Pathologically Proven Gadolinium-enhanced MRI Lesions in the Bilateral Corticospinal Tracts in Lymphomatosis Cerebri

Yamaura, Genpei; Ogasawara, Akihiro; Ito, Takeshi; Ohsugi, Shizuka; Kanatsuka, Yoichi; Hayashi, Ryuichiro; Iwashita, Hiromichi; Hayashi, Hiroyuki; Koyano, Shigeru; Yamaguchi, Shigeki; Tanaka, Fumiaki

doi:10.2169/internalmedicine.4382-19

Cited by 3 publications

(2 citation statements)

References 14 publications

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“…Performed in the operating room, the procedure involves the removal of a small piece of tissue, most commonly from the brain, but may include samples from the scalp, blood vessels or dura mater, and in most cases, the neurosurgeon uses stereotactic equipment to localize the preferable site for the biopsy (7). At present, early diagnosis of LC is challenging and misdiagnosis is not uncommon (1)(2)(3)5,(8)(9)(10), but specific epidemiological data are lacking because it is a rare disease and most of the previous studies are case reports (1)(2)(3)(4)(5)(6).…”

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confidence: 99%

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Lymphomatosis cerebri: Multimodality imaging features and misdiagnosis analysis

et al. 2021

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Lymphomatosis cerebri (LC) is a significant challenge in terms of its clinical diagnosis due to it being a rare disease. The purpose of the present study was to investigate the multimodality imaging characteristics, clinical features and reasons for misdiagnosis of LC with the goal of potentially facilitating early and accurate diagnosis of this frequently misdiagnosed disease. In the present study, clinical data and cerebral multimodality imaging findings from 11 patients with LC proven based on pathology were retrospectively analyzed and reviewed with consultation of the literature. The results indicated that the common symptoms included cognitive decline (8/11), gait disturbance (9/11) and behavioral abnormalities (5/11). Cerebrospinal fluid analysis indicated that the number of cells and level of protein increased (8/10). All patients had both deep and lobar lesion distribution of bilateral cerebral white matter with equal or slightly low-density shadows on CT plain scan and slightly longer signals on T1and T2-weighted MRI. Most of the lesions (9/11) exhibited isointensity or slight hyperintensity on diffusion-weighted imaging and hyperintensity on apparent diffusion coefficient maps. In addition, five patients presented with a marked decrease in N-acetylaspartate/creatine (Cr) and increase in choline/Cr on 1 H-magnetic resonance spectroscopy, including an increase in lipid/Cr in 3 cases. Of these, one case exhibited no increase in lesion metabolism and 2 cases had slightly increased uptake on positron emission tomography/CT. The present study indicated that the multimodality imaging findings of LC have certain distinct characteristics and prompt recognition of these features may significantly improve early diagnosis and patient prognosis. Misdiagnosis may be mainly due to insufficient understanding knowledge of the condition and improper brain biopsy.

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confidence: 99%

“…Therefore, early diagnosis is significantly crucial for those patients, which mostly depends on imaging. However, the differentiated imaging features of LC have remained to be established in detail ( 1 – 3 , 5 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

Lymphomatosis cerebri: Multimodality imaging features and misdiagnosis analysis

et al. 2021

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Clinical and imaging features of lymphomatosis cerebri: analysis of 8 cases and systematic review of the literature

Fan,

Zhao,

Chen

et al. 2023

Clin Exp Med

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Lymphomatosis cerebri (LC) is a rare type of primary central nervous system lymphoma with diffuse, nonenhancing infiltrative lesions and is often misdiagnosed. Our study aimed to investigate the clinical characteristics and prognosis of LC through analyzing patients from the literature and our own center, so as to improve early diagnosis and treatment. PubMed, Web of Science and our hospital databases were reviewed, and information on demographic, clinical, pathological, cerebrospinal fluid (CSF), neuroimaging and treatment options was extracted. Univariate survival analysis was conducted by generating survival curves and comparing them using the log-rank test. Multivariate analysis was performed using the Cox proportional hazards regression model to identify the prognostic predictors. A total of 81 patients (median age: 58 years; interquartile range, IQR: 50–66.5 years), 45 males and 36 females, were included. The most common symptoms were cognitive impairment (65.4%) and gait impairment (50.6%). Imaging studies indicated that all 81 patients had supratentorial structure involvement, and 93.8% (76/81) had bilateral hemisphere involvement. There were 53.3% (32/60) patients with CSF pleocytosis and 65% (39/60) patients with increased CSF protein levels. The median time of diagnosis was 4.8 months (IQR: 2.3–6.9 months). Compared with 4 (95% CI: 1.78–6.22) months for all 81 patients, the median OS was 20 (95% CI: 8.24–31.76) months for those who had chemotherapy plus radiotherapy. Multivariate Cox analysis revealed that chemoradiotherapy (HR: 0.12; 95% CI: 0.02–0.68) and higher CSF glucose level (HR: 0.01; 95% CI: 0.00–0.26) were inversely associated with death. The diagnosis of LC should be alerted when neuroimaging with bilateral hemispheric involvement and CSF abnormality with pleocytosis and increased protein. Once the diagnosis is confirmed, the combination of chemotherapy and radiotherapy can be considered if the patient’s physical condition permits.Journal standard instruction requires an unstructured abstract. Kindly check and confirm.We have checked and confirmed that there is no problem.

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Case Report: Paraneoplastic Hashimoto's Encephalopathy Associated With Lymphomatosis Cerebri With Periodic Synchronous Discharges Resembling Creutzfeldt–Jakob Disease

Amano¹,

Tsukada²,

Kosuge³

et al. 2021

Front. Neurol.

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Hashimoto's encephalopathy (HE) is an autoimmune encephalopathy that presents with various clinical symptoms, including cognitive deterioration, convulsive seizures, and personality changes. HE is associated with thyroid autoimmunity; however, few cases have been reported to develop as paraneoplastic syndrome. Herein, we report the case of a 73-year-old woman with onset of rapidly progressive dementia. Brain magnetic resonance imaging showed diffuse T2 hyperintensity areas involving the bilateral cerebral white matter, right midbrain tegmental area, left cerebral peduncle, and right middle cerebellar peduncle without clear diffusion hyperintensities and gadolinium enhancement. Her neurological symptoms worsened rapidly, and she presented with the apallic syndrome. Electroencephalogram showed periodic synchronous discharge, suggestive of Creutzfeldt–Jakob disease. However, a brain biopsy revealed infiltration of atypical lymphoid cells expressing CD20, and the anti-NH2 terminal of the α-enolase antibody was detected, diagnosing the complication with lymphomatosis cerebri and HE. High-dose intravenous methylprednisolone therapy and oral prednisolone with whole cranial irradiation enabled her to have simple conversations and consume food orally; however, severe cognitive impairment persisted. Although HE is a rare complication of malignant lymphoma, clinicians should be aware that it could be strongly suspected if the clinical symptoms worsen in the absence of imaging changes.

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Pathologically Proven Gadolinium-enhanced MRI Lesions in the Bilateral Corticospinal Tracts in Lymphomatosis Cerebri

Cited by 3 publications

References 14 publications

Lymphomatosis cerebri: Multimodality imaging features and misdiagnosis analysis

Lymphomatosis cerebri: Multimodality imaging features and misdiagnosis analysis

Clinical and imaging features of lymphomatosis cerebri: analysis of 8 cases and systematic review of the literature

Case Report: Paraneoplastic Hashimoto's Encephalopathy Associated With Lymphomatosis Cerebri With Periodic Synchronous Discharges Resembling Creutzfeldt–Jakob Disease

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