The effects of multidisciplinary rehabilitation on neuroimaging, biological, cognitive and motor outcomes in individuals with premanifest Huntington's disease
Huntington's disease (HD) is a chronic, progressive neurodegenerative condition for which there are currently no proven disease-modifying therapies. Lifestyle factors have been shown to impact on the age of disease onset and progression of disease features. We therefore investigated the effects of a nine-month multidisciplinary rehabilitation intervention on neuroimaging, biological and clinical disease outcomes in individuals with premanifest HD. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 11, 2020 Category: Neurology Authors: Danielle M. Bartlett, Andrew Govus, Timothy Rankin, Amit Lampit, Kirk Feindel, Govinda Poudel, Wei-Peng Teo, Johnny Lo, Nellie Georgiou-Karistianis, Mel R. Ziman, Travis M. Cruickshank Source Type: research

Heart rate entropy is associated with mortality after intracereberal hemorrhage
Autonomic nervous system changes have been associated with outcome after intracerebral hemorrhage (ICH) previously. We aimed to investigate the association of heart rate entropy (HRE) with mortality after ICH. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 11, 2020 Category: Neurology Authors: Marek Sykora, Jozef Szabo, Pavel Siarnik, Peter Turcani, Stefan Krebs, Wilfried Lang, Marek Czosnyka, Peter Smielewski Source Type: research

Pseudotumor cerebri syndrome and essential thrombocythaemia: Case report and literature review
Letter to the Editor (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 10, 2020 Category: Neurology Authors: Michele Trimboli, Antonietta Romaniello, Letizia Troisi, Nicola Paciello Tags: Letter to the Editor Source Type: research

Cerebral hemorrhage related to vein thrombosis in Covid-19 patients in different Italian hospitals: View point for clinical and imaging implications
The Covid-19 pandemic have spread to 185 countries affecting almost 5 million patients worldwide with more than two million recovering and more than 300 thousand deaths globally as of 17th May [1]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 10, 2020 Category: Neurology Authors: R. Argir ò, C. Cirelli, A. Sgreccia, B. Del Sette, V. Da Ros Tags: Letter to the Editor Source Type: research

Cumulative effects of long-term treatment with abobotulinumtoxinA in cervical dystonia: Findings from a prospective, observational study
Patients with cervical dystonia (CD) require regular injections of botulinum neurotoxin type A (BoNT-A) to maintain treatment effectiveness. In this secondary analysis of a large routine-practice study, we assessed the response to abobotulinumtoxinA over repeated cycles and compared the responses of patients previously na ïve to BoNT treatment to those already treated with BoNT-A. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 9, 2020 Category: Neurology Authors: Carlo Colosimo, David Charles, Vijay P. Misra, Pascal Maisonobe, Savary Om, INTEREST IN CD2 study group Source Type: research

Posterior reversible encephalopathy syndrome in patients with COVID-19
To report four patients with coronavirus disease 2019 (COVID-19) who developed posterior reversible encephalopathy syndrome (PRES). (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 9, 2020 Category: Neurology Authors: Sarah C. Parauda, Virginia Gao, Alexandra N. Gewirtz, Neal S. Parikh, Alexander E. Merkler, Joshua Lantos, Halina White, Dana Leifer, Babak B. Navi, Alan Z. Segal Tags: Clinical short communication Source Type: research

Cerebral small vessel disease in community-dwelling older adults living in remote rural settings
In this study, we aimed to assess the burden of neuroimaging biomarkers of cSVD and its association with risk factors in community-dwelling older adults residing in rural Ecuador. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 8, 2020 Category: Neurology Authors: Oscar H. Del Brutto, Robertino M. Mera, Bettsy Y. Recalde, Victor J. Del Brutto Tags: Clinical short communication Source Type: research

