Central Neurological Manifestations Research Articles

COVID-19 and Alzheimer's disease: Impact of lockdown and other restrictive measures during the COVID-19 pandemic.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection initially results in respiratory distress symptoms but can also lead to central nervous system (CNS) and neurological manifestations, significantly impacting coronavirus disease 2019 (COVID-19) patients with neurodegenerative diseases. Additionally, strict lockdown measures introduced to curtail the spread of COVID-19 have raised concerns over the wellbeing of patients with dementia and/or Alzheimer's disease. The aim of this review was to discuss the overlapping molecular pathologies and the potential bidirectional relationship between COVID-19 and Alzheimer's dementia, as well as the impact of lockdown/restriction measures on the neuropsychiatric symptoms (NPS) of patients with Alzheimer's dementia. Furthermore, we aimed to assess the impact of lockdown measures on the NPS of caregivers, exploring its potential effects on the quality and extent of care they provide to dementia patients.We utilized the PubMed and Google Scholar databases to search for articles on COVID-19, dementia, Alzheimer's disease, lockdown, and caregivers. Our review highlights that patients with Alzheimer's disease face an increased risk of COVID-19 infection and complications. Additionally, these patients are likely to experience greater cognitive decline. It appears that these issues are primarily caused by the SARS-CoV-2 infection and appear to be further exacerbated by restrictive/lockdown measures. Moreover, lockdown measures introduced during the pandemic have negatively impacted both the NPSs of caregivers and their perception of the wellbeing of their Alzheimer's patients. Thus, additional safeguard measures, along with pharmacological and non-pharmacological approaches, are needed to protect the wellbeing of dementia patients and their caregivers in light of this and possible future pandemics.

Recurrent Non-stroke Central Neurologic Manifestations in Primary Antiphospholipid Syndrome.

Article Recurrent non-stroke central neurologic manifestations in primary antiphospholipid syndrome was published on June 1, 2022 in the journal Rheumatology and Immunology Research (volume 3, issue 2).

Brain iron deposition and movement disorders in hereditary haemochromatosis without liver failure: A cross-sectional study.

Hereditary haemochromatosis (HH) is the most common inherited disorder of systemic iron excess in Northern Europeans. Emerging evidence indicates that brain iron overload occurs in HH. Despite this observation, there is a paucity of literature regarding central neurological manifestations, in particular movement disorders, in HH. The current study documents deep gray matter (DGM) nuclei iron deposition, movement disorders, and clinicoradiological correlations in HH without liver failure. This is a cross-sectional study. Consecutive subjects with HFE-haemochromatosis without liver disease were recruited from an outpatient gastroenterology clinic. Age- and sex-matched healthy controls (HCs) were enrolled. Iron content in individual DGM nuclei was measured as mean susceptibility on magnetic resonance imaging using quantitative susceptibility mapping-based regions of interest analysis. Occurrence and phenotype of movement disorders were documented and correlated with patterns of DGM nuclei iron deposition in subjects with HH. Fifty-two subjects with HH and 47 HCs were recruited. High magnetic susceptibility was demonstrated in several DGM nuclei in all HH subjects compared to HCs. Thirty-five subjects with HH had movement disorders. Magnetic susceptibility in specific DGM nuclei correlated with individual movement disorder phenotypes. Serum ferritin, phlebotomy frequency, and duration were poor predictors of brain iron deposition. Abnormal brain iron deposition can be demonstrated on imaging in all subjects with HH without liver failure. A significant proportion of these subjects manifest movement disorders. Peripheral iron measurements appear not to correlate with brain iron deposition. Therefore, routine neurological examination and quantitative brain iron imaging are recommended in all subjects with HH.

