scholarly journals Case reports of unexplained weight loss in autoimmune central neurological syndromes with anti-GAD antibodies

2019 ◽  
Vol 6 (2) ◽  
pp. 5
Author(s):  
James Broadley ◽  
Mastura Monif
Keyword(s):  
Central Nervous System ◽  
Weight Loss ◽  
Neurological Disorders ◽  
Case Reports ◽  
Acid Decarboxylase ◽  
Gad Antibodies ◽  
Unexplained Weight Loss ◽  
Immune Mediated ◽  
Disease Associations ◽  
The Central Nervous System

Glutamic acid decarboxylase (GAD) is becoming increasingly recognised as an antigenic target in autoimmune disorders of the central nervous system. There are currently no reports of weight loss being a manifestation in such disorders. We describe two cases of anti-GAD associated neurological disorders with profound and otherwise unexplained weight loss. Both patients had incomplete response to immunotherapy, as is becoming typical of these disorders. The variable disease associations of anti-GAD antibodies is incompletely understood, and leads us to question whether weight loss in these patients could possibly be immune-mediated.

Case reports of unexplained weight loss in autoimmune central neurological syndromes with anti-GAD antibodies

2018 ◽  
pp. 1-4
Keyword(s):  
Central Nervous System ◽  
Weight Loss ◽  
Neurological Disorders ◽  
Case Reports ◽  
Acid Decarboxylase ◽  
Gad Antibodies ◽  
Unexplained Weight Loss ◽  
Immune Mediated ◽  
Disease Associations ◽  
The Central Nervous System

Glutamic acid decarboxylase (GAD) is becoming increasingly recognised as an antigenic target in autoimmune disorders of the central nervous system. There are currently no reports of weight loss being a manifestation in such disorders. We describe two cases of anti-GAD associated neurological disorders with profound and otherwise unexplained weight loss. Both patients had incomplete response to immunotherapy, as is becoming typical of these disorders. The variable disease associations of anti-GAD antibodies is incompletely understood, and leads us to question whether weight loss in these patients could possibly be immune-mediated.

Paraneoplastic and Idiopathic Autoimmune Neurological Disorders Affecting the Central Nervous System and Autoimmune Dysautonomia

2019 ◽  
pp. 245-265
Author(s):  
Nicholas L. Zalewski ◽  
Sean J. Pittock
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Diagnostic Evaluation ◽  
Rapid Rate ◽  
Disease Category ◽  
Cns Disorders ◽  
Immune Mediated ◽  
The Central Nervous System

This chapter is an examination of immune-mediated central nervous system (CNS) disorders, which have increasingly been recognized as a critical disease category in the field of neurology. The chapter looks at clinical presentation, diagnostic evaluation, and treatment. The chapter also looks to the future. The field of immune-mediated neurological diseases is rapidly growing. New autoantibodies are being discovered at a rapid rate, helping unveil the mystery behind the challenging neurological presentations in many patients.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

scholarly journals Meningitis Caused by Salmonella Newport in a Five-Year-Old Child

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Ana De Malet ◽  
Sheila Ingerto ◽  
Israel Gañán
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurological Disorders ◽  
Invasive Disease ◽  
Salmonella Typhi ◽  
Gram Negative ◽  
Negative Bacillus ◽  
Salmonella Newport ◽  
Gram Negative Bacillus ◽  
The Central Nervous System

Salmonella Newport is a Gram-negative bacillus belonging to the Enterobacteria family and the nontyphi Salmonella (NTS), usually related to gastroenteritis. Main difference between NTS and Salmonella typhi is that the last one evolves to an invasive disease easier than NTS. These can progress to bacteremias in around 5% of cases and secondary focuses can appear occasionally, as in meningitis. An infection of the central nervous system is uncommon, considering its incidence in 0.6–8% of the cases; most of them are described in developing countries and mainly in childhood, especially neonates. Bacterial meningitis by NTS mostly affects immunosuppressed people in Europe. Prognosis is adverse, with a 50% mortality rate, mainly due to complications of infection: hydrocephalus, ventriculitis, abscesses, subdural empyema, or stroke. Choice antibiotic treatments are cefotaxime, ceftriaxone, or ceftazidime. The aim of this paper is to present a case of meningitis caused by Salmonella Newport diagnosed in a five-year-old girl living in a rural area of the province of Ourense (Spain), with favorable evolution and without neurological disorders.

scholarly journals Emerging Synaptic Molecules as Candidates in the Etiology of Neurological Disorders

2017 ◽  
Vol 2017 ◽  
pp. 1-25 ◽  
Author(s):  
Viviana I. Torres ◽  
Daniela Vallejo ◽  
Nibaldo C. Inestrosa
Keyword(s):  
Central Nervous System ◽  
Neurological Disorders ◽  
Neuronal Development ◽  
Protein Complexes ◽  
Synaptic Proteins ◽  
Excitatory Synapses ◽  
Complex Structures ◽  
Disease Phenotype ◽  
The Central Nervous System ◽  
Invertebrate Models

Synapses are complex structures that allow communication between neurons in the central nervous system. Studies conducted in vertebrate and invertebrate models have contributed to the knowledge of the function of synaptic proteins. The functional synapse requires numerous protein complexes with specialized functions that are regulated in space and time to allow synaptic plasticity. However, their interplay during neuronal development, learning, and memory is poorly understood. Accumulating evidence links synapse proteins to neurodevelopmental, neuropsychiatric, and neurodegenerative diseases. In this review, we describe the way in which several proteins that participate in cell adhesion, scaffolding, exocytosis, and neurotransmitter reception from presynaptic and postsynaptic compartments, mainly from excitatory synapses, have been associated with several synaptopathies, and we relate their functions to the disease phenotype.

