Non-inflammatory cerebrospinal fluid delays the diagnosis and start of immunotherapy in anti-NMDAR encephalitis

Espínola-Nadurille, Mariana; Bautista-Gómez, Paola; Flores, José de Jesús Vargas; Rivas‐Alonso, Verónica; Pérez‐Esparza, Rodrigo; Solís-Vivanco, Rodolfo; Vargas‐Cañas, Steven

doi:10.1590/0004-282x20170179

Cited by 18 publications

(12 citation statements)

References 16 publications

(17 reference statements)

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“…In addition, in the present study more than a third of patients with anti-NMDAR encephalitis did not exhibit pleocytosis via routine CSF examination. This result is concordant with a previously reported case series in which initial non-inflammatory CSF varied widely in 15%-96% of patients at the first evaluation [9]. It is notable that initial noninflammatory CSF evidently does not exclude a diagnosis of anti-NMDAR encephalitis, and in the past some patients may have been misdiagnosed leading to delays in the initiation of immunotherapy.…”

Section: Discussionsupporting

confidence: 90%

Characteristics of cerebrospinal fluid cytology in anti-N-methyl-D-aspartate receptor encephalitis

Liu

Ren

Zhao

et al. 2019

Preprint

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Background The aim of the current study was to explore the characteristics of cerebrospinal fluid (CSF) cytology in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods CSF was collected from patients with anti-NMDAR encephalitis at Peking Union Medical College Hospital and Henan Provincial People’s Hospital from 01 January 2015 to 31 December 2018, and cytological characteristics and other parameters of the CSF were analyzed.Results CSF cytological data were obtained from 164 patients with anti-NMDAR encephalitis. Visible signs of inflammation were identified in cytological analyses of 112 patients’ CSF, including 46 cases of mild inflammation, 58 of moderate inflammation, and 8 of severe inflammation. With regard to inflammation type, 89 cases were classified as lymphocytic inflammation, 22 as mixed inflammation with both lymphocytes and neutrophils, and 1 case was classified as mixed inflammation with lymphocytes, neutrophils, and eosinophils. Activated lymphocytes were detected in 51 patients, and plasma cells were detected in 16. Oligoclonal bands were detected in 111/164 patients, and 51 patients were positive for specific oligoclonal bands in CSF. The positivity rate was 45.9%. In comparison with routine CSF examination with an inflammation positivity threshold of > 5 × 10 6 cells/L, the inflammation positivity rate of CSF cytology was significantly higher (68.3% versus 64.0%, p < 0.001).Conclusions The comparatively higher inflammation positivity rate and the superiority of evaluating cell morphology render CSF cytology an effective tool for identifying anti-NMDAR encephalitis and investigating its pathogenesis.

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Section: Discussionsupporting

confidence: 90%

Characteristics of cerebrospinal fluid cytology in anti-N-methyl-D-aspartate receptor encephalitis

Liu

Ren

Zhao

et al. 2019

Preprint

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“…The most common antibody found in our study was anti-NMDAR, as reported in other series of AE patients (13). A recent study of 29 cases of anti-NMDAR encephalitis reported an absence of inflammatory markers in CSF in almost 50% of patients, and a delayed time to diagnosis in these cases (14). In our study, time to diagnosis was similar in anti-NMDAR encephalitis patients regardless of their inflammatory status.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Inflammatory Markers in Autoimmune Encephalitis Associated With Antibodies Against Neuronal Surface in Brazilian Patients

et al. 2019

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Acute encephalitis is a debilitating neurological disorder associated with brain inflammation and rapidly progressive encephalopathy. Autoimmune encephalitis (AE) is increasingly recognized as one of the most frequent causes of encephalitis, however signs of inflammation are not always present at the onset which may delay the diagnosis. We retrospectively assessed patients with AE associated with antibodies against neuronal surface diagnosed in reference centers in Northeast of Brazil between 2014 to 2017. CNS inflammatory markers were defined as altered CSF (pleocytosis >5 cells/mm 3 ) and/or any brain parenchymal MRI signal abnormality. Thirteen patients were evaluated, anti-NMDAR was the most common antibody found (10/13, 77%), followed by anti-LGI1 (2/13, 15%), and anti-AMPAR (1/13, 7%). Median time to diagnosis was 4 months (range 2–9 months). Among these 13 patients, 6 (46.1%) had inflammatory markers and when compared to those who did not present signs of inflammation, there were no significant differences regarding the age of onset, time to diagnosis and modified Rankin scale score at the last visit. Most of the patients presented partial or complete response to immunotherapy during follow-up. Our findings suggest that the presence of inflammatory markers may not correlate with clinical presentation or prognosis in patients with AE associated with antibodies against neuronal surface. Neurologists should be aware to recognize clinical features of AE and promptly request antibody testing even without evidence of inflammation in CSF or MRI studies.

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“…2 In previous reports, initial non-inflammatory CSF were noted in 15%-96% of the patients. 3 EEG could be a diagnostic aid, given that in most case series, it was abnormal in more than 90% of the cases. 4 But findings were usually non-specific.…”

Section: Conflict Of Interestmentioning

confidence: 99%

“…5 The absence of CSF pleocytosis and/or increased proteins are known to delay diagnosis, as well as the length of time to initiate immunotherapy in patients with anti-NMDA encephalitis. 3 Additionally, EEG and MRI could be a diagnostic aid, but the absence of abnormality of them does not exclude anti-NMDAR encephalitis. In conclusion, follow up tests with close observation may be considered in patients with the atypical psychiatric symptoms, although there are initially no significant abnormal findings on CSF, MRI, and EEG.…”

Section: Conflict Of Interestmentioning

confidence: 99%

A Case of Anti-NMDA Receptor Encephalitis with Normal Findings on Initial Diagnostic Tests

Kwon

Park

et al. 2020

Dement Neurocogn Disord

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Non-inflammatory cerebrospinal fluid delays the diagnosis and start of immunotherapy in anti-NMDAR encephalitis

Cited by 18 publications

References 16 publications

Characteristics of cerebrospinal fluid cytology in anti-N-methyl-D-aspartate receptor encephalitis

Characteristics of cerebrospinal fluid cytology in anti-N-methyl-D-aspartate receptor encephalitis

Clinical Features and Inflammatory Markers in Autoimmune Encephalitis Associated With Antibodies Against Neuronal Surface in Brazilian Patients

A Case of Anti-NMDA Receptor Encephalitis with Normal Findings on Initial Diagnostic Tests

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