Global Publications on Covid-19 and Neurosciences: A Bibliometric Assessment during 2020-21

<p style="text-align: justify;"><strong>Background: </strong>Covid-19 is a significant global health burden. The pulmonary morbidity and mortality of Covid-19 is well described, however, there is mounting evidence of neurological manifestations of SARS-CoV-2, which may be of paramount significance. This paper provides a bibliometric assessment of global literature of the peripheral and central neurological manifestations of Covid-19, using bibliometric methods. <strong>Methods: </strong>The study downloaded publications on this topic from the Scopus database using a well-defined research strategy. The search strategy was initially based on using different keywords related to Covid-19 in &ldquo;Keyword&rdquo; and &ldquo;Title&rdquo; search tags of Scopus database. The search was subsequently restricted to &ldquo;Neurosciences&rdquo; subject, yielding 5245 global records. <strong>Results:</strong> The 5245 global publications were published on &lsquo;Covid-19 and Neurosciences&rdquo; as covered in Scopus database and they have received 5245 citations, averaging 13.46 citations per paper. Of these 5245 publications, 24.54% (1287) received external funding support from 150+ agencies and averaged 20.17 citations per paper. 160 countries participated in global research on this topic, with USA, U.K. and Italy contributing the largest publication share (26.43%, 12.14% and 10.51%) and China (23.8 and 1.78), Spain (9.13 and 0.68) and Italy (8.56 and 0.64) registering the highest citation impact, based on citations per paper and relative citation index. &ldquo;Adults&rdquo;, among population age groups, contributed the largest share (30.31%), followed by &ldquo;Aged&rdquo; (9.48%), &ldquo;Middle Aged&rdquo; (9.02%), &ldquo;Children&rdquo;(4,73%) and &ldquo;Adolescents&rdquo; (2.71%). &ldquo;Stroke&rdquo;, &ldquo;Multiple Sclerosis&rdquo; and &ldquo;Headache&rdquo; among type of neurological diseases impacted by Covid-19, contributed the largest global publication share (6.31%, 6.22% and 6.06%), followed by &ldquo;Seizure&rdquo;, &ldquo;Parkinson&rsquo;s Disease&rdquo;, Cerebrovascular Accident&rdquo;, &ldquo;Anosmia&rdquo;, &ldquo;Guillain-Barre Syndrome&rdquo; and &ldquo;Epilepsy&rdquo; (from 4.39% to 5.62%), etc. &ldquo;Clinical Studies&rdquo;, among various types of research, contributed the largest publication share (29.38%) in total output impacted by Covid-19, followed by &ldquo;Complications&rdquo; (16.03%), &ldquo;Treatment&rdquo; (13.99%), &ldquo;Pathophysiology&rdquo; (12.18), &ldquo;Risk Factors&rdquo;(10.79%), &ldquo;Epidemiology&rdquo; (9.38%) and &ldquo;Genetics&rdquo;. Among participating organizations, Harvard Medical School, USA, University of Toronto, Canada and INSERM, France contributed the largest output (with 142, 101 and 99 papers) and University of Cambridge, U.K. (72.97 and 5.46), King&rsquo;s College London, U.K. (49.97 and 3.74), University College London, U.K. (47.58 and 3.56) registered the largest citation impact. Among participating authors, J. Sterer (24 papers), F.A. Scorza (20 papers) and E. Moro (14 papers) contributed the largest number of papers and Z. Liu (169.7 and 12.7), M.L.R. Neto (54.92 and 4.11) and G. Tsivgoulis (45.54 and 3.41) registered the highest citation impact. Among journals participating on this theme,<em> Psychiatry Research</em> (311 papers), <em>The Lancet Psychiatry </em>(175 papers) and <em>Frontiers in Neurology </em>(172 papers) contributed the largest number of papers and <em>Brain, Behavior and Immunity </em>(61.35), <em>The Lancet Psychiatry </em>(54.58) and <em>Nature Human Behavior </em>(44.37) registered the largest citation impact per paper. <strong>Conclusion: </strong>This paper evaluates the rapidly evolving literature on the neurological manifestations of Covid-19, which will help to inform and improve decision-making among physicians treating Covid-19 and scholars conducting research on this area. It will also aid in the recognition of significant extra-pulmonary manifestations of the disease among attending front-line clinicians and consulting neurologists and also help them in understanding the pandemic&rsquo;s broader impact on chronic disease management.

