Leptomeningeal Contrast Enhancement Research Articles

Primary central nervous system lymphoma: Imaging features and differential diagnosis.

Primary central nervous system lymphoma (PCNSL) represents 5% of malignant primary brain tumors. The clinical presentation typically includes focal neurological symptoms, increased intracranial pressure, seizures, and psychiatric symptoms. Although histological examination remains the gold standard for diagnostic confirmation, non-invasive imaging plays a crucial role for the diagnosis. In immunocompetent individuals, PCNSL usually appears as a single, well-defined, supratentorial lesion with a predilection for periventricular areas, iso- or hypointense on T1- and T2-weighted magnetic resonance imaging, with restricted diffusion, slightly increased perfusion, and homogenous gadolinium-enhancement. Differential diagnoses include high-grade glioma and pseudotumoral demyelinating disease. In immunocompromised patients, PCNSL may present as multiple lesions, with a higher likelihood of hemorrhage and necrosis and less restricted diffusion than immunocompetent individuals. Differential diagnoses include neurotoxoplasmosis, progressive multifocal leukoencephalopathy, and cerebral abscess. Atypical forms of lymphoma are characterized by extra-axial lymphoma, lymphomatosis cerebri, and intravascular lymphoma. Extra-axial lymphoma presents as single or multiple extra-axial dural lesions with diffuse leptomeningeal contrast-enhancement. Lymphomatosis cerebri appears as an infiltrative and symmetric lesion, primarily affecting deep white matter and basal ganglia, appearing hyperintense on T2-weighted imaging, without significant contrast-enhancement or perfusion changes. Intravascular lymphoma presents as multiple rounded or oval-shaped "infarct-like" lesions, located cortically or subcortically. This study aims to highlight the imaging characteristics of PCNSL, focusing on magnetic resonance imaging and its differential diagnosis.

Glial cell injury and atrophied lesion volume as measures of chronic multiple sclerosis inflammation

BackgroundAtrophied lesion volume (aLV), a proposed biomarker of disability progression in multiple sclerosis (MS) and transition into progressive MS (PMS), depicts chronic periventricular white matter (WM) pathology. Meningeal infiltrates, imaged as leptomeningeal contrast enhancement (LMCE), are linked with greater cortical pathology. ObjectivesTo determine the relationship between serum-derived proteomic data with the development of aLV and LMCE in a heterogeneous group of people with MS (pwMS). MethodsProteomic and MRI data for 202 pwMS (148 clinically isolated syndrome /relapsing-remitting MS and 54 progressive MS (PMS)) were acquired at baseline and at 5.4-year follow-up. The concentrations of 21 proteins related to multiple MS pathophysiology pathways were derived using a custom-developed Proximity Extension Assay on the Olink™ platform. The accrual of aLV was determined as the volume of baseline T2-weighted lesions that were replaced by cerebrospinal fluid over the follow-up. Regression models and age-adjusted analysis of covariance (ANCOVA) were used. ResultsOlder age (standardized beta = 0.176, p = 0.022), higher glial fibrillary acidic protein (standardized beta = 0.312, p = 0.001), and lower myelin oligodendrocyte glycoprotein levels (standardized beta = −0.271, p = 0.002) were associated with accrual of aLV over follow-up. This relationship was driven by the pwPMS population. The presence of LMCE at the follow-up visit was not predicted by any baseline proteomic biomarker nor cross-sectionally associated with any protein concentration. ConclusionProteomic markers of glial activation are associated with chronic lesional WM pathology (measured as aLV) and may be specific to the progressive MS phenotype. LMCE presence in MS does not appear to relate to proteomic measures.

Highly Sensitive 3-Tesla Real Inversion Recovery MRI Detects Leptomeningeal Contrast Enhancement in Chronic Active Multiple Sclerosis.