False-positive anti-neuronal nuclear antibody type 1 in a patient with RFC1 repeat expansion: Preventing “phenotype creep” in autoimmune neurology
A 61-year-old man was referred for evaluation of anti-neuronal nuclear antibody type 1 (ANNA-1, or “anti-Hu”)-associated sensory neuronopathy. Past medical history was significant for a right subcortical stroke involving the subthalamic nucleus three years prior. Family history was unremarkable. He reported insidiously progressive gait imbalance over 15 years, and worsening balance with hea d movement. He also noted mild dysarthria and left hemiparesis following his stroke. He had undergone plasma exchange one year prior to referral without clinical improvement. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 8, 2020 Category: Neurology Authors: Adrian Budhram, John R. Mills, Kamal Shouman, P. James B. Dyck, Anhar Hassan, Nicholas L. Zalewski Tags: Letter to the Editor Source Type: research

Treatment of intracerebral hemorrhage: Current approaches and future directions
Intracerebral hemorrhage (ICH) stands out among strokes, both for the severely morbid outcomes it routinely produces, and for the striking deficiency of defenses possessed against the same. The brain damage caused by ICH proceeds through multiple pathophysiological mechanisms, broadly differentiated into those considered primary, arising from the hematoma itself, and the secondary consequences of hematoma presence and expansion thereof. A number of interventions against ICH and its sequelae have been investigated (e.g., hemostatic therapies, blood pressure control, hematoma evacuation, and a variety of neuroprotective stra...
Source: Journal of the Neurological Sciences - July 8, 2020 Category: Neurology Authors: Wenbo Zhao, Chuanjie Wu, Christopher Stone, Yuchuan Ding, Xunming Ji Tags: Review Article Source Type: research

Long-term safety and efficacy of safinamide as add-on therapy in levodopa-treated Japanese patients with Parkinson's disease with wearing-off: Results of an open-label study
This study aimed to confirm the long-term safety and efficacy of safinamide in Japanese PD patients with wearing-off. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 6, 2020 Category: Neurology Authors: Yoshio Tsuboi, Nobutaka Hattori, Akihiko Yamamoto, Yuji Sasagawa, Masahiro Nomoto, on behalf of the ME2125-4 Study Group Source Type: research

Reliability and validity of the multiple sclerosis resiliency scale (MSRS)
The objective of this study was to determine the internal reliability and construct validity of the Multiple Sclerosis Resiliency Scale (MSRS) in comparison with a common measure of global resilience. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 5, 2020 Category: Neurology Authors: Abbey J. Hughes, Krina Patel, Kathryn C. Fitzgerald, Aeysha Brown, Elizabeth S. Gromisch, Ellen M. Mowry Source Type: research

Galenic Pial arteriovenous fistulas in adults
Vein of Galen aneurysmal malformations (VOGMs) are pial arteriovenous fistulas possessing Galenic venous drainage most commonly presenting during the neonatal period and infancy, with initial discovery during adulthood quite rare. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 5, 2020 Category: Neurology Authors: Michael George Zaki Ghali, Ulf P. Arborelius, Erol Veznedaroglu, Robert F. Spetzler, M. Gazi Ya şargil, Vitaliy Marchenko Source Type: research

Correlations between COVID-19 and burden of dementia: An ecological study and review of literature
This study aims to investigate the associations between COVID-19 caseload and the burden of dementia. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 4, 2020 Category: Neurology Authors: M. Reza Azarpazhooh, Amin Amiri, Negar Morovatdar, Sandy Steinwender, Amir Rezaei Ardani, Nawaf Yassi, Jose Biller, Saverio Stranges, Masoud Tokazebani, Sepideh Kazemi Neya, Bita Khorram, Mohamad Sobhan Sheikh Andalibi, Shahram Arsang-Jang, Naghmeh Mokhbe Source Type: research

Seroconversion and indolent course of COVID-19 in patients with multiple sclerosis treated with fingolimod and teriflunomide
The recent pandemic of COVID 19, caused by SARS-CoV-2, has been confirmed on May 8th in more than 3.5 million cases worldwide, carrying a mortality of approximately 2 –7% [1]. The elderly and patients with comorbidities may develop a severe progression of COVID-19, even whether the rate risk in immunosuppressed is still unknown [2]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 3, 2020 Category: Neurology Authors: Bollo Luca, Guerra Tommaso, Bavaro Davide Fiore, Monno Laura, Saracino Annalisa, Angarano Gioacchino, Paolicelli Damiano, Trojano Maria, Iaffaldano Pietro Tags: Letter to the Editor Source Type: research