Cochlear Implant Patients with Superficial Siderosis

2009 ◽  
Vol 20 (06) ◽  
pp. 348-352 ◽  
Author(s):  
Sarah A. Sydlowski ◽  
Michael J. Cevette ◽  
Jon Shallop ◽  
David M. Barrs
Keyword(s):  
Central Nervous System ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Case Reports ◽  
Superficial Siderosis ◽  
Viable Option ◽  
Retrospective Case ◽  
Fatal Disease ◽  
The Central Nervous System

Background: Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. Purpose: To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation. Research Design: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease. Results: Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option. Conclusions: It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.

scholarly journals Insights Into the Role of CSF1R in the Central Nervous System and Neurological Disorders

2021 ◽  
Vol 13 ◽  
Author(s):  
Banglian Hu ◽  
Shengshun Duan ◽  
Ziwei Wang ◽  
Xin Li ◽  
Yuhang Zhou ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Colony Stimulating Factor ◽  
Loss Of Function ◽  
Axonal Spheroids ◽  
The Central Nervous System ◽  
Stimulating Factor

The colony-stimulating factor 1 receptor (CSF1R) is a key tyrosine kinase transmembrane receptor modulating microglial homeostasis, neurogenesis, and neuronal survival in the central nervous system (CNS). CSF1R, which can be proteolytically cleaved into a soluble ectodomain and an intracellular protein fragment, supports the survival of myeloid cells upon activation by two ligands, colony stimulating factor 1 and interleukin 34. CSF1R loss-of-function mutations are the major cause of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) and its dysfunction has also been implicated in other neurodegenerative disorders including Alzheimer’s disease (AD). Here, we review the physiological functions of CSF1R in the CNS and its pathological effects in neurological disorders including ALSP, AD, frontotemporal dementia and multiple sclerosis. Understanding the pathophysiology of CSF1R is critical for developing targeted therapies for related neurological diseases.

scholarly journals The incidence and pathophysiology of neurological symptoms in COVID-19

2021 ◽  
Vol 40 (4) ◽  
pp. 33-42
Author(s):  
Igor V. Litvinenko ◽  
Miroslav M. Odinak ◽  
Nikolay V. Tsygan ◽  
Aleksander V. Ryabtsev
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Complication ◽  
Cranial Nerves ◽  
Neurological Symptoms ◽  
Immune Mediated ◽  
High Incidence ◽  
The Central Nervous System ◽  
Coronavirus Infection

The central nervous system seems to be quite vulnerable to SARS-CoV-2, leading to a variety of alteration pathways, high incidence and variability of the neurological symptoms of COVID-19. The COVID-19 symptoms, possibly associated with alteration to the central nervous system, include hyperthermia, shortness of breath, fatigue, headache, dizziness, dysphonia, dysphagia, hyposmia and anosmia, hypogeusia and ageusia, impairment of consciousness. The impairment of olfaction and gustation are the most common symptoms of the nervous system alteration (98% and 70%, respectively), which is most likely a consequence of the alteration of the receptors. Presumably the pathogenesis of dysphonia and dysphagia may involve neurodegenerative mechanisms or may be associated with a predominantly demyelinating alteration of the caudal cranial nerves. Pathomorphological findings in the brain of the COVID-19 patients include diffuse hypoxic and focal ischemic injuries of various sizes up to ischemic infarctions (in thrombosis of large arteries); microangiopathy; vasculitis; diapedetic and confluent hemorrhages with possible progression to hemorrhagic infarctions and rarely intracerebral hematomas. Acute cerebrovascular accident worsens the course of COVID-19 and can worsen the clinical outcome, taking into account the mechanisms of the central nervous system alteration in highly contagious coronavirus infections (SARS-CoV, MERS, SARS-CoV-2), including embolism, hypoxia, neurodegeneration, systemic inflammatory response and immune-mediated alteartion to the nervous tissue. A fairly rare complication of coronavirus infection, however, acute myelitis requires attention due to the severity of neurological disorders. The literature data show high incidence and polymorphism of the symptoms of the central nervous system alteration, as well as the important role of the cerebrovascular and neurodegenerative pathogenesis of brain alteration in COVID-19, which is taken into account in examining and treating the patients with new coronavirus infection. (1 figure, bibliography: 61 refs)

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