Leptomeningeal contrast enhancement (LME) on T2-weighted Fluid-Attenuated Inversion Recovery (T2-FLAIR) MRI is a reported marker of leptomeningeal inflammation, which is known to be associated with progression of multiple sclerosis (MS). However, this MRI approach, as typically implemented on clinical 3-tesla (T) systems, detects only a few enhancing foci in ~25% of patients and has thus been criticized as poorly sensitive. To compare an optimized 3D real-reconstruction inversion recovery (Real-IR) MRI sequence on a clinical 3 T scanner to T2-FLAIR for prevalence, characteristics, and clinical/radiological correlations of LME. We obtained 3D T2-FLAIR and Real-IR scans before and after administration of standard-dose gadobutrol in 177 scans of 154 participants (98 women, 64%; mean ± SD age: 49 ± 12 years), including 124 with an MS-spectrum diagnosis, 21 with other neurological and/or inflammatory disorders, and 9 without neurological history. We calculated contrast-to-noise ratios (CNR) in 20 representative LME foci and determined association of LME with cortical lesions identified at 7 T (n = 19), paramagnetic rim lesions (PRL) at 3 T (n = 105), and clinical/demographic data. We observed focal LME in 73% of participants on Real-IR (70% in established MS, 33% in healthy volunteers, P < 0.0001), compared to 33% on T2-FLAIR (34% vs. 11%, P = 0.0002). Real-IR showed 3.7-fold more LME foci than T2-FLAIR ( P = 0.001), including all T2-FLAIR foci. LME CNR was 2.5-fold higher by Real-IR ( P < 0.0001). The major determinant of LME status was age. Although LME was not associated with cortical lesions, the number of PRL was associated with the number of LME foci on both T2-FLAIR ( P = 0.003) and Real-IR ( P = 0.0003) after adjusting for age, sex, and white matter lesion volume. Real-IR a promising tool to detect, characterize, and understand the significance of LME in MS. The association between PRL and LME highlights a possible role of the leptomeninges in sustaining chronic inflammation.

Does sevoflurane sedation in pediatric patients lead to "pseudo" leptomeningeal enhancement in the brain on 3 Tesla magnetic resonance imaging?

Prominent leptomeningeal contrast enhancement (LMCE) in the brain is observed in some pediatric patients during sedation for imaging. However, based on clinical history and cerebrospinal fluid analysis, the patients are not acutely ill and do not exhibit meningeal signs. Our study determined whether sevoflurane inhalation in pediatric patients led to this pattern of 'pseudo' LMCE (pLMCE) on 3 Tesla magnetic resonance imaging (MRI). To highlight the significance of pLMCE in pediatric patients undergoing enhanced brain MRI under sedation to avoid misinterpretation in reports. A retrospective cross-sectional evaluation of pediatric patients between 0-8 years of age was conducted. The patients underwent enhanced brain MRI under inhaled sevoflurane. The LMCE grade was determined by two radiologists, and interobserver variability of the grade was calculated using Cohen's kappa. The LMCE grade was correlated with duration of sedation, age and weight using the Spearman rho rank correlation. A total of 63 patients were included. Fourteen (22.2%) cases showed mild LMCE, 48 (76.1%) cases showed moderate LMCE, and 1 case (1.6%) showed severe LMCE. We found substantial agreement between the two radiologists in detection of pLMCE on post-contrast T1 imaging (kappa value = 0.61; P < 0.001). Additionally, we found statistically significant inverse and moderate correlations between patient weight and age. There was no correlation between duration of sedation and pLMCE. pLMCE is relatively common on post-contrast spin echo T1-weighted MRI of pediatric patients sedated by sevoflurane due to their fragile and immature vasculature. It should not be misinterpreted for meningeal pathology. Knowing pertinent clinical history of the child is an essential prerequisite to avoid radiological overcalling and the subsequent burden of additional investigations.

