Atypical development of neurosyphilis mimicking limbic encephalitis

Beiruti, Karine; Awad, Anan Abu; Keigler, Galina; Ryder, Chen Hanna; Shahien, Radi

doi:10.1177/0956462418797873

Cited by 13 publications

(13 citation statements)

References 5 publications

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“…[ 18 ] Recently, 2 cases of serologically confirmed neurosyphilis presenting as LE accompanied with anti-NMDAR antibodies were published. [ 19 , 20 ] These cases suggest that antibodies might control the symptoms of neurosyphilis. Nevertheless, cases with negativity for NSAs and onconeural antibodies, including the current one, were also reported.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review

2022

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Rationale: Neurosyphilis presenting as limbic encephalitis (LE) is an important differential diagnosis of autoimmune LE (ALE) defined by Graus in 2016. However, data on the clinical differences and similarities between neurosyphilis presenting as LE and ALE are limited. Herein, we report neurosyphilis presenting as ALE that fulfilled the main items of the Graus ALE criteria. Moreover, a literature review of neurosyphilis presenting as LE was performed. Patient concerns: A 66-year-old Japanese man developed nonconvulsive status epilepticus. He presented with progressive personality change and working memory deficits within 3 months prior to admission. A hyperintense lesion localized in the bilateral medial temporal area was observed on T2-weighted fluid-attenuated inversion recovery brain magnetic resonance imaging. Cerebrospinal fluid analysis showed mild pleocytosis and the presence of oligoclonal band. However, in-house assays did not detect antineuronal antibodies. Electroencephalogram showed lateralized rhythmic delta activity in the right temporal area. The serum and cerebrospinal fluid serological and antigen tests for syphilis had positive results. Diagnosis: ALE was initially suspected based on the patient’s symptoms and ancillary test findings that fulfilled the Graus ALE criteria. However, based on the positive confirmatory test results for syphilis, a diagnosis of neurosyphilis was eventually made. Intervention: The patient received intravenous midazolam, oral levetiracetam, and lacosamide to control nonconvulsive status epilepticus. In addition, he was treated with intravenous benzylpenicillin at a dose of 24 million units/day for 14 days. Outcomes: The patient’s cognitive function relatively improved after antibiotic treatment. However, he presented with persistent mild working memory deficit, which was evaluated with the Wechsler Adult Intelligence Scale, 3rd edition. Therefore, on day 103 of hospitalization, he was transferred to another hospital for rehabilitation and long-term care due to limitations in performing activities of daily living. Lessons: The present case was diagnosed with neurosyphilis presenting as ALE, but meanwhile, in most case, neurosyphilis presenting as LE developed at a slower progressive rate, and it had a broader or restricted involvement on brain MRI than ALE based on the literature review. Therefore, an appropriate differential diagnosis of LE can be obtained by identifying clinical differences between the 2 conditions.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review

2022

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“…In addition, the patient showed steroid-responsiveness, and the MRI findings at relapse were consistent with HSVE-related changes, suggesting that HSVE can trigger the expression of NMDA receptor antibodies ( 8 , 9 ). Neurologists have reported detecting an abnormal intensity on brain MRI scans in the hippocampus and/or temporal lobe in patients with neurosyphilis ( 10 , 11 ). Recently, Beiruti et al reported a neurosyphilis case mimicking limbic encephalitis with anti-NMDA receptor antibody, without MRI abnormalities ( 10 ).…”

Section: Discussionmentioning

confidence: 99%

“…Neurologists have reported detecting an abnormal intensity on brain MRI scans in the hippocampus and/or temporal lobe in patients with neurosyphilis ( 10 , 11 ). Recently, Beiruti et al reported a neurosyphilis case mimicking limbic encephalitis with anti-NMDA receptor antibody, without MRI abnormalities ( 10 ). Tsukita et al reported a case of neurosyphilis with limbic encephalitis and anti-GluR antibody ( 11 ).…”

Section: Discussionmentioning

confidence: 99%

Cryptococcus Meningitis Can Co-occur with Anti-NMDA Receptor Encephalitis

et al. 2020

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We herein report a 50-year-old man with alcoholic cirrhosis who developed loss of consciousness and tremor of the upper limbs. Magnetic resonance imaging findings were suggestive of limbic encephalitis with bilateral hippocampal damage, and a cerebrospinal fluid (CSF) examination confirmed anti-N-methyl-D-aspartate (NMDA) and anti-glutamate receptor antibodies. Despite initial corticosteroid therapy, meningeal irritation symptoms appeared, owing to the development of cryptococcal meningitis (CM), diagnosed by the detection of cryptococcal capsular polysaccharide antigen in the follow-up CSF analysis. Cerebral infarction with reversible stenosis of major cerebral arteries during the clinical course was also observed. Following administration of antifungals and corticosteroids, the number of cells in the CSF gradually declined, and NMDA receptor antibodies disappeared. Our study demonstrates the unique coexistence of CM with anti-NMDA receptor encephalitis in adults.

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“…The aetiology is attributed to tumours in 38% of cases of which teratoma is the most common type (8). Anti-NMDAR Ab-mediated encephalitis has also been associated with infective encephalitides, including herpes simplex virus (HSV) encephalitis and cryptococcal meningitis, with neuroinflammation possibly acting as a mechanism triggering anti-NMDAR Ab production (9)(10)(11)(12)(13)(14)(15).…”

Section: Introductionmentioning

confidence: 99%

Autoimmune Encephalitis in Long-Standing Schizophrenia: A Case Report

et al. 2022

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Anti-N-methyl-D-aspartate (NMDA) receptor antibody (anti-NMDAR Ab)-mediated encephalitis is an autoimmune disorder involving the production of antibodies against NMDARs in the central nervous system that leads to neurological or psychiatric dysfunction. Initially described as a paraneoplastic syndrome in young women with teratomas, increased testing has found it to be a heterogeneous condition that affects both the sexes with varying clinical manifestations, severity, and aetiology. This case report describes a 67-year-old man with a 40-year history of relapsing, severe, treatment-refractory schizophrenia. Due to the worsening of his condition during a prolonged inpatient admission for presumed relapse of psychosis, a revisit of the original diagnosis was considered with extensive investigations performed including an autoimmune panel. This revealed anti-NMDAR Abs in both the serum and cerebrospinal fluid on two occasions. Following treatment with intravenous immunoglobulin and methylprednisolone, he demonstrated rapid symptom improvement. This is a rare case of a long-standing psychiatric presentation with a preexisting diagnosis of schizophrenia subsequently found to have anti-NMDAR Ab-mediated encephalitis. Whether the case is one of initial NMDAR encephalitis vs. overlap syndrome is unknown. Most importantly, this case highlights the need for vigilance and balanced consideration for treatment in cases of long-standing psychiatric presentation where the case remains treatment refractory to antipsychotics or when atypical features including seizures and autonomic dysfunction or focal neurology are observed.

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Atypical development of neurosyphilis mimicking limbic encephalitis

Cited by 13 publications

References 5 publications

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review

Cryptococcus Meningitis Can Co-occur with Anti-NMDA Receptor Encephalitis

Autoimmune Encephalitis in Long-Standing Schizophrenia: A Case Report

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