High-Risk Screening for Fabry Disease: A Nationwide Study in Japan and Literature Review.

Fabry disease (FD) is an X-linked inherited disorder caused by mutations in the GLA gene, which encodes the lysosomal enzyme α-galactosidase A (α-Gal A). FD detection in patients at an early stage is essential to achieve sufficient treatment effects, and high-risk screening may be effective. Here, we performed high-risk screening for FD in Japan and showed that peripheral neurological manifestations are important in young patients with FD. Moreover, we reviewed the literature on high-risk screening in patients with renal, cardiac, and central neurological manifestations. Based on the results of this study and review of research abroad, we believe that FD can be detected more effectively by targeting individuals based on age. In recent years, the methods for high-risk screening have been ameliorated, and high-risk screening studies using GLA next-generation sequencing have been conducted. Considering the cost-effectiveness of screening, GLA sequencing should be performed in individuals with reduced α-Gal A activity and females with certain FD manifestations and/or a family history of FD. The findings suggest that family analysis would likely detect FD patients, although GLA sequencing of asymptomatic family members requires adequate genetic counseling.

Stroke-Like Episodes in Charcot-Marie-Tooth X1 (CMTX1) Disease

Background: Charcot-Marie-Tooth disease (CMT) is a heterogeneous group of disorders best known for causing inherited forms of peripheral neuropathy. History of CMT disease is not considered relevant in patients presenting with acute central neurologic manifestations of acute stroke. The case described here should alert clinicians to the possibility of transient stroke-like episodes in young patients suffering from CMTX1, X‐linked variant of CMT.&#x0D; Case description: A 21-year-old man presented to our emergency department with acute onset dysarthria, right facial weakness and right hemiparesis, which started 2 hours before his admission. He was treated with intravenous thrombolysis with a suspected diagnosis of acute ischemic stroke with full resolution of symptoms. The following day he experienced another stroke-like episode of acute onset of dysarthria and left facial weakness that resolved within 3 hours. The patient underwent stroke in the young work-up which was normal. MRI of the brain showed symmetric, non-enhancing areas of restricted diffusion in the corona radiata bilaterally and corpus callosum, also demonstrated on T2/fluid-attenuated inversion recovery (FLAIR) sequences. Clinical and familial investigations resulted in diagnosing CMTX1, X‐linked variant of CMT.&#x0D; Discussion: This case illustrates that CMTX1 disease can have central nervous system (CNS) manifestations that could mimic stroke-like neurological deficits with pathological MRI findings. The prognosis of the CNS phenotype of CMTX1 is usually good, with spontaneous resolution without

SARS-CoV-2: its potential neurological manifestations and plausible mechanism: a review article.

Coronavirus disease (COVID-19) was first identified in late December 2019. The disease began in Wuhan, Hubei province in China and since then it has spread quickly to many countries all over the world. COVID-19 is caused by a novel coronavirus, named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The virus was majorly seen to overwhelm the respiratory system with mild to severe acute respiratory syndrome considered pathognomic for the disease. However, with time a plethora of symptoms was observed in the patients infected with COVID-19 including strong evidence for neurological symptoms. Evidence suggests that the virus has both central and peripheral nervous system manifestations. Patients, particularly those who suffer from a severe illness, have a central nervous system (CNS) involvement and neurological manifestations. There is precise and targeted documentation of neurological symptoms with details of clinical, neurological, and electrophysiological findings. This review article thus gives an insight into the neuro-invasive potential of COVID-19 and discusses the possible pathogenesis.

A Review of Neuro-Ophthalmological Manifestations of Human Coronavirus Infection.

