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IgG regulation through FcRn blocking: A novel mechanism for the treatment of myasthenia gravis
Gil I.WolfeaE. SallyWardbHansde HaardcPeterUlrichtscTahseenMozaffardMamathaPasnooreGesturVidarssonf
doi : 10.1016/j.jns.2021.118074
Volume 430, 15 November 2021, 118074
The neonatal Fc receptor (FcRn) is an MHC class I–like molecule that is widely distributed in mammalian organs, tissues, and cells. FcRn is critical to maintaining immunoglobulin G (IgG) and albumin levels through rescuing these molecules from lysosomal degradation. IgG autoantibodies are associated with many autoimmune diseases, including myasthenia gravis (MG), a rare neuromuscular autoimmune disease that causes debilitating and, in its generalized form (gMG), potentially life-threatening muscle weakness. IgG autoantibodies are directly pathogenic in MG and target neuromuscular junction proteins, causing neuromuscular transmission failure. Treatment approaches that reduce autoantibody levels, such as therapeutic plasma exchange and intravenous immunoglobulin, have been shown to be effective for gMG patients but are not indicated as ongoing maintenance therapies and can be associated with burdensome side effects. Agents that block FcRn-mediated recycling of IgG represent a rational and promising approach for the treatment of gMG. Blocking FcRn allows targeted reduction of all IgG subtypes without decreasing concentrations of other Ig isotypes; therefore, FcRn blocking could be a safe and effective treatment strategy for a broad population of gMG patients. Several FcRn-blocking antibodies and one antibody Fc fragment have been developed and are currently in various stages of clinical development. This article describes the mechanism of FcRn blockade as a novel approach for IgG-mediated disease therapy and reviews promising clinical data using such FcRn blockers for the treatment of gMG.
Glucocorticoids for therapeutic immunosuppression: Clinical pearls for the practicing neurologist
AlexandraGalatiaE. SherwoodBrownbRileyBoveaAnandVaidyacJeffreyGelfanda
doi : 10.1016/j.jns.2021.120004
Volume 430, 15 November 2021, 120004
Given widespread use of glucocorticoid therapy in neurologic disease, understanding glucocorticoid pharmacology and risk is paramount for the practicing neurologist. While dosing and tapering regimens vary depending on the neurological disease and indication being treated, there are important general principles of glucocorticoid prescribing and monitoring that can guide clinical decision-making. Glucocorticoid-related toxicities can occur across multiple organ systems, including hypertension; dyslipidemia; weight gain; hyperglycemia; osteoporosis and avascular necrosis; myopathy; gastrointestinal bleeding; infection; and neuropsychiatric effects with sleep, mood disturbance and cognition. This narrative review provides a practical framework for safe and responsible prescribing of this therapeutic class of medications, including appreciation of immunosuppressive consequences, risk mitigation strategies, dosing and tapering, and recognition of adrenal insufficiency and glucocorticoid withdrawal.
Consensus on the treatment of dysphagia in Parkinson's disease
AntonioSchindlera1NicolePizzornia1EmanueleCeredabGiuseppeCosentinocdMicolAvenaliceCristinaMontomolifGiovanniAbbruzzesegAngeloAntoninihFilippoBarbieraiMarcoBenazzojEduardoBenarrochkGiuliaBertinojPereClavèlmPietroCortellinoRobertoEleoprapChiaraFerrariaShaheenHamdyqMaggie-LeeHuckabeerLeonardoLopianosRosarioMarchese-RagonatStefanoMasierouEmiliaMichouvAntonioOcchiniiClaudioPacchettiwRonald F.PfeifferxDomenico A.RestivoyMariangelaRondanellizagGiovanniRuoppoloaaGiorgioSandrinicAnthonySchapiraabFabrizioStocchiacEduardoTolosaadFrancescaValentinowMauroZamboniaeRobertaZangagliawMarioZappiaafCristinaTassorelliceEnricoAlfonsid
doi : 10.1016/j.jns.2021.120008
Volume 430, 15 November 2021, 120008
Dysphagia is common in Parkinson's disease (PD). The effects of antiparkinsonian drugs on dysphagia are controversial. Several treatments for dysphagia are available but there is no consensus on their efficacy in PD.
