scholarly journals Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

2021 ◽  
Vol 14 ◽  
pp. 175628642199890
Author(s):  
Samantha A. Banks ◽  
Elia Sechi ◽  
Eoin P. Flanagan
Keyword(s):  
Objective Assessment ◽  
Neuropsychological Testing ◽  
Thyroid Peroxidase ◽  
Rapid Progression ◽  
Antibody Testing ◽  
Progressive Dementia ◽  
Csf Analysis ◽  
Bedside Testing ◽  
Magnetic Resonance Imaging Mri ◽  
Neurodegenerative Dementias

The terms autoimmune dementia and autoimmune encephalopathy may be used interchangeably; autoimmune dementia is used here to emphasize its consideration in young-onset dementia, dementia with a subacute onset, and rapidly progressive dementia. Given their potential for reversibility, it is important to distinguish the rare autoimmune dementias from the much more common neurodegenerative dementias. The presence of certain clinical features [e.g. facio-brachial dystonic seizures that accompany anti-leucine-rich-glioma-inactivated-1 (LGI1) encephalitis that can mimic myoclonus] can be a major clue to the diagnosis. When possible, objective assessment of cognition with bedside testing or neuropsychological testing is useful to determine the degree of abnormality and serve as a baseline from which immunotherapy response can be judged. Magnetic resonance imaging (MRI) head and cerebrospinal fluid (CSF) analysis are useful to assess for inflammation that can support an autoimmune etiology. Assessing for neural autoantibody diagnostic biomarkers in serum and CSF in those with suggestive features can help confirm the diagnosis and guide cancer search in paraneoplastic autoimmune dementia. However, broad screening for neural antibodies in elderly patients with an insidious dementia is not recommended. Moreover, there are pitfalls to antibody testing that should be recognized and the high frequency of some antibodies in the general population limit their diagnostic utility [e.g., anti-thyroid peroxidase (TPO) antibodies]. Once the diagnosis is confirmed, both acute and maintenance immunotherapy can be utilized and treatment choice varies depending on the accompanying neural antibody present and the presence or absence of cancer. The target of the neural antibody biomarker may help predict treatment response and prognosis, with antibodies to cell-surface or synaptic antigens more responsive to immunotherapy and yielding a better overall prognosis than those with antibodies to intracellular targets. Neurologists should be aware that autoimmune dementias and encephalopathies are increasingly recognized in novel settings, including post herpes virus encephalitis and following immune-checkpoint inhibitor use.

scholarly journals Progressive Dysphasic Dementia with Bucco-Facial Apraxia: A Case Report

1993 ◽  
Vol 6 (3) ◽  
pp. 159-163 ◽  
Author(s):  
S. F. Cappa ◽  
C. A. De Fanti ◽  
R. De Marco ◽  
E. Magni ◽  
C. Messa ◽  
...  
Keyword(s):  
Single Photon ◽  
Neuropsychological Testing ◽  
Photon Emission ◽  
Temporal Cortex ◽  
Resonance Imaging ◽  
Single Photon Emission ◽  
Progressive Dementia ◽  
Fluent Aphasia ◽  
Magnetic Resonance Imaging Mri ◽  
Resolution Single

A patient with progressive dementia, prominent non-fluent aphasia and signs of frontal lobe involvement, was evaluated by neuropsychological testing, magnetic resonance imaging (MRI) and high resolution single photon emission tomography (SPET). The presence of severe bucco-facial apraxia, associated with spared imitation of limb movements, correlated well with a marked reduction of cerebral perfusion in the left fronto-temporal cortex. This case emphasizes the usefulness of SPET as a valuable alternative to PET for the diagnosis of conditions, such as progressive neuropsychological syndromes, where a coupled reduction of metabolism and blood flow can be expected.

scholarly journals The value of contrast-enhanced FLAIR magnetic resonance imaging in detecting meningeal abnormalities in suspected cases of meningitis compared to conventional contrast-enhanced T1WI sequences

2020 ◽  
Vol 51 (1) ◽  
Author(s):  
Wael Hamza Kamr ◽  
Mohamed Gaber Eissawy ◽  
Amr Saadawy
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Early Diagnosis ◽  
Diagnosis And Treatment ◽  
Effective Management ◽  
Resonance Imaging ◽  
Flair Sequence ◽  
Csf Analysis ◽  
Contrast Enhanced ◽  
Magnetic Resonance Imaging Mri

