Tumefactive Demyelinating Lesions Research Articles

Catastrophic tumefactive acute disseminated encephalomyelitis in patient with dengue virus: a case report.

Tumefactive demyelinating lesions (TDL) are a rare occurrence among inflammatory demyelinating diseases of the central nervous system, distinguished by tumor-like lesions exceeding 2 cm in diameter. While various etiologies have been associated with TDL, only a limited number of case reports document the coexistence of acute disseminated encephalomyelitis (ADEM) and TDL. Here, we present the case of a female diagnosed with dengue fever two weeks prior, who subsequently developed left hemiparesis and encephalopathy. Both her brain magnetic resonance imaging (MRI) and clinical course align with the characteristics of tumefactive ADEM.

Solitary Tumefactive Demyelinating Lesions in Children: Clinical and MRI Features, Pathological Characteristics, and Outcomes

BackgroundIsolated tumefactive demyelinating lesions (≥ 2 cm) may be difficult to distinguish from contrast enhancing brain tumors, central nervous system infections, and (rarely) tissue dysgenesis, which may all occur with increased signal on T2-weighted images. Establishing an accurate diagnosis is essential for management, and we delineate our single center experience. MethodsWe performed a retrospective review of medical records, imaging, and biopsy specimens for patients under 18 presenting with isolated tumefactive demyelination over a 10-year period. ResultsTen children (eight female) met inclusion criteria, with a median age of 14.1 years. Lesions were most likely to involve the thalamus (6/10), brainstem (5/10), basal ganglia (4/10) or corpus callosum (4/10). Eighty percent had perilesional edema at presentation and 60% had midline shift. Biopsies demonstrated demyelination with perivascular lymphocytic infiltration and axonal damage ranging from mild to severe. All patients were initially treated with high dose corticosteroids and 8/10 required additional medical therapies such as IVIG, plasmapheresis, cyclophosphamide, or rituximab. Increased intracranial pressure was managed aggressively with 2/10 patients requiring decompressive craniectomies. Clinical outcomes varied. ConclusionsSolitary tumefactive demyelinating lesions are rare and aggressive management of inflammation and increased intracranial pressure is essential. Biopsy is helpful to evaluate for other diagnoses on the differential and maximize therapies. Treatment beyond initial therapy with corticosteroids is often required. Isolated tumefactive demyelinating lesions are uncommon; multi-center natural history studies are needed to better delineate differential diagnoses and optimal therapies.

Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Tumefactive demyelinating lesions (TDL), characterized by large (≥ 2 cm) demyelinating lesions mimicking tumors, are a rare manifestation of the central nervous system inflammatory demyelinating diseases (CNS-IDD). Distinguishing TDL from other brain lesions can be challenging, often necessitating biopsy or advanced diagnostics. The natural history of TDL varies among races. This study aimed to assess demographics, clinical and radiological features, laboratory findings, management, and outcomes of Thai patients with TDL. We retrospectively reviewed records of twenty-six patients with TDL from the Multiple Sclerosis and Related Disorders registry from two tertiary medical centers. Among 1102 CNS-IDD patients, 26 (2.4%) had TDL. The median age at TDLs onset was 34.5 years (range 17–75); 69.2% were female. Over 70% manifested TDL as their initial CNS-IDD presentation. Common presenting symptoms included motor deficits, sensory disturbances, and cognitive problems. About two-fifths exhibited multiple lesions, most frequently in the frontoparietal region (46.2%). Half of the patients showed an incomplete ring on post-contrast T1-weighted imaging, with peripheral diffusion-weighted imaging restriction in twenty-one patients. T2-hypointense rims were present in thirteen (56.5%) patients. Brain biopsy was performed in 12 cases (46.1%). Serum aquaporin-4 immunoglobulin was positive in 16.7% of tested (4/24) cases. Serum myelin oligodendrocyte glycoprotein immunoglobulin was negative in all thirteen patients tested. Twenty patients (76.9%) received intravenous corticosteroids for TDL attacks. After the median follow-up period of 48 months (range 6–300), 23.1% experienced CNS-IDD relapses. Median Expanded Disability Status Scale at TDL diagnosis was 4.3 (range 0.0–9.5), and improved to 3.0 (range 0.0–10.0) at the last follow-up. This study suggested that TDL were rare among Thai CNS-IDD patients, frequently presenting as a monophasic condition with a favorable outcome.

