risk for PML. However, younger patients without lymphopenia were also observed to develop PML. DMF-associated PML has occurred in patients with absolute lymphocyte counts (ALCs) above the guideline threshold, suggesting that changes in specific subsets might be more important than total ALC. Furthermore, since DMF has been found to decrease immune cell migration by decreasing the expression of adhesive molecules, the cerebrospinal fluid (CSF) immune profile may also be useful for assessing PML risk in DMF-treated patients. This review provides an up-to-date assessment of PML cases occurring in DMF-treated patients and discusses other potential considerations in light of our current understanding of DMF's mechanism of action on the immune system in the periphery and in the central nervous system (CNS).

Transverse myelitis associated with Mpox infection.

Neurologic manifestations of mpox (monkeypox) infection are common. Rarely, transverse myelitis has been associated with mpox infection. We describe a case of longitudinally extensive transverse myelitis in a patient with recently diagnosed mpox, presenting as acute flaccid paraplegia. The patient underwent an extensive work-up that included serological and cerebrospinal fluid (CSF) testing and magnetic resonance imaging (MRI). They were treated with tecoviromat, high dose steroids, and intravenous immunoglobulin, followed by plasma exchange. Despite these interventions, there was minimal neurologic improvement. This case underscores the importance of instituting measures designed to prevent mpox infection, including public education initiatives.

If you want to develop an effective autism training, ask autistic students to help you.

LAY ABSTRACT: Autistic university students are often left out because people do not understand autism. We wanted to help people understand autism. Most autism trainings are not made by autistic people. Autistic people know what it is like to be autistic. So autistic people may be the best teachers when it comes to teaching about autism. Autistic students and non-autistic professors made an autism training. The students made videos for the training. They also helped make questions to see what people learned from the trainings. Professors who are not autistic made a training on their own. Students in New York City tried out the trainings. After they answered questions, they did either the training the autistic students helped make or the training made by only professors. Then, they answered questions again. We learned from the students how to make our trainings better. Then, students from two universities in the United States and one university in Lebanon did our trainings and questions. Both trainings made hidden feelings about autism better. The training autistic students helped make taught students more than the training professors made on their own. The autistic-led training also helped students accept autism more. These studies show that autistic students can make autism research and trainings better. At the end of this article, autistic students share their ideas for how to make autism trainings even better in the future.

How is autistic identity in adolescence influenced by parental disclosure decisions and perceptions of autism?

LAY ABSTRACT: There is a lot of research about how parents think about their child's autism but we don't know much about how parents talk with their kids about autism. How parents talk with their kids about autism may shape how kids see autism. A team of autistic and non-autistic people (including a mother of an autistic person) did a study. We wanted to know if how parents talk with their kids about autism shapes how their kids see autism. Nineteen teens from a summer camp did interviews and surveys. Their mothers did surveys. Teens learned about if they had autism in different ways. Some teens still didn't know they were autistic. Teens whose moms chose to tell them about their autism talked about autism and themselves more positively than teens whose moms didn't choose to talk with them about autism. Only teens whose moms chose to talk with them about autism described themselves as having social strengths. Teens had a harder time defining autism than moms. However, teens and moms talked about autism in similar ways. Our study shows that parents can help their kids see autism and themselves more positively by talking with their kids about autism early in development.

Elevated Creatinine Kinase in Peripheral Neuropathy Is Associated With Muscle Cramping.

Introduction: Serum Creatinine Kinase (CK) is a non-specific marker of muscle damage. There has been limited investigation of the association between peripheral neuropathy and CK elevation (hyperCKemia). Methods: We performed a chart review to investigate the CK level in peripheral neuropathies. Demographics, clinical history, physical exam, electrodiagnostic data, CK level, statin use, etiology of neuropathy, and concomitant neuromuscular disorders were recorded. HyperCKemia was defined using our laboratory cutoff values of >180 U/L (women) and >220 U/L (men). Results: We identified 450 patients with peripheral neuropathy who had CK testing, 92 (20.4%) of whom had hyperCKemia. Sixty-one of those patients (13.5% of the total figure) had a concomitant etiology that could explain the CK elevation. Thirty-one patients (6.9%) had no other identifiable etiology for their hyperCKemia beyond the neuropathy. The average CK level in the latter cohort with hyperCKemia was 376 U/L (women: 312 U/L; men: 444 U/L). The frequency of cramping was greater in patients with elevated vs. normal CK (p < 0.0001). Discussion: HyperCKemia can occur in patients with peripheral neuropathy and appears to associate with cramping.