Good recanalization is associated with long term favorable outcomes in acute stroke patients with large vessel occlusion treated with endovascular therapy
The long-term outcome in acute stroke patients with large vessel occlusion (LVO) treated with endovascular therapy (EVT) are unclear. We investigated functional outcomes one year after EVT in acute stroke patients with LVO. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 2, 2020 Category: Neurology Authors: Masafumi Arakawa, Kentaro Suzuki, Akihito Kutsuna, Takehiro Katano, Takuya Kanamaru, Junya Aoki, Yuki Sakamoto, Satoshi Suda, Kazumi Kimura Source Type: research

Cerebral white matter tau-positive granular glial pathology as a characteristic pathological feature in long survivors of multiple system atrophy
It is unclear whether tau-positive granular glial pathology is a characteristic feature of MSA. We aimed to analyse the prevalence and significance of tau-positive granular glial pathology in MSA. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - July 2, 2020 Category: Neurology Authors: Taku Homma, Yoko Mochizuki, Shinsuke Tobisawa, Takashi Komori, Eiji Isozaki Source Type: research

The discerning eye of computer vision: Can it measure Parkinson's finger tap bradykinesia?
The worldwide prevalence of Parkinson's disease is increasing. There is urgent need for new tools to objectively measure the condition. Existing methods to record the cardinal motor feature of the condition, bradykinesia, using wearable sensors or smartphone apps have not reached large-scale, routine use. We evaluate new computer vision (artificial intelligence) technology, DeepLabCut, as a contactless method to quantify measures related to Parkinson's bradykinesia from smartphone videos of finger tapping. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 29, 2020 Category: Neurology Authors: Stefan Williams, Zhibin Zhao, Awais Hafeez, David C. Wong, Samuel D. Relton, Hui Fang, Jane E. Alty Source Type: research

Apathy and its impact on carer burden and psychological wellbeing in primary progressive aphasia
While patients with primary progressive aphasia (PPA) typically present with predominant language impairment, behavioural symptoms, such as apathy, are often under-recognised. We aimed to systematically characterise apathy across the three recognised subtypes of PPA, plus atypical right-lateralised presentations of semantic dementia, and to evaluate the impact of apathy on carer burden and psychological wellbeing. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 29, 2020 Category: Neurology Authors: Stephanie Wong, Muireann Irish, Masud Husain, John R. Hodges, Olivier Piguet, Fiona Kumfor Source Type: research

Novel quantitative approach for crossed cerebellar diaschisis detection in acute ischemic stroke using CT perfusion
Crossed cerebellar diaschisis (CCD) is a common finding in hyper-acute ischemic stroke, related to supratentorial dysfunction of the contralateral hemisphere. Several studies investigated a possible relationship between CCD and clinical outcomes but still no evidence emerged. We used a novel quantitative whole cerebellum analysis using CT perfusion (CTP) imaging to investigate the relationship between CCD and stroke severity, hypoperfused volume and outcome measures. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 29, 2020 Category: Neurology Authors: Marcello Naccarato, Milo š Ajčević, Giovanni Furlanis, Carlo Lugnan, Alex Buoite Stella, Ilario Scali, Paola Caruso, Lara Stragapede, Maja Ukmar, Paolo Manganotti Source Type: research

Neurological impact of emboli during adult cardiac surgery
This study draws on advances in Doppler ultrasound bubble sizing to investigate whether high volumes of macro-bubbles entering the brain during cardiac surgery increase the risk of new cerebral microbleeds (CMBs), ischemic MR lesions, or post-operative cognitive decline (POCD). (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 26, 2020 Category: Neurology Authors: Nikil Patel, Caroline Banahan, Justyna Janus, Mark A. Horsfield, Anthony Cox, David Marshall, Jordon Colman, John Morlese, David H. Evans, Claire Hannon, Vincent Egan, Peter Garrard, James P. Hague, Emma M.L. Chung Source Type: research