Clinical and radiological characteristics and outcomes of patients with recurrent or relapsing tumefactive demyelination

BackgroundRelapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports. ObjectiveWe examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs). MethodsWe used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient. ResultsWe identified 18 cases (11F, 7 M). The median age at onset of the index TDL was 37 years (range 12–72) and most were solitary lesions 72 % (13/18). CSF-restricted oligoclonal bands (OCBs) were detected in 25 % (4/16). Only one of those tested (n = 13) was positive for AQP4-IgG. A moderate-to-marked treatment response (high dose corticosteroid with or without additional plasmapheresis, IVIg or disease modifying therapies) was evident in 89 % of treated patients. Median EDSS at the median follow-up of 36 months (range 6–144) was 2 (range 1–10). Most remained ambulatory (EDSS < 4 in 13/18), but 1 patient died. ConclusionThe median age of patients with relapsing TDLs is similar to that of typical MS, but differences include a lower female:male sex ratio, larger lesions, and a comparative lack of CSF-restricted OCBs. Outcomes vary among this group of patients ranging from minimal disability through to death.

Tumefactive demyelinating lesions versus CNS neoplasms, a comparative study.

Differentiating tumefactive demyelinating lesions (TDL) from neoplasms of the central nervous system continues to be a diagnostic dilemma in many cases. Our study aimed to examine and contrast the clinical and radiological characteristics of TDL, high-grade gliomas (HGG) and primary CNS lymphoma (CNSL). This was a retrospective review of 66 patients (23 TDL, 31 HGG and 12 CNSL). Clinical and laboratory data were obtained. MRI brain at presentation were analyzed by two independent, blinded neuroradiologists. Patients with TDLs were younger and predominantly female. Sensorimotor deficits and ataxia were more common amongst TDL whereas headaches and altered mental status were associated with HGG and CNSL. Compared to HGG and CNSL, MRI characteristics supporting TDL included relatively smaller size, lack of or mild mass effect, incomplete peripheral rim enhancement, absence of central enhancement or restricted diffusion, lack of cortical involvement, and presence of remote white matter lesions on the index scan. Paradoxically, some TDLs may present atypically or radiologically mimic CNS lymphomas. Careful evaluation of clinical and radiological features helps in differentiating TDLs at first presentation from CNS neoplasms.

Kidney Transplant Recipient With Tumefactive Demyelinating Lesions: A Case Report and Literature Review

Tumefactive demyelinating lesions (TDLs) are rare and specific types of inflammatory demyelinating lesions. Its clinical manifestations are nonspecific, and the imaging findings are similar to those of other intracranial space-occupying lesions, which are usually misdiagnosed as tumors or abscesses and require a pathologic examination to confirm the diagnosis. Tumefactive demyelinating lesions after kidney transplantation are even rarer. This article reports a case of TDLs after kidney transplantation. A 60-year-old female patient underwent kidney transplantation 15 years ago and took anti-rejection drugs such as tacrolimus, tacrolimus, and corticosteroids after surgery. The patient was admitted with headache and left limb weakness, and magnetic resonance imaging of the head showed multiple space-occupying lesions with surrounding edema. The patient underwent a stereotactic biopsy of the encephalopathy lesion, and postoperative pathology confirmed TDLs. She was treated with corticosteroids and discharged after the improvement of her symptoms. Here, to our knowledge, we report the first case of TDLs after kidney transplantation. We report this case to provide clinicians with useful information on intracranial demyelinating disease after kidney transplantation.