IntroductionHuman coronavirus (HCoVs) are a group of viruses with recognized neurotropic and neuroinvasive capabilities. The reports on the neurological and ocular findings are increasing day after day and several central and peripheral neurological manifestations are already described. However, none specifically describes the neuro-ophthalmological manifestation of HCoVs. This is the first article specifically reviewing neuro-ophthalmological manifestations of HCoVs infection.MethodsPubMed and Google Scholar databases were searched using the keywords: coronaviridae, coronavirus, COVID-19, SARS-CoV-2, SARS-CoV-1, MERS, ocular, ophthalmology, ophthalmological, neuro-ophthalmology, neurological, manifestations. A manual search through the reference lists of relevant articles was also performed. There were no restrictions concerning language or study type and publications not yet printed but available online were considered.ResultsCoronavirus eye involvement is not frequent and includes mostly a typical viral follicular conjunctivitis. Recently, retinal anatomical alterations were described using optic coherence tomography. Neuro-ophthalmological symptoms and signs can appear isolated or associated with neurological syndromes. The manifestations include headache, ocular pain, visual impairment, diplopia, and cranial nerve palsies secondary to Miller Fisher syndrome, Guillain-Barré syndrome, or encephalitis, and nystagmus.ConclusionNeurological and neuro-ophthalmological syndromes, symptoms, and signs should not be neglected and a complete ophthalmological examination of these patients should be performed to fully describe ocular manifestations related to HCoVs. We believe that major ocular and neuro-ophthalmological manifestations reports lack due to safety issues concerning detailed ophthalmological examination; on the other hand, in a large number of cases, the presence of life-threatening coronavirus disease hinders ocular examination and ophthalmologist’s visit to the intensive care unit.

SARS-CoV-2 pathophysiology and its clinical implications: An integrative overview of the pharmacotherapeutic management of COVID-19

Common manifestations of COVID-19 are respiratory and can extend from mild symptoms to severe acute respiratory distress. The severity of the illness can also extend from mild disease to life-threatening acute respiratory distress syndrome (ARDS). SARS-CoV-2 infection can also affect the gastrointestinal tract, liver and pancreatic functions, leading to gastrointestinal symptoms. Moreover, SARS-CoV-2 can cause central and peripheral neurological manifestations, affect the cardiovascular system and promote renal dysfunction. Epidemiological data have indicated that cancer patients are at a higher risk of contracting the SARS-CoV-2 virus. Considering the multitude of clinical symptoms of COVID-19, the objective of the present review was to summarize their pathophysiology in previously healthy patients, as well as in those with comorbidities. The present review summarizes the current, though admittedly fluid knowledge on the pathophysiology and symptoms of COVID-19 infection. Although unclear issues still remain, the present study contributes to a more complete understanding of the disease, and may drive the direction of new research. The recognition of the severity of the clinical symptoms of COVID-19 is crucial for the specific therapeutic management of affected patients.

Neurological manifestations in coronavirus disease 2019.

Coronavirus disease 2019 (COVID-19), an infection caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is currently hitting the world in the form of a pandemic. Given that some reports suggest that this infection can also occur with neurologic manifestations, this narrative review addresses the basic and clinical aspects concerning the nervous system involvement associated with this disease. More than one third of patients hospitalized for COVID-19 can present with both central and peripheral neurological manifestations. The former include dizziness and headache, while the latter include taste and smell disturbances. Other reported neurological manifestations are cerebrovascular disease and epileptic seizures. According to published reports, neurological disorders are not uncommon in COVID-19 and can sometimes represent the first manifestation of the disease; therefore, neurologists should consider this diagnostic possibility in their daily practice. Since maybe not all COVID-19 neurological manifestations are due to SARS-CoV-2 direct effects, it is important to monitor the rest of the clinical parameters such as, for example, oxygen saturation. Similarly, follow-up of patients is advisable, since whether neurological complications may develop lately is thus far unknown.

Rheumatoid Meningitis a Rare Extra-Articular Manifestation of Rheumatoid Arthritis: Report of 6 Cases and Literature Review.