Neuroimaging findings in headache with normal neurologic examination: Systematic review and meta-analysis
To determine if pooled estimates of the prevalence of unexpected findings in patients with headache and normal neurologic examination support current expert opinion-based neuroimaging guidelines. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 26, 2020 Category: Neurology Authors: Joseph Kamtchum-Tatuene, Bernold Kenteu, Yannick F. Fogang, Joseline G. Zafack, Ulrich Flore Nyaga, Jean Jacques Noubiap Source Type: research

Efficacy and safety of sonothrombolysis in patients with acute ischemic stroke: A systematic review and meta-analysis
Accumulating clinical evidence has indicated that sonothrombolysis can aid in the treatment of ischemic stroke; however, these findings remain controversial. The purpose of the present meta-analysis was to assess randomized clinical studies concerning the effects of sonothrombolysis on ischemic stroke to evaluate its safety and efficacy. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 25, 2020 Category: Neurology Authors: Xiaoqiang Li, Hui Du, Zhibin Song, Hui Wang, Zhijian Tan, Mufang Xiao, Fu Zhang Tags: Review Article Source Type: research

Age-dependent efficacy of subthalamic nucleus deep brain stimulation in young- and late-onset Parkinson's disease based on a 10  year follow-up
Long-term efficacy and safety of subthalamic nucleus deep brain stimulation (STN DBS) in patients with young-onset Parkinson's disease (YOPD) and late-onset PD (LOPD) (i.e. motor symptom initial appearance at ages ≤40 and > 40 years, respectively) was compared to identify relationships between PD onset age and the efficacy of DBS. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 25, 2020 Category: Neurology Authors: Mi Sun Kim, Ho-Sung Ryu, Kye Won Park, Nari Choi, Sooyeoun You, Mi-Jung Kim, Young Jin Kim, Juyeon Kim, Kiju Kim, Sun Ju Chung Source Type: research

Orofacial dystonia and associated bulbar symptoms in multiple system atrophy: A blinded video analysis
We read with interest the article by Tongchuam and colleagues [1] on orofacial dystonia and associated bulbar symptoms in multiple system atrophy (MSA). Dystonia is an important but under-investigated symptom that may be present in both idiopathic Parkinson's disease and atypical parkinsonian conditions, including MSA [2]. Cranial dystonia, and in particular orofacial dystonia, is a relatively common manifestation in MSA, consisting of tonic spasms of the lower face and lips, and conveying an expression that resembles the so-called “tetanic risus sardonicus” [3]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 23, 2020 Category: Neurology Authors: Luca Marsili, Daniel D. Truong Tags: Editorial Source Type: research

World Brain Day 2020: Move together to end Parkinson's disease
Neurological disorders are the leading cause of disability worldwide [1]. The latest iteration of the global burden of neurological diseases (GBD) is a true reflection of the current global impact of neurological disorders [2]. The collaborators noted 9.0 million deaths (95% uncertainty interval [UI] 8.8 –9.4) and 16.5% (16.1–17.0) of global deaths due to neurological disorders, making them the second leading cause of death after heart disease [1]. It is worth noting that 276 million (247–308) disability-adjusted life years (DALYs) were caused by neurological disorders [1]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 23, 2020 Category: Neurology Authors: Tissa Wijeratne, Wolfgang Grisold, Claudia Trenkwalder, A.M. William Carroll Tags: Letter to the Editor Source Type: research

Peculiar muscle imaging findings in a patient with alphaB-crystallinopathy and axial myopathy
Myofibrillar myopathies (MFM) are muscle disorders characterized by myofibrillar disruption beginning at the Z-discs and abnormal accumulation of sarcoplasmic proteins [1]. Mutations in the gene encoding for alphaB-crystallin (CRYAB) are a rare cause of MFM. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 23, 2020 Category: Neurology Authors: Sara Bortolani, Fabiana Fattori, Mauro Monforte, Enzo Ricci, Giorgio Tasca Tags: Letter to the Editor Source Type: research