Psychiatric sequelae of stroke affecting the non-dominant cerebral hemisphere
CynthiaGuoaChristophStretzbcJordan RoderickAndersondNadaEl-HusseinieBrianMac GroryeBlakeWernerfStephanieYarnellf
doi : 10.1016/j.jns.2021.120007
Volume 430, 15 November 2021, 120007
There are a plethora of cognitive sequelae in addition to neglect and extinction that arise with unilateral right hemispheric stroke (RHS). Cognitive deficits following non-dominant (right) hemisphere stroke are common with unilateral neglect and extinction being the most recognized examples. The severity of RHS is usually underestimated by the National Institutes of Health Stroke Scale (NIHSS), which in terms of lateralized right hemisphere cognitive deficits, tests only for visual inattention/extinction. They account for 2 out of 42 total possible points. Additional neuropsychiatric sequelae include but are not limited to deficiencies in affective prosody comprehension and production (aprosodias), understanding and expressing facial emotions, empathy, recognition of familiar faces, anxiety, mania, apathy, and psychosis. These sequelae have a profound impact on patients' quality of life; affecting communication, interpersonal relationships, and the ability to fulfill social roles. They also pose additional challenges to recovery. There is presently a gap in the literature regarding a cohesive overview of the significant cognitive sequelae following RHS. This paper serves as a narrative survey of the current understanding of the subject, with particular emphasis on neuropsychiatric poststroke syndromes not predominantly associated with left hemisphere lesions (LHL), bilateral lesions, hemiplegia, or paralysis. A more comprehensive understanding of the neuropsychological consequences of RHS extending beyond the typical associations of unilateral neglect and extinction may have important implications for clinical practice, including the ways in which clinicians approach diagnostics, treatment, and rehabilitation.
Insight in booster COVID-19 vaccine and disease modifying therapy in multiple sclerosis
KatherineBeardabShitizSriwastavaabc
doi : 10.1016/j.jns.2021.120034
Volume 430, 15 November 2021, 120034
Sonographic and ophthalmic assessment of optic nerve in patients with idiopathic intracranial hypertension: A longitudinal study
S.KnodelaS.N.RoemeraK.MoslemanibA.WykrotabB.Käsmann-KellnerbB.SeitzbG.WagenpfeilcK.FassbenderaA.NaldidS.KalampokinieP.Lochnera
doi : 10.1016/j.jns.2021.118069
Volume 430, 15 November 2021, 118069
To assess the validity of neurosonological parameters (transorbital sonography (TOS)) for detection and monitoring of patients with idiopathic intracranial hypertension (IIH).
Biomarkers of kidney function and cognitive ability: A Mendelian randomization study
Erin L.RichardabLinda K.McEvoybcSteven Y.CaobEyalOrendJohn E.AlcarazdAndrea Z.LaCroixbRany M.Salemb
doi : 10.1016/j.jns.2021.118071
Volume 430, 15 November 2021, 118071
Estimated glomerular filtration rate (eGFR), albuminuria and serum uric acid (SUA) are markers of kidney function that have been associated with cognitive ability. However, whether these associations are causal is unclear.
The level of urbanization influences acute ischemic stroke care: A nationwide ecological study from Germany
DanielRichteraJensEydingbcRalphWeberbdDirkBartigaBjörnMisselwitzeArminGraufChristosKrogiasab
doi : 10.1016/j.jns.2021.119983
Volume 430, 15 November 2021, 119983
Regional differences in acute ischemic stroke (AIS) care are still reported for Germany but not fully understood. We aimed to analyze the impact of the level of urbanization on the application of proved treatment procedures and the incidence of hospitalized AIS cases in Germany.