Objectives. Central neurological manifestations of rheumatoid arthritis (RA) like rheumatoid meningitis (RM) are rare, little known and have a high rate of morbi-mortality. METHODS. We described six cases of RM that were directly related to RA activity after exhaustive assessment. RESULTS. They were mainly women, aged of 50 to 69. All were positive for anti-cyclic citrullinated peptide antibodies and half for rheumatoid factors. RA activity, duration, and treatments were heterogeneous including oral steroids, conventional synthetic disease modifying anti-rheumatic drugs (DMARDs) and biologic DMARDs. Symptoms were various, with acute or progressive beginning; main were: generalized or focal seizure (4/6), fever (3/6), headaches (3/6), and frontal syndrome (2/6). Imaging lesions were four leptomeningitis, one pachymeningitis, and one association of both. MRI usually showed hypersignal in various territories in T2-FLAIR (fluid attenuated inversion recovery) mode, and enhancement in T1-weighted mode after gadolinium injection. All patients had lumbar puncture that found sterile cerebrospinal fluid, no neoplasic cell, elevated cell count in 5/6 cases and elevated proteins concentration in 3/6 cases. Cerebral biopsy was possible for three patients, and definitively confirmed the diagnosis of aseptic lepto- or pachymenintis, excluding vasculitis and lymphoma. Different treatments were used like intravenous high dose steroids, immunoglobulins or biologic DMARDs, with variable clinical and imaging outcome: one death, one complete recovery, and four recoveries with sequelae. Conclusions. Clinical symptoms, imaging, lumbar puncture, and serological studies are often nonspecific, only histologic examination can confirm the diagnosis of RM. Any central neurological manifestation in RA patients, even in quiescent and ancient RA, should warn the physician.

Neurological Involvement in Systemic Lupus Erythematosus (SLE): Our Recent Experience

Introduction: The central, psychiatric and peripheral neurological manifestations of lupus are among the most severe visceral disorders and are grouped under the general term of “neuro-psychiatric systemic lupus erythematosus” (NPSLE). We conducted a cross-sectional observational study within our Department of Internal Medicine aimed at describing the clinical and evolutionary aspects of central neurological disorders of SLE, excluding lupus myelopathy. Patients and Methods: This was a retrospective and observational cross-sectional study carried out from 1 January 2015 to 31 October 2017, in the Department of Internal Medicine of Aristide le Dantec University Hospital in Dakar (Senegal). All patients hospitalized during this period who met the 1997 ACR classification criteria of SLE and who presented with a central neuropsychiatric syndrome attributable to SLE (as defined by ACR 1999) were included. Patients with isolated headache, acute myelitis or secondary neurological involvement attributable to a toxic, metabolic, infectious or tumour-related cause were excluded from our study. Results: During the study period, 10 patients with neuropsychiatric lupus involvement were treated at our institution, including 9 women and 1 man; the median age was 29 years (20 - 55 years). Neurological involvement occurred during the course of lupus evolution in 9/10 cases. The median time to SLE evolution was 18 months (0 - 60 months). Neuropsychiatric syndromes as defined by the 1999 ACR were commonly associated and more than half of our patients had multiple neuropsychiatric syndromes. There were 5 cases of confusion syndrome and coma, 4 cases of seizure, 3 cases of psychosis, 2 cases of acute cerebrovascular disease and 1 case of aseptic meningitis. Among the extra-neurological manifestations of SLE, haematological and dermatological involvements were common. Renal involvement affected half of the patients. The other manifestations were: polyarthritis in 3 patients, serositis in 2 patients, 5 cases of fever, 4 cases of deterioration of the general state, and one isolated case of ophthalmological involvement. Therapeutically, 8 patients received a bolus of methylprednisolone and 3 patients received a bolus of cyclophosphamide. Oral corticosteroids and hydroxychloroquine were administered to all patients, and azathioprine was administered in 2 patients. The evolution was favorable in 4 patients, other 2 patients maintained neurological sequelae and 2 patients were transferred to intensive care. Death was recorded in 4 patients. Conclusion: Neuropsychiatric manifestations of lupus are rare and sometimes severe, potentially life-threatening. In our patients, we have identified some of the most severe neurological syndromes according to the ACR nomenclature. The neurological involvement is exceptionally revealing, as these syndromes are often associated and integrated into a systemic context of lupus. The evolution is rapidly unfavorable and requires early diagnosis and optimal management.