Clinical spectrum and prognosis of pathologically confirmed atypical tumefactive demyelinating lesions

To describe the clinical spectrum and prognosis of atypical tumefactive demyelinating lesions (TDLs), which were confirmed by pathology. A total of 11 patients were diagnosed with atypical TDLs confirmed by brain biopsy and surgery between January 2006 and December 2017. The clinical spectrum and prognosis in these patients were analyzed. The patients’ ages ranged from 29 to 62 years, with a mean age of 48.9 years; 72.7% were males. The Expanded Disability Status Scale (EDSS) of the patients with first onset was 2.36. Most of the patients started with limb numbness and weakness (45.5%) or alalia (27.2%). The mean time from symptom onset to biopsy or surgery was 12.9 days (3–30 days). Most of the patients had solitary lesions (72.7%), supratentorial lesions (90.9%, particularly predominant in the frontal, temporal, and parietal lobes), moderate edema (63.6%), mild mass effect (54.5%), and patchy lesions (54.5%). Among them, three patients were positive for myelin basic protein (MBP) and one patient was positive for myelin oligodendrocyte glycoprotein (MOG). The patients were followed up for an average of 6.9 years (2–14 years), and recurrent TDLs were observed in 2 patients. Except for the 2 patients who relapsed, only 1 of the 9 patients died; the other 8 patients improved or maintained the status quo (the EDSS scores were lower or unchanged). The patients did not have any serious nervous system injury at onset, and the main presentation included extremity weakness, headache or dizziness, and alalia. The most common form was patchy on MRI enhancement. Cerebrospinal fluid and demyelination test can be an indicator of TDLs, and seizures may be a poor prognostic indicator. Most atypical TDLs have monophasic courses and good outcomes. The effect of neurosurgery alone was good in our group, and the effect of surgery on atypical TDLs can be further studied.

Tumefactive multiple sclerosis – a diagnostic and therapeutic challenge

Tumefactive multiple sclerosis with the presence of tumefactive demyelinating lesions (TDL) is an infrequent manifestation of multiple sclerosis. It poses a diagnostic challenge, especially in attempts to differentiate it from a central nervous system neoplasm or other demyelinating disorders, such as neuromyelitis optica spectrum disorder (NMOSD). The article reports a case of a 40-year-old female during chronic steroid therapy administered due to an episode of idiopathic transverse myelitis. The patient developed tumour-like brain lesions in the left hemispheric parietal-occipital region. The diagnostic process was hindered by extended steroid treatment and a lack of patient compliance. This case is an example of insufficient knowledge about rare forms of multiple sclerosis and the need for a holistic approach involving a through analysis of medical history and previous medical records.

The value of convolutional neural networks-based deep learning model in differential diagnosis of space-occupying brain diseases.

It is still a challenge to differentiate space-occupying brain lesions such as tumefactive demyelinating lesions (TDLs), tumefactive primary angiitis of the central nervous system (TPACNS), primary central nervous system lymphoma (PCNSL), and brain gliomas. Convolutional neural networks (CNNs) have been used to analyze complex medical data and have proven transformative for image-based applications. It can quickly acquire diseases' radiographic features and correct doctors' diagnostic bias to improve diagnostic efficiency and accuracy. The study aimed to assess the value of CNN-based deep learning model in the differential diagnosis of space-occupying brain diseases on MRI. We retrospectively analyzed clinical and MRI data from 480 patients with TDLs (n = 116), TPACNS (n = 64), PCNSL (n = 150), and brain gliomas (n = 150). The patients were randomly assigned to training (n = 240), testing (n = 73), calibration (n = 96), and validation (n = 71) groups. And a CNN-implemented deep learning model guided by clinical experts was developed to identify the contrast-enhanced T1-weighted sequence lesions of these four diseases. We utilized accuracy, sensitivity, specificity, and area under the curve (AUC) to evaluate the performance of the CNN model. The model's performance was then compared to the neuroradiologists' diagnosis. The CNN model had a total accuracy of 87% which was higher than senior neuroradiologists (74%), and the AUC of TDLs, PCNSL, TPACNS and gliomas were 0.92, 0.92, 0.89 and 0.88, respectively. The CNN model can accurately identify specific radiographic features of TDLs, TPACNS, PCNSL, and gliomas. It has the potential to be an effective auxiliary diagnostic tool in the clinic, assisting inexperienced clinicians in reducing diagnostic bias and improving diagnostic efficiency.

