Syoko Tsubouchi, Haeru Hayashi, Koichiro Tahara, Kayo Ishii, Takuya Yasuda, Yusuke Yamamoto, Takahiro Mizuuchi, Hiroaki Mori, Mayu Tago, Eri Kato, Tetsuji Sawada
Neuropsychiatric (NP) manifestations of systemic lupus erythematosus (SLE) are diverse, but involvement of basal ganglia is rare. We describe here a 28-year-old woman with NPSLE presenting aseptic meningitis accompanied by elevated interleukin-6 levels in the cerebrospinal fluid, who developed symmetrical basal ganglia lesions, containing a cytotoxic oedematous core, surrounded by vasogenic oedema upon magnetic resonance imaging. We were able to observe these lesions from a de novo appearance during the disease onset to its disappearance during immunosuppressive treatment...
January 2020: Modern rheumatology case reports
Lena Sannemann, Ann-Katrin Schild, Slawek Altenstein, Claudia Bartels, Frederic Brosseron, Katharina Buerger, Nicoleta Carmen Cosma, Klaus Fliessbach, Silka Dawn Freiesleben, Wenzel Glanz, Michael T Heneka, Daniel Janowitz, Ingo Kilimann, Xenia Kobeleva, Christoph Laske, Coraline D Metzger, Matthias H J Munk, Robert Perneczky, Oliver Peters, Alexandra Polcher, Josef Priller, Boris Rauchmann, Christina Rösch, Janna Rudolph, Anja Schneider, Annika Spottke, Eike Jakob Spruth, Stefan Teipel, Ruth Vukovich, Michael Wagner, Jens Wiltfang, Steffen Wolfsgruber, Emrah Duezel, Frank Jessen
BACKGROUND: Early identification of individuals at risk of dementia is mandatory to implement prevention strategies and design clinical trials that target early disease stages. Subjective cognitive decline (SCD) and neuropsychiatric symptoms (NPS) have been proposed as potential markers for early manifestation of Alzheimer's disease (AD). We aimed to investigate the frequency of NPS in SCD, in other at-risk groups, in healthy controls (CO), and in AD patients, and to test the association of NPS with AD biomarkers, with a particular focus on cognitively unimpaired participants with or without SCD-related worries...
October 16, 2020: Alzheimer's Research & Therapy
Kai Xin Chia, Sonali Polakhare, Stefania Dafne Bruno
Early case series suggest that about one-third of patients with COVID-19 present with neurological manifestations, including cerebrovascular disease, reported in 2%-6% of hospitalised patients. These are generally older patients with severe infection and comorbidities. Here we discuss the case of a previously fit and well 39-year-old man who presented with fever and respiratory symptoms, evolving in pneumonia with hypoxia but only requiring continuous positive airway pressure. After resolution of the respiratory disease, the patient developed focal neurology and was found to have bilateral occipital, thalamic and cerebellar infarcts...
October 15, 2020: BMJ Case Reports
Yukun Song, Lin Zou, Jing Zhao, Xiangxue Zhou, Yingqian Huang, Haishan Qiu, Haiwei Han, Zhiyun Yang, Xunhua Li, Xiaoying Tang, Jianping Chu
Wilson's disease (WD) is an inherited autosomal recessive disorder of copper metabolism, and its neurological and neuropsychiatric manifestations are associated with copper accumulation in brain. A few neuroimaging studies have shown that gray matter atrophy in WD affects both subcortical structures and cortex. This study aims to quantitatively evaluate the morphometric brain abnormalities in patients with WD in terms of whole brain volume and cortical thickness and their associations with clinical severity of WD...
October 14, 2020: Brain Imaging and Behavior
Raffaello Bonacchi, Maria A Rocca, Giuseppe A Ramirez, Enrica P Bozzolo, Valentina Canti, Paolo Preziosa, Paola Valsasina, Gianna C Riccitelli, Alessandro Meani, Lucia Moiola, Patrizia Rovere-Querini, Angelo A Manfredi, Massimo Filippi
Neuropsychiatric manifestations are highly prevalent in systemic lupus erythematosus (SLE)-patients. We aimed to unravel the substrates of these manifestations by investigating abnormalities of resting state (RS) functional connectivity (FC) and their correlations with neuropsychiatric variables in SLE-patients. Thirty-two SLE-patients and 32 age- and sex-matched healthy controls (HC) underwent brain 3T RS fMRI. Neuropsychological assessment was performed for all SLE-patients. The main large-scale cognitive and psychiatric functional networks were derived and between-group comparisons and correlations with neuropsychological measures were performed...
October 13, 2020: Molecular Psychiatry
Sonali N Reisinger, Spyros Sideromenos, Orsolya Horvath, Sophia Derdak, Ana Cicvaric, Francisco J Monje, Martin Bilban, Martin Häring, Micaela Glat, Daniela D Pollak
The signal transducer and activator of transcription 3 (STAT3) signalling pathway is activated through phosphorylation by Janus kinases in response to a diverse set of immunogenic and non-immunogenic triggers. Several distinct lines of evidence propose an intricate involvement of STAT3 in neural function relevant to behaviour in health and disease. However, in part due to the pleiotropic effects resulting from its DNA binding activity and the consequent regulation of expression of a variety of genes with context-dependent cellular consequences, the precise nature of STAT3 involvement in the neural mechanisms underlying psychopathology remains incompletely understood...