The journey from gene knockout to clinical medicine: telotristat and sotagliflozin.

Gene knockout has been a powerful technique to evaluate the physiologic role of selected gene products. Lexicon pioneered high-throughput gene knockout technology and went further in designing agents to inhibit products of gene expression. Two agents have entered late-stage development. Telotristat is an inhibitor of tryptophan hydroxylase (TPH), preventing the production of serotonin. Although this agent blocks the two isoforms of TPH, it does not cross the blood–brain barrier, thus avoiding central neurologic manifestations. It inhibits the peripheral production of serotonin, and in particular prevents serotonin action in the intestines, resulting in decreased peristaltic action. Lexicon successfully developed telotristat to treat carcinoid syndrome not responding adequately to somatostatin inhibitors. Sotagliflozin development proceeded from the observation that dual inhibition of SGLT2 in the kidneys and SGLT1 in the intestines resulted in increased renal glucose excretion, reduced early-phase glucose absorption, as well as increased blood levels of GLP-1 and PYY. Initial development efforts focused on type 1 diabetes and have shown reduced postprandial glucose levels, less tendency to hypoglycemia, and lower HbA1c. Several other SGLT2 inhibitors have been associated with increased frequency of diabetic ketoacidosis (DKA). In the type 1 trials, sotagliflozin-treated individuals experienced DKA at a higher rate than placebo-treated patients. The sotagliflozin development program has now been extended to trials on type 2 diabetes. Long-term clinical trials will determine the benefits and risks of the agent in comparison to other currently marketed SGLT2 inhibitors.

Neurologic manifestations associated with cryoglobulinemia: A single center experience

IntroductionLimited information is available describing the spectrum of neurological complications of cryoglobulinemia. MethodsSingle center retrospective review of patients with neurologic symptoms and elevated serum cryoglobulins, with their potential association being classified as definite, possible, or unlikely using defined criteria. ResultsAmong 492 patients, 131 (87 classified as definite and 44 as possible) had neurologic symptoms associated with cryoglobulinemia. Common comorbidities included hepatitis C (N = 43), monoclonal gammopathy of undetermined significance (N = 20), Sjogren's syndrome (N = 17), membranoproliferative glomerulonephritis (N = 17), and systemic lupus erythematosus (N = 10). Features supporting an association between cryoglobulinemia and neurological symptoms were the presence of purpura (p < .001), positive rheumatoid factor (p = .001) and low C4 (p = .002). Common peripheral neurological diagnoses were symmetric polyneuropathy (N = 84), small fiber neuropathy (N = 25), and mononeuritis multiplex (N = 16). Central neurological manifestations were infrequent and included seizures (N = 3), posterior reversible encephalopathy syndrome (N = 2), intracerebral hemorrhage (N = 1), vasculitis (N = 1), rapidly progressive dementia (N = 1), lymphoma (N = 1), and myelitis/meningitis (N = 1). Treatments utilized included corticosteroids (N = 74), rituximab (N = 42), cyclophosphamide (N = 27), methotrexate, azathioprine, or mycophenolate mofetil (N = 28), anti-viral therapy (N = 20), plasmapheresis (N = 16), and intravenous immunoglobulin (N = 20). Neurologic symptoms associated with cryoglobulinemia remained stable or improved in 86% of patients. ConclusionThis study describes a wide spectrum of patients with neurologic symptoms attributed to cryoglobulinemia and provides a framework to approach this challenging diagnosis.