Central nervous system tumefactive demyelinating lesions: Risk factors of relapse and follow-up observations.

To track the clinical outcomes in patients who initially presented with tumefactive demyelinating lesions (TDLs), we summarized the clinical characteristics of various etiologies, and identified possible relapse risk factors for TDLs. Between 2001 and 2021, 116 patients initially presented with TDLs in our hospital were retrospectively evaluated. Patients were followed for relapse and clinical outcomes, and grouped according to various etiologies. Demographic information, clinical data, imaging data, and laboratory results of patients were obtained and analyzed. The risk factors of relapse were analyzed by the Log-Rank test and the Cox proportional hazard model in multivariate analysis. During a median follow-up period of 72 months, 33 patients were diagnosed with multiple sclerosis (MS), 6 patients with Balo, 6 patients with neuromyelitis optica spectrum disorders (NMOSD), 10 patients with myelin oligodendrocyte glycoprotein antibody-associated demyelination (MOGAD), 1 patient with acute disseminated encephalomyelitis (ADEM), and the remaining 60 patients still have no clear etiology. These individuals with an unknown etiology were categorized independently and placed to the other etiology group. In the other etiology group, 13 patients had recurrent demyelinating phases, while 47 patients did not suffer any more clinical events. Approximately 46.6% of TDLs had relapses which were associated with multiple functional system involvement, first-phase Expanded Disability Status Scale score, lesions morphology, number of lesions, and lesions location (P<0.05). And diffuse infiltrative lesions (P=0.003, HR=6.045, 95%CI:1.860-19.652), multiple lesions (P=0.001, HR=3.262, 95%CI:1.654-6.435) and infratentorial involvement (P=0.006, HR=2.289, 95%CI:1.064-3.853) may be independent risk factors for recurrence. Relapse free survival was assessed to be 36 months. In clinical practice, around 46.6% of TDLs relapsed, with the MS group showing the highest recurrence rate, and lesions location, diffuse infiltrative lesions, and multiple lesions might be independent risk factors for relapse. Nevertheless, despite extensive diagnostic work and long-term follow-up, the etiology of TDLs in some patients was still unclear. And these patients tend to have monophase course and a low rate of relapse.

Integrative and comparative single-cell analysis reveals transcriptomic difference between human tumefactive demyelinating lesion and glioma

Tumefactive demyelinating lesion (TDL) is an immune-mediated disease which can be misdiagnosed as glioma. At present, there is no study comparing difference between the two disorders at the cellular level. Here, we perform integrative and comparative single-cell RNA sequencing (ScRNA-seq) transcriptomic analysis on TDL and glioma lesions. At single-cell resolution, TDL is comprised primarily of immune cells, which is completely different from glioma. The integrated analysis reveals a TDL-specific microglial subset involving in B cell activation and proliferation. Comparative analysis highlights remyelination function of glial cells and demyelination function of T cells in TDL. Subclustering and pseudotime trajectory analysis of T cells in TDL reveal their heterogeneity and diverse functions involving in TDL pathogenesis and recovery process. Our study identifies substantial differences between TDL and glioma at single-cell resolution. The observed heterogeneity and potentially diverse functions of cells in TDL may be critical in disease progression.

Neuroimaging and clinicopathological differences between tumefactive demyelinating lesions and sentinel lesions of primary central nervous system lymphoma.

