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Approach to patient with unilateral optic disc edema and normal visual function
Laura Donaldson,Edward Margolin
doi : 10.1016/j.jns.2021.117414
VOLUME 424, 117414, MAY 15, 2021
In patients with swollen optic nerve head and normal visual function, optic disc drusen (ODD) is the most common diagnosis. The best tests for detecting ODD are funds autofluorescence and enhanced-depth imaging ocular coherence tomography (EDIOCT). After ODD has been ruled out, asymmetric papilledema should be assumed to be the cause and MRI of the brain and orbits with contrast and venography should be performed in all patients.
Roles of inflammation in the natural history of intracranial saccular aneurysms
Jienan Wang,Liming Wei,Haitao Lu,Yueqi Zhu
doi : 10.1016/j.jns.2020.117294
VOLUME 424, 117294, MAY 15, 2021
Aneurysmal subarachnoid hemorrhage is caused by intracranial aneurysm (IA) rupture and results in high rates of mortality and morbidity. Factors contributing to IA generation, growth and rupture can involve genetics, injury, hemodynamics, environmental factors, and inflammation, in which inflammatory factors are believed to play central roles in the whole natural history.
A systematic review of stereotactic radiofrequency ablation for hypothalamic hamartomas
Aditya M. Kondajji,Audree Evans,Meachelle Lum,...Courtney Duong,Kunal Patel,Isaac Yang
doi : 10.1016/j.jns.2021.117428
VOLUME 424, 117428, MAY 15, 2021
The seizure activity associated with hypothalamic hamartomas (HHs) is refractory to medical management and surgical intervention is often required. Stereotactic Radiofrequency Ablation (SRFA) is a minimally invasive technique offering targeted lesion ablation with a reduced risk of complications.
Rating scales and biomarkers for CAG-repeat spinocerebellar ataxias: Implications for therapy development
Meng-Ling Chen,Chih-Chun Lin,Liana S. Rosenthal,Puneet Opal,Sheng-Han Kuo
doi : 10.1016/j.jns.2021.117417
VOLUME 424, 117417, MAY 15, 2021
Spinocerebellar ataxias (SCAs) are a group of dominantly-inherited cerebellar ataxias, among which CAG expansion-related SCAs are most common. These diseases have very high penetrance with defined disease progression, and emerging therapies are being developed to provide either symptomatic or disease-modifying benefits.
MRI activity and extended interval of Natalizumab dosing regimen: a multicentre Italian study
Stefania Federica De Mercanti,Alessio Signori,Cinzia Cordioli,...Ruggero Capra,Maria Pia Sormani,Marinella Clerico
doi : 10.1016/j.jns.2021.117385
VOLUME 424, 117385, MAY 15, 2021
To minimize the risk of Progressive Multifocal Leukoencephalopathy and rebound in JCV-positive multiple sclerosis (MS) patients after 24 natalizumab doses, it has been proposed to extend the administrations interval. The objective is to evaluate the EID efficacy on MRI activity compared with the standard interval dosing (SID).
Risk factors and outcomes of hospitalized stroke patients in Lusaka, Zambia
Aparna Nutakki,Mashina Chomba,Lorraine Chishimba,...Rebecca F. Gottesman,Mona N. Bahouth,Deanna Saylor
doi : 10.1016/j.jns.2021.117404
VOLUME 424, 117404, MAY 15, 2021
Limited data exists about stroke risk factors and outcomes in sub-Saharan African countries, including Zambia. We aim to fill this gap by describing features of hospitalized stroke patients at University Teaching Hospital (UTH), the national referral hospital in Lusaka, Zambia.
Angular vestibulo ocular reflex loss with preserved saccular function in Machado-Joseph disease
Dario Geisinger,Zohar Elyoseph,Roy Zaltzman,Matti Mintz,Carlos R. Gordon
doi : 10.1016/j.jns.2021.117393
VOLUME 424, 117393, MAY 15, 2021
To provide a comprehensive evaluation of the vestibular function in Machado-Joseph Disease (MJD).
Autoimmune encephalitis after Japanese encephalitis in children: A prospective study
Benke Liu,Jie Liu,Hong Sun,...Hengsheng Chen,Jiannan Ma,Li Jiang
doi : 10.1016/j.jns.2021.117394
VOLUME 424, 117394, MAY 15, 2021
To explore anti-neuronal surface antibodies and identify associated serum predictors of autoimmune encephalitis after Japanese encephalitis (JE).