Novel monoallelic variant in ERLIN2 causes spastic paraplegia converted to amyotrophic lateral sclerosis
KodaiKumeaMasakiKamadabYoshimitsuShimatanicdTadayukiTakataeYuishinIzumicHideshiKawakamia
doi : 10.1016/j.jns.2021.119984
Volume 430, 15 November 2021, 119984
Long-term outcomes in patients presenting with optic neuritis: Analyses of the MSBase registry
RachelKenneyabMenglingLiubSachiPatilaRaedAlroughanicRadekAmpapadRobertoBergamaschieCavitBozfHelmutButzkuevengJose CabreraGomezhElisabettaCartechiniiSara EichauMadueñojDianaFerrarokFrancoisGrand-MaisonlFrancoGranellamDanaHorakovanGuillermoIzquierdo AyusojTomasKalincikoJanaLizrova PreiningerovapAlessandraLugaresiqrMarcoOnofrjsSerkanOzakbastFrancescoPattiuPatriziaSolavAysunSoysalwDaniele Litterio A.SpitalerixMuratTerziyRecaiTurkogluzVincentvan PeschaaShivSaidhaabLorna E.ThorpebSteven L.GalettaaacLaura J.BalcerabacIlyaKisteraTimSpelmanadaethe MSBase Study Group
doi : 10.1016/j.jns.2021.118067
Volume 430, 15 November 2021, 118067
Short-term outcomes of optic neuritis (ON) have been well characterized. Limited data exists on longer-term visual outcomes in patients who present with ON. The large MSBase registry allows for characterization of long-term visual outcomes after ON.
Investigating RFC1 expansions in sporadic amyotrophic lateral sclerosis
YevgenyaAbramzonab1RamitaDewana1AndreaCortesedeSusanResnickfLuigiFerruccigHenryHouldendBryan J.Traynorabc
doi : 10.1016/j.jns.2021.118061
Volume 430, 15 November 2021, 118061
A homozygous AAGGG repeat expansion within the RFC1 gene was recently described as a common cause of CANVAS syndrome. We examined 1069 sporadic ALS patients for the presence of this repeat expansion. We did not discover any carriers of the homozygous AAGGG expansion in our ALS cohort, indicating that this form of RFC1 repeat expansions is not a common cause of sporadic ALS. However, our study did identify a novel repeat conformation and further expanded on the highly polymorphic nature of the RFC1 locus.
Post-COVID-19 opsoclonus-myoclonus syndrome and encephalopathy associated with leucine-rich glioma-inactivated 1 (LGI-1) antibodies
DuncanSmythabKaung MyatKyawcAmyLegisterdGeorgeMacFarlanedUdayaraj UmaSankardMehoolPateldChrisCloughcAngelaKulendrandEoinMulroyab
doi : 10.1016/j.jns.2021.119982
Volume 430, 15 November 2021, 119982
Impact of systemic inflammatory response syndrome on acute ischemic stroke patients treated with mechanical thrombectomy
MostafaJafaria1KalmanKatlowitzb1CarlosDe la GarzaaAlexanderSellersaShawnMooreaHaydenHallaAaronDesaiaVikramjeetSinghcRahulDamaniab
doi : 10.1016/j.jns.2021.119988
Volume 430, 15 November 2021, 119988
Systemic inflammatory response syndrome (SIRS) has been associated with poor outcomes after acute ischemic stroke (AIS). The primary goal of this study was to determine whether SIRS status on admission correlated with functional outcomes in AIS treated with mechanical thrombectomy (MT).
Color-coded multiphase computed tomography angiography may predict outcome in anterior circulation acute ischemic stroke
FabioPilatoa1TommasoVerdolottib1RosalindaCalandrellibIacopoValentebEdoardoMonellicSimoneCottonarodFioravanteCaponeaFrancescoMotoleseaGianmarcoIaccarinoaSergio SoerenRossiaCesareColosimobcVincenzoDi Lazzaroa
doi : 10.1016/j.jns.2021.119989
Volume 430, 15 November 2021, 119989
To evaluate whether arterial and venous color-coded mCTA score may predict clinical outcome in anterior circulation acute ischemic stroke.