Abstract Background Early diagnosis of meningitis with magnetic resonance imaging (MRI) would be useful for appropriate and effective management, decrease morbidity and mortality, and provide better diagnosis and treatment. The objective of the current study is to compare the accuracy of contrast-enhanced FLAIR (CE-FLAIR) and contrast-enhanced T1WI (CE-T1WI) in the detection of meningeal abnormalities in suspected cases of meningitis. Results Out of 45 patients, 37 patients were confirmed to have meningitis on CSF analysis. Out of the 37 patients, 34 patients were positive on CE-FLAIR sequence and 27 were positive on CE-T1WI. The sensitivity of CE-FLAIR sequence was 91.9% and specificity 100%, while the sensitivity of CE-T1WI sequence was 73% and specificity 100%. Conclusion CE-FLAIR is more sensitive than CE-T1WI in diagnosis of meningitis. It is recommended to be used in any cases with clinically suspected meningitis.

Apraxia screening predicts Alzheimer pathology in frontotemporal dementia

2018 ◽  
Vol 90 (5) ◽  
pp. 562-569 ◽  
Author(s):  
Matthias Pawlowski ◽  
Viktoria Joksch ◽  
Heinz Wiendl ◽  
Sven G Meuth ◽  
Thomas Duning ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Presentation ◽  
Neuropsychological Testing ◽  
High Sensitivity ◽  
Clinical Syndrome ◽  
Test Results ◽  
Discriminative Power ◽  
Bedside Test ◽  
Neurodegenerative Dementias ◽  
Alzheimer Pathology

ObjectivesFrontotemporal dementia (FTD) is a heterogeneous clinical syndrome linked to diverse types of underlying neuropathology. Diagnosis is mainly based on clinical presentation and accurate prediction of underlying neuropathology remains difficult.MethodsWe present a large cohort of patients with FTD spectrum diseases (n=84). All patients were thoroughly characterised by cerebrospinal fluid (CSF) Alzheimer’s disease (AD) biomarkers, neuroimaging, neuropsychological testing and standardised apraxia screening.ResultsA potential AD pathology was found in 43% of patients with FTD. CSF AD biomarker levels positively correlated with AD-typical apraxia scores in patients with FTD. The discriminative power of apraxia test results indicative of AD pathology was high (sensitivity: 90%, specificity: 66%).ConclusionsApraxia is common in neurodegenerative dementias but under-represented in clinical workup and diagnostic criteria. Standardised apraxia screening may serve as bedside test to objectify an AD-typical apraxia profile as an early and robust sign of AD pathology in patients with FTD.

Memory Impairment

2014 ◽  
pp. 77-82
Author(s):  
David L Brody
Keyword(s):  
Cognitive Rehabilitation ◽  
Illicit Drugs ◽  
Speech Therapy ◽  
Memory Function ◽  
Neuropsychological Testing ◽  
Vitamin B12 Deficiency ◽  
Memory Training ◽  
Electrolyte Disorders ◽  
Cardiovascular Exercise ◽  
Bedside Testing

Many complaints of memory problems after concussion actually turn out to be attention deficit. Take a careful collateral history regarding memory function in everyday life. Consider both bedside testing and formal neuropsychological testing of memory, but treat the patient, not the test results. Reduce barriers to optimal memory function: Optimize sleep; treat chronic pain; taper or stop cognitively impairing medications; stop alcohol and illicit drugs; prescribe moderate cardiovascular exercise; test for vitamin B12 deficiency, hypothyroidism, electrolyte disorders, hypo- or hypergylcemia, renal failure, liver failure, and anemia. Refer to speech therapy and occupational therapy for memory training. Stimulants can allow more intense cognitive rehabilitation when attention or fatigue are limiting. Consider pharmacological enhancers of memory including caffeine, donepezil, or rivastigmine. These have modest benefits, and the nonpharmacological interventions are more important.