Influence of plasma matrix metalloproteinase levels on longitudinal changes in Alzheimer's disease (AD) biomarkers and cognitive function in patients with mild cognitive impairment due to AD registered in the Alzheimer's Disease Neuroimaging Initiative database
The present study investigated the effects of plasma matrix metalloproteinases (MMPs) on longitudinal changes in Alzheimer's disease (AD)-related biomarkers in cerebrospinal fluid (CSF), brain atrophy, and cognitive function in patients with mild cognitive impairment due to AD (MCI-AD). (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 22, 2020 Category: Neurology Authors: Kie Abe, Yuhei Chiba, Saki Hattori, Asuka Yoshimi, Takeshi Asami, Omi Katsuse, Akira Suda, Akitoyo Hishimoto, Alzheimer's Disease Neuroimaging Initiative Source Type: research

Severe visual loss after one episode of anti-MOG optic neuritis
We describe a case of optic neuritis secondary to myelin oligodendrocyte glycoprotein protein antibody (MOG-Ab) that has resulted in no light perception vision after the first attack. This case demonstrates that the visual outcome can be very poor in MOG-associated optic neuritis (MOG-ON) if treatment is not administered promptly. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 22, 2020 Category: Neurology Authors: Anna Kabanovski, Trishal Jeeva-Patel, Edward Margolin Tags: Letter to the Editor Source Type: research

The pressing questions in multiple sclerosis Care in the era of COVID-19
The rapidly evolving pandemic of the severe acute respiratory syndrome corona virus type-2 (SARS-CoV-2) commonly referred to as COVID-19 has raised many questions in the neuroimmunology clinic. The implications on MS patients are complex and multifaceted. Patients and providers share similar concerns and are challenged by this unprecedented situation. The Centers for Disease Control and Prevention (CDC) designated immunocompromised patients and those with disabilities as possible COVID-19 high-risk groups [1]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 22, 2020 Category: Neurology Authors: Hesham Abboud, Alessandro Serra Tags: Letter to the Editor Source Type: research

Excellent reliability of the ALSFRS-R administered via videoconferencing: A study of people with motor neuron disease in Scotland
Motor neuron disease is characterised by progressive weakness and disability, meaning that travel to specialist MND care and research participation can quickly become difficult. Many pwMND, particularly those living in remote areas, face troublesome commutes to access care and to participate in research. Travel-related restrictions have been shown to impact on attrition in clinical trials [1] and are likely to contribute to drop-out of clinic-based assessment. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 21, 2020 Category: Neurology Authors: Judith Newton, Kiran Jayaprakash, Stella A. Glasmacher, Alison McEleney, Andrew Bethell, Elizabeth Fraser, Janice Hatrick, Hanne Haagendruud, Gill Stott, Rachel Dakin, Siddharthan Chandran, Suvankar Pal Tags: Letter to the Editor Source Type: research

Association between PNPLA3 rs738409 G variant and MRI cerebrovascular disease biomarkers
Nonalcoholic fatty liver disease (NAFLD) has been associated with greater cerebral white matter hyperintensity (WMH) volume and microbleeds. The adiponutrin (PNPLA3) rs738409 G variant, a robust NAFLD susceptibility variant, has been variably associated with carotid atherosclerosis. We hypothesized that this variant is associated with WMH volume, microbleeds, covert brain infarction (CBI), and small perivascular spaces. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 19, 2020 Category: Neurology Authors: Neal S. Parikh, Nicole Dueker, Dalila Varela, Victor J. Del Brutto, Tatjana Rundek, Clinton B. Wright, Ralph L. Sacco, Mitchell S.V. Elkind, Jose Gutierrez Source Type: research

Hereditary spastic paraplegia type 11 (SPG11) is associated with obesity and hypothalamic damage
In this study, we investigate whether SPG11 pathogenic variants might affect metabolic regulation, leading to weight changes and if this could relate to hypothalamic damage. In this cross-sectional case-control study, we selected a group of individuals with confirmed SPG11 mutations (n  = 20), paired with healthy controls - both groups underwent brain MRI, from which we performed manual hypothalamic segmentation - and patients with Friedreich Ataxia (FRDA), having collected weight and height data for BMI-comparison. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 19, 2020 Category: Neurology Authors: Ana Luisa de Carvalho Cardozo Hern ández, Thiago Junqueira Ribeiro de Rezende, Marcondes Cavalcante França Tags: Clinical short communication Source Type: research