Genetic and demographic predisposing factors associated with pediatric sleepwalking in the Philadelphia Neurodevelopmental Cohort
YuheiChibaadehOwen R.PhillipsbfShinichiTakenoshitaagHanna M.OllilacJoachim F.HallmayerbSeijiNishinoaManpreet K.Singhb
doi : 10.1016/j.jns.2021.119997
Volume 430, 15 November 2021, 119997
Sleepwalking is a parasomnia associated with non-rapid eye movement (NREM) sleep and is formally diagnosed using polysomnography (PSG). However, PSG are difficult to perform on children or adolescents due to needed compliance. To understand this condition in youth, few studies have been conducted on a large cohort of youths with a diverse distribution of ages and races to characterize it better in the absence of PSG. The present study aimed to evaluate the prevalence of sleepwalking in youth, as well as associated demographic and genetic characteristics, using questionnaires in a large pediatric cohort.
The association between developing Parkinson's disease and ?-Adrenoceptor acting agents use: A systematic review and meta-analysis
WeerawatSaengphatrachaiKulsatreePraditukritWeerapatOwattanapanichYuvadeePitakpatapeePrachayaSrivanitchapoom
doi : 10.1016/j.jns.2021.120009
Volume 430, 15 November 2021, 120009
Parkinson's disease (PD) ranks the second most common neurodegenerative disease. Aside from genetic predisposition, many external factors such as traumatic brain injury and exposure of substances including pesticides also contribute to PD's pathogenesis. Many previous studies observed the association between the use of ?-adrenoceptor acting agents and risk of PD.
Accuracy and outcomes of diffusion tensor imaging tractography in resection for vestibular schwannoma for facial nerve preservation
NolanUngaPanayotis E.PelargosaKhashayarMozaffariaMonicaMathuraCourtneyDuongaLawrance K.ChungaKevinDingaNikhilesh S.BhattaNatalieBarnetteaBrittanyVothaQuintonGopencdeIsaacYangabcdefg
doi : 10.1016/j.jns.2021.120006
Volume 430, 15 November 2021, 120006
Impairment of facial nerve (FN) function is a common postoperative complication in surgical resections of Vestibular Schwannomas (VS). Diffusion tensor imaging (DTI) tractography creates in vivo imaging of the anatomical location of white matter tracts that can be preoperatively used to visualize the displaced FN. We present an analysis of patients who underwent DTI tractography imaging prior to VS resection.
Frontal lobe dysfunction is associated with reduced DAT-SPECT accumulation in Lewy body disease
AtsuoNakaharaab1RenpeiSengokuab1TadashiUmeharabHiromasaMatsunobMikihiroYamazakiabHisayoshiOkaab
doi : 10.1016/j.jns.2021.119998
Volume 430, 15 November 2021, 119998
Lewy body disease (LBD) causes olfactory or cognitive dysfunction even before motor symptoms emerge. Recent reports indicate that the dopamine transporter (DAT), which can be imaged using single-photon emission computed tomography (123I-ioflupane SPECT), is related to olfactory and cognitive dysfunction in LBD patients. We suspected that decreased cerebral blood flow (CBF) in the frontal lobe might be involved in these relationships. If so, then the results of these examinations may be useful in assessing the pathological progression of Lewy bodies.
MRI findings at neurological onset predict neurological prognosis in hemorrhagic shock and encephalopathy syndrome
IchiroKukiaTakeshiInoueaMegumiNukuiaShinOkazakiaHisashiKawawakiaJunichiIshikawabdKiyokoAmobMasaoTogawabAtsushiUjirocHiroshiRinkadMasashiShiomie
doi : 10.1016/j.jns.2021.120010
Volume 430, 15 November 2021, 120010
Hemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease and has an uncertain pathogenesis. The aim of this study was to predict neurological outcomes for HSES using magnetic resonance imaging (MRI) findings at neurological onset and elucidate the pathophysiology of HSES in the acute phase from serial MRI changes.
Clinical comorbidities correlated with a response to the cerebrospinal fluid tap test in idiopathic normal-pressure hydrocephalus
HiroyukiNaitoaTakamichiSugimotoabKazukiKimotoaTakafumiAbeaTomohitoKawanoaChikaMatsuokaaNarumiOhnoaMayumiGigaaTomoyukiKonoaHirokiUenoaEiichiNomuraa
doi : 10.1016/j.jns.2021.120024
Volume 430, 15 November 2021, 120024
The mainstay of treatment for idiopathic normal-pressure hydrocephalus (iNPH) is spinal fluid shunting. A tap test (TT) is recommended as an indication of shunting. Patients with iNPH are often elderly and have multiple comorbidities affecting the shunting outcome. We investigated the factors affecting TT in patients with iNPH.
