Clinical Neuropathology practice guide 4-2013: post-herpes simplex encephalitis: N-methyl-Daspartate receptor antibodies are part of the problem

Höftberger, Romana; Armangué, Thaís; Leypoldt, Frank; Graus, Francesc; Dalmau, Josep

doi:10.5414/np300666

Cited by 41 publications

(32 citation statements)

References 33 publications

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“…The strongest association appears to be in children developing choreoathetosis and cognitive impairment weeks to months after proven HSVE, suggesting that NGSAbs can arise as a result of virally induced neuronal damage 78–81. Given the marked similarity of this presentation to many typical patients with NMDAR antibodies, and the observed response to immunotherapy, these NMDAR antibodies are likely to be pathogenic 78,82…”

Section: Red Flags To the Presence Of A Cell-surface Cns-directed Autmentioning

confidence: 97%

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

Irani

Gelfand

Al-Diwani

et al. 2014

Annals of Neurology

167

149

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The recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface–expressed neuronal or glial proteins such as LGI1, N-methyl-D-aspartate receptor, and aquaporin-4. The original descriptions of the associated clinical syndromes were phenotypically well circumscribed. However, as availability of antibody testing has increased, the range of associated patient phenotypes and demographics has expanded. This in turn has led to the recognition of more immunotherapy-responsive syndromes in patients presenting with cognitive and behavioral problems, seizures, movement disorders, psychiatric features, and demyelinating disease. Although antibody detection remains diagnostically important, clinical recognition of these distinctive syndromes should ensure early and appropriate immunotherapy administration. We review the emerging paradigm of cell-surface–directed antibody–mediated neurological diseases, describe how the associated disease spectrums have broadened since the original descriptions, discuss some of the methodological issues regarding techniques for antibody detection and emphasize considerations surrounding immunotherapy administration. As these disorders continue to reach mainstream neurology and even psychiatry, more cell-surface–directed antibodies will be discovered, and their possible relevance to other more common disease presentations should become more clearly defined.

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Section: Red Flags To the Presence Of A Cell-surface Cns-directed Autmentioning

confidence: 97%

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

Irani

Gelfand

Al-Diwani

et al. 2014

Annals of Neurology

167

149

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“…12 It has been demonstrated that some types of viral encephalitis can trigger autoimmune LE, 53,54 particularly anti-Nmethyl-D-aspartate receptor encephalitis. 55 This phenomenon occurs when prolonged or atypical neurologic symptoms recur after successful control of the viral infection. Some patients with negative viral results develop a syndrome described as relapsing post-HSE or choreoathetosis post-HSE.…”

Section: Infectious Lementioning

confidence: 99%

Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders

Rocha

Nunes

Maia

et al. 2015

AJNR Am J Neuroradiol

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SUMMARY: Limbic encephalitis is far more common than previously thought. It is not always associated with cancer, and it is potentially treatable. Autoantibodies against various neuronal cell antigens may arise independently or in association with cancer and cause autoimmune damage to the limbic system. Neuroimaging plays a key role in the management of patients with suspected limbic encephalitis by supporting diagnosis and excluding differential possibilities. This article describes the main types of autoimmune limbic encephalitis and its mimic disorders, and emphasizes their major imaging features.ABBREVIATIONS: AME ϭ autoimmune-mediated encephalopathy; AMPAR ϭ ␣-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; CASPR2, contactin-asso-

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“…They were found to be HSV-negative by polymerase chain reaction in CSF, but positive for anti-NMDA-R antibody, and subsequently did not respond to antiviral therapy. This subgroup did respond to immunotherapy 35,36…”

Section: Differential Diagnosismentioning

confidence: 91%

Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges

Mann¹,

Grebenciucova²,

Lukas³

2014

TCRM

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ObjectiveAnti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune disorder, often paraneoplastic in nature, presenting with complex neuropsychiatric symptoms. Diagnosed serologically, this disorder is often responsive to immunosuppressant treatment. The objective of this review is to educate clinicians on the challenges of diagnosis and management of this disorder.Materials and methodsA review of the relevant literature on clinical presentation, pathophysiology, and recommended management was conducted using a PubMed search. Examination of the results identified articles published between 2007 and 2014.ResultsThe literature highlights the importance of recognizing early common signs and symptoms, which include hallucinations, seizures, altered mental status, and movement disorders, often in the absence of fever. Although the presence of blood and/or cerebrospinal fluid autoantibodies confirms diagnosis, approximately 15% of patients have only positive cerebrospinal fluid titers. Antibody detection should prompt a search for an underlying teratoma or other underlying neoplasm and the initiation of first-line immunosuppressant therapy: intravenous methylprednisolone, intravenous immunoglobulin, or plasmapheresis, or a combination thereof. Second-line treatment with rituximab or cyclophosphamide should be implemented if no improvement is noted after 10 days. Complications can include behavioral problems (eg, aggression and insomnia), hypoventilation, catatonia, and autonomic instability. Those patients who can be managed outside an intensive care unit and whose tumors are identified and removed typically have better rates of remission and functional outcomes.ConclusionThere is an increasing need for clinicians of different specialties, including psychiatrists, neurologists, oncologists, neurooncologists, immunologists, and intensivists to become familiar with this disorder and its potential complications. Remission can be optimized with prompt detection and aggressive, collaborative treatment within a multidisciplinary team.

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Clinical Neuropathology practice guide 4-2013: post-herpes simplex encephalitis: N-methyl-Daspartate receptor antibodies are part of the problem

Cited by 41 publications

References 33 publications

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

Cell‐surface central nervous system autoantibodies: Clinical relevance and emerging paradigms

Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders

Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges

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