THUR 159 Radiological findings in two patients with autoimmune GFAP astrocytopathy

2018 ◽  
Vol 89 (10) ◽  
pp. A19.3-A19
Author(s):  
White Daniel ◽  
Patel Markand ◽  
Muhammad Wasim ◽  
Hayton Tom ◽  
Ramalingam Satheesh ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cognitive Impairment ◽  
Clinical Presentation ◽  
Radiological Findings ◽  
Radial Pattern ◽  
Immune Mediated ◽  
Clinical Presentations ◽  
The Central Nervous System ◽  
Complex Forms ◽  
Bilateral Papilloedema

BackgroundAutoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or subacute onset of cognitive impairment, seizures, encephalopathy, meningeal symptoms to more complex forms of encephalomyelitis.Materials and MethodsWe present a radiological review of two cases which were recently diagnosed in our institution. They presented with fever, meningoencephalitis and bilateral papilloedema. CSF antibody analysis (GFAPα-IgG) from Mayo Clinic confirmed the diagnosis of GFAP in both cases.ResultsThe typical radiological findings in both of the cases were a radial pattern of enhancement in brain and longitudinally extensive myelitic lesions in the spinal cord. The first patient improved with immunosupression treatment. The second patient had a significantly more severe clinical presentation with drug-refractory progression, who later died.ConclusionPatients presenting with subacute onset of cognitive impairment, meningoencephalomyelitis and papilloedema should raise the suspicion of autoimmune GFAP astrocytopathy. Though it is a relatively new disease entity, the radial pattern of enhancement and long spinal cord lesions on imaging are striking and CSF and serum antibodies are highly specific.

scholarly journals Cognitive Impairment in Multiple Sclerosis

Folia Medica ◽  
2016 ◽  
Vol 58 (3) ◽  
pp. 157-163 ◽  
Author(s):  
Anastasiya G. Trenova ◽  
Georgi S. Slavov ◽  
Maria G. Manova ◽  
Jana B. Aksentieva ◽  
Lyuba D. Miteva ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Impairment ◽  
Cognitive Disorders ◽  
Social Burden ◽  
Immune Mediated ◽  
The Social ◽  
The Central Nervous System ◽  
Axonal Transection ◽  
The Relationship

Abstract Multiple sclerosis (MS) is a socially significant immune-mediated disease, characterized by demyelination, axonal transection and oligodendropathy in the central nervous system. Inflammatory demyelination and neurodegeneration lead to brain atrophy and cognitive deficit in up to 75% of the patients. Cognitive dysfunctions impact significantly patients’ quality of life, independently from the course and phase of the disease. The relationship between pathological brain findings and cognitive impairment is a subject of intensive research. Summarizing recent data about prevalence, clinical specificity and treatment of cognitive disorders in MS, this review aims to motivate the necessity of early diagnosis and complex therapeutic approach to these disturbances in order to reduce the social burden of the disease.

Recurrent Hypothalamic Dysfunction in Seropositive Neuromyelitis Optica

2020 ◽  
pp. 10.1212/CPJ.0000000000001012
Author(s):  
Mary Clare McKenna ◽  
Nuala McNicholas ◽  
Conor Fearon ◽  
David Bradley
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Neuromyelitis Optica ◽  
Clinical Manifestations ◽  
Inflammatory Disorder ◽  
Diverse Range ◽  
Hypothalamic Dysfunction ◽  
Inappropriate Secretion ◽  
Clinical Presentations ◽  
The Central Nervous System

Background:Neuromyelitis optica (NMO) is a rare autoimmune inflammatory disorder of the central nervous system1. Pathogenic aquaporin 4 (AQP4) antibodies are present in 65-88% of cases1. The majority of cases follow a relapsing course preferentially involving the optic nerves, spinal cord, brainstem, diencephalon or cerebral regions1, 2. Within the acute diencephalic clinical presentations, symptomatic hypothalamic lesions may have a diverse range of clinical manifestations including homeostatic dysfunction of neuroendocrine systems2-4. We report a case of recurrent hypothalamic dysfunction secondary to NMO manifesting as syndrome of inappropriate secretion of antidiuretic hormone (SIADH), thermal dysregulation, dysautonomia and disorder of alertness.

