Background Encephalitis, characterised as inflammation of the brain tissue, is an important cause of acquired brain injury in children. Objective clinical outcomes vary significantly between affected patients, however they do not always correlate with quality of life as reported by parents.
JOURNAL ARTICLE Thymoma associated paraneoplastic encephalitis (TAPE), a potential cause of limbic encephalitis Henry Liu, Randall S Edson BMJ Case Reports 2019 August 30, 12 (8) A 59-year-old man presents with expressive aphasia and short term memory deficits. Shortly thereafter, he started developing staring spells and intermittent right hand spasms, preliminarily thought to be simple partial seizures. Subsequent MRI brain imaging was highly suggestive of herpes simplex virus (HSV) encephalitis; however, HSV PCR from cerebrospinal fluid was negative. On further testing, the patient was found to have an autoimmune encephalitis thought to be related to an incidentally found thymoma. His clinical presentation, in conjunction with imaging and response to therapy, was strongly suggestive of thymoma associated paraneoplastic encephalitis. Early recognition is the only way to ensure prompt initiation of appropriate treatment. Immunotherapy and cancer directed therapy (including tumour resection, if indicated) have been shown to have favourable outcomes, improved speed of neurological recovery and reduced risk of relapses. Without treatment, progressive neurologic deterioration can occur over months to years, eventually resulting in death. Comments You need to log in or sign up for an account to be able to comment. No comments yet, be the first to post one! Related Papers
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Autoimmune diseases associated with antineuronal and antiglial autoantibodies (Abs) is one of the most rapidly expanding research fields in clinical neuroimmunology, with more than 30 autoantibodies described so far.
Autoimmune encephalitis typically manifests with seizures and neuropsychiatric symptoms and may occur with or without cancer. The most frequent and best characterised type is anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. The target antigen of the autoantibodies is the membrane-spanning channel subunit NR1 of the NMDA receptor. Symptoms of anti-NMDA receptor encephalitis encompass psychosis, catatonia, seizures, dyskinesia, autonomic dysfunction and decreased consciousness. It is most common in young adults, and approximately 40-50 per cent of patients present with a neoplasm, predominantly ovarian teratoma. Other forms of autoimmune encephalitis are linked to autoantibodies against a multitude of further antigens. LGI1 and CASPR2 are specific target antigens of autoantibodies formerly thought to be directed against voltage-gated potassium channels. Anti-LGI1 reactivity is tightly associated with limbic encephalitis, with tumours occurring in 5-10 per cent of cases. Anti-CASPR2 autoantibodies have been described in patients with mostly encephalitis and/or peripheral nerve dysfunction (Morvan’s syndrome). 20-50 per cent of cases are linked to thymoma. Anti-DPPX encephalitis is a multifocal neurological disorder with prominent hyperexcitability of the central nervous system and rare (<10 per cent) association with lymphoma. Autoimmune encephalitis with reactivity against GABAB receptors is characterised by very prominent seizures, memory loss and confusion. Neoplasms, especially small-cell lung carcinoma, occur in about half of patients. Patients with anti-AMPA receptor encephalitis commonly exhibit subacute confusion, memory deficits, seizures and sometimes dementia. 70 per cent of cases are paraneoplastic, affecting the lungs, thymus and breast. There is evidence that autoantibodies in autoimmune encephalitis play a direct pathogenic role through antibody-driven inflammation and/or functional manipulation of the target antigen, which results in impairment of synaptic signal transduction. This is supported by the fact that these disorders can be effectively treated by immunotherapy.
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