Developing the Multidimensional Visual Scale Assessing Affect, Anxiety, Pride, and Energy Through a Research Partnership with Autistic Scholars.

Autism research studies have traditionally failed to represent the full diversity of the autism spectrum due to the lack of measures available for use with participants who prefer to express themselves visually. A multidimensional measure of emotions, which can include both picture- and text-based prompts, may improve accessibility of emotion rating measures and broaden participation in research and educational evaluations to include those who communicate in diverse ways. Picture-based measures designed to assess participants' emotions may be useful for research concerning autistic identity and service evaluation, two areas where representation of diverse perspectives is needed. Our participatory group of autistic and nonautistic researchers developed a Multidimensional Visual Scale Assessing Affect, Anxiety, Pride, and Energy (AAPE) by adapting and expanding upon an existing emotion rating scale. When testing the AAPE with autistic college students (n = 72), their open-ended responses indicated that the AAPE's dimensions of affect (97.2% correct), anxiety (79.2% correct), and energy (84.7% correct) were well comprehended without text-based labels with potential for improvement in how pride (52.8% correct) was represented. When provided with the labels that each dimension was intended to represent, participants generally agreed that each emotional dimension was well represented. When tested in an informal educational summer camp with autistic children and adolescents (n = 50), the AAPE was well received and revealed insights about the students' emotional responses to different instructional strategies that can guide curricular improvements. The AAPE has utility as a tool to help diverse autistic individuals self-advocate and improve research and services. Lay summary: Why was this study done?: There are very few tools that autistic people can use to share how they feel. We wanted to develop a tool to help autistic people express their emotions using pictures. Pictures can help autistic people share how they feel.What was the purpose of this study?: We wanted to make an easy-to-understand tool that autistic people can use to share how they feel.What did the researchers do?: Our research group is participatory, meaning that autistic and nonautistic researchers worked together to make our tool. An autistic artist drew the tool. We called it the AAPE, which stands for the emotions it assesses: Affect, Anxiety, Pride, and Energy. We worked together to see how well the AAPE worked. We used a survey to see if autistic high school and college students understood our first try at the AAPE and we learned how to make the AAPE better from these students. We worked together to make the AAPE better. Then, we did another survey with autistic college students to see if our second try at the AAPE worked better. Then, we asked autistic kids and teenagers to use the AAPE to share how they felt about different ways of teaching.What were the results of the study?: In our final test, we asked 72 autistic college students to tell us what emotions they thought the AAPE was showing. College students thought that affect (97.2%), anxiety (79.2%), and energy (84.7%) showed the emotions we aimed to show with room for improvement in how pride (52.8%) was shown. After we told participants which emotion each scale was showing, they agreed that affect (average score 4.28 of 5) and anxiety (4.29 of 5) showed the emotions best, followed by energy (4.08 of 5) and pride (3.5 of 5) on a scale from 1 to 5 (strongly agree).Students preferred using the AAPE compared with text-based surveys we used in the past. Results showed that the AAPE does a good job at measuring emotions. Edits may still be needed to better show "pride." Work is needed with nonspeaking people to make sure our measure works well for people who communicate in different ways.What are potential weaknesses in the study?: The autistic adolescents and adults we have tested the AAPE with so far have not been very diverse. We did not include participants who communicate without speaking in these first tests of the AAPE. We plan to use the AAPE with more diverse groups in future studies.How will these findings help autistic adults now or in the future?: In a future study, we will use the AAPE to rate pictures that show experiences of being autistic, like feeling outside a social group, having sensory difficulties, and making patterns. We would like to do this because some studies talk about negative experiences of autism, like feeling the need to hide autistic traits, but other studies describe strengths of autism, like strong memories and advanced knowledge in particular subjects. However, these studies do not talk about the emotions that come with these experiences and if these experiences are shared with autistic people who do not use speech to communicate. The AAPE is a tool that might help us understand how diverse autistic people feel about autism.

Dying on Hospice in the Midst of an Opioid Crisis: What Should We Do Now?