Cognitive impairment predicts disability progression and cortical thinning in MS: An 8-year study

2016 ◽  
Vol 23 (6) ◽  
pp. 848-854 ◽  
Author(s):  
Marco Pitteri ◽  
Chiara Romualdi ◽  
Roberta Magliozzi ◽  
Salvatore Monaco ◽  
Massimiliano Calabrese
Keyword(s):  
Cognitive Impairment ◽  
Structural Mri ◽  
Disability Progression ◽  
Cortical Thinning ◽  
Immune Mediated ◽  
Relapsing Remitting ◽  
The Central Nervous System ◽  
Magnetic Resonance Imaging Mri ◽  
Few Data

Background: Multiple sclerosis (MS) is a chronic immune-mediated disease of the central nervous system (CNS). Although cognitive impairment (CI) affects a large proportion of MS patients, only few data are available about its prognostic value associated with advanced magnetic resonance imaging (MRI) metrics. Objectives: We aimed at investigating the relationship between the early CI and the disease progression over 8-year follow-up in MS patients. Methods: We conducted a retrospective 8-year longitudinal study involving 78 patients with relapsing-remitting MS, who completed neuropsychological examination and structural MRI at the time of diagnosis. Each patient was clinically evaluated every 6 months, and cortical thickness was quantified at baseline and at the end of the follow-up. Patients were classified as having normal cognition and mild or severe CI. Results: The results show that CI at the time of diagnosis is a good predictor of conversion to definite MS ( p < 0.001), disability progression ( p < 0.001), as well as of transition to secondary progressive phase ( p < 0.001) and of cortical thinning ( p < 0.001). Conclusion: We confirmed and extended the evidence that early CI might be helpful in the identification of MS patients at high risk of disability progression and poor clinical outcome and should be considered as a marker of most aggressive pathology.

Autoimmune Dementia

2018 ◽  
Vol 38 (03) ◽  
pp. 303-315 ◽  
Author(s):  
Justin Long ◽  
Gregory Day
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cognitive Impairment ◽  
Relevant Information ◽  
Paraneoplastic Encephalitis ◽  
Immune Mediated ◽  
Paraneoplastic Disease ◽  
Chronic Complication ◽  
The Central Nervous System ◽  
Diagnostic Framework

AbstractDementia refers to an acquired syndrome of intraindividual cognitive decline that ultimately interferes with an individual's ability to manage their usual duties at work or home. As experience with the diagnosis and management of patients with autoimmune and paraneoplastic encephalitis (AE) has expanded, it has become increasingly apparent that dementia may arise as a subacute or chronic complication of immune-mediated injury to the central nervous system. Progressive memory and thinking problems may represent the first (or only) sign of an underlying autoimmune or paraneoplastic disease. Accordingly, there is a need to routinely consider the diagnosis of AE in patients with dementia, and to evaluate patients recovering from AE for clinically meaningful cognitive impairment. We review and summarize the available evidence concerning the diagnosis and care of AE patients with associated cognitive impairment, herein referred to as autoimmune dementia (AiD). Relevant information is used to propose a novel diagnostic framework that may be applied to improve recognition, and facilitate the expedited evaluation and treatment of patients with AiD.

