| Literature DB >> 35984192 |
Tomotaka Mizoguchi1, Makoto Hara, Hideto Nakajima.
Abstract
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.Entities:
Mesh:
Year: 2022 PMID: 35984192 PMCID: PMC9388039 DOI: 10.1097/MD.0000000000030062
Source DB: PubMed Journal: Medicine (Baltimore) ISSN: 0025-7974 Impact factor: 1.817
Figure 1.Axial section of T2-FLAIR brain MRI upon admission. (A, B) T2-FLAIR brain MRI upon admission revealed localized high-intensity lesions in the bilateral (highlighted in the right side) medial temporal lobes (indicated by yellow arrows heads). (C) Abnormal signals were not observed in other limbic area such as the insula. MRI = magnetic resonance imaging, T2-FLAIR = T2-weighted fluid-attenuated inversion recovery.
Figure 2.EEG findings upon admission. The initial EEG recording with longitudinal bipolar deviation showed a lateralized rhythmic delta activity in the right temporal area without specific epileptic discharges. EEG montage was recorded with the standard 10–20 electrodes. The blue and black lines indicate recording in the left and right sides, respectively. The vertical and horizontal bars indicate 50 µV and 1 s, respectively. EEG = electroencephalogram.
Figure 3.Axial section of T2-FLAIR brain MRI performed on day 75. (A, B) The high-intensity lesions in the bilateral medial temporal lobes on T2-FLAIR brain MRI performed on day 75 partly improved, with weak abnormal signals remaining (indicated by yellow arrows heads). (C) No newly abnormal signals were detected in other limbic area, such as the insula. MRI = magnetic resonance imaging, T2-FLAIR = T2-weighted fluid-attenuated inversion recovery.
Cases of neurosyphilis that fulfilled the Graus ALE criteria.
| Authors (year) | ||||
|---|---|---|---|---|
| Bash et al (2001)[ | Jeong et al (2009)[ | Abdelerahman et al (2012)[ | Current case | |
| Age (yr), sex | 50, male | 35, male | 51, male | 66, male |
| Main symptoms | WMD, seizure | WMD, disorientation | Personality change, seizure | Personality change, WMD, NCSE |
| Symptom duration (mo) | 3 | 1 | 3 | ≤3 |
| CSF cell level (/μL) | 19 | 48 | 220 | 25 |
| HIV status | N.D | N.D | Positive | Negative |
| Treatments | Benzylpenicillin | Benzylpenicillin | Benzylpenicillin | Benzylpenicillin |
| Follow-up period (mo) | 4 | 1 | 1.5 | 3.5 |
| Outcome | Improved | Improved | Partly improved | Partly improved (persistence of mild WMD) |
ALE = autoimmune limbic encephalitis, CSF = cerebrospinal fluid, HIV = human immunodeficiency virus, NCSE = nonconvulsive status epilepticus, N.D = not described, WMD = working memory deficits.