Clinical approach to neuro-rheumatology

SarahGoglinaTracey A.Chob

doi : 10.1016/j.jns.2021.120048

Volume 431, 15 December 2021, 120048

Recognizing the neurologic manifestations of systemic rheumatologic diseases and certain isolated autoimmune neurologic diseases poses challenges to the clinician. Using a systematic approach allows the clinician to diagnose these conditions more readily and to initiate treatment more rapidly. Specific neurological syndromes frequently associated with rheumatologic or specific autoimmune conditions can suggest the diagnosis. A targeted history and examination can identify neurological and systemic clues that help to identify an underlying rheumatologic condition. Judicious use of laboratory and radiographic studies can help confirm suspected diagnoses. This article will review some of the neurological syndromes typical of rheumatologic disease and outline an approach to evaluating for unknown rheumatologic disease in this context.

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Facial skin temperature in acute stroke patients with delirium - A pilot study

JannikStokholmabAbd Al Bari OmarAhmedabLars Kristian HebsgaardBirkmoseabClaudioCsillagbcTroels WesenbergKjærbdThomasChristensenab

doi : 10.1016/j.jns.2021.120036

Volume 431, 15 December 2021, 120036

Facial skin temperature depends strongly on blood flow in small blood vessels in the skin. These are regulated by the sympathetic part of the autonomic nervous system. Delirium may pathophysiologically be associated to changes in the sympathetic part of the autonomic nervous system. In this observational study, we evaluated the influence of various exogenous and endogenous covariables on the regional facial temperatures in acute stroke patients with and without delirium. Facial thermography (FT) was performed using an infrared digital camera. Screening for delirium was done using the Confusion Assessment Method (CAM). Sixty-four patients were enrolled. Eight patients developed delirium. Sex and body temperature were positively associated to facial skin temperature, and so was ambient temperature but to an overall lesser magnitude. Stroke severity, diabetes, infection, facial palsy, facial sensory deficit, and physical activity did not influence facial skin temperature. Overall, there was no association between facial temperature and the occurrence of delirium except in one facial region, the medial palpebral commissure.

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Age at onset predicts outcome in aquaporin-4-IgG positive neuromyelitis optica spectrum disorder from a United Kingdom population

AthanasiosPapathanasiouaRaduTanasescuabChris R.TenchbMaria FranciscaRochaaSmritiBosecCris S.ConstantinescuabSaijuJacobcd

doi : 10.1016/j.jns.2021.120039

Volume 431, 15 December 2021, 120039

There are few studies exploring the prognostic factors in patients with aquaporin-4 (AQP4)-IgG positive neuromyelitis optica spectrum disorder (NMOSD).

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King's college progression rate at first clinical evaluation: A new measure of disease progression in amyotrophic lateral sclerosis

AlessandroIntronaaGiammarcoMilellaaAntonellaMoreaaMariaUcciaAngelaFraddosioaStefanoZoccolellabEustachioD'ErricoaIsabella LauraSimonea

doi : 10.1016/j.jns.2021.120041

Volume 431, 15 December 2021, 120041

To estimate King's college clinical stage progression rate (?KC) at first clinical evaluation in order to define its predictive and prognostic role on survival in a large cohort of Amyotrophic Lateral Sclerosis (ALS) patients.

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Late-life terminal seizure freedom in drug-resistant epilepsy: “Burned-out epilepsy”

S.RajakulendranaM.BelluzzoabJ.NovyacS.M.SisodiyaaM.J.KoeppaJ.S.DuncanaJ.W.Sanderade

doi : 10.1016/j.jns.2021.120043

Volume 431, 15 December 2021, 120043

The course of established epilepsy in late life is not fully known. One key question is whether the resolution of an epileptic diathesis is a natural outcome in some people with long-standing epilepsy. We investigated this with a view to generating a hypothesis. We retrospectively explored whether terminal seizure-freedom occurs in older people with previous drug-resistant epilepsy at the Chalfont Centre for Epilepsy over twenty years. Of the 226 people followed for a median period of 52 years, 39 (17%) achieved late-life terminal seizure-freedom of at least two years before death, which occurred at a median age of 68 years with a median duration of 7 years. Multivariate analysis suggests that a high initial seizure frequency was a negative predictor (p < 0.0005). Our findings indicate that the ‘natural’ course of long-standing epilepsy in some people is one of terminal seizure freedom. We also consider the concept of “remission” in epilepsy, its definition challenges, and the evolving terminology used to describe the state of seizure freedom. The intersection of ageing and seizure freedom is an essential avenue of future investigation, especially in light of current demographic trends. Gaining mechanistic insights into this phenomenon may help broaden our understanding of the neurobiology of epilepsy and potentially provide targets for therapeutic intervention.