Orofacial dystonia and asssociated bulbar symptoms in multiple system atrophy: A blinded video analysis
Orofacial dystonia (OFD) is considered a supporting feature for a diagnosis of Multiple System Atrophy (MSA). However, the association of OFD with other adjacent symptoms has not been explored. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Yuwadee Thongchuam, Pattamon Panyakaew, Roongroj Bhidayasiri Tags: Clinical short communication Source Type: research

Arrival blood pressure in hypertensive and non-hypertensive spontaneous intracerebral hemorrhage
Hypertension is a known risk factor for intracerebral hemorrhage (ICH), but it is unclear whether blood pressure (BP) at hospital arrival can be used to distinguish hypertensive ICH from non-hypertensive etiologies. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Michael E. Reznik, Nasir Fakhri, Scott Moody, Kayleigh Murray, Samantha Costa, Shadi Yaghi, Matthew Schrag, Tracy E. Madsen, Tina M. Burton, Shawna Cutting, Ali Mahta, Linda C. Wendell, Bradford B. Thompson, Shyam S. Rao, Christoph Stretz, Karen L. Furie, Source Type: research

Clinical characteristics and brain MRI findings in myeloproliferative neoplasms
Myeloproliferative neoplasms (MPNs) including polycythemia vera (PV) and essential thrombocythemia (ET) have an increased risk of ischemic stroke. However, little is known about brain morphological changes and the cerebral vasculature in MPNs. The aim of the present study is to clarify the prevalence rates of brain infarcts (BIs) on magnetic resonance imaging (MRI) and to assess the detailed clinical and MRI characteristics in those patients. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Koichiro Nagai, Takashi Shimoyama, Hiroki Yamaguchi, Yuki Sakamoto, Satoshi Suda, Satoshi Wakita, Yasuhiro Nishiyama, Koiti Inokuchi, Kazumi Kimura Source Type: research

Therapeutic considerations in a case of progressive encephalomyelitis with rigidity and myoclonus
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an inflammatory disorder of the stiff-person syndrome spectrum that presents subacutely with trunk and limb rigidity, muscle spasms, brainstem dysfunction, and autonomic instability [1,2]. PERM has been strongly associated with IgG1 and IgG3 autoantibodies against the glycine receptor alpha-1 subunit (GlyR α1) [3,4]. Glycine receptors facilitate inhibitory signaling in the brainstem and spinal cord. Autoantibodies against GlyRα1 show pathogenic characteristics in vitro such as complement activation and receptor internalization [4]. (Source: Jou...
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Lauren Gluck, Amanda L. Hernandez, Sarah F. Wesley, Robert K. Fulbright, Erin E. Longbrake, Panos Stathopoulos Tags: Letter to the Editor Source Type: research

Huntington's disease: A new evidence of neurovascular dysfunction
Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder characterized by motor, cognitive and psychiatric abnormalities. The onset of the disease is usually in the third-fourth decade of life, however 5 –10% of subjects become symptomatic before 20 years (“juvenile Huntington's disease”). The disease is caused by an abnormal CAG repeat expansion in exon 1 of the “huntingtin” gene localized on the short arm of chromosome 4. This mutation leads to expression of the mutant huntingtin (mHtt ) protein which forms toxic intracellular aggregates that are ubiquitously found in ...
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Claudio Campa Tags: Letter to the Editor Source Type: research

The association of SARS-CoV-2 infection and acute disseminated encephalomyelitis without prominent clinical pulmonary symptoms
Dear Editor, (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 18, 2020 Category: Neurology Authors: Siamak Abdi, Askar Ghorbani, Farzad Fatehi Tags: Letter to the Editor Source Type: research