Neurocutaneous melanocytosis with manifestation in adulthood

A 20-year-old patient was presented with subacute onset of headache, nausea and vomiting. Testing of nasal/oropharyngeal swabs indicated the presence of SARS-CoV-2 RNA, and later the antibodies to this virus were found. The treatment in the hospital for Coronavirus 19 Disease (COVID-19) provided only temporary relief, and the patient then was referred to the Regional Stroke Center (RSC) to exclude a subarachnoid hemorrhage. RSC neurologists drew attention to multiple skin nevi in the patient. Brain MRI demonstrated abnormal T1 hyperintensity in the brain leptomeninges, with leptomeningeal contrast enhancement as well as hyperintensity in amygdala regions on T1 weighted images, bilaterally. The anomaly of the Dandy-Walker malformation complex was also revealed. Cerebrospinal fluid (CSF) analysis showed elevated protein (0.52 g/L), low lymphocytosis (lymphocytes, 6 in mm3), and decreased glucose (1.8 mmol/L). Neurocutaneous melanocytosis (NCM) was diagnosed, which neurological manifestation was probably triggered by COVID-19. The patient’s vision gradually progressively worsened. In 2.5 months after the clinical manifestation of NCM, fundoscopy revealed optic discs atrophy (despite the absence of previous edema), and repeated CSF analysis showed atypical cells with characteristics corresponding to melanoma. Malignant transformation of cerebral melanocytosis was suspected, and the patient was referred to an oncological dispensary for further therapy. In the presented literature review, special attention is paid to the issues of neuroimaging, cytological and immunocytochemical diagnostics of NCM.

Pediatric spinal pilocytic astrocytomas form a distinct epigenetic subclass from pilocytic astrocytomas of other locations and diffuse leptomeningeal glioneuronal tumours

Pediatric spinal low-grade glioma (LGG) and glioneuronal tumours are rare, accounting for less 2.8–5.2% of pediatric LGG. New tumour types frequently found in spinal location such as diffuse leptomeningeal glioneuronal tumours (DLGNT) have been added to the World Health Organization (WHO) classification of tumours of the central nervous system since 2016, but their distinction from others gliomas and particularly from pilocytic astrocytoma (PA) are poorly defined. Most large studies on this subject were published before the era of the molecular diagnosis and did not address the differential diagnosis between PAs and DLGNTs in this peculiar location. Our study retrospectively examined a cohort of 28 children with LGGs and glioneuronal intramedullary tumours using detailed radiological, clinico-pathological and molecular analysis. 25% of spinal PAs were reclassified as DLGNTs. PA and DLGNT are nearly indistinguishable in histopathology or neuroradiology. 83% of spinal DLGNTs presented first without leptomeningeal contrast enhancement. Unsupervised t-distributed stochastic neighbor embedding (t-SNE) analysis of DNA methylation profiles showed that spinal PAs formed a unique methylation cluster distinct from reference midline and posterior fossa PAs, whereas spinal DLGNTs clustered with reference DLGNT cohort. FGFR1 alterations were found in 36% of spinal tumours and were restricted to PAs. Spinal PAs affected significantly younger patients (median age 2 years old) than DLGNTs (median age 8.2 years old). Progression-free survival was similar among the two groups. In this location, histopathology and radiology are of limited interest, but molecular data (methyloma, 1p and FGFR1 status) represent important tools differentiating these two mitogen-activated protein kinase (MAPK) altered tumour types, PA and DLGNT. Thus, these molecular alterations should systematically be explored in this type of tumour in a spinal location.

Malignant transformation of a cerebral dermoid cyst into a squamous cell carcinoma with malignant intraperitoneal spreading along a ventriculoperitoneal shunt: illustrative case.

BACKGROUNDMalignant progression of intracranial dermoid cysts into squamous cell carcinoma is extremely rare with only three reports published so far. Intracranial dermoid cysts are uncommon benign tumors lined by stratified squamous epithelium of embryonic ectodermal origin.OBSERVATIONSHere, the authors present the case of a 64-year-old female with a recurrent temporal dermoid cyst. After surgery for the recurrent dermoid cyst, once in the early 1990s and another 16 years later, the patient presented with headache and nausea due to hydrocephalus. After implantation of a ventriculoperitoneal shunt, she deteriorated rapidly and died only 60 days after admission. Autopsy revealed malignant transformation of the epithelial lining of the dermoid cyst into a squamous cell carcinoma resulting in neoplastic meningiosis and intraperitoneal tumor spread along a previously implanted ventriculoperitoneal shunt.LESSONSMalignant transformation should be considered in patients with dermoid cyst who show new leptomeningeal contrast enhancement. In the case of hydrocephalus, alternatives to peritoneal shunting should be considered.