Italian cohort of Lafora disease: Clinical features, disease evolution, and genotype-phenotype correlations
Antonella Riva,Alessandro Orsini,Marcello Scala,...Berge Arakel Minassian,Pasquale Striano, on behalf of Italian League Against Epilepsy Genetic Commission
doi : 10.1016/j.jns.2021.117409
VOLUME 424, 117409, MAY 15, 2021
Lafora disease (LD) is characterized by progressive myoclonus, refractory epilepsy, and cognitive deterioration. This complex neurodegenerative condition is caused by pathogenic variants in EPM2A/EPM2B genes, encoding two essential glycogen metabolism enzymes known as laforin and malin. Long-term follow-up data are lacking. We describe the clinical features and genetic findings of a cohort of 26 Italian patients with a long clinical follow-up.
Atherosclerotic event risk and risk reduction therapies among Ghanaian hemorrhagic stroke survivors
Fred Stephen Sarfo,Sheila Adamu,Vida Obese,Manolo Agbenorku,Priscilla Abrafi Opare-Addo,Bruce Ovbiagele
doi : 10.1016/j.jns.2021.117389
VOLUME 424, 117389, MAY 15, 2021
Intracerebral hemorrhage (ICH) stroke constitute up to 40% of incident strokes in Africa. While ICH patients are at high risk for atherosclerotic events, the risk-benefit of anti-atherosclerotic therapies in this patient population is uncertain.
Adherence to anticoagulant guideline for atrial fibrillation: A large care gap among stroke patients in a rural population
Johan Diaz,Eric Koza,Durgesh Chaudhary,...Paul Griffin,Vida Abedi,Ramin Zand
doi : 10.1016/j.jns.2021.117410
VOLUME 424, 117410, MAY 15, 2021
This study aimed to investigate the prevalence and factors associated with oral anticoagulant undertreatment of atrial fibrillation (AF) among a cohort of rural patients with stroke outcomes and examine how undertreatment may influence a patient's one-year survival after stroke.
Calcium Channel blockers are associated with reduced risk of Parkinson's disease in patients with hypertension: A population-based retrospective cohort study
Yuan-Fu Tseng,Hsiu-Chen Lin,Jane Chen-Jui Chao,Chien-Yeh Hsu,Hsiu-Li Lin
doi : 10.1016/j.jns.2021.117412
VOLUME 424, 117412, MAY 15, 2021
The use of dihydropyridine calcium channel blockers (DCCBs) was proposed to reduce the risk of Parkinson's disease (PD). This study aimed to evaluate the association between DCCB and its dose effect and the risk of PD in patients with newly diagnosed hypertension.
A novel PHKA1 mutation associating myopathy and cognitive impairment: Expanding the spectrum of phosphorylase kinase b (PhK) deficiency
Michela Bisciglia,Roseline Froissart,Anne Laure Bedat-Millet,...Jean-Yves Hogrel,François M. Petit,Tanya Stojkovic
doi : 10.1016/j.jns.2021.117391
VOLUME 424, 117391, MAY 15, 2021
Muscle phosphorylase kinase b deficiency (PhK) is a rare disorder of glycogen metabolism characterized by exercise-induced myalgia and cramps, myoglobinuria and progressive muscle weakness. PhK deficiency is due to mutations in the PHKA1 gene inherited in an X-linked manner and is associated to glycogenosis type VIII (GSD VIII also called GSD IXd). PHKA1 gene codes for the ?M subunit of the PhK, a multimeric protein complex responsible for the control of glycogen breakdown in muscle.
Non-white cases of sporadic Creutzfeldt-Jakob disease: A 28 year review of United Kingdom National Surveillance Data
Gavin Langlands,Jan Mackenzie,Cat Graham,...Alison Green,Colin Smith,Suvankar Pal
doi : 10.1016/j.jns.2021.117416
VOLUME 424, 117416, MAY 15, 2021
Descriptions of sporadic Creutzfeldt-Jakob disease (sCJD) in non-White populations are limited. Improved understanding may aid diagnoses and case ascertainment within surveillance programmes. We aimed to: 1) Ascertain the proportion of sCJD cases with non-White ethnicity in the United Kingdom (UK); 2) Compare clinical and investigation findings between non-White and White cases.
