Treatment of Movement Disorder Emergencies in Autoimmune Encephalitis in the Neurosciences ICU

AbstractImmune response against neuronal and glial cell surface and cytosolic antigens is an important cause of encephalitis. It may be triggered by activation of the immune system in response to an infection (para-infectious), cancer (paraneoplastic), or due to a patient ’s tendency toward autoimmunity. Antibodies directed toward neuronal cell surface antigens are directly pathogenic, whereas antibodies with intracellular targets may become pathogenic if the antigen is transiently exposed to the cell surface or via activation of cytotoxic T cells. Immune-mediated encephalitis is well recognized and may require intensive care due to status epilepticus, need for invasive ventilation, or dysautonomia. Patients with immune-mediated encephalitis may become critically ill and display clinically complex and challenging to treat movement disorders in over 80% of the cases (Zhang et al. in Neurocrit Care 29(2):264–272, 2018). Treatment options include immunotherapy and symptomatic agents affecting dopamine or acetylcholine neurotransmission. There has been no prior published guidance for management of these movement disorders for the intensivist. Herein, we d iscuss the immune-mediated encephalitis most likely to cause critical illness, clinical features and mechanisms of movement disorders and propose a management algorithm.
Source: Neurocritical Care - Category: Neurology Source Type: research

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AbstractBackgroundParaneoplastic syndromes (PNS) are autoimmune disorders specifically associated with cancer. There are few data on anti-PD-1 or anti-PD-L1 immunotherapy in patients with a PNS. Our objective was to describe the outcome for patients with a pre-existing or newly diagnosed PNS following the initiation of anti-PD-1 or anti-PD-L1 immunotherapy.MethodsWe included all adult patients (aged ≥18) treated with anti-PD-1 or anti-PD-L1 immunotherapy for a solid tumor, diagnosed with a PNS, and registered in French pharmacovigilance databases. Patients were allocated to cohorts 1 and 2 if the PNS had been diagnosed ...
Source: Journal for Immunotherapy of Cancer - Category: Cancer & Oncology Source Type: research
Conclusion: Anti-CV2 autoimmune encephalitis can present as Parkinsonism with bilateral leukoencephalopathy on MRI. PET scanning can be useful to reveal an occult cancer. Treatment of the cancer may improve the paraneoplastic neurological syndrome without the need of immunosuppressive therapy.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Conclusions We show a clear association between ICI use and increased diagnosis of Ma2-PNS. Physicians need to be aware that ICIs can trigger Ma2-PNS because clinical presentation can be challenging.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: All Immunology, Autoimmune diseases, All Oncology, Paraneoplastic syndrome Article Source Type: research
Ma2 antibody-associated encephalitis is an inflammatory brain disease that associates with a systemic tumor in more than 90% of patients, most commonly a testicular germ-cell tumor, lung cancer, or breast cancer. The Ma2 antibody-mediated autoimmune encephalitis presents mostly as limbic, mesodiencephalic, or brain stem encephalitis. Cranial MRI often detects T2-hyperintense lesions that may progress to atrophy. However, other areas of the CNS, such as the brainstem, the thalamus, the hypothalamus, the cerebellum, or the basal ganglia may also be affected. Approximately one-third of patients with Ma2 antibody-associated en...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Chorea Clinical/Scientific Notes Source Type: research
Conclusion Paraneoplastic limbic encephalitis is a rare paraneoplastic syndrome in nervous system caused by malignant neoplasms often characterized by facial neurological symptoms. The disease are usually associated with lung cancer (especially SCLC). Its nervous system symptoms occur earlier than the tumor diagnosis. Early diagnosis and treatment for primary tumors will increase the benefit. 
 DOI: 10.3779/j.issn.1009-3419.2019.03.02
Source: Chinese Journal of Lung Cancer - Category: Cancer & Oncology Source Type: research
Semin Neurol 2018; 38: 330-343 DOI: 10.1055/s-0038-1660821The understanding of the manifestations, mechanisms, and management of autoimmune encephalitis has expanded dramatically in recent decades. Immune-mediated encephalitides are comparable in incidence and prevalence to infectious etiologies, and are associated with significant morbidity, especially when there is a delay in recognition and treatment. As such, clinicians from many specialties must develop a functional understanding of these disorders. Herein we provide an overview of the autoimmune and paraneoplastic encephalitides, including those associated with eithe...
Source: Seminars in Neurology - Category: Neurology Authors: Tags: Review Article Source Type: research
Purpose of Review: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. Recent Findings: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies. Recently, the pathogenic mechanisms and antigens of these disorders have been further elucidated. Paraneoplastic...
Source: CONTINUUM: Lifelong Learning in Neurology - Category: Neurology Tags: Review Articles Source Type: research
We report a unique case of brainstem encephalitis with a sudden lethal outcome following ipilimumab and pembrolizumab therapy in a patient with malignant melanoma. The autopsy showed a diffuse nodular activation of microglia in the whole encephalon with prominent intraparenchymal and perivascular lymphocytic infiltration of the brainstem. Non‐infectious brainstem encephalitis is a well‐recognized subset of paraneoplastic encephalitis. Brainstem involvement is usually accompanied by a wide spectrum of signs and symptoms, which were not observed in this case. The timing of the clinical symptoms as well as the histopathol...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Symptom Management and Supportive Care, Genitourinary Cancer Brief Communications Source Type: research
We report a unique case of brainstem encephalitis with a sudden lethal outcome following ipilimumab and pembrolizumab therapy in a patient with malignant melanoma. The autopsy showed a diffuse nodular activation of microglia in the whole encephalon with prominent intraparenchymal and perivascular lymphocytic infiltration of the brainstem. Non-infectious brainstem encephalitis is a well-recognized subset of paraneoplastic encephalitis. Brainstem involvement is usually accompanied by a wide spectrum of signs and symptoms, which were not observed in this case. The timing of the clinical symptoms as well as the histopathologic...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Oncologist Source Type: research
Conclusions:Paraneoplastic neurological syndromes are associated with antibodies against intracellular neuronal antigens and are often resistant to treatment, whereas autoimmune processes (with or without cancer) have antibodies to cell-surface antigens and respond to immunotherapy. Subacute cognitive decline, new seizures, focal neurologic exam, CSF and MRI features should arouse suspicion. Autoantibodies are often inaccessible to many centers or are absent.Disclosure: Dr. Ahmed has nothing to disclose. Dr. Venkatachalam has nothing to disclose. Dr. Khan has nothing to disclose. Dr. Kentris has nothing to disclose. Dr. Pi...
Source: Neurology - Category: Neurology Authors: Tags: General Neurology: Inflammation, Infections, and Autoimmunity Source Type: research
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