RARE-27. Treatment and outcomes in atypical choroid plexus papilloma: a single institution experience

BACKGROUND: Atypical choroid plexus papillomas (aCPP) are rare central nervous system (CNS) tumors often occurring in very young children. While surgical resection has been a mainstay of therapy, there is no consensus and limited data on the treatment of relapsed or metastatic tumors. METHODS: Retrospective review of the treatment and outcome of patients diagnosed with aCPP since 2011 was performed. RESULTS: Of the seven patients, 4 were male and 3 were female with a median age of 3 years at diagnosis (range: antenatal to 18 years old). All non-metastatic patients (six) were treated with surgery and all achieved gross total resection. Two patients had diffuse leptomeningeal contrast enhancement on diagnosis MRI that resolved after resection of primary tumor alone. One patient developed local relapse underwent re-resection with a GTR then was treated with 4 cycles of chemotherapy based on CPT-SIOP-2000 protocol (carboplatin, etoposide) and has not had further relapse in 24 months. One patient had metastatic disease at the time of diagnosis. They were treated with adjuvant chemotherapy, which stabilized disease for 36 months until they had progression. Additional four cycles were given and has again stabilized disease now 8 months from completion of that therapy. One non-metastatic patient died of unknown causes 28 months from diagnosis. CONCLUSIONS: Surgical resection remains the standard of care for patients with aCPP. However, chemotherapy based on the SIOP backbone may be useful to reduce the need for or to delay radiation therapy in select patients in the relapsed or metastatic setting.

Rare nervous system involvement in an anti-myelin oligodendrocyte-positive case: spinal leptomeningeal involvement

Anti-myelin oligodendrocyte (MOG)-positive cases have a variable clinical presentation. MOG autoantibodies are associated with acute disseminated encephalomyelitis, multiple sclerosis, clinically isolated syndrome, neuromyelitis optica, isolated optic neuritis, and transverse myelitis. Our patient had bilateral optic neuritis with acute tetraparesis. Cranial magnetic resonance imaging (MRI) showed leptomeningeal contrast involvement and contrast enhancement in all cranial nerve nuclei. Spinal MRI revealed leptomeningeal contrast enhancement, and a contrast-enhancing lesion was found in the cervical spinal cord. This case is worth presenting because spinal leptomeningeal involvement is rare in anti-MOG-positive patients.

High Levels of Cerebrospinal Fluid Kappa Free Light Chains Relate to IgM Intrathecal Synthesis and Might Have Prognostic Implications in Relapsing Multiple Sclerosis.

Cerebrospinal kappa free light chain (KFLC)-index is a marker of intrathecal immunoglobulin synthesis that aids in the diagnosis of multiple sclerosis (MS). However, little evidence exists on its prognostic role. Our aim is to analyze the relationship between KFLC-index and other MS biomarkers and to explore its prognostic role. This is a monocentric observational study in a cohort of 52 people with relapsing MS (pwRMS) performed on prospectively acquired clinical data and with retrospective evaluation of biomarkers. We measured KFLC-index, immunoglobulin intrathecal synthesis, cerebrospinal fluid (CSF) chitinase 3-like 1 (CHI3L1), and neurofilament light protein (NFL) and reviewed MRI to detect leptomeningeal contrast enhancement (LMCE). We compared time to Expanded Disability Status Scale (EDSS) 3 and to initiation of high-efficacy disease-modifying therapies (heDMTs) by multivariate Cox regression analysis. Median KFLC-index correlated with IgG/IgM indexes (p < 0.0001/p < 0.05) and IgG-oligoclonal bands (OCGBs) (p < 0.001). Patients with IgM-oligoclonal bands (OCMBs) had a higher KFLC-index (p = 0.049). KFLC-index was higher in patients with LMCE (p = 0.008) and correlated with CHI3L1 (p = 0.007), but disease activity had no effect on its value. Bivariate and multivariate analyses confirmed KFLC-index > 58 as an independent risk factor for reaching an EDSS of 3 (hazard ratio (HR) = 12.4; 95% CI = 1.1–147; p = 0.047) and for the need of treatment with heDMTs (HR = 3.0; 95% CI = 1.2–7.1; p = 0.0013). To conclude, our data suggest a potential prognostic role of the KFLC-index during the MS course.