Cerebral adaptation to lower motor neuron injury
Pierre-Francois Pradat
doi : 10.1016/j.jns.2021.117360
VOLUME 424, 117360, MAY 15, 2021
Increased cerebral integrity metrics in poliomyelitis survivors: putative adaptation to longstanding lower motor neuron degeneration
Stacey Li Hi Shing,Jasmin Lope,Mary Clare McKenna,Rangariroyashe H. Chipika,Orla Hardiman,Peter Bede
doi : 10.1016/j.jns.2021.117361
VOLUME 424, 117361, MAY 15, 2021
Post-polio syndrome (PPS) has been traditionally considered a slowly progressive condition that affects poliomyelitis survivors decades after their initial infection. Cerebral changes in poliomyelitis survivors are poorly characterised and the few existing studies are strikingly conflicting.
A multicenter survey on access to care in Multiple Sclerosis-related trigeminal neuralgia
D. Ferraro,P. Annovazzi,R. Lanzillo,...E. Cocco,C. Gasperini,C. Solaro
doi : 10.1016/j.jns.2021.117430
VOLUME 424, 117430, MAY 15, 2021
The prevalence of trigeminal neuralgia (TN) in patients with Multiple Sclerosis (MS) is higher than in the general population and its management can be particularly challenging due to a number of reasons including high recurrence rates, lack of MS-specific treatment guidelines and uncertainties about pain pathophysiology.
Nitrous oxide-induced motor-predominant axonal peripheral neuropathy: A phenotype distinct from isolated vitamin B12 deficiency
Manon Lee,Ahmed Abbas,Omay Lee,Christopher J. Record,Kuven K. Moodley,Niranjanan Nirmalananthan
doi : 10.1016/j.jns.2021.117390
VOLUME 424, 117390, MAY 15, 2021
Assessment of vertical component of gaze deviation in acute PICA occlusion on Computed Tomography
Nan N. Jiang,Wei Wu
doi : 10.1016/j.jns.2021.117415
VOLUME 424, 117415, MAY 15, 2021
The prostate as a good candidate organ for proving amyloid deposition in non-endemic late onset hereditary transthyretin amyloidosis: Report of two cases
Kentaro Maeda,Yasuaki Kubota,Satoshi Kitagawa,Mitsuharu Ueda,Yukio Ando,Yasuhiro Ito
doi : 10.1016/j.jns.2021.117418
VOLUME 424, 117418, MAY 15, 2021
Re: Predictors and outcomes of hemorrhagic stroke in reversible cerebral vasoconstriction syndrome (Garg et al., Volume 421, 117312, February 15, 2021)
Aneesh B. Singhal
doi : 10.1016/j.jns.2021.117427
VOLUME 424, 117427, MAY 15, 2021
Reply to the letter to the editor regarding “predictors and outcomes of hemorrhagic stroke in reversible cerebral vasoconstriction syndrome”
Aayushi Garg,Santiago Ortega-Gutierrez
doi : 10.1016/j.jns.2021.117429
VOLUME 424, 117429, MAY 15, 2021
Rabies: Presentation, case management and therapy
Pasin Hemachudha,Thiravat Hemachudha
doi : 10.1016/j.jns.2021.117413
VOLUME 424, 117413, MAY 15, 2021
Several Lyssaviruses are known to be a causative agent of rabies and rabies like syndrome. There are no proven effective treatment strategies for symptomatic rabies patient. Risk of infection from dog variant of rabies virus is highest with deep bite reaching muscular layer and much higher when compared to scratch. Failure of viral eradication at the central nervous system (CNS) is partly due to inadequate immune response.
Neurological complications of cancer immunotherapy (CAR T cells)
Daniel B. Rubin,Henrikas Vaitkevicius
doi : 10.1016/j.jns.2021.117405
VOLUME 424, 117405, MAY 15, 2021
Chimeric antigen receptor (CAR) T cell therapy has become an indispensable tool in the treatment of advanced malignancy, however, it is associated with significant neurologic toxicity. The pathophysiology of CAR T-cell associated neurotoxicity is incompletely understood, and the specific risk factors have only recently begun to be characterized.
The neurology of lupus
John G. Hanly,Murat Inanç
doi : 10.1016/j.jns.2021.117419
VOLUME 424, 117419, MAY 15, 2021
No Abstract
The neurology of IGG4-related disease
G. Saitakis,B.K. Chwalisz
doi : 10.1016/j.jns.2021.117420
VOLUME 424, 117420, MAY 15, 2021
IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches.