Accuracy of clinical scoring systems for the diagnosis of tuberculosis meningitis in a case mix of meningitides a retrospective cohort study
Tuberculosis meningitis (TBM) is elusive to diagnosis. Two widely used clinical scores are the Thwaites diagnostic score (TDS) and The Lancet Consensus score (LCS). We aim to evaluate the accuracy of these scores in a retrospective cohort of meningitis patients. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 14, 2020 Category: Neurology Authors: Yahia Z. Imam, Hassan Ahmedullah, Prem Chandra, Muna Almaslamani, Abdulatif Alkhal, Dirk Deleu Source Type: research

Serum neurofilament light chain studies in neurological disorders, hints for interpretation
Neurofilaments are cylindric proteins located in the dendrites, soma, and in particular axons of neurons, where they confer structural stability, promote axonal growth, and allow intracellular transport. With a diameter of 10  nm, neurofilaments are classified as intermediate (i.e., larger than actin but smaller than myosin) filaments, and are heteropolymers composed by four different subunits based on the length of the carboxy-terminal region: neurofilament light chain (NfL), neurofilament middle chain (NfM), neurofil ament heavy chain (NfH), and alpha-internexin [1]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 14, 2020 Category: Neurology Authors: Sara Mariotto, Elia Sechi, Sergio Ferrari Tags: Letter to the Editor Source Type: research

The role of CAMKK2 polymorphisms in HIV-associated sensory neuropathy in South Africans
Human immunodeficiency virus-associated sensory neuropathy (HIV-SN) is a common neurological complication of HIV infection. It affected 57% of South African patients whose antiretroviral therapy (ART) included stavudine and was influenced by genotypes of the P2X-block (P2X7R, P2X4R and CAMKK2). We investigate associations between HIV-SN and P2X-block genotypes in patients who never received stavudine. An adjacent gene, ANAPC5, was included.75 HIV+ individuals were assessed using the Brief Peripheral Neuropathy Screen before treatment and after 6-8 months on stavudine-free regimens. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 14, 2020 Category: Neurology Authors: Jessica Gaff, Prinisha Pillay, Catherine Cherry, Simon M. Laws, Patricia Price, Peter Kamerman Source Type: research

Risk of recurrent ischemic stroke in young cryptogenic patients with embolic stroke of undetermined source
Elderly cryptogenic ischemic stroke (IS) patients with embolic stroke of undetermined source (ESUS) have a high risk of recurrent IS (RIS) compared to other stroke subtypes. In young ESUS patients, different sources of embolism may be a cause and the risk of RIS remains not enough established. The aim was to assess and compare risk of RIS between ESUS and non ESUS patients (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 12, 2020 Category: Neurology Authors: Daniel Šaňák, Petra Divišová, Martin Hutyra, Michal Král, Andrea Bártková, Jana Zapletalová, Jan Látal, Tomáš Dorňák, Štěpán Hudec, David Franc, Petr Polidar, Tomáš Veverka, Petr Kaňovský Source Type: research

Prognostic role of the controlling nutritional status score in acute ischemic stroke among stroke subtypes
The association of malnutrition on stroke subtypes has not been well established. We investigated the relationship between malnutrition and functional outcome according to stroke subtypes. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 11, 2020 Category: Neurology Authors: Hiroyuki Naito, Naohisa Hosomi, Tomohisa Nezu, Daisuke Kuzume, Shiro Aoki, Yuko Morimoto, Takeshi Yoshida, Yuji Shiga, Naoto Kinoshita, Hiroki Ueno, Hirofumi Maruyama Source Type: research

Real world application of ocrelizumab in multiple sclerosis: Single-center experience of 128 patients
Pivotal trials showed good clinical efficiency of the monoclonal antibody ocrelizumab while being well tolerated and manageable in multiple sclerosis (MS). However, data on adverse events in everyday practice are scarce. Hence, our study aims at investigating short-term tolerability of ocrelizumab in a “real-world” setting. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 11, 2020 Category: Neurology Authors: Victoria Prockl, Florian T. Nickel, Kathrin S. Utz, Kilian Fr öhlich, Tobias Engelhorn, Max-Josef Hilz, De-Hyung Lee, Ralf A. Linker, Konstantin Huhn Source Type: research