Neuroimaging in patients with COVID-19: a neuroradiology expert group consensus.

Neurological and neuroradiological manifestations in patients with COVID-19 have been extensively reported. Available imaging data are, however, very heterogeneous. Hence, there is a growing need to standardise clinical indications for neuroimaging, MRI acquisition protocols, and necessity of follow-up examinations. A NeuroCovid working group with experts in the field of neuroimaging in COVID-19 has been constituted under the aegis of the Subspecialty Committee on Diagnostic Neuroradiology of the European Society of Neuroradiology (ESNR). The initial objectives of this NeuroCovid working group are to address the standardisation of the imaging in patients with neurological manifestations of COVID-19 and to give advice based on expert opinion with the aim of improving the quality of patient care and ensure high quality of any future clinical studies.Key Points • In patients with COVID-19 and neurological manifestations, neuroimaging should be performed in order to detect underlying causal pathology. • The basic MRI recommended protocol includes T2-weighted, FLAIR (preferably 3D), and diffusion-weighted images, as well as haemorrhage-sensitive sequence (preferably SWI), and at least for the initial investigation pre and post-contrast T1 weighted-images. • 3D FLAIR should be acquired after gadolinium administration in order to optimise the detection of leptomeningeal contrast enhancement.

Cortical involvement and leptomeningeal inflammation in myelin oligodendrocyte glycoprotein antibody disease: A three-dimensional fluid-attenuated inversion recovery MRI study

Background: Cortical demyelination and meningeal inflammation have been detected neuropathologically in multiple sclerosis (MS) and recently in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). Objectives: To assess in vivo cortical and leptomeningeal involvement in MOGAD. Methods: We prospectively evaluated 11 MOGAD and 12 relapsing-remitting MS (RRMS) patients combining three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) and 3D-T1-weighted (3D-T1w) sequences at 3-Tesla magnetic resonance imaging (MRI). Leptomeningeal contrast enhancement (LMCE) was assessed on 3D-FLAIR post-gadolinium (3D-FLAIRGd). Cerebral cortical lesions (CCLs) were classified as either intracortical–subpial (IC–SP) or leukocortical (LC). Results: CCLs were present in 8/11 MOGAD and 12/12 RRMS patients, with the number of CCLs being significantly lower in MOGAD (median (interquartile range (IQR)) 3 (0.5–4) vs 12 (4.75–19), p = 0.0032). In MOGAD, IC–SP lesions were slightly more prevalent than LC lesions (2 (0–2.5) vs 1 (0–2), p = 0.6579); whereas in RRMS, IC–SP lesions were less prevalent than LC lesions (3.5 (2.75–5.5) vs 9 (2–12.75), p = 0.27). LMCE was observed in 3/11 MOGAD and 1/12 RRMS patients; MOGAD with LMCE showed an increased median number of CCLs compared with MOGAD without LMCE (8 (4–9) vs 2.5 (0.75–3.25), p = 0.34). No correlation was observed between MOGAD MRI findings and (a) MOGAD duration, (b) serum MOG-immunoglobulin G1 titers, and (c) oligoclonal band presence. Conclusion: We described cortical lesion topography and detected for the first time LMCE using 3D-FLAIRGd sequences in MOGAD patients.

Reply.