Rare giant cell ependymoma in an octogenarian

2006 ◽  
Vol 105 (6) ◽  
pp. 908-911 ◽  
Author(s):  
Patrick B. Cooper ◽  
Matthew Katus ◽  
Leon Moores ◽  
Dennis Geyer ◽  
James G. Smirniotopoulos ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Giant Cell ◽  
Unusual Case ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Relevant Literature ◽  
World Health ◽  
Radiological Findings ◽  
The World ◽  
Health Organization

✓Ependymomas are glial tumors that occur most often in children. In adults, ependymomas most often appear in the spinal cord. The World Health Organization recognizes several rare ependymoma subtypes, including the giant cell ependymoma of the terminal filum. The authors describe an unusual case of a posterior fossa giant cell ependymoma in an 89-year-old man presenting with vertigo and disequilibrium. Only seven cases of this tumor have been reported in the literature to date. The authors discuss the clinical presentation, radiological findings, pathological considerations, and surgical intervention in this patient and review the relevant literature.

scholarly journals Tethered spinal cord following repair of myelomeningocele

2004 ◽  
Vol 16 (2) ◽  
pp. 1-4 ◽  
Author(s):  
Roger J. Hudgins ◽  
C. Lynn Gilreath
Keyword(s):  
Spinal Cord ◽  
Clinical Presentation ◽  
Tethered Cord ◽  
Tethered Spinal Cord ◽  
Terminal Portion ◽  
Clinical Criteria ◽  
Orthopedic Surgeons ◽  
Clinical Presentations ◽  
Multidisciplinary Clinic ◽  
Surgical Release

Object The goal of this paper is to elucidate the clinical presentation of tethered cord syndrome (TCS) following repair of a myelomeningocele. Methods Approximately 10 to 30% of children will develop TCS following repair of a myelomeningocele. Because essentially all children with repaired myelomeningocele will have a tethered spinal cord, as demonstrated on MR imaging, the diagnosis of TCS is made based on clinical criteria. The six common clinical presentations of TCS are increased weakness (55%), worsening gait (54%), scoliosis (51%), pain (32%), orthopedic deformity (11%), and urological dysfunction (6%). The primary goal of surgery is to detach the spinal cord where it is adherent to the thecal sac, relieving the stretch on the terminal portion of the cord. Conclusions Early diagnosis and surgical release of the tethered cord results in stabilization or improvement in most cases. Because TCS may present with orthopedic and/or urological signs or symptoms, children with myelomeningocele should by followed, ideally in a multidisciplinary clinic, by neurosurgeons, orthopedic surgeons, and urologists who are aware of this condition.

scholarly journals Brainstem cavernous malformation

2013 ◽  
Vol 32 (01) ◽  
pp. 31-36
Author(s):  
Ariel Roberto Estramiana ◽  
Diana Lara Pinto de Santana ◽  
Eberval Gadelha Figueiredo ◽  
Manoel Jacobsen Teixeira
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Clinical Presentation ◽  
Cavernous Malformation ◽  
Vascular Malformations ◽  
Resonance Imaging ◽  
The Central Nervous System ◽  
Magnetic Resonance Imaging Mri ◽  
Brainstem Cavernous Malformation

AbstractCavernous malformation (CM) of the central nervous system (CNS) are acquired or developmental vascular malformations that represent the 5% to 15% of all vascular malformations of the CNS. Eighty to ninety percent of CM are supratentorial, 15% infratentorial, and 5% occur in the spinal cord. The subset of brainstem malformation presents as a very difficult paradigm for treating clinicians. The widespread use of magnetic resonance imaging (MRI) has increased the recognition of this disease. Clinical presentation, pathophysiology and treatment are discussed in this article.

scholarly journals Macrophage Infiltration, but Not Apoptosis, Is Correlated with Immune-Mediated Demyelination following Murine Infection with a Neurotropic Coronavirus

1999 ◽  
Vol 73 (10) ◽  
pp. 8771-8780 ◽  
Author(s):  
Gregory F. Wu ◽  
Stanley Perlman
Keyword(s):  
Spinal Cord ◽  
Adoptive Transfer ◽  
Hepatitis Virus ◽  
Virus Antigen ◽  
Moderate Increase ◽  
Apoptotic Cells ◽  
Progressive Development ◽  
Immune Mediated ◽  
Before And After ◽  
The Central Nervous System