October 12, 2020: Molecular Psychiatry
Lorenzo Muccioli, Umberto Pensato, Ilaria Cani, Luca Guerra, Federica Provini, Giorgio Bordin, Luca Albini Riccioli, Raffaele Lodi, Paolo Tinuper, Francesca Bisulli
Encephalopathy is emerging as a recurrent complication of COVID-19 yet remains poorly characterized. We report the case of a middle-aged woman with COVID-19-related encephalopathy presenting as expressive aphasia and inattentiveness, subsequently progressing to agitation and marked confusion. Brain MRI and CSF analysis were unremarkable, while EEG showed slowing with frontal sharp waves. Neuropsychiatric symptoms resolved following treatment with tocilizumab. CNS involvement in COVID-19 may present as a subacute encephalopathy characterized by prominent frontal lobe dysfunction, with language disturbances as first neurological manifestation...
September 24, 2020: Journal of Neuroimmunology
Lorenzo Muccioli, Umberto Pensato, Giorgia Bernabè, Lorenzo Ferri, Maria Tappatà, Lilia Volpi, Ilaria Cani, Olivia J Henry, Francesca Ceccaroni, Sabina Cevoli, Gloria Stofella, Elena Pasini, Giacomo Fornaro, Caterina Tonon, Simone Vidale, Rocco Liguori, Paolo Tinuper, Roberto Michelucci, Pietro Cortelli, Francesca Bisulli
OBJECTIVE: To report on efficacy and safety of intravenous immunoglobulin (IVIg) therapy in a case series of patients with COVID-19-related encephalopathy. METHODS: We retrospectively collected data on all patients with COVID-19 hospitalized at two Italian hospitals who developed encephalopathy during disease course and were treated with IVIg. RESULTS: Five patients (two females, mean age 66.8 years) developed encephalopathy after a mean of 12...
October 8, 2020: Journal of Neurology
Piyumi Sachindra Alwis Wijewickrama, Henry N Rajaratnam
Introduction: Hypoparathyroidism, which is a common complication following total thyroidectomy can be transient in majority and permanent in 1.5% of the patients and usually occurs secondary to an inadvertent removal of parathyroid glands, mechanical or thermal injury or disruption of the vasculature. In some patients, it is observed that symptoms of hypocalcemia can occur for the first time several years after the surgery, which is known as "delayed hypoparathyroidism." We report three cases of delayed hypoparathyroidism from Sri Lanka, presenting several years after total thyroidectomy...
2020: Case Reports in Endocrinology
Parménides Guadarrama-Ortiz, José Alberto Choreño-Parra, Claudia Marisol Sánchez-Martínez, Francisco Javier Pacheco-Sánchez, Alberto Iván Rodríguez-Nava, Gabriela García-Quintero
The human infection of the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a public health emergency of international concern that has caused more than 16.8 million new cases and 662,000 deaths as of July 30, 2020. Although coronavirus disease 2019 (COVID-19), which is associated with this virus, mainly affects the lungs, recent evidence from clinical and pathological studies indicates that this pathogen has a broad infective ability to spread to extrapulmonary tissues, causing multiorgan failure in severely ill patients...
2020: Frontiers in Neurology
Ana Rita Silva, Patrícia Regueira, Elisabete Albuquerque, Inês Baldeiras, Ana Luísa Cardoso, Isabel Santana, Joaquim Cerejeira
OBJECTIVES: Delirium is an acute neuropsychiatric syndrome associated with poor outcomes. Older adults undergoing surgery have a higher risk of manifesting perioperative delirium, particularly those having associated comorbidities. It remains unclear whether delirium frequency varies across surgical settings and if it has remained stable across the years. We conducted a systematic review to (1) determine the overall frequency of delirium in older people undergoing noncardiac surgery; (2) explore factors explaining the variability of the estimates; and (3) determine the changing of the estimates over the past 2 decades...
September 30, 2020: Journal of the American Medical Directors Association
Michael C Spies, Johannes A Gutjahr-Holland, James V Bertouch, Anthony M Sammel
OBJECTIVES: Psychosis is a rare manifestation of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). Current guidelines do not make a recommendation regarding the use of Antinuclear Antibodies (ANA) testing in the assessment of patients with psychosis. This study was undertaken to determine the prevalence of NPSLE in patients with psychosis who have had positive ANA. METHODS: Retrospective review of patients admitted to the mental health service of two metropolitan tertiary referral centres with a diagnosis of psychosis, who had been tested for ANA...
October 1, 2020: Arthritis Care & Research
Viktor Kožich, Sally Stabler
The metabolism of sulfur-containing amino acids (SAAs) requires an orchestrated interplay among several dozen enzymes and transporters, and an adequate dietary intake of methionine (Met), cysteine (Cys), and B vitamins. Known human genetic disorders are due to defects in Met demethylation, homocysteine (Hcy) remethylation, or cobalamin and folate metabolism, in Hcy transsulfuration, and Cys and hydrogen sulfide (H2S) catabolism. These disorders may manifest between the newborn period and late adulthood by a combination of neuropsychiatric abnormalities, thromboembolism, megaloblastic anemia, hepatopathy, myopathy, and bone and connective tissue abnormalities...