Peripheral nervous system manifestations of rheumatological diseases
Kelly G. Gwathmey,Kelsey Satkowiak
doi : 10.1016/j.jns.2021.117421
VOLUME 424, 117421, MAY 15, 2021
Rheumatological diseases result in immune-mediated injury to not only connective tissue, but often components of the peripheral nervous system. These overlap conditions can be broadly categorized as peripheral neuropathies and overlap myositis. The peripheral neuropathies are distinctive as many have unusual presentations such as non-length-dependent, small fiber neuropathies and sensory neuronopathies (both due to dorsal root ganglia dysfunction), multiple mononeuropathies (e.g. vasculitic neuropathies), and even cranial neuropathies.
Primary central nervous system vasculitis – An update on diagnosis, differential diagnosis and treatment
Markus Kraemer,Peter Berlit
doi : 10.1016/j.jns.2021.117422
VOLUME 424, 117422, MAY 15, 2021
Primary angiitis of the central nervous system (PACNS) is a rare condition which is often misdiagnosed. In order to avoid mistakes in the management, a step by step approach is necessary.
CNS involvement in systemic vasculitides
Neil Scolding
doi : 10.1016/j.jns.2021.117423
VOLUME 424, 117423, MAY 15, 2021
Both the CNS and the PNS can be involved in almost all of the vasculitides – including the primary systemic vasculitic disorders, such as microscopic polyangiitis and polyarteritis nodosa, and in non-vasculitic systemic disorders, such as systemic lupus erythematosis and sarcoidosis.
Neurological complications of immune checkpoint inhibitor cancer immunotherapy
Cecilia Zivelonghi,Anastasia Zekeridou
doi : 10.1016/j.jns.2021.117424
VOLUME 424, 117424, MAY 15, 2021
Neurological autoimmunity is increasingly recognized as a complication of immune checkpoint inhibitor (ICI) cancer immunotherapy. ICIs act by enhancing endogenous anti-tumor immune responses and can also lead to autoimmunity affecting all organs. ICI-related neurological autoimmunity is rare, most often manifests with neuromuscular involvement and more rarely affects the central nervous system.
Cerebral amyloid angiopathy and related inflammatory disorders
B.K. Chwalisz
doi : 10.1016/j.jns.2021.117425
VOLUME 424, 117425, MAY 15, 2021
Inflammatory cerebral amyloid angiopathy is a largely reversible inflammatory vasculopathy that develops in an acute or subacute fashion in reaction to amyloid protein deposition in the central nervous system blood vessels. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Both variants produce a clinical picture that resembles primary angiitis of the CNS but is distinguished by a characteristic radiologic appearance.
Neurology of inflammatory bowel disease
José M. Ferro,Miguel Oliveira Santos
doi : 10.1016/j.jns.2021.117426
VOLUME 424, 117426, MAY 15, 2021
Inflammatory bowel diseases (IBD) are chronic inflammatory conditions affecting the digestive system, comprising two main distinctive entities, ulcerative colitis (UC) and Crohn's disease (CD). Besides gastrointestinal manifestations, IBD causes extraintestinal manifestations in the central and peripheral nervous system. The incidence of neurological complications in IBD ranges from 0.25% to 47.5%.
Corrigendum to ‘Neurology of COVID-19 in Singapore’ [Journal of the Neurological Sciences Volume 418, 15 November 2020, 117118]
Jasmine Shimin Koh,Deidre Anne De Silva,Amy May Lin Quek,...David Chien Boon Lye,Kevin Tan,Thirugnanam Umapathi
doi : 10.1016/j.jns.2021.117406
VOLUME 424, 117406, MAY 15, 2021
Corrigendum to’ Long-term outcomes of endovascular simple coiling versus neurosurgical clipping of unruptured intracranial aneurysms: A systematic review and meta-analysis’ [Journal of the Neurological Sciences Volume 422, 15 March 2021, 117338]
Christian H. Krag,Lasse Speiser,Rikke B. Dalby
doi : 10.1016/j.jns.2021.117411
VOLUME 424, 117411, MAY 15, 2021
Corrigendum to “Pneumonia risk and its associated factors in Parkinson's disease: A national database study” [Journal of the Neurological Sciences, Volume 415, 15 August 2020, 116949]
Jun Hee Won,Seong Jun Byun,Byung-Mo Oh,Han-Joon Kim,Sang Jun Park,Han Gil Seo
doi : 10.1016/j.jns.2021.117431
VOLUME 424, 117431, MAY 15, 2021