It is still a challenge to distinguish sentinel lesions of primary central nervous system lymphoma (PCNSL) from atypical tumefactive demyelinating lesions (TDLs) in clinical practice. We aimed to investigate potential differences of clinical features, neuroimaging findings and pathological characteristics between PCNSL and TDLs, improving early accurate diagnosis. It was a retrospective study involving 116 patients with TDLs and 150 patients with PCNSLs. All cases were pathologically confirmed. Clinical features, neuroimaging findings and pathological characteristics between two groups were analyzed. The onset age was 37 ± 14 years in TDLs and 58 ± 13 years in PCNSL(p=0.000). Main onset symptom was headache in TDLs, while cognitive impairment was frequently noted in PCNSL. CT brain scan image showed hypodense lesions in most cases of TDL (110/116, 94.8%), while approximately 80% patients (120/150) with PCNSL had hyperdense lesions. Furthermore, we found that the presence of Creutzfeldt-Peters cells (might be misdiagnosed as tumor cells) may serve as an important feature in TDLs. Onset age of patients with TDLs was younger than PCNSL. Neuroimaging features on brain CT scan might provide clues to make a differential diagnosis. Pathological features of PCNSL with sentinel lesions or following steroids therapy might mimic TDLs. Dynamic neuroimaging pathological and follow-up information were essential for an accurate diagnosis.

Tumefactive multiple sclerosis versus high grade glioma: A diagnostic dilemma.

Background: Tumefactive demyelinating lesions (TDL) share similar clinical features and magnetic resonance imaging (MRI) characteristics with high grade glioma (HGG). This study develops an approach to navigating this diagnostic dilemma, with significant treatment implications as the management of both entities is drastically different.Methods: A retrospective analysis of 41 TDLs and 91 HGG with respect to demographics, presentation and classical MRI characteristics was performed. A diagnostic pathway was then developed to help diagnose TDLs based on whole neuraxis MRI and cerebrospinal fluid (CSF) examination.Results: The diagnosis of TDL is more likely than HGG in younger females who present with subacute or chronic symptoms. MRI characteristics favoring TDL over HGG include smaller size, open rim enhancement, little or no associated edema or mass effect and the presence of a T2 hypointense rim. MRI of the whole neuraxis for detection of other lesions typical of multiple sclerosis (MS), in combination with a lumbar puncture (LP) showing positive CSF-specific oligoclonal bands (OCB), was positive in 90% of the TDL cohort.Conclusion: The diagnostic pathway, proposed on the basis of specific clinicoradiological features, should be followed in patients with suspected TDL. If MRI demonstrates other lesions typical of MS and LP demonstrates positive CSF-specific OCBs, then patients should undergo a short course of IV steroids to look for clinical improvement. Patients, who continue to deteriorate, do not demonstrate other lesions on MRI or where the LP is negative for CSF-specific OCB, should be considered for biopsy if safe to do so. This pathway will give the patients the best chance at neurological preservation.

Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases.

Tumefactive demyelinating lesions (TDL) represent a diagnostic dilemma for clinicians, and in rare atypical cases a collaboration of a neuroradiologist, a neurologist, and a neuropathologist is warranted for accurate diagnosis. Recent advances in neuropathology have shown that TDL represent an umbrella under which many different diagnostic entities can be responsible. TDL can emerge not only as part of the spectrum of classic multiple sclerosis (MS) but also can represent an idiopathic monophasic disease, a relapsing disease with recurrent TDL, or could be part of the myelin oligodendrocyte glycoprotein (MOG)- and aquaporin-4 (AQP4)-associated disease. TDL can appear during the MS disease course, and increasingly cases arise showing an association with specific drug interventions. Although TDL share common features with classic MS lesions, they display some unique features, such as extensive and widespread demyelination, massive and intense parenchymal infiltration by macrophages along with lymphocytes (mainly T but also B cells), dystrophic changes in astrocytes, and the presence of Creutzfeldt cells. This article reviews the existent literature regarding the neuropathological findings of tumefactive demyelination in various disease processes to better facilitate the identification of disease signatures. Recent developments in immunopathology of central nervous system disease suggest that specific pathological immune features (type of demyelination, infiltrating cell type distribution, specific astrocyte pathology and complement deposition) can differentiate tumefactive lesions arising as part of MS, MOG-associated disease, and AQP4 antibody-positive neuromyelitis optica spectrum disorder. Lessons from immunopathology will help us not only stratify these lesions in disease entities but also to better organize treatment strategies. Improved advances in tissue biomarkers should pave the way for prompt and accurate diagnosis of TDL leading to better outcomes for patients.

