Case | Sex, Age, yrs | Underlying Disease and Duration | IST Before PML | IST at PML | PML Symptoms | MRI Findings | CSF JCV DNA PCR | PML Diagnosis | PML-specific Treatment | PML Outcome |
---|---|---|---|---|---|---|---|---|---|---|
113 | F 52 | DM, 5 yrs | Prednisone | Prednisone | ↓ Psychomotor performance, memory loss, aphasia, N VII palsy | Supratentorial and infratentorial T2 WM lesions, Gd+ cranial nerve roots VII and VIII | Negative × 2 | Brain biopsy: pos JCV DNA PCR | CYT-ARA | Improved |
213 | M 44 | DM, 2.5 yrs | Prednisone MMF, IVIG, CYC | Prednisone MMF, IVIG, CYC | ↓ Visual acuity hemianopsia | Supratentorial T2 WM lesions | Positive 104–105 JCV copies/ml | Pos CSF JCV DNA PCR | CYT-ARA, mirtazapine | Improved |
314 | F 72 | DM, unknown | AZA | AZA | Cognitive deficits, personality changes | Supratentorial Gd+ T2 WM lesions | ? | Brain biopsy and autopsy: pos JCV DNA ISH | CYT-ARA | Death in 1 mo |
411 | F 45 | DM, 7 mo, chronic renal insufficiency | Prednisone, IVIG, CYC | Prednisone, IVIG, CYC | Altered consciousness, progressive tetraparesis | Supratentorial T2 WM lesions | ? | Autopsy: intranuclear viral bodies and inflammation | None | Death in 1 mo |
512 | F 43 | DM, 5 yrs | Prednisone, AZA | Prednisone | Gait ataxia, dysarthria, corticospinal tract involvement | T2 WM lesions cerebellum and brainstem | Negative | Brain biopsy: pos JCV ab | Cidofovir, cytarabine | Death in 3 mo |
69 | F 29 | DM, unknown | HCQ | None | Left hemiparesis, diplopia, nystagmus, N III, N VI palsy | Supratentorial WM lesions, 1 thalamic lesion | ? | “Pos brain biopsy” | IV steroids, mirtazapine | Improved |
716 | M 54 | PM, unknown | Prednisone, chlorambucil | ? | Altered behavior, impaired memory, ataxia | ? | Not done? | Brain biopsy: pos JCV ab | None | Death in 5 mo |
815 | M 67 | DM, 4 yrs | Dexamethasone, CPP, IVIG | Dexamethasone, sirolismus, IVIG | Dysarthria, trunk ataxia, confusion, balance distribution | Supratentorial T2 WM lesions | Positive | Not performed | Cidofovir | Death within weeks |
9 | F 67, present case | PM, 2 yrs | Prednisone, CPP, IVIG, MTX, AZA | Prednisone | Limb ataxia, nystagmus, L hemiparesis, dysarthria | T2 WM lesions cerebellum and brainstem | Positive | Pos CSF JCV DNA PCR; brain biopsy: pos JCV DNA ISH | None | Death in 4.5 mo |
1017 | F 43 | PM 6.5 yrs | Prednisone, MTX, AZA, MMF, CYC, IVIG, rituximab | Prednisone, rituximab | Bilat hemianopsia, ↓ visual acuity | Supratentorial subcortical and cortical T2 and FLAIR WM lesions | Positive | Pos CSF JCV DNA PCR | None | Death in 8 mo |
IST: immunosuppressive treatment; DM: dermatomyositis; PM: polymyositis; MMF: mycophenolate mofetil; AZA: azathioprine; CPP: cyclophosphamide; CYC: cyclosporine; HCQ: hydroxychloroquine; MTX: methotrexate; IVIG: intravenous immunoglobulins; Gd+: gadolinium enhancement; CYT-ARA: cytosine-arabinoside; ISH: in situ hybridization; ab: antibody; WM: white matter; CSF: cerebrospinal fluid; JCV: John Cunningham virus; MRI: magnetic resonance imaging; PCR: polymerase chain reaction.