Two Patients With an Anti-N-Methyl-d-Aspartate Receptor Antibody Syndrome-Like Presentation and Negative Results of Testing for Autoantibodies

Shah, Rikin; Veerapandiyan, Aravindhan; Winchester, Sara A.; Gallentine, William B.; Mikati, Mohamad A.

doi:10.1016/j.pediatrneurol.2011.09.012

Cited by 10 publications

(7 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Meanwhile other cell surface antigens—all elements of receptors—have been identified in patients with central nervous system (CNS) disorders that frequently have epilepsy: the N ‐methyl‐ d ‐aspartate receptor (NMDAR) subunit NR1 (Dalmau et al., ), the AMPA receptor subunits 1 and 2 (Lai et al., ), γ‐aminobutyric acid (GABA) B receptor subunit B1 (Lancaster et al., ), and metabotropic glutamate receptor type 5 (mGluR5) (Lancaster et al., 2011b). Finally, there are other patients with autoimmune epilepsies but negative results even upon extensive conventional antibody testing (as in the case of two children with an immunotherapy‐responsive disease that was indistinguishable from anti‐NMDAR encephalitis except for the presence of antibodies [Shah et al., ]). This can have different reasons: (1) false negative results, for example, due to insufficiently sensitive assays; (2) presence of still unrecognized antibodies; (3) other elements of the adaptive immune system like T cells exert adverse effects but are not accompanied by “marker antibodies” (as in the case of onconeural antibodies).…”

Section: Autoantibodies and Their Antigenic Targetsmentioning

confidence: 99%

See 1 more Smart Citation

Value of autoantibodies for prediction of treatment response in patients with autoimmune epilepsy: Review of the literature and suggestions for clinical management

Bien

2013

Epilepsia

View full text Add to dashboard Cite

SUMMARYThe detection of antineural autoantibodies in patients with epilepsy has led to the new concept of "autoimmune epilepsy." A particularly important implication is that knowledge of the antigenic target of the underlying antibody permits prognostic estimates. Patients with antibodies to the potassium channel complex (mostly to its leucinerich glioma inactivated 1 [LGI1] component) have a high chance of becoming seizure free within days to months upon immunotherapy but less so with antiepileptic drug (AED) treatment alone. Seizures in the setting of antibodies to the N-methyl-D-aspartate receptor also have a high likelihood to remit, again especially with rapid institution of immunotherapy. In contrast to these antibodies to neuronal surface molecules, antibodies directed to intracellular antigens (onconeural antibodies, antibodies to glutamic acid decarboxylase) portend a low likelihood of seizure remission, regardless of the treatments chosen. These outcome differences are probably related to the underlying pathophysiology-with largely reversible functional effects of antibodies to surface antigens and irreversible destructive sequelae (probably caused by T cells) in patients with antibodies to intracellular antigens. With ongoing experience with these conditions, clinical and paraclinical clues to the diagnosis of autoimmune epilepsies are emerging.

show abstract

Section: Autoantibodies and Their Antigenic Targetsmentioning

confidence: 99%

“… The full spectrum of “autoimmune epilepsy” still needs to be explored. Single case reports suggest epileptic manifestations that have not been considered to be autoimmune in origin yet but may in fact be so and may respond to immunotherapy (Shah et al., ; Suleiman et al., 2011b). …”

Section: Clinical Clues To Autoimmune Epilepsymentioning

confidence: 99%

Value of autoantibodies for prediction of treatment response in patients with autoimmune epilepsy: Review of the literature and suggestions for clinical management

Bien

2013

Epilepsia

View full text Add to dashboard Cite

show abstract

“…Antibody screening with currently available tests may result negative in some patients, even when clinical presentation is archetypal [104][105][106][107]. These patients may also improve under immunotherapy, which is strongly advised in cases with highly suggestive clinical presentation, after alternative etiologies have been appropriately excluded; tumor screening should also be carried out in these cases [108].…”

Section: Resultsmentioning

confidence: 99%

Neuropsychiatric symptoms in autoimmune encephalopathies: a clinician’s guide

Carvalho¹,

Massano²,

Coelho³

2014

IJCNMH

View full text Add to dashboard Cite

Background: The spectrum of central nervous system autoimmune disorders has recently expanded with the discovery of disorders associated with antibodies directed against the neuronal membrane surface. Although many of these disorders have an underlying malignancy and present with signs of dysfunction of the limbic system (paraneoplastic limbic encephalitis, PLE), a high proportion of cases is non-paraneoplastic. They may occur with milder symptoms, and present no abnormalities in the exams usually used in the investigation of PLE. A striking number of cases may be misdiagnosed as primary psychiatric or other neurological disorders. This paper aims to review the current knowledge on this topic, and provide physicians with an updated text on the neuropsychiatric presentation, diagnostic approach and current management of autoimmune encephalopathies.

show abstract

“…Detection of elevated antibody titer of N-type VGCC in his serum and his positive response to steroids and IVIG point towards an autoimmunity as the cause of his symptoms [6]. A case report of two boys with encephalitis of unknown etiology demonstrated the positive effect of immunotherapy even in the absence of a known antibody as the cause [7]. Patients with immune-mediated encephalitis can present with the wide spectrum of symptoms including psychosis, catatonia, alterations of behavior and memory, seizures, abnormal movements, and autonomic dysregulation [2].…”

Section: Discussionmentioning

confidence: 97%

N-type calcium channel antibody-mediated autoimmune encephalitis: An unlikely cause of a common presentation

Finkel

Koh

2013

Epilepsy & Behavior Case Reports

View full text Add to dashboard Cite

We report, to our knowledge, the only known pediatric case with encephalopathy and significantly elevated titers of N-type voltage-gated calcium channel antibody (N-type VGCC). The patient, an 8th grader, was previously healthy and presented with a one-week history of confusion, aphasia, transient fever, headaches, and dizziness. An underlying autoimmune process was suspected because of inflammatory changes in the brain MRI and multiple focal electrographic seizures captured in the EEG in the absence of CSF pleocytosis. Within 24 h of presentation, the patient was empirically started on immune-modulatory therapy, and a full recovery was achieved within 3 months of the initial presentation. Immune therapy included high-dose intravenous (IV) methylprednisolone followed by a 2-week course of dexamethasone and 2 monthly courses of IV immunoglobulin (IVIG). He was also treated with anticonvulsants for one month. No tumor has been found to date. There is a paucity of reports on autoimmune epilepsy or encephalopathy associated with N-type VGCC. Complete resolution of brain lesion, seizure freedom, and full recovery of function following early and aggressive immunotherapy demonstrate that a high index of suspicion is crucial for early recognition and treatment of autoimmune encephalitis.

show abstract

Two Patients With an Anti-N-Methyl-d-Aspartate Receptor Antibody Syndrome-Like Presentation and Negative Results of Testing for Autoantibodies

Cited by 10 publications

References 12 publications

Value of autoantibodies for prediction of treatment response in patients with autoimmune epilepsy: Review of the literature and suggestions for clinical management

Value of autoantibodies for prediction of treatment response in patients with autoimmune epilepsy: Review of the literature and suggestions for clinical management

Neuropsychiatric symptoms in autoimmune encephalopathies: a clinician’s guide

N-type calcium channel antibody-mediated autoimmune encephalitis: An unlikely cause of a common presentation

Contact Info

Product

Resources

About