We thank Drs Velonakis, Karavasilis, Almyroudi, Filippiadis, and Korompoki for the in-depth commentary on our article. We value the important observations they made regarding the differences in the rates of leptomeningeal contrast enhancement in patients with coronavirus disease 2019 among the

Symptoms: Sudden Hearing Loss and Facial Paralysis

Symptoms: Sudden Hearing Loss and Facial Paralysis

A cytology negative rare tumor with the presentation of pseudotumor cerebri clinical symptoms: diffuse leptomeningeal glioneuronal tumor

Diffuse leptomeningeal glioneuronal tumor is characterized by hydrocephalus, leptomeningeal involvement in the absence of a primary parenchymal mass, and negative cerebrospinal fluid (CSF) cytology. It is an extremely rare and difficult tumor to diagnose as no mass can be biopsied and it mimics infectious, rheumatologic, and inflammatory pathologies. An 11-year-old girl presented with complaints of headache, vomiting, and double vision. On examination, there was papilledema. Initial MRI scanning did not yield any significant findings. Clinical progression was observed in four months in the follow-ups. The symptoms included seizures, gait disturbances, and severely increased intracranial pressure. The screening of the patient for infectious, rheumatologic, endocrinologic, and inflammatory pathologies was normal. CSF pressure was elevated without any malignancy. Repeated cranial MRI revealed hydrocephalus and pituitary expansion. Leptomeningeal thickening and contrast enhancement were observed in spinal MRI. After a negative dural biopsy, the patient was diagnosed with a spinal leptomeningeal biopsy. The authors believed that the prevalence of this rare pediatric tumor, diagnosed with a leptomeningeal biopsy, is underestimated as it has an insidious course and signs of increased intracranial pressure in the absence of a definite solid mass.

Magnetic resonance findings of meningoencephalitis in a dog seroreactive to Ehrlichia canis in United Kingdom

AbstractWe describe a novel clinical presentation and imaging findings in a dog seropositive to Ehrlichia canis. A dog presented with an acute non‐ambulatory tetraparesis and severe obtundation. MRI findings showed multifocal intra‐axial changes centered in the grey matter. The lesions caused mild mass effect and leptomeningeal contrast enhancement. The dog responded to treatment with the progressive reduction of antibody titers detected on the indirect fluorescent antibody test and complete resolution of the clinical signs. Canine monocytic ehrlichiosis should be considered a differential in dogs with thrombocytopenia and meningoencephalitis.

Leptomeningeal, dura mater and meningeal vessel wall enhancements in multiple sclerosis

Background: Leptomeningeal contrast enhancement (LMCE) has previously shown potential to be an indirect marker for leptomeningeal inflammation in multiple sclerosis (MS). Dura mater (DME), inclusive falx cerebri (FCE) enhancement and meningeal vessel wall enhancement (VWE) represent two other meningeal enhancement patterns in MS that have not been extensively studied.Objectives: To investigate the frequency of LMCE, DME/FCE and VWE in patients with MS and their associations with demographic, clinical and MRI characteristics in a longitudinal retrospective study.Methods: 217 MS patients (193 relapsing-remitting MS, 24 progressive MS) were assessed at baseline and over 18 months follow-up using 3T 3D FLAIR pre- and post-contrast and subtraction images. Lesion and brain volume outcomes were additionally calculated. Analyses were adjusted for age, and corrected for multiple comparisons.Results: LMCE and VWE frequency was associated with higher age (p<0.02), but the presence of DME/FCE was not (p=0.402). 24% of MS patients revealed LMCE and VWE, respectively, and 47% showed DME/FCE. Presence of LMCE, VWE and DME/FCE was not significantly associated with clinical or imaging markers of disease severity. All three patterns of meningeal enhancement showed a high persistence in shape and size at follow-up.Conclusions: LMCE, DME/FCE and VWE can be identified by gadolinium-enhanced 3D FLAIR MR imaging. Meningeal enhancement is associated with higher age. DME/FCE is the most frequent meningeal enhancement pattern in MS, however further case-control studies should determine whether this represents abnormal lymphatic drainage in these patients or is an age-dependent physiologic phenomenon.

MRI Brain Findings in 126 Patients with COVID-19: Initial Observations from a Descriptive Literature Review.