Newly Diagnosed Tumefactive Demyelinating Lesion and Multiple Sclerosis After COVID-19 Infection.

To describe the parainfectious or postinfectious effects of COVID-19 infection on the first demyelinating presentation of Multiple Sclerosis and tumefactive demyelinating lesion (TDL) developing with Longitudinally Extensive Transverse Myelitis (LETM). We present six patients who presented with a first CNS demyelination event or whose demyelinating lesions had aggravated after COVID-19 infection between May and December 2020. Nasopharyngeal swab SARS-CoV-2 PCR positivity was detected in five cases and cerebrospinal fluid (CSF) PCR was positive in one. The symptoms, neurological signs, radiological and CSF findings of the cases were examined. A 24-year-old woman presented with LETM aggravated by COVID-19, accompanied by a newly developed open-ring enhanced TDL. Four patients were diagnosed with the first presentation of MS, and one presented with clinically isolated syndrome according to the McDonald 2017 criteria. The interval between SARS-CoV-2 infection and the onset of clinical symptoms ranged from 4-93 days. All of the cases present with pyramidal or brain stem findings and have high brain and/or spinal MRI load. This suggests the moderate activity of CNS demyelinating disease after COVID-19 infection. Based on this case series, all these first demyelinating events suggested that COVID-19 infection might trigger or exacerbate CNS demyelinating disease. SARS-CoV-2 plays a role in the clinical onset of Multiple Sclerosis. Active delayed demyelination developed within the first three months. This can be explained by COVID-triggered neuroimmune response that had been latent, and the initiation of the active disease process began with triggering or aggravation of the lesions in MRI. Multiple Sclerosis should be maintained during the COVID-19 pandemic.

Deep Learning With Data Enhancement for the Differentiation of Solitary and Multiple Cerebral Glioblastoma, Lymphoma, and Tumefactive Demyelinating Lesion.

ObjectivesTo explore the MRI-based differential diagnosis of deep learning with data enhancement for cerebral glioblastoma (GBM), primary central nervous system lymphoma (PCNSL), and tumefactive demyelinating lesion (TDL).Materials and MethodsThis retrospective study analyzed the MRI data of 261 patients with pathologically diagnosed solitary and multiple cerebral GBM (n = 97), PCNSL (n = 92), and TDL (n = 72). The 3D segmentation model was trained to capture the lesion. Different enhancement data were generated by changing the pixel ratio of the lesion and non-lesion areas. The 3D classification network was trained by using the enhancement data. The accuracy, sensitivity, specificity, and area under the curve (AUC) were used to assess the value of different enhancement data on the discrimination performance. These results were then compared with the neuroradiologists’ diagnoses.ResultsThe diagnostic performance fluctuated with the ratio of lesion to non-lesion area changed. The diagnostic performance was best when the ratio was 1.5. The AUCs of GBM, PCNSL, and TDL were 1.00 (95% confidence interval [CI]: 1.000–1.000), 0.96 (95% CI: 0.923–1.000), and 0.954 (95% CI: 0.904–1.000), respectively.ConclusionsDeep learning with data enhancement is useful for the accurate identification of GBM, PCNSL, and TDL, and its diagnostic performance is better than that of the neuroradiologists.