scholarly journals Rapidly progressive dementia due to neurosarcoidosis

2013 ◽  
Vol 7 (4) ◽  
pp. 428-434
Author(s):  
Gabriela Carneiro C. Fortes ◽  
Marcos Castello B. Oliveira ◽  
Laura Cardia G. Lopes ◽  
Camila S. Tomikawa ◽  
Leandro T. Lucato ◽  
...  
Keyword(s):  
Correct Diagnosis ◽  
Oral Prednisone ◽  
Brain Biopsy ◽  
High Dose ◽  
Mri Findings ◽  
Progressive Dementia ◽  
Granulomatous Angiitis ◽  
Severe Impairment ◽  
Magnetic Resonance Imaging Mri ◽  
One Year

ABSTRACT Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. Methods: We report two cases of neurosarcoidosis presenting as RPD. Results: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. Conclusion: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

scholarly journals Metabolomic Biomarkers of Multiple Sclerosis: A Systematic Review

2020 ◽  
Vol 7 ◽  
Author(s):  
Lachlan Porter ◽  
Alireza Shoushtarizadeh ◽  
George A. Jelinek ◽  
Chelsea R. Brown ◽  
Chai K. Lim ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
High Throughput Sequencing ◽  
Cochrane Library ◽  
Clinical Criteria ◽  
Diagnosis And Prognosis ◽  
Csf Analysis ◽  
Potential Biomarker ◽  
Magnetic Resonance Imaging Mri ◽  
Potential Biomarkers

BackgroundMagnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and the McDonald’s clinical criteria are currently utilized tools in diagnosing multiple sclerosis. However, a more conclusive, consistent, and efficient way of diagnosing multiple sclerosis (MS) is yet to be discovered. A potential biomarker, discovered using advances in high-throughput sequencing such as nuclear magnetic resonance (NMR) spectroscopy and other “Omics”-based techniques, may make diagnosis and prognosis more reliable resulting in a more personalized and targeted treatment regime and improved outcomes. The aim of this review was to systematically search the literature for potential biomarkers from any bodily fluid that could consistently and accurately diagnose MS and/or indicate disease progression.MethodsA systematic literature review of EMBASE, PubMed (MEDLINE), The Cochrane Library, and CINAHL databases produced over a thousand potential studies. Inclusion criteria stated studies with potential biomarker outcomes for people with MS were to be included in the review. Studies were limited to those with human participants who had a clinically defined diagnosis of MS and published in English, with no limit placed on date of publication or the type of bodily fluid sampled.ResultsA total of 1,805 studies were recorded from the literature search. A total of 1,760 studies were removed based on their abstract, with a further 18 removed after considering the full text. A total of 30 studies were considered relevant and had their data retrieved and analyzed. Due to the heterogeneity of focus and results from the refined studies, a narrative synthesis was favored.ConclusionSeveral promising candidate biomarkers suitable for clinical application in MS have been studied. It is recommended follow-up studies with larger sample sizes be completed on several potential biomarkers.

scholarly journals Fronto-limbic disconnection in patients with multiple sclerosis and depression

2018 ◽  
Vol 25 (5) ◽  
pp. 715-726 ◽  
Author(s):  
Quinten van Geest ◽  
Rosa E Boeschoten ◽  
Matthijs J Keijzer ◽  
Martijn D Steenwijk ◽  
Petra JW Pouwels ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Disease Duration ◽  
Neuropsychological Testing ◽  
Uncinate Fasciculus ◽  
Resting State Functional Connectivity ◽  
Major Depressive ◽  
Brain Volumes ◽  
Functional Changes ◽  
Severity Of Depression ◽  
Magnetic Resonance Imaging Mri

Background: The biological mechanism of depression in multiple sclerosis (MS) is not well understood. Based on work in major depressive disorder, fronto-limbic disconnection might be important. Objective: To investigate structural and functional fronto-limbic changes in depressed MS (DMS) and non-depressed MS (nDMS) patients. Methods: In this retrospective study, 22 moderate-to-severe DMS patients (disease duration 8.2 ± 7.7 years), 21 nDMS patients (disease duration 15.3 ± 8.3 years), and 12 healthy controls underwent neuropsychological testing and magnetic resonance imaging (MRI; 1.5 T). Brain volumes (white matter (WM), gray matter, amygdala, hippocampus, thalamus), lesion load, fractional anisotropy (FA) of fronto-limbic tracts, and resting-state functional connectivity (FC) between limbic and frontal areas were measured and compared between groups. Regression analysis was performed to relate MRI measures to the severity of depression. Results: Compared to nDMS patients, DMS patients (shorter disease duration) had lower WM volume ( p < 0.01), decreased FA of the uncinate fasciculus ( p < 0.05), and lower FC between the amygdala and frontal regions ( p < 0.05). Disease duration, FA of the uncinate fasciculus, and FC of the amygdala could explain 48% of variance in the severity of depression. No differences in cognition were found. Conclusion: DMS patients showed more pronounced (MS) damage, that is, structural and functional changes in temporo-frontal regions, compared to nDMS patients, suggestive of fronto-limbic disconnection.