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Effectiveness, relevance, and feasibility of an online neurocritical care course for African healthcare workers

KhadijahTiamiyuaJose I.SuarezbcdMorenikeji AdeyoyinKomolafeeJudith K.KwasafDeannaSaylorbg

doi : 10.1016/j.jns.2021.120045

Volume 431, 15 December 2021, 120045

The majority of neurological disorders exist in low- and middle-income countries, but these nations have the fewest neurologists and neurological training opportunities worldwide. The objective of this study was to assess the effectiveness, relevance, and feasibility of a five-day neurocritical care course delivered online to African healthcare workers and to understand participants' prior neurological and neurocritical care training experiences. We offered the Neurocritical Care Society's Emergency Neurological Life Support (ENLS) course covering 14 neurocritical conditions via Zoom to 403 African healthcare workers over a 4-day period. An additional day was devoted to management of neurological emergencies in resource-limited settings. Participants completed pre- and post-course surveys to assess the effectiveness, relevance, and feasibility of the overall course to their settings. 318 participants (46% female; 56% residents; 24% neurologists; 9.0 ± 6.7 years practicing medicine) from 11 African countries completed the pre-course self-assessment, and 232 completed the post-course self-assessment. 97% reported prior experience caring for patients with neurological emergencies but only 35% reported prior neurology training and 9% prior neurocritical care training. Pre-course and post-course comfort levels showed statistically significant improvements (p < 0.001) across all fourteen neurocritical topics. 95% of participants found the course relevant to their current practice setting, 94% agreed the Zoom online platform was easy to use, and 93% reported it facilitated their learning. Suggestions for course improvement included addition of non-critical neurological conditions and inclusion of locally available diagnostics and treatment modalities. Study results suggest virtual platforms may offer a way to improve neurology training in areas with reduced neurological workforce.

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Intrathecal methotrexate – Another tool for the treatment of refractory autoimmune encephalitis - Single institution cohort and literature review

James E.EatonaPatriciaKleinholz-OwensbSubramaniamSriramaSiddharamaPawatea

doi : 10.1016/j.jns.2021.120042

Volume 431, 15 December 2021, 120042

Autoimmune encephalitis (AIE) encompasses a range of inflammatory disorders manifesting with some combination of encephalopathy, seizures, behavioral changes, movement disorders, dysautonomia or other neurologic symptoms. Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is the most common AIE and is an autoantibody mediated disorder, often paraneoplastic. Untreated or undertreated AIE has a high degree of morbidity and mortality. Immunosuppressive treatment regimens including glucocorticoids, plasma exchange (PLEX), intravenous immunoglobulin (IVIG) and rituximab used alone or in combination for such patients. Patients' refractory to such treatments requires more aggressive and potentially toxic therapies. We report favorable outcomes in patients with refractory AIE who received intrathecal methotrexate (IT-MTX) as part of treatment.

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Feasibility and effectiveness appraisal of a neurology residency health equities curriculum

AdysMendizabalaJessica H.FanbRaymond S.PricecRoy H.Hamiltonc

doi : 10.1016/j.jns.2021.120040

Volume 431, 15 December 2021, 120040

Despite increasing awareness of inequities in healthcare in neurology, health equity is not a core competency of neurology training. To meet this need, we implemented a health equities curriculum for neurology residents at the Hospital of the University of Pennsylvania.

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DNA methylation-based surrogates of plasma proteins are associated with Parkinson's disease risk

Katherine A.FuaKimberly C.PaulbAke T.LucSteveHorvathcdAdrienne M.KeeneraeYvetteBordelonaJeff M.BronsteinaBeateRitzab

doi : 10.1016/j.jns.2021.120046

Volume 431, 15 December 2021, 120046

The epigenome may reflect Parkinson's disease (PD) risk, which serves as a point of convergence of genetic and environmental risk factors. Here, we investigate whether blood DNA methylation (DNAm) markers are associated with PD risk.

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Non-infectious mechanisms of neurological damage due to infection

FelixBenningerabcIsraelSteinerbc

doi : 10.1016/j.jns.2021.120057

Volume 431, 15 December 2021, 120057

Infections of the nervous system is a growing aspect of clinical neurology. Accumulating knowledge in early diagnosis, course, therapy and prognosis is enlarging the clinical tools required for effective therapy. Of special importance is the ability to differentiate between proper infections, where anti-microbial agents, when available, should be introduced and used and post infectious conditions where therapy is mainly directed against the host immune system. The two conditions sometimes overlap, a situation that requires the ability to combine clinical skills with the use of laboratory tools such as polymerase chain reaction (PCR), serology, and antigenic detection. In the era of the SARS-CoV-2 pandemic, the need to make this distinction is emphasized as correct diagnosis of post infectious conditions and expedited therapy is important and sometimes lifesaving. We here attempt to present several infectious agents and their possible indirect damage to the nervous system causing in some cases significant neurological deficits. We try to limit our focus on those mechanisms which do not involve the direct tissue damage by the infectious agents but rather are connected to para- and post-infectious mechanisms. We attempt to delineate the features that will enable to tailor the correct diagnosis and following the effective therapy.

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CACNA1S mutation associated with a case of juvenile-onset congenital myopathy

EleonoraMauriaDanielaPigaaSerenaPagliaranibFrancescaMagriaAriannaManinibMonicaSciaccocMichelaRipolonecLauraNapolicLindaBorellinidClaudiaCinnanteeDeniseCassandrinifStefaniaCortiabNereoBresolinabGiacomo PietroComibcAlessandraGovonia

doi : 10.1016/j.jns.2021.120047

Volume 431, 15 December 2021, 120047

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