Prognostic factors in Tumefactive demyelinating lesions: A retrospective study

IntroductionDemyelinating lesions occasionally present as mass-like lesions on imaging, raising concern for malignancy. The disease course of such tumefactive demyelinating lesions (TDLs) is still being defined. MethodsWe retrospectively analyzed 21 patients with new-onset neurologic symptoms and mass-like lesions on brain magnetic resonance imaging (MRI), which resulted in biopsy-proven diagnoses of demyelination. 18 patients had a median follow-up of 52 months. The clinical, radiologic and histologic features were associated with disease course. ResultsAn aggressive disease course (ADC) was noted in 33% of the patients and was associated with an initial largest lesion size ≥35 mm (p = 0.0007), mass effect (p = 0.01) and perilesional edema (p = 0.01) on MRI. Age 30 years and older, at presentation (p = 0.05), as well as the absence of a prior tonsillectomy (p = 0.0128) were also associated with an ADC. ConclusionsWe identified several factors, including initial larger lesion size, mass effect and perilesional edema on MRI, presentation after 30 years of age and the absence of a prior tonsillectomy, that predict an ADC in patients presenting with TDLs. These predictors of disease course can help guide patient follow-up and stratification for intervention.

Tumefactive Demyelination Appearing as Multiple Cystic Brain Lesions

Tumefactive demyelinating lesions (TDLs) are a rare sequelae of idiopathic inflammatory demyelinating diseases of the central nervous system. Their propensity to mimic tumor and abscess poses a diagnostic challenge for the clinician. Our case depicts TDLs causing right-hand focal sensory seizures in an otherwise healthy 35-year-old female. The differential diagnosis of metastatic disease and infection were excluded on histology. Ensuing magnetic resonance imaging of the cord, in addition to cerebral spinal fluid analysis, supported the diagnosis of idiopathic inflammatory demyelinating diseases. This case highlights the need to consider the rare diagnosis of TDL when imaging shows cystic brain lesions in an otherwise healthy young adult.

Tumefactive multiple sclerosis versus high-grade glioma: A diagnostic dilemma.

Background: Tumefactive demyelinating lesions (TDLs) share similar clinical features and MRI characteristics with high-grade glioma (HGG). This study develops an approach to navigating this diagnostic dilemma, with significant treatment implications as the management of both entities is drastically different.Methods: A retrospective analysis of 41 TDLs and 91 HGG with respect to demographics, presentation, and classical MRI characteristics was performed. A diagnostic pathway was then developed to help diagnose TDLs based on whole neuraxis MRI and cerebrospinal fluid (CSF) examination.Results: The diagnosis of TDL is more likely than HGG in younger females who present with subacute or chronic symptoms. MRI characteristics favoring TDL over HGG include smaller size, open rim enhancement, little or no associated edema or mass effect, and the presence of a T2 hypointense rim. MRI of the whole neuraxis for detection of other lesions typical of multiple sclerosis (MS), in combination with a lumbar puncture (LP) showing positive CSF-specific oligoclonal bands (OCB), was positive in 90% of the TDL cohort.Conclusion: The diagnostic pathway, proposed on the basis of specific clinicoradiological features, should be followed in patients with suspected TDL. If MRI demonstrates other lesions typical of MS and LP demonstrates positive CSF-specific OCBs, then patients should undergo a short course of IV steroids to look for clinical improvement. Patients who continue to deteriorate, do not demonstrate other lesions on MRI or where the LP is negative for CSF-specific OCB, should be considered for biopsy if safe to do so. This pathway will give the patients the best chance at neurological preservation.

Tumefactive demyelinating lesion (TDL)

Tumefactive demyelinating lesion(TDL)is defined as a large lesion, size >2 cm, mass effect, perilesional edema and/or ring enhancement. TDL could occur in multiple sclerosis(MS), neuromyelitis optica spectrum disorder(NMOSD), acute disseminated encephalomyelitis(ADEM)or other immunological diseases. Non-invasive methods including MR imaging and assay of several autoantibodies(e.g. aquaporin-4 autoantibodies)are recommended when each TDL is identified. The radiological findings on MRI are characterized by size >2 cm, mass effect, perilesional edema, T2 weighted hypointense rim, peripheral diffusion restriction, open ring enhancement, vascular enhancement, and central vein sign. When atypical clinical and radiological presentations are present in patients with TDL, diagnosis may necessitate brain biopsy due to exclude alternative pathology(e.g. primary central nervous system lymphoma). Because treatments and outcomes for patients with TDL are dependent on each disease etiology including MS, NMOSD, ADEM or others, we should always clarify the entire picture behind the disease.