Presentation
Analyses of the CSF and serum revealed anti SOX-1 antibodies at initial presentation but PCA-2 antibodies seven months after diagnosis. [ncbi.nlm.nih.gov]
Patients can express more than one autoantibody and present with more than one neuronal syndrome. [mdedge.com]
This edition presents expanded coverage of neurophysiology and motor neurone disease. [books.google.com]
[…] dysfunction is the most common manifestation in patients with anti-Ma2 encephalitis which is one of the major causes of paraneoplastic limbic encephalitis (LE), it has been reported that less than 30% of the patients with anti-Ma2 LE exhibit clinical presentations [unboundmedicine.com]
Lacking generally accepted diagnostic criteria of NPLE, we recommend a long-term follow-up with repeated neurological examination and oncological survey of patients presenting without evidence of a malignancy. [neurologia.com.pl]
Immune System
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Mediastinal Lymphadenopathy
After three cycles of therapy, he was re-evaluated with a chest computed tomography which showed a 50% reduction in the tumor mass and in the size of the hilar and mediastinal lymphadenopathy. [jmedicalcasereports.biomedcentral.com]
Entire Body System
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Excessive Daytime Sleepiness
Of the remaining, many exhibit excessive daytime sleepiness, vertical ophthalmoparesis, or both associated with LE, because of frequent involvement of the diencephalon and/or upper brainstem. [unboundmedicine.com]
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Pathologist
She is a licensed physician since 1987, licensed pathologist since 1991 and she gained her title as Doctor in Medical Sciences in 1986. [books.google.com]
Psychiatrical
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Compulsive Disorder
Anti-Ma2 LE can also be manifested as a pure psychiatric disturbance such as obsessive-compulsive disorder in a few cases. [unboundmedicine.com]
Workup
If a primary tumor is discovered during the workup, it should be removed and treated with the most up-to-date oncologic treatment available. [ncbi.nlm.nih.gov]
An extensive search for an underlying malignancy should be undertaken, guided by the type of antibody discovered on the workup. Most authorities recommend screening with whole body FDG-PET/CT. [ahcmedia.com]
• Onset Painful paresthesias & dysesthesias Asymmetric; Distal or Proximal No tumor at initial workup: 50% • Sensory loss (95%) All modalities involved Proprioceptive loss: Prominent Ataxia: Sensory Pseudoathetosis Distribution Proximal [slideshare.net]
Results of autoimmune workup revealed positive LGI1 antibody which is part of VGKC. That confirmed our diagnosis. [jneuro.com]
EEG
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Epileptiform Activity
EEG showed paroxysmal epileptiform activity in both temporal lobes. The results of viral serologic tests were negative. The results of CSF polymerase chain reaction for herpes simplex virus 1 and 2 were negative. [ajnr.org]
Other Pathologies
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Lymphocytic Infiltrate
Examination of the peripheral nervous system revealed subperineurial lymphocytic infiltration of the myelin. F ig 3. Reactive astrocytosis and lymphocytic infiltration with T cells were found. A, Glial fibrillary acidic protein immunostaining. [ajnr.org]
A stereotactic brain biopsy was performed and the pathology specimen revealed brain tissue with areas of lymphocyte infiltration and gliosis, with no evidence of tumor cells (Fig. 3 ). Figure 2 Magnetic resonance spectroscopy of the brain. [jmedicalcasereports.biomedcentral.com]
Treatment
The limited stage of the disease, irrespective of anti-neuronal antibody status, is associated with neurological improvement after tumor treatment. The treatment of LE is generally unsatisfactory. [jmedicalcasereports.biomedcentral.com]
While it can cause severe neurological deficits or death in a substantial proportion of the patients, approximately one-third show neurological improvement and another 20 - 40% stabilize in response to treatment, including immunotherapy and/or tumor treatment [unboundmedicine.com]
