감염내과_ PML (Progressive Multifocal Leukoencephalopathy)

1. Clinical Features and Pathology

1) Multifocal areas of demyelination of varying size distributed throughout the brain but sparing the spinal cord and optic nerves 

2) Cytologic alterations in both astrocytes and oligodendrocytes

- Astrocytes: enlarged, hyperchromatic, deformed, bizarre nuclei, frequent mitotic figures

- Oligodendrocytes: enlarged, densely staining nuclei (crystalline arrays of JC virus particles)

- JC virus: John Cunnignham virus (human polyomavirus)

3) 환자 증상

- Visual deficit (45%), Mental impairment (38%, Dementia, confusion, personally change)

- Weakness, ataxia

- Seizures in 20%

4) 동반 질병 (Underlying immunosuppressive disorder)

- AIDS (80%): 5% AIDS 환자까지

- Hematologic malignancies (13%)

- Transplant receipients (5%)

- Chronic inflammatory diseases (2%)

2. Diagnostic Studies

1) MRI: multifocal asymmetric, coalescing white matter lesion

- T2, FLAIR: increased signal

- T1-weighted images: decreased signal

- HIV-PML lesions: classically nonenhancing (90%)

2) CSF: typically normal

3. Treatment: 효과적인 치료제는 아직 없다.

1) 5-HT_2a receptor antagonist: potential beneficial effect

- Oligodendrocyte에서 JCV의 binding을 억제

2) Highly Active Antiretroviral Therapy (HAART): HIV 양성인 환자에 치료

- Nucleoside/nucleotide reverse transcriptase inhibitors: abacavir, emtricitabine, tenofovir

- Nonnucleoside reverse transcriptase inhibitors (NNRTIs): efavirenz, etravirine, nevirapine

- Protease inhibitors (PIs): atazanavir, darunavir, ritonavir

- Entry inhibitors: enfuvirtide maraviroc

- Integrase inhibitors: dolutegravir, raltegravir