October 1, 2020: Journal of Nutrition
Eliasz Engelhardt, Felipe Kenji Sudo, Gilberto Sousa Alves, Jerson Laks
Neuropsychiatric symptoms, which may appear alone or combined with cognitive and neurological manifestations, are frequent in many brain dysfunctions or lesions due to vascular, traumatic, neurodegenerative, or systemic conditions. Throughout history, many of the most prominent names have contributed to the clinical definition of the currently recognized mental symptoms and syndromes. The present paper aims at providing a comprehensive overview of the development, from ancient to modern times, of some widely known concepts and constructs about such neuropsychiatric disorders...
July 2020: Dementia & Neuropsychologia
Gürkan Demir, Onur Balaban, Muhammet Halit Tekeci, Zeynep Issı, Ali Fuat Erdem
BACKGROUND: Fahr's syndrome (or Fahr's disease) is a rare, neurological disorder characterized by bilateral calcification in the cerebellum, thalamus, basal ganglia, and cerebral cortex as a result of calcium and phosphorus metabolism disorder. The patients may be asymptomatic and clinical symptoms represent a wide range of neurologic manifestations and nonspecific neuropsychiatric disorders. We report an unusual case of Fahr's syndrome which was asymptomatic and incidentally diagnosed by generalized tonic-clonic seizure in a patient with SARS-CoV-2 (COVİD-19) pneumonia...
September 23, 2020: Neurological Sciences
Selima Chebli, Yosra Zgueb, Uta Ouali, Sana Taleb, Fethi Nacef
Neuropsychiatric manifestations in Sjögren's syndrome are common and can occur not only during its course, but also at the onset of the disease. Depression and anxiety were the most frequently described symptoms. However, the association with bipolar disorder seems to be rare and not well documented. This case report presents a patient with bipolar disorder as comorbidity with Sjögren's syndrome, suggesting that bipolar disorder could be associated with this autoimmune disease, which could lead to delaying diagnosis and treatment...
2020: Case Reports in Psychiatry
Laura E Godínez-Baca, Gustavo E Lugo-Zamudio, Lucía V Maya-Piña
Las manifestaciones neuropsiquiátricas en los pacientes con lupus eritematoso sistémico (LES) y síndrome antifosfolípido (SAF) secundario son muy frecuentes. En ambos casos, la fisiopatología se correlaciona con vasculopatía asociada a anticuerpos antifosfolípido y neurotoxicidad por anticuerpos y citocinas. La corea es el único trastorno del movimiento incluido en los 19 síndromes neuropsiquiátricos del LES según el American College of Rheumatology, con presentación inusual (prevalencia del 2%), y puede ocurrir como primera manifestación de la enfermedad...
2020: Cirugia y Cirujanos
Cassandra Doyno, Diana M Sobieraj, William L Baker
INTRODUCTION: While chloroquine, a derivative of quinine, has been used as an antimalarial for 70 years, hydroxychloroquine is now used to treat conditions such as rheumatoid arthritis and systemic lupus erythematosus. In 2020, hydroxychloroquine (and to a lesser extent chloroquine) also received attention as a possible treatment for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). During investigation for treating coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2, concerns for serious adverse events arose...
September 22, 2020: Clinical Toxicology
Keri Wallace, Julie Uchitel, Lyndsey Prange, Joan Jasien, Melanie Bonner, Richard D'Alli, Gary Maslow, Mohamad A Mikati
BACKGROUND: Alternating hemiplegia of childhood often manifests severe or extreme behavioral problems, the nature of which remains to be fully characterized. METHODS: We analyzed 39 consecutive patients with alternating hemiplegia of childhood for occurrence of behavioral problems and categorized those by severity: mild (not requiring intervention), moderate (requiring intervention but no risk), severe (minor risk to self, others, or both), and extreme (major risk)...
October 2020: Pediatric Neurology
Anna Alkelai, Shahar Shohat, Lior Greenbaum, Tanya Schechter, Benjamin Draiman, Eti Chitrit-Raveh, Shlomit Rienstein, Neha Dagaonkar, Daniel Hughes, Vimla S Aggarwal, Erin L Heinzen, Sagiv Shifman, David B Goldstein, Yoav Kohn
Childhood-onset schizophrenia (COS) is a rare form of schizophrenia with an onset before 13 years of age. There is rising evidence that genetic factors play a major role in COS etiology, yet, only a few single gene mutations have been discovered. Here we present a diagnostic whole-exome sequencing (WES) in an Israeli Jewish female with COS and additional neuropsychiatric conditions such as obsessive-compulsive disorder (OCD), anxiety, and aggressive behavior. Variant analysis revealed a de novo novel stop gained variant in GRIA2 gene (NM_000826...
September 18, 2020: Journal of Human Genetics
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