Her seizures were refractory to treatment, and she was given pentobarbital to induce a coma. [mdedge.com]
The fourth edition has been fully revised and updated to take into account current developments in the investigation and treatment of neurological disorders. [books.google.com]
Prognosis
PMID: 20212230 Prognosis Li H, Zhang A, Hao Y, Guan H, Lv Z Medicine (Baltimore) 2018 May;97(19):e0696. doi: 10.1097/MD.0000000000010696. PMID: 29742721 Free PMC Article McKeon A, Tracy JA Muscle Nerve 2017 Jul;56(1):15-27. [ncbi.nlm.nih.gov]
Source MeSH Adult Animals Antigens, Neoplasm Autoantibodies Biomarkers Carcinoma, Non-Small-Cell Lung Diagnosis, Differential Female Humans Limbic Encephalitis Lung Neoplasms Male Middle Aged Neoplasms, Germ Cell and Embryonal Nerve Tissue Proteins Prognosis [unboundmedicine.com]
Incidence, clinical presentation, laboratory evaluation, differential diagnosis, prognosis and treatment of this entity are discussed. [jmedicalcasereports.biomedcentral.com]
A remarkable finding was the poor prognosis of patients with dysautonomia; a tumour was found in 37 out of 51 cases and in 28 of these the diagnosis followed the neurological syndrome with a median time of 4.6 months. [cordis.europa.eu]
Anti-Ma2-associated encephalitis may improve after a combination of immunotherapy and tumor removal 21 ; the others have a poor prognosis. [mdedge.com]
Etiology
Etiology Handb Clin Neurol 2017;145:285-299. doi: 10.1016/B978-0-12-802395-2.00020-1. PMID: 28987176 Dash D, Tripathi M, Ihtisham K, Tripathi M BMJ Case Rep 2016 Oct 13;2016 doi: 10.1136/bcr-2016-217083. [ncbi.nlm.nih.gov]
Keywords Small Cell Lung Cancer Seminoma Paraneoplastic Neurological Syndrome Cerebral Gliomatosis Typical Clinical Picture Introduction The etiology of seizure disorders in patients with cancer is broad. [jmedicalcasereports.biomedcentral.com]
"Etiologies of epilepsy: a comprehensive review". Expert Rev Neurother. 11 (6): 861–76. doi : 10.1586/ern.11.51. PMID 21651333. Retrieved from " " Categories : Encephalitis [en.wikipedia.org]
Further imaging to look for etiology revealed no evidence of any mass lesion, tumour or secondaries anywhere in the body. [jneuro.com]
In fact, viral etiology is the most frequent of encephalitis. However, among all viral factors there is one that is especially important in the case of EL, herpes simplex virus type 1 (HSV-1). [lifepersona.com]
Epidemiology
The epidemiology of tumor-related autoimmune encephalitis mimics that of the underlying malignancy. Those with non-tumor related autoimmune encephalitis have a variable epidemiology but are mostly young patients with a female predilection 8. [radiopaedia.org]
While the database was not designed for the conduction of epidemiological studies, the data is considered to represent the relative prevalence of each syndrome. [cordis.europa.eu]
Scott R Plotkin MV Dorfman is studying for a Master of Public Health in Epidemiology at the Boston University School of Public Health. Molly V Dorfman JS Loeffler is the Herman and Joan Suit Professor of Radiation Oncology at Harvard Medical School. [nature.com]
Epidemiology With an annual incidence of 2–3/100,000 in northern Europe, 15 encephalitis of any aetiology is half as common as newly diagnosed multiple sclerosis(4–8/100,000/year). 16 While 40 % of cases are infectious and 40 % are due to unknown causes [touchneurology.com]
Pathophysiology
It is basically autoimmune in nature, with its pathophysiology determined by a number of antibodies to neural surface proteins. [jneuro.com]
Prevention
Degeneration Within a few months it reaches its peak and then stabilizes most cannot walk without support cannot sit unsupported handwriting is impossible eating independently difficult speech very difficult to understand oscillopsia may prevent [slideshare.net]
Since it is therapeutically responsive, so timely diagnosing and proper intervention will prevent permanent neurologic damage. 1. Brierley JB, Corsellis JA, Hierons R, Nevin S. Subacute encephalitis of later adult life. [annalsofian.org]