Endocrine ophthalmopathy

2019 ◽  
Vol 44 (2) ◽  
pp. 22-27
Author(s):  
T. L. Pavlova ◽  
G. A. Kotova ◽  
G. A. Gerasimov
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
Objective Assessment ◽  
The State ◽  
Resonance Imaging ◽  
Treatment Methods ◽  
Ultrasound Computed Tomography ◽  
Endocrine Ophthalmopathy ◽  
Magnetic Resonance Imaging Mri

The urgency of the problem of endocrine ophthalmopathy (EOP) is currently in no doubt. This is due to the fact that relatively recently methods of objective assessment of the state of the eyeball and orbital tissues using ultrasound (ultrasound), computed tomography (CT) and magnetic resonance imaging (MRI) have appeared. At the same time, the etiology and pathogenesis of EOP are not well understood, which undoubtedly affects the validity and effectiveness of various treatment methods.

scholarly journals The Role of Advanced MRI in the Development of Treat-to-Target Therapeutic Strategies, Patient Stratification and Phenotyping in Rheumatoid Arthritis

2019 ◽  
Author(s):  
Ali Mobasheri ◽  
Mark Hinton ◽  
Olga Kubassova
Keyword(s):  
Rheumatoid Arthritis ◽  
Bone Marrow Edema ◽  
Objective Assessment ◽  
Treat To Target ◽  
Resonance Imaging ◽  
Advanced Imaging ◽  
Dce Mri ◽  
Contrast Enhanced ◽  
Magnetic Resonance Imaging Mri

Abstract In this commentary we discuss the potential of advanced imaging, particularly Dynamic Contrast Enhanced (DCE) magnetic resonance imaging (MRI) for the objective assessment of disease progression in rheumatoid arthritis (RA). We emphasise the potential DCE-MRI in advancing the field and exploring new areas of research and development in RA. We believe that different grades of bone marrow edema (BME) and synovitis in RA can be examined and monitored in a more sensitive manner with DCE-MRI. Future treatments for RA will be significantly improved by enhanced imaging of BMEs and synovitis. DCE-MRI will also facilitate enhanced stratification and phenotyping of patients enrolled in clinical trials.

scholarly journals Is there a link between COVID-19 and Creutzfeldt-Jakob Disease? a Case Report

2021 ◽  
Vol 9 (1) ◽  
pp. 26-26
Author(s):  
Ehsan Nasiri ◽  
Amirreza Naseri ◽  
Mohammad Yazdchi ◽  
Mahnaz Talebi
Keyword(s):  
Protein Detection ◽  
Behavioral Changes ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Progressive Dementia ◽  
Abnormal Signal ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Abnormal Signal Intensity ◽  
Magnetic Resonance Imaging Mri

Creutzfeldt-Jakob Disease (CJD) is a rare rapidly progressive neurodegenerative disease. The diagnosis of CJD is based on magnetic resonance imaging (MRI) findings, electro-encephalography (EEG), or 14-3-3 protein detection. We report a case of a previously-healthy 72 years old woman, with evidence of coronavirus disease 2019 (COVID-19), who complained of behavioral changes and rapidly progressive dementia. While hospitalization, she didn't have orientation to time and place and repeated an irrelevant sentence in response to questions. Also, anomia and impaired comprehension was observed. Myoclonic jerks, abnormal signal intensity at bilateral parieto-occipital cortices in MRI, periodic sharp wave complexes in EEG, and increased lactate dehydrogenase in cerebrospinal fluid (CSF), highly recommended CJD for her. This is the second case of CJD after COVID-19 during this pandemic, which can be an alarm to clinicians about the silent impact of COVID-19 on the central nervous system.

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