Zonisamide improves wearing off in Parkinson's disease without exacerbating dyskinesia: Post hoc analysis of phase 2 and phase 3 clinical trials
YoshioTsuboiaMasatoshiNakamurabHidenoriMaruyamacYujiMatsumotoc
doi : 10.1016/j.jns.2021.120026
Volume 430, 15 November 2021, 120026
Although phase 2 and 3 clinical trials in Japan showed that zonisamide improved wearing off in patients with Parkinson's disease (PD), no studies to date have evaluated whether zonisamide improves wearing off in patients with PD without exacerbating dyskinesia. Therefore, we examined this hypothesis in a post hoc analysis of pooled data from the previous phase 2 and 3 trials.
Intrathecal inflammatory responses in the absence of SARS-CoV-2 nucleic acid in the CSF of COVID-19 hospitalized patients
EricaNormandinab1Kathryn B.Holroydc1Sarah I.CollenscBennett M.ShawadKatherine J.SiddleabGordonAdamsaMelissaRudyaIsaac H.SolomoneMelis N.AnahtarfJacob E.LemieuxadBianca A.TrombettacPiaKivisakkcSteven E.ArnoldcOttoRapalinogAnne L.PiantadosihPrithaSendEric S.RosenbergdJohnBrandafPardis C.Sabetiabijk1Shibani S.Mukerjic1
doi : 10.1016/j.jns.2021.120023
Volume 430, 15 November 2021, 120023
Little is known about CSF profiles in patients with acute COVID-19 infection and neurological symptoms. Here, CSF was tested for SARS-CoV-2 RNA and inflammatory cytokines and chemokines and compared to controls and patients with known neurotropic pathogens.
Hospital-based observational study of neurological disorders in patients recently vaccinated with COVID-19 mRNA vaccines
Jasmine ShiminKohaRebecca Hui MinHoea1Ming HuiYongb1Hui JinChiewa1YihuiGohc1Kok PinYongbd1Tian MingTuaeDerrick Wei ShihChanfBenjamin Yong-QiangTancLeonard Leong LittYeocAmy May LinQuekcIsabelSiowgMonicaSainiaJasmynAngonhSharminiRathakrishnanhGeraldine JiangyanCheniAndrew Che-FaiHuijAftabAhmadjMoe PearlShwejKevinTanaRaymond Chee SeongSeetcMingshiCaikSi MinKuokLing LiFookThirugnanamUmapathia
doi : 10.1016/j.jns.2021.120030
Volume 430, 15 November 2021, 120030
We describe the spectrum of acute neurological disorders among hospitalized patients who recently had COVID-19 mRNA vaccination.
Investigating the neuroanatomy underlying proprioception using a stroke model
Matthew J.ChilversaRachel L.HaweabStephen H.ScottcSean P.Dukelowa
doi : 10.1016/j.jns.2021.120029
Volume 430, 15 November 2021, 120029
Neuroanatomical investigations have associated cortical areas, beyond Primary Somatosensory Cortex (S1), with impaired proprioception. Cortical regions have included temporoparietal (TP) regions (supramarginal gyrus, superior temporal gyrus, Heschl's gyrus) and insula. Previous approaches have struggled to account for concurrent damage across multiple brain regions. Here, we used a targeted lesion analysis approach to examine the impact of specific combinations of cortical and sub-cortical lesions and quantified the prevalence of proprioceptive impairments when different regions are damaged or spared.