ABSTRACT Mice infected with mouse hepatitis virus strain JHM (MHV-JHM) develop a chronic demyelinating encephalomyelitis that is in large part immune mediated. Potential mechanisms of immune activity were assessed using an adoptive transfer system. Mice deficient in recombinase-activating gene function (RAG1−/−), defective in B- and T-cell maturation, become persistently infected with MHV but do not develop demyelination. Adoptive transfer of splenocytes from mice immunized to MHV into RAG1−/− mice infected with an attenuated strain of the virus results in the rapid and progressive development of demyelination. Most striking, adoptive transfer resulted, within 5 to 6 days, in extensive recruitment of activated macrophages/microglia to sites of demyelination within the spinal cord. Clearance of virus antigen occurred preferentially from the gray matter of the spinal cord. Apoptotic cells were identified in both the gray and white matter of the central nervous system (CNS) from RAG1−/− mice before and after adoptive transfer, with a moderate increase in number, but not distribution, of apoptotic cells following the development of demyelination. These results suggest that apoptosis following MHV-JHM infection of the murine CNS is not sufficient to cause demyelination. These results, showing that macrophage recruitment and myelin destruction occur rapidly after immune reconstitution of RAG−/− mice, suggest that this will be a useful system for investigating MHV-induced demyelination.

scholarly journals MRI-Negative Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Antibody Spectrum Demyelinating Disease

2019 ◽  
Vol 6 ◽  
pp. 2329048X1983047 ◽  
Author(s):  
Carlos A. Pérez ◽  
Stephanie Garcia-Tarodo ◽  
Regina Troxell
Keyword(s):  
Demyelinating Disease ◽  
Disease Recurrence ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Antibody Testing ◽  
Immune Mediated ◽  
Clinical Presentations ◽  
The Central Nervous System

Myelin oligodendrocyte glycoprotein is expressed in the central nervous system on the surface of oligodendrocytes and is associated with a broad range of adult and pediatric demyelinating phenotypes. The entire spectrum of clinical and radiologic features of myelin oligodendrocyte glycoprotein antibody spectrum disorder remains to be fully elucidated. We describe the case of a 9-year-old boy with immune-mediated myelitis undetectable by conventional magnetic resonance imaging in the context of relapsing anti-myelin oligodendrocyte glycoprotein spectrum disorder. Despite the severe clinical presentation, his symptoms improved significantly following treatment with corticosteroids. Because timely diagnosis and treatment is imperative to prevent disease recurrence and reduce long-term morbidity, serum anti-myelin oligodendrocyte glycoprotein antibody testing should be considered in all children with acute demyelinating syndromes and unusual clinical presentations—including seizures—both at presentation and at follow-up.

Autoimmune diseases of the brain, imaging and clinical review

2021 ◽  
pp. 197140092110428
Author(s):  
Ghazal Shadmani ◽  
Tyrell J Simkins ◽  
Reza Assadsangabi ◽  
Michelle Apperson ◽  
Lotfi Hacein-Bey ◽  
...  
Keyword(s):  
Critical Role ◽  
Daily Practice ◽  
Diagnostic Approach ◽  
Laboratory Evaluation ◽  
Imaging Features ◽  
Radiological Findings ◽  
Immune Mediated ◽  
Clinical Scenarios ◽  
The Central Nervous System ◽  
The Brain

There is an extensive spectrum of autoimmune entities that can involve the central nervous system, which has expanded with the emergence of new imaging modalities and several clinicopathologic entities. Clinical presentation is usually non-specific, and imaging has a critical role in the workup of these diseases. Immune-mediated diseases of the brain are not common in daily practice for radiologists and, except for a few of them such as multiple sclerosis, there is a vague understanding about differentiating them from each other based on the radiological findings. In this review, we aim to provide a practical diagnostic approach based on the unique radiological findings for each disease. We hope our diagnostic approach will help radiologists expand their basic understanding of the discussed disease entities and narrow the differential diagnosis in specific clinical scenarios. An understanding of unique imaging features of these disorders, along with laboratory evaluation, may enable clinicians to decrease the need for tissue biopsy.

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