Fampridine-induced changes in walking kinetics are associated with clinical improvements in patients with multiple sclerosis
Gait dysfunction is common in patients with multiple sclerosis (PwMS). Treatment with prolonged-release fampridine (PR-fampridine) improves walking ability in some PwMS. Associated fampridine-induced changes in the walking pattern are still poorly understood but may provide a better understanding of the mechanisms underlying the beneficial drug effects. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 8, 2020 Category: Neurology Authors: D. Weller, L. Loerincz, T. Sutter, K. Reuter, M. Linnebank, M. Weller, B. Z örner, L. Filli Source Type: research

Multiple internal border zone infarcts in a patient with COVID-19 and CADASIL
Dear Editor, (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 8, 2020 Category: Neurology Authors: Owain Hedd Williams, Saamir Mohideen, Arup Sen, Olivera Martinovic, Jonathan Hart, Peter Anthony Brex, Laszlo Krisztian Sztriha Tags: Letter to the Editor Source Type: research

Novel homozygous mutation in the FBXL4 gene is associated with mitochondria DNA depletion syndrome-13
Mitochondrial DNA depletion syndrome-13 (MTDPS13) is caused by mutations in FBXL4 (F-box and leucine-rich repeat protein 4), a nuclear gene encoding an F-box protein that plays a role in maintaining mtDNA integrity and stability. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 7, 2020 Category: Neurology Authors: Simei Wang, Longlong Lin, Yilin Wang, Anqi Wang, Zhao Liu, Shengnan Wu, Xiaoping Lan, Jia Jia, Yuanfeng Zhang, Fang Yuan, Chunmei Wang, Xiaona Luo, Xiaomin Sun, Sreenivas K. Avula, Abdullah Tolaymat, Changsheng Liu, Ren Yun, Yucai Chen Source Type: research

Stent-unsheathed effect predicts acute distal middle cerebral artery atherosclerotic disease-related occlusion
The differentiation of intracranial atherosclerosis (ICAS) and embolism is important. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 6, 2020 Category: Neurology Authors: Wen-huo Chen, Ting-yu Yi, A-lai Zhan, Yan-Min Wu, Yan-yu Lu, Yi-min Li, Zhi-nan Pan, Ding-lai Lin, Xiao-hui Lin Source Type: research

Revisiting the role of brain and peripheral a β in the pathogenesis of Alzheimer's disease
Amyloid beta (A β) is an intricate molecule that interacts with several biomolecules and/or produces insoluble assemblies and eventually the nonphysiological depositions of its alternate with normal neuronal conditions leading to Alzheimer’s disease (AD). Aβ is formed through the proteolytic cleavage of an inte gral type I membrane protein amyloid precursor protein (APP). Significant efforts are being made to explore the exact role of Aβ in AD pathogenesis. It is believed that the deposition of Aβ in the brain takes place from Aβ components which are derived from the brain itself. (Source: Jo...
Source: Journal of the Neurological Sciences - June 6, 2020 Category: Neurology Authors: Md. Sahab Uddin, Md. Tanvir Kabir, Devesh Tewari, Abdullah Al Mamun, Bijo Mathew, Lotfi Aleya, George E. Barreto, May N. Bin-Jumah, Mohamed M. Abdel-Daim, Ghulam Md Ashraf Tags: Review Article Source Type: research

Long-term test-retest evaluation of the King-Devick test in youth soccer athletes
Despite the clinical utility of baseline comparisons during concussion assessments, little evidence exists on long-term test-retest reliability of baseline tests in youth athletes. In addition, sex differences in baseline performance are inconsistent in youth athletes, warranting further research. The purpose was to examine sex differences, prevalence of false-positive scores, and long-term test-retest reliability of the King-Devick (KD) test. Healthy youth athletes (23 males, 28 females) completed the KD test prior to the Spring 2016 and Fall 2017 seasons. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - June 5, 2020 Category: Neurology Authors: Abigail C. Bretzin, Morgan Anderson, Ryan N. Moran, Tracey Covassin Source Type: research