TY - JOUR
T1 - Autoimmune encephalopathies
AU - Vernino, Steven
AU - Geschwind, Michael
AU - Boeve, Bradley
N1 - Funding Information:
Y. Hacohen has no financial disclosures. M. Lim receives research grants from Action Medical Research (SP4472), DES society (A20249PID10583), GOSH charity (V1214), NIHR (HTA11/129/148; EME12/212/15), MS Society (893/08), SPARKS charity (11OUH01; 12KCL02), London Clinical Research Network (2011FSF; 2014FSF), Evelina Appeal (X141005); has received consultation fees from CSL Behring ; received travel grants from Merck Serono ; and awarded educational grants to organize meetings by Novartis , Biogen Idec , Merck Serono, and Bayer . A. Vincent serves/has served on scientific advisory boards for the Patrick Berthoud Trust, the Brain Research Trust, and the Myasthenia Gravis Foundation of America; has received funding for travel and a speaker honorarium from Baxter International and Biogen ; receives royalties from the publication of Clinical Neuroimmunology ( Blackwell Publishing , 2005) and Inflammatory and Autoimmune Disorders of the Nervous System in Children ( Mac Keith Press , 2010); receives/has received research support from the European Union , NIHR Biomedical Research Centre Oxford, Euroimmun AG , and the Sir Halley Stewart Trust ; has received Musk antibody royalties and consulting fees from Athena Diagnostics Inc ; and holds patents and/or receive royalties and payments for antibody assays in neurologic diseases.
Funding Information:
Funding: This work was supported by Oxford University Clinical Academic Graduate School , Oxford (Y. Hacohen) and the National Institute for Health Research (NIHR) Oxford Biomedical Research Centre based at Oxford University Hospitals NHS Trust and the University Of Oxford (Y. Hacohen and A. Vincent).
PY - 2007/5
Y1 - 2007/5
N2 - BACKGROUND: Evaluation of patients with recent onset of progressive cognitive and behavioral problems can be challenging. Psychiatric disorders, metabolic derangements, toxins and infections are generally considered in the differential diagnosis along with prion disorders (Creutzfeldt-Jakob disease) and rapidly progressive degenerative dementias. Some subacute encephalopathies are caused by autoimmune or inflammatory mechanisms, recognized by the association with autoantibody markers and/or clear response to immunomodulatory treatment. This review describes the clinical features of these potentially reversible autoimmune encephalopathies. REVIEW SUMMARY: Morvan syndrome, paraneoplastic limbic encephalitis (PLE), and nonparaneoplastic autoimmune limbic encephalitis have characteristic clinical and serological features. Limbic encephalitis is characterized by short-term memory impairment, complex partial temporal lobe seizures and psychiatric symptoms. Signal abnormalities in the mesial temporal lobes without contrast enhancement are the typical MRI findings. Morvan syndrome presents with behavioral changes, hallucinations, severe insomnia, autonomic hyperactivity and neuromyotonia (spontaneous muscle activity). Corticosteroid-responsive encephalopathy associated with evidence of thyroid autoimmunity (sometimes called Hashimoto encephalopathy) has a broad range of clinical presentation. Cognitive impairment with tremor, seizures, stroke-like events (including transient aphasia) and normal thyroid hormone levels is a common scenario. In the absence of diagnostic serological findings, clinical improvement with corticosteroids may be the only evidence of autoimmune encephalopathy. CONCLUSIONS: Autoimmune encephalopathies are an important cause of rapidly progressive cognitive and behavioral decline that probably remain under recognized. Electroencephalography, brain MRI, cerebrospinal fluid examination and serological tests are useful diagnostic tools. With increased clinical suspicion, these diseases may be diagnosed and treated successfully.
AB - BACKGROUND: Evaluation of patients with recent onset of progressive cognitive and behavioral problems can be challenging. Psychiatric disorders, metabolic derangements, toxins and infections are generally considered in the differential diagnosis along with prion disorders (Creutzfeldt-Jakob disease) and rapidly progressive degenerative dementias. Some subacute encephalopathies are caused by autoimmune or inflammatory mechanisms, recognized by the association with autoantibody markers and/or clear response to immunomodulatory treatment. This review describes the clinical features of these potentially reversible autoimmune encephalopathies. REVIEW SUMMARY: Morvan syndrome, paraneoplastic limbic encephalitis (PLE), and nonparaneoplastic autoimmune limbic encephalitis have characteristic clinical and serological features. Limbic encephalitis is characterized by short-term memory impairment, complex partial temporal lobe seizures and psychiatric symptoms. Signal abnormalities in the mesial temporal lobes without contrast enhancement are the typical MRI findings. Morvan syndrome presents with behavioral changes, hallucinations, severe insomnia, autonomic hyperactivity and neuromyotonia (spontaneous muscle activity). Corticosteroid-responsive encephalopathy associated with evidence of thyroid autoimmunity (sometimes called Hashimoto encephalopathy) has a broad range of clinical presentation. Cognitive impairment with tremor, seizures, stroke-like events (including transient aphasia) and normal thyroid hormone levels is a common scenario. In the absence of diagnostic serological findings, clinical improvement with corticosteroids may be the only evidence of autoimmune encephalopathy. CONCLUSIONS: Autoimmune encephalopathies are an important cause of rapidly progressive cognitive and behavioral decline that probably remain under recognized. Electroencephalography, brain MRI, cerebrospinal fluid examination and serological tests are useful diagnostic tools. With increased clinical suspicion, these diseases may be diagnosed and treated successfully.
KW - Cancer
KW - Dementia
KW - Methylprednisolone
KW - Prednisone
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U2 - 10.1097/01.nrl.0000259483.70041.55
DO - 10.1097/01.nrl.0000259483.70041.55
M3 - Review article
C2 - 17495758
AN - SCOPUS:34248394293
SN - 1074-7931
VL - 13
SP - 140
EP - 147
JO - Neurologist
JF - Neurologist
IS - 3
ER -