P264 Sjögren’s syndrome presenting with myelitis and without SICCA symptoms

Sjogren’s syndrome (SS) is a chronic inflammatory disorder of exocrine glands with autoimmune etiology. Sjogren’s syndrome affect the nervous system in approximately 20% of cases. The prevalence of peripheral and central neurological manifestations in Sjogren syndrome are about 15% and 5% respectively. Neurological symptoms may occur before the onset of SICCA symptoms (dry eyes and dry mouth). Anti-Ro/SSA and anti-La/SSB antibodies are present 50–70% of patients with Sjogren syndrome. Patients with SS that have neurological symptoms, only 21% have anti-SSA or anti-SSB antibodies. In this article, we report a patient with SS, presenting as myelitis without any SICCA symptoms and without any Anti-Ro/SSA and anti-La/SSB antibodies in the sera of patient. Most common peripheral nervous system (PNS) presentations are axonal polyneuropathies (distal axonal sensory and sensorimotor), sensory ganglionopathy, motor neuropathy, small fiber neuropathy, multiple mononeuritis, trigeminal and other cranial neuropathies, autonomic neuropathies, demyelinating polyradiculoneuropathy. The sensory neuropathies constitute the most frequent PNS complication. Central nervous system (CNS) manifestations of Sjogren’s syndrome may affect spinal cord, brain stem, optic nerves, cerebellum, and cerebral hemispheres. CNS involvement is much less common than peripheral nervous system involvement. Transverse myelitis is an inflammatory disorder of the spinal cord that presents acutely. The presence of sensorial axonal polyneuropathy and longitudinally extensive transverse myelitis that spans the servical and thoracic spinal cord led us thinking about SS. The positive Schirmer’ test and lip biopsy supported the diagnosis of SS. So, it is important to consider SS in differential diagnosis even if there is no SICCA syndrome or even if autoantibodies are negative.

Myelit bulguları ile prezente olan ve sicca semptomları olmayan sjögren vakası

Sjögren’s syndrome (SS) is a chronic inflammatory disorder of exocrine glands with autoimmune etiology. Neurological symptoms occur in approximately 20% of patients with Sjögren’s syndrome.  Neurological symptoms may occur before the onset of sicca symptoms such as; dry eyes and dry mouth. The prevalence of peripheral and central neurological manifestations in  Sjögren syndrome are about 15% and 5% respectively. CNS involvement is much less common than peripheral nervous system involvement. In this case, our patient presented symptoms of transverse myelitis; however, the serum Anti-Ro/SSA and anti-La/SSB antibodies were negative and there were no dry eyes and dry mouth.

Hemophagocytic Syndrome in Systemic Lupus Erythematosus : A Monocentric Review of 13 Cases

Hemophagocytic syndrome (HS) can be either primary, with a genetic etiology, or secondary, associated with malignancies or infections. Among rheumatic or auto-immune disorders, HS occurs most frequently in systemic juvenile idiopathic arthritis. In recent years this syndrome has been increasingly reported in patients with systemic lupus erythematosus (SLE). We retrospectively studied SLE-associated HS in a monocentric lupus cohort patients.Methods :We reviewed the medical records of adult patients with SLE for a recent 6 years period (2010-2015) and identified patients who had developed HS. The diagnosis of SLE was made using ACR criteria (4 or more criteria) and HS has been diagnosed using Hunter criteria (5 or more). We conducted statistical analyses to identify the characteristics of those patients in comparison with SLE patients without HSResults :Among 110 consecutive lupus patients, 13 patients (12 women) was identified having HS. The mean age of patients was 37.69 +/- 11.4 years (21-68). HS revealed lupus in 3 patients; in the others the delay between diagnosis of SLE and HS was 12 months (1 months - 108). Fever, pericarditis and splenomegaly were found in 100%, 54% and 46% of patients at presentation of HS. Bone marrow aspiration indicated hemophagocytosis in all patients. Cutaneo-mucous and arthritis were present in 92% patients of patients at presentation of HS. Laboratory features were diverse, bicytopenia or pancytopenia, high C-reactive protein level (mean 93 mg/L) and hyperferritinemia (mean 11.082 ng/ml), hypertriglyceridemia (mean 4.2 g/L )were present in all patients. The mean hemoglobin level was 74 g/L (55-88 g/L), the mean neutrophils count was 6004 /µl (930-13.080), mean lymphocyte count 926/µl (350-1350) and the mean platelets 68.230/µl (10.000-98.000). All patients had anti-nuclear antibodies when the HS occurred. Anti-double-stranded DNA antibodies were present in 7 patients. Serum complement C3 was low in 10 patients.HS was associated with a lupus flare in 8 patients (3 renal flare, 3 central neurologic manifestations, one patient had proliferative nephropathy and polyradiculoneuritis and one patient pancreatitis). Infections was diagnosed in 11 patients (4 bacterial infections, 4 tuberculosis infection, 3 viral infections). Recognition of the cause of HS was particularly challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication; both conditions was considered present in 6 patients.The most commonly used therapy was corticosteroids, which were initially administered in all patients. All patients respond first to steroids. Immunosuppressant therapy was used together with corticosteroids in 7 patients (intravenous cyclophosphamide in 6, mycophenolate mofetil in 1). Intravenous immunoglobulin was given in 3 cases and Rituximab was used as the third line treatment in one patients (with polyradiculonevritis). Anti-tuberculosis treatment was used also as first line treatment in 4 patients with life threatening presentation. All patients had a good outcome without any mortality with a mean follow-up of 25 months.Compared with SLE patients without HS, those with HS was older and showed a higher serum C-reactive protein level (p = 0.039), a higher ferritinemia (p = 0.004), higher SLEDAI score (p = 0.003), a lower levels of plasma neutrophils (p < 0.001), lower level of hemoglobin (p = 0.013), as well as lower platelets count (p=0.03).Conclusion :HS was observed in 11.8 % Moroccan patients with SLE. The occurrence of HS was most frequently associated with the SLE disease activity and bacterial infection. Profound cytopenia, a high SLEDAI score, and notable changes in the level of acute-phase reactants are the characteristics of SLE patients with HS in our series. DisclosuresNo relevant conflicts of interest to declare.