The current opioid crisis in the United States is a major problem facing health-care providers, even at the end of life. Opioids continue to be the mainstay treatment for pain at the end of life, with the prevalence of pain reported in up to 80% of patients and tends to increase as one gets closer toward the end of life. In the past year, 20.2 million Americans had a substance use disorder (SUD) and SUDs are disabling disorders that largely go untreated. In addition, the coexistence of both a mental health and SUD is very common with the use of opioids often as a means of chemical coping. Most hospice programs do not have standardized SUD policies/guidelines in place despite the increasing concerns about substance abuse within the United States. The goal of this article is to review the literature on this topic and offer strategies on how to manage pain in patients who have active SUD or who are at risk for developing SUD in those dying on hospice.

Recent advances in understanding and managing myasthenia gravis.

Autoimmune myasthenia gravis (MG) is a neuromuscular junction disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies against acetylcholine receptors (AChRs), muscle-specific kinase (MuSK), or lipoprotein-related protein 4 (LPR4). Over the past few decades, the mortality of patients with MG has seen a dramatic decline secondary to evolving interventions in critical care and medical management. In the past 2 to 3 years, there have been several changes in standard of care for the treatment of MG. These changes include confirmation of the benefit of thymectomy versus medical management alone in AChR patients and a new US Food and Drug Administration-approved medication for refractory MG. There are also several exciting new prospective drugs in the pipeline, which are in different stages of clinical trial testing.

Safety and Efficacy of Rituximab: Experience of a Single Multiple Sclerosis Center.

OBJECTIVES: Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system. B cells play an important pathogenic role in MS. Rituximab (RTX), a B-cell depleting drug, has been used to treat MS and neuromyelitis optica (NMO). Patient characteristics, safety, and efficacy measures are reviewed to ascertain the therapeutic benefit and safety of RTX in a real-world setting with long-term follow-up. METHODS: This is a retrospective chart review of patients who received RTX at The Ohio State University's MS clinic from January 2005 to October 2016. RESULTS: Of the 64 patient charts reviewed, 23 had a relapsing remitting MS, 17 had primary progressive MS (PPMS), and 24 had NMO. In the relapsing remitting MS cohort, there was an annual relapse rate of 0.005 and 87% were reported as clinically stable at the end of the chart review period. In the primary progressive MS cohort, 47% were reported as clinically stable at the end of the chart review period. In the NMO cohort, there was an annual relapse rate of 0.0074 and 79% were reported as clinically stable at the end of the chart review period. A total of 29 infusion reactions were reported in 21 patients. None were serious and only 1 patient elected to stop RTX due to an adverse event. CONCLUSIONS: Rituximab demonstrated good tolerability and efficacy in cases of both relapsing and progressive forms of MS and NMO.

Depression in Older Adults: A Palliative Medicine Perspective.

This article aims to discuss the rapidly growing field of palliative medicine and its unique approach to treating depression in older adults.

Serotonin reuptake inhibitor use in pregnancy and the neonatal behavioral syndrome.

OBJECTIVE: To assess the severity of neonatal behavioral syndrome (NBS) in infants of serotonin reuptake inhibitor (SRI)-treated pregnancies, compared with infants of women with psychiatric illness not treated with medication. METHODS: This was a retrospective cohort study of pregnancies followed in a prenatal clinic for women with psychiatric illness. Infants of women who received SRI medication through delivery (SRI-treated) were compared with those who did not receive treatment or discontinued medication before the last month of pregnancy (SRI-untreated). NBS was defined as one or more of the following: jitteriness, irritability, lethargy, hypotonia, hypertonia, hyperreflexia, apnea, respiratory distress, vomiting, poor feeding, or hypoglycemia. RESULTS: Findings of NBS were identified in 28% of 46 SRI-treated pregnancies and 17% of 59 untreated pregnancies. There were no differences in rates of prematurity (4% vs. 7%), fetal growth restriction (6% vs. 2%), transfer to a higher nursery for NBS (11% vs. 10%), respiratory abnormality (7% vs. 5%), or hospitalization duration among infants with NBS findings (2 vs. 6 days). CONCLUSIONS: Findings of NBS were identified in 28% of SRI-exposed neonates. However, these infants were not more likely than unexposed infants to be admitted to a higher nursery, experience respiratory abnormalities, or have prolonged hospitalization.