Recently, numerous investigational studies, case series, and case reports have been published describing various MR imaging brain findings in patients with COVID-19. The purpose of this literature review was to compile and analyze brain MR imaging findings in patients with COVID-19-related illness. Literature searches of PubMed, publicly available Internet search engines, and medical journal Web sites were performed to identify articles published before May 30, 2020 that described MR imaging brain findings in patients with COVID-19. Twenty-two articles were included in the analysis: 5 investigational studies, 6 case series, and 11 case reports, encompassing MR imaging of the brain in 126 patients. The articles originated from 7 different countries and were published in 14 medical journals. MR imaging brain findings included specific diagnoses (such as acute infarct, posterior reversible encephalopathy syndrome) or specific imaging features (such as cortical FLAIR signal abnormality, microhemorrhages). The most frequent diagnoses made on brain MR imaging in patients with COVID-19 were acute and subacute infarcts. Other common findings included a constellation of leukoencephalopathy and microhemorrhages, leptomeningeal contrast enhancement, and cortical FLAIR signal abnormality.

Sarcoïdose médullaire mimant une myélopathie cervico-arthrosique : mauvais pronostic après chirurgie

IntroductionCervical spinal sarcoidosis can mimic compressive cervical myelopathy leading to potentially harmful surgical procedures before the diagnosis can be made. MethodsRetrospective description of 3 patients and review of the literature. ResultsTwenty-seven patients (16 men/11 women), median age 58 years [range 29–74] were described. Neurosurgical procedures consisted of laminectomy (n=10), laminoplasty (n=15) and anterior discectomy (n=2). Immediately after surgery, 17 patients (63%) worsened or remained disabled. Among the 10 patients who improved, 9 worsened secondarily. The analysis of preoperative MRI showed T2 hypersignal lesions and contrast enhancement in all patients. Neurological symptoms were inaugural in 25/27 patients, and systemic involvement of the sarcoidosis was found after surgery in 15/27 patients. After surgery, all patients received corticosteroids, along with immunosuppressive therapy in 8 cases/27. After a follow-up of 24 [16–72] months; 13 patients were stabilized or worsened, 7 were partially improved. Three died of other cause. Only 5 recovered without sequelae. ConclusionIn patients with compressive cervical myelopathy, leptomeningeal contrast enhancement, a T2-weighted hypersignal exceeding the compression level on MRI, and the presence of extraneurological symptoms should point to inflammatory disease. These rare manifestations may be the first symptoms of sarcoidosis and should be recognized to avoid harmful surgical procedures and to provide appropriate medical treatment.

Case report: chronic relapsing cryptococcal meningitis in a patient with low mannose-binding lectin and a low na\xefve CD4 cell count

BackgroundCryptococcal meningitis is most commonly found in HIV-infected patients. In HIV-negative patients, its low incidence can lead to prolonged time to diagnosis. Detailed case reports of chronic cryptococcal meningitis are scarce, but could provide clues for earlier diagnosis in this patient category.Case presentationA 60-year old man presented June 2015 with intermittent headaches for several months without any fever. Initial work-up showed a leukocytosis, raised CSF opening pressure and raised leukocytes and protein in the CSF. An MRI revealed leptomeningeal contrast enhancement and cerebellar oedema. While malignancy and various infectious causes were excluded, the patient had a spontaneous clinical and radiological recovery. One year later, the patient returned with complaints of headaches. Also, cerebellar oedema and leptomeningeal contrast enhancement had recurred. Eventually in March 2017, the novel cryptococcal antigen lateral flow assay (CrAg LFA) was positive on CSF, and one colony of Cryptococcus neoformans was cultured from CSF. The patient was treated with the standard antifungal regimen which resulted in resolution of his headaches. In retrospect, the cryptococcal antigen test was already positive on a serum sample from June 2015. Interestingly, post-treatment immunological analysis revealed both a low mannose-binding lectin (MBL) concentration and low naïve CD4 counts.ConclusionsWe present a patient with cryptococcal meningitis in an HIV-negative patient with low MBL and low naïve CD4 count suffering a chronic relapsing meningo-encephalitis with relatively mild symptoms for around 2 years. In patients with an unexplained meningo-encephalitis such as this case, early performance of CrAg LFA on serum and/or CSF is an inexpensive and rapid method to reduce time-to diagnosis.