White matter changes in drug-naïve Parkinson's disease patients with impulse control & probable REM sleep behavior disorders
MahsaDolatshahiabAmirAshraf-GanjoueiaI-WeiWucYuZhangdMohammad HadiAarabieDuyguTosuncfor the Parkinson's Progression Marker Initiative
doi : 10.1016/j.jns.2021.120032
Volume 430, 15 November 2021, 120032
According to epidemiological studies, Parkinson's disease (PD) patients with probable REM sleep behavior disorder (pRBD) are more prone to develop impulse control disorders (ICDs), which is shown to be present in drug-naïve PD patients, and vice versa.
Optic perineuritis: A Canadian case series and literature review
Jim ShenchuXieaLauraDonaldsonbEdwardMargolinbc
doi : 10.1016/j.jns.2021.120035
Volume 430, 15 November 2021, 120035
Optic perineuritis (OPN) is a rare orbital inflammatory disorder that primarily involves the optic nerve sheath (ONS). This study aimed to describe the clinical features of idiopathic OPN as well as OPN secondary to other infectious and inflammatory conditions in a Canadian case series as current literature on secondary OPN in Western countries is mostly limited to case reports.
The diagnostic spectrum of ATP1A3-related disorders: 3 new patients
Daniel N.Laxa1PhyllisBieribPujaPatela
doi : 10.1016/j.jns.2021.120003
Volume 430, 15 November 2021, 120003
ATP1A3-related disorders are rare but increasingly recognized syndromes with overlapping phenotypes.
Beyond electives: Rethinking training in global neurology
MathuraRavishankaraAaron L.Berkowitzb
doi : 10.1016/j.jns.2021.120025
Volume 430, 15 November 2021, 120025
Global health electives in neurology residencies provide opportunities for high-income country trainees, but have limited benefits–and may create burdens–for lower-income country hosts. Current suspension of global health electives for U.S. neurology residents due to the COVID-19 pandemic provides an opportunity to reflect on ways to reimagine global health education during this period and beyond. The framework proposed in this article underscores the need for equitable, bidirectional international partnerships and highlights global health educational innovations developed during the pandemic.
A Chinese Han pedigree with Huntington disease mimicking spinocerebellar ataxia
ChengyuanMaoabc1YunSuabc1HuiyunWangd1LiyuanFanabcHuiminZhengabcTaiWangabXinweiLiabcShuoZhangabcZhengweiHuabcHaiyangLuoabcJingYangabcChangheShiacYumingXuace
doi : 10.1016/j.jns.2021.119985
Volume 430, 15 November 2021, 119985
Asymptomatic retinal vasculopathy in neuropsychiatric systemic lupus erythematosus
Peter V.SguignaaLaurenTardoaDarrelCongeraRobertHoganabcdBenjamin M.Greenberga
doi : 10.1016/j.jns.2021.118053
Volume 430, 15 November 2021, 118053
Multiple retinal aneurysms in probable cerebral amyloid angiopathy
panelAmelieScharfaNils G.MargrafbJanTodecUlfJensen-Konderingde
doi : 10.1016/j.jns.2021.120005
Volume 430, 15 November 2021, 120005
Neuropathy due to coexistent vitamin B12 and B6 deficiencies in patients with Parkinson's disease: A case series
RyanCanissarioaMichaelStantonaJoseph S.ModicaaEric L.LogigianaKarlo J.Lizarragaab
doi : 10.1016/j.jns.2021.120028
Volume 430, 15 November 2021, 120028
Mutation analysis of the ATP13A2 gene in patients with PD and MSA from Italy
MonicaGagliardiaRadhaProcopiobGiuseppeNicoletticMaurizioMorellibLauraBrighinadAndreaQuattronebGiuseppeBonapaceeDonatellaMalangafgAldoQuattronechGraziaAnnesia
doi : 10.1016/j.jns.2021.120031
Volume 430, 15 November 2021, 120031
Cross-sectional associations of cerebrospinal ?-synuclein, tau and amyloid-? with dyskinesias, motor fluctuations, non-motor symptoms in a cohort of Parkinson's disease patients
DianaEstelleraRalucaModreanuabSonia CatalinaCerquera-ClevesacAlmudenaSánchez-GómezaManelFernándezaAnaCámaraaYaroslauComptaa
doi : 10.1016/j.jns.2021.120033
Volume 430, 15 November 2021, 120033
