EEG Differences in Two Clinically Similar Rapid Dementias: Voltage-Gated Potassium Channel Complex-Associated Autoimmune Encephalitis and Creutzfeldt-Jakob Disease

Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex-associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015. More patients in the VGKC AE group had seizures (12/17) than those with CJD (3/14; P = .008). Serum sodium levels were lower in those with VGKC AE ( P = .001). Cerebrospinal fluid (CSF) white blood cell count was higher in VGKC AE ( P = .008). CSF protein 14-3-3 ( P = .018) was more commonly detected in CJD, and tau levels were higher in those with CJD ( P < .006). On neuroimaging, diffusion restriction in the cortex ( P = .001), caudate ( P < .001), and putamen ( P = .001) was more frequent in CJD. Periodic sharp wave complexes ( P = .001) and generalized suppressed activity ( P = .008) were more common on initial EEG in CJD. On serial EEGs, generalized periodic discharges ( P = .004), generalized suppressed activity (P=0.008), and periodic sharp wave complexes ( P < .001) were detected more in CJD. This study shows that there are a number of differentiating features between CJD and VGKC AE, and electroencephalography can aid in their diagnoses. Performing serial EEGs better delineates these conditions

Electroencephalographic and fluorodeoxyglucose-positron emission tomography correlates in anti-N-methyl-d-aspartate receptor autoimmune encephalitis

Importance: Anti-N-methyl-d-aspartate receptor (anti-NMDAR) autoimmune encephalitis is an increasingly recognized cause of limbic encephalitis (LE). Prolonged LE and limbic status epilepticus (LSE) share many features. The ability to distinguish between the two is crucial in directing appropriate therapy because of the potential iatrogenesis associated with immunosuppression and anesthetic-induced coma. Observations: A 34-year-old woman with recurrent LE developed behavioral changes, global aphasia, and repetitive focal and generalized tonic–clonic seizures. Because asymmetric rhythmic delta patterns recurred on electroencephalography (EEG) despite treatment with nonsedating antiepileptic drugs followed by anesthetic-induced coma, an investigation to distinguish LSE from LE was undertaken. Implanted limbic/temporal lobe depth electrodes revealed no epileptiform activity. Brain single-photon emission computerized tomography (SPECT) showed no hyperperfusion, and brain fluorodeoxyglucose-positron emission tomography (FDG-PET) showed hypermetabolism in the left frontal, temporal, and parietal cortices. Anti-N-methyl-d-aspartate receptor autoimmune encephalitis was diagnosed based detection of anti-NMDAR antibody in the cerebrospinal fluid (CSF). With chronic immunosuppression, the resolution of brain FDG-PET abnormalities paralleled clinical improvement. Conclusions and relevance: This case of anti-NMDAR autoimmune encephalitis illustrates the challenges of distinguishing prolonged LE from LSE. We discuss the parallels between these two conditions and propose a management paradigm to optimize evaluation and treatment

AE burden supplementary information

Supplemental Figures and Table

Supplemental Material - Images in Clinical Neurology Relapsing Anti-GAD65-Associated Encephalitis Responsive to Thymoma Resection, Radiation, and Immunomodulatory Therapy

Supplemental Material for Images in Clinical Neurology Relapsing Anti-GAD65-Associated Encephalitis Responsive to Thymoma Resection, Radiation, and Immunomodulatory Therapy by Jonathan D. Krett, Maria E. Molinaro, Justin C. McArthur, David O. Kamson, Kemar E. Green, and John C. Probasco in he Neurohospitalist.</p

Data from: Autoimmune encephalitis: a costly condition

Objective: To assess the inpatient hospitalization burden and costs of patients with autoimmune encephalitis (AE) at a tertiary care institution. Methods: Adult inpatients with AE were identified retrospectively from July 1, 2005 – June 30, 2015. Demographic and clinical data were collected and analyzed. Billing data were compared to that of patients with herpes simplex encephalitis (HSE). Charges were adjusted for inflation. Results: Of 244 admissions for encephalitis reviewed, 63 patients met criteria for probable or definite AE. Thirty-one (49%) patients were antibody-positive, and twenty-seven (43%) were admitted to the intensive care unit (ICU). Median hospital charges per AE patient were over $70k, median length of stay (LOS) was 15 days, and in hospital mortality was 6$173k/ admission vs. non-ICU $50k/ admission, p<0.001). LOS was strongly associated with charges and was driven by delay in diagnosis of AE, prolonged treatment courses, and lack of response to therapy. In comparison with HSE, median hospital charges per AE patient were nearly 4 times higher, median AE LOS was 3 times higher, and total charges over the study period were nearly twice as high. Conclusions: AE patients utilized more inpatient healthcare resources per patient during a ten-year period than HSE at our institution. ICU-admitted AE patients were responsible for a substantially higher financial burden than non-ICU-admitted AE patients. Our data underscore the need for the development of novel diagnostic and therapeutic modalities to improve patient outcomes and decrease hospital burden in AE