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Classic Paraneoplastic Limbic Encephalitis
Classic Paraneoplastic Limbic Encephalitis with or without Intracellular Antigens

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]

Some patients present more slowly with psychiatric manifestations. The MRI may be normal or may demonstrate medial temporal T2 hyperintensity. [ahcmedia.com]

Clinical presentation is variable but typically is gradual with short-term memory loss (anterograde memory impairment) and mental status changes. [radiopaedia.org]

Immune System

  • 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]

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]

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]

Prognosis

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]

Affected patients are often older and have an unfavorable prognosis. [paperity.org]

• Early diagnosis and treatment are associated with a better prognosis and decreased risk of relapse. • Treatment of the underlying disorder usually improves the movement disorders, but symptomatic treatment may be necessary as well. [medlink.com]

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]

Etiology

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]

Therefore, for the clinician, markers are important which on the one hand prove the paraneoplastic etiology of the given neurological syndrome, and on the other hand help to diagnose the associated tumor. [degruyter.com]

Clin Infect Dis 49:1838–1847 Article PubMed Google Scholar Glaser CA, Honarmand S, Anderson LJ, et al (2006) Beyond viruses: clinical profiles and etiologies associated with encephalitis. [link.springer.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]

Clin Infect Dis 35:254–260 Article PubMed Google Scholar Mailles A, Stahl J, Brouard C, Vaillant V (2008) Epidemiology, improvement of etiological diagnosis, and outcome of infectious encephalitis in France in 2007: preliminary results of a national prospective [link.springer.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]

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