Wernickeʼs Encephalopathy Following Roux-en-Y Gastric Bypass Complicated by Gastrojejunal Stenosis and Small Bowel Obstruction: 2016 ACG Presidential Poster Award

A 28 year-old woman was admitted to our hospital with 3 weeks of abdominal pain, nausea, and vomiting. At another facility, she had undergone Roux-en-Y gastric bypass 3 months prior for morbid obesity, followed by laparoscopic cholecystectomy one week prior for presumed chronic cholecystitis. The patient suffered from multiple daily episodes of nausea and vomiting, resulting in a 95 pound weight loss since her bariatric surgery. The cholecystectomy did not improve her symptoms. Neurologic examination was remarkable for horizontal rotary nystagmus, patellar and ankle areflexia, impaired pin-prick and temperature sensation of anterior aspects of the chest and abdomen, as well as distal legs and feet, impaired vibration sense in both feet, and wide-based ataxic gait. Empiric high dose intravenous thiamine repletion was immediately begun; whole blood thiamine level was later confirmed to be low. An upper GI series/small bowel follow-through did not reveal an obstruction initially, though CT scan showed a distended and fluid-filled gastric remnant and proximal small bowel, with possible transition point near the jejunojejunal anastomosis. EGD, however, demonstrated a normal appearing jejunojejunal anastomosis, but a mild gastrojejunal stenosis, which was successfully dilated. The patient rapidly improved following the dilation and with aggressive thiamine repletion. However, she was re-admitted three weeks later with recurrence of pain, nausea, and vomiting. CT now showed an evolving small bowel obstruction, prompting exploratory laparotomy with lysis of an adhesive band causing obstruction of the common channel. A feeding gastrostomy tube was also placed. Her neurological signs and symptoms have since improved and she is no longer requiring tube feeds. This case illustrates the importance of prompt attention to the development of any central or peripheral neurologic manifestations following bariatric surgery. This is especially important for thiamine deficiency, as Wernicke's encephalopathy only rarely presents with the classical triad of altered mental status, ocular motility abnormalities, and ataxia. If Wernicke's encephalopathy is suspected, it should be treated urgently, as confirmatory laboratory results would result in delays during which neurologic damage may progress. Additionally, although gastric bypass itself can predispose to thiamine deficiency, an underlying etiology, such as complications of surgery, should be sought and addressed.Figure 1Figure 2Figure 3

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

ObjectivesNeurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort...

Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases.