Table 1 Summary of findings of intracerebral light chain deposition diseases (No. 1–4) in comparison to intracerebral amyloidomas (No.5)
No. and Source | 1 | 2 | 3 | 4 | 5* |
|---|---|---|---|---|---|
Fischer et al. [18] 2006 | Popovic et al. [19] 2007 | Pantazis et al. [17] 2010 | Present case | Foreid et al. [16] 2010 | |
Fischer et al. [14] 2007 - (1935–2010) - | |||||
Age | 19 | 35 | 72 | 61 | 49 (15–71) |
Sex | M | M | M | F | 13F, 17M |
Initial clinical presentation | Generalized epileptic seizure | A) Progressive | Focal epileptic seizure (hemiparesis) | Hemiparesis | Epileptic seizures (12) |
bulbar palsy | |||||
Hemiparesis (6) | |||||
B) Putative | |||||
Gait disturbance (2) | |||||
Paranoid | Visual impairment (4) | ||||
schizophrenia | |||||
Cognitive disturbance (5) | |||||
Hearing loss (1) | |||||
Duration of symptoms | No | A) 1 ½ years | 8 years | 3 ½ years | NA |
B) 13 years | |||||
First location | Subcortical | Subcortical | Subcortical | Subcortical parieto-insular lobe & basal ganglia | Subcortical (21) |
periventricular posterior horn | gyrus rectus & | periventricular parietal lobe | Cortical (1) | ||
periventricular | |||||
Subcortical & cortical (8) | |||||
Diagnostic | Stereotactic Biopsy | Autopsy | Stereotactic Biopsy | Resection | Resection (9) |
Free-hand Biopsy (3) | |||||
Stereotactic Biopsy (13) | |||||
Autopsy (5) | |||||
CT and MRI-Findings | T2: Diffuse hyperintense mass with perifocal edema | NA in detail | T1 + GL: | T1: Hypointense | CT: Hyperdense |
Not enhanced | T1+GL: Enhanced | CT+CM: Enhanced | |||
T2: Hyperintense | T1: Hypointense | ||||
T2: Iso- to | T1+GL: Enhanced | ||||
hyperintense | T2: Iso- to | ||||
hyperintense | |||||
Histomorphology | - Light chain deposits within vessel wall | - Light chain deposits | - Light chain deposits around blood vessels | - Light chain deposits | - Amyloid deposits |
- B cell lymphoma with plasma-cellular different | - around blood vessels | - B cell lymphoma with abundant plasma cells | - Diffuse lympho- plasmacytic B-cell lymphoma | - around blood vessels | |
- Infiltrating T-cells | - within vessel wall | - Infiltrating T-cells | Infiltrating T-cells | - within vessel wall | |
- Monoclonal B- cell proliferation | - In most cases NA, but in some cases lymphocytes and plasma cells | ||||
- Infiltrating T-cells | |||||
Immunohistochemistry | - Congo red neg. | - Congo red neg. | - Congo red neg. | - Congo red neg. | - Congo red positive |
- λ-Light chain deposits | - λ-Light chain deposits | - λ-Light chain deposits | - λ-Light chain deposits and IgA | - λ-Light chain deposits | |
- Plasma cells: λ-light chain >> κ-light chain | - Plasma cells: λ-light chain >> κ-light chain | - Ki67: 2% | - Ki67: 3% | - 7–12-nm fibrils | |
- Ki67: 1-3% | - CD3+, CD5+, CD20+, CD23-, Cyclin D- | - CD5+, CD38+, CD19+, CD10, CD79a-, CD138- | - CD20+, CD5+, CD3+ CD38+, CD19-, CD10-, MUM1-, bcl6- | - λ-light chain ++ 14/16 | |
- κ-light chain + 2/16 |