The Open Ring or White-Matter-Crescent Pattern of Enhancement Differentiates Demyelinating Brain Disease with Tumor-Like Presentation from Infection and Neoplasm

Masdeu, Joseph C.; Quinto, Christine; Olivera, Caridad Villar; Leslie, Denise; Tenner, Michael; Visintainer, Paul

doi:10.1177/19714009980110s206

Cited by 2 publications

(1 citation statement)

References 4 publications

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“…In particular, the open-ring sign was present in 70% of the demyelinating disease 26 . The open portions of the open-ring face the cortical gray matter or basal ganglia and the portions of enhancement face the white matter 27 .The lesions of enhancement are regarded as demyelination and the lesions that are not enhanced are regarded as chronic inflammation 28 . In our study, all the cases appeared as hypointensities on T1-weighted MRI and hyperintensities on T2-weighted MRI.…”

Section: Discussionmentioning

confidence: 99%

Clinical spectrum and prognosis of pathologically confirmed atypical tumefactive demyelinating lesions

Zhang

Yan

et al. 2023

Sci Rep

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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.

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Section: Discussionmentioning

confidence: 99%

Clinical spectrum and prognosis of pathologically confirmed atypical tumefactive demyelinating lesions

Zhang

Yan

et al. 2023

Sci Rep

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Tumefactive demyelinating lesions: nine cases and a review of the literature

et al. 2009

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Tumefactive demyelinating lesions (TDLs) are misdiagnosed frequently. To investigate the characteristics of TDLs, clinical and radiological data from nine cases with TDLs were analyzed after admission. All cases underwent surgery and pathological examination; some received postoperative steroid therapy. Onsets were mostly within 3 weeks and main presentation included intracranial hypertension, extremity weakness, epilepsy, and visual disturbance. Symptoms in children were acute and severe, frequently including headache, vomiting, and visual disturbance. Most intracephalic lesions were in cerebral hemispheres. All intraspinal lesions were in cervical segments. Radiological features included mass effect, perifocal edema and enhancement (of which open-ring enhancement was diagnostic), and decreased relative cerebral blood volume. Intraoperative frozen section did not confirm the diagnosis, while postoperative paraffin section did confirm it (by evidence of macrophage infiltration). The patients responded well to steroid therapy and no relapse was found during following up. Thus, intensive analysis of both clinical and radiological data may provide some clues for diagnosis. For suspected cases, it is advisable to take steroid therapy or undergo advanced radiological examinations, such as serial magnetic resonance spectroscopy. However, in difficult cases, pathological evidence is beneficial to a final diagnosis.

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The Open Ring or White-Matter-Crescent Pattern of Enhancement Differentiates Demyelinating Brain Disease with Tumor-Like Presentation from Infection and Neoplasm

Cited by 2 publications

References 4 publications

Clinical spectrum and prognosis of pathologically confirmed atypical tumefactive demyelinating lesions

Clinical spectrum and prognosis of pathologically confirmed atypical tumefactive demyelinating lesions

Tumefactive demyelinating lesions: nine cases and a review of the literature

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