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종결 Case No. 733 2025-10-01~2025-10-31
출제자 : 정소영 Hit : 453
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Period 2025-10-01~2025-10-31
Diagnosis Progressive Multifocal Leukoencephalopathy
Gender
  • M
  •  
  • F
Age 53
Clinical information M/53Y
C.C> A patient presented with cognitive impairment that began two weeks ago.
Past medical history> uncontrolled DM, HIV Ab Positive
Images> FLAIR, T1WI, DWI, Contrast T1WI
What was the your diagnosis?
Discussion Progressive Multifocal Leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by reactivation of the JC virus, predominantly in immunocompromised patients. It is characterized by multifocal areas of demyelination affecting the white matter, leading to neurological deficits such as altered mental status, motor impairment, ataxia, visual disturbances, and seizures.
Radiologic features are critical for diagnosis and monitoring. Brain MRI is the imaging modality of choice for PML and reveals multiple asymmetric, confluent lesions predominantly in the supratentorial white matter, especially in the parieto-occipital regions. On MRI, lesions typically appear hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR sequences, with a characteristic scalloped or irregular lateral margin. Diffusion-weighted imaging may show variable restricted diffusion depending on the activity of the lesions. Contrast enhancement is usually absent but may be present in early or immune reconstitution inflammatory syndrome (IRIS) phases.
Lesions show no significant mass effect or edema in most cases, although mass effect can occur and correlates with poorer prognosis. Small punctate high T2 signal lesions surrounding the main areas (“Milky Way” sign) and a "barbell" sign are other described imaging features. MRI not only assists in early diagnosis but also in follow-up to assess progression or response to therapy.

Reference
Mori, K., Kurokawa, M., Harada, M., Nakamichi, K., Arai, H., Takao, M., ... & Miura, Y. (2025). Overview of MRI findings in progressive multifocal leukoencephalopathy. Japanese Journal of Radiology, 1-18.
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  • 조서범 연세대학교 세브란스병원
  • 김신영 양산부산대학교병원
  • Murat Ulusoy GOP Avrasya Hospital Istanbul/Turkey
  • 이진영 동국대학교 일산병원
  • 정하경 서울아산병원
  • 홍혁기 서울의료원
  • 정하택 서울의료원
  • 고지수 서울아산병원
  • 도윤아 분당서울대학교병원
  • 이강휘 서울대학교병원
  • 최현일 강동경희대학교병원
  • 김찬순 계명대학교 동산의료원
  • 류혜선 강동경희대학교병원

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Murat Ulusoy

GOP Avrasya Hospital Istanbul/Turkey 2025-10-11 14:41:17
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