PMID- 16768760
OWN - NLM
STAT- MEDLINE
DCOM- 20060921
LR  - 20210904
IS  - 1015-6305 (Print)
IS  - 1750-3639 (Electronic)
IS  - 1015-6305 (Linking)
VI  - 16
IP  - 2
DP  - 2006 Apr
TI  - A 19-year-old male with generalized seizures, unconsciousness and a deviation of 
      gaze.
PG  - 185-6, 187
AB  - Light chain deposition disease (LCDD) is a form of monoclonal immunoglobulin 
      deposition diseases (MIDD) which in contrast to light-chain derived (AL) 
      amyloidosis is characterized by non-congophilic, non-fibrillary monoclonal 
      protein deposits. Systemic organ deposits are common with the kidney being a 
      major target organ. A clonal lymphoplasmocytic proliferation, e.g. plasmacytoma, 
      is present in the majority of cases. Here we report on a 19-year-old male who 
      presented with generalized seizures and an enhancing white matter lesion on MRI 
      scans. A stereotactic brain biopsy revealed a low-grade B cell lymphoma with 
      plasmacellular differentiation as well as lambda light chain deposits without 
      birefringence under polarized microscopy. No systemic lymphoma manifestations or 
      systemic light chain deposits were found, nor was a monoclonal gammopathy 
      detectable in serum and urine. After systemic chemotherapy with three courses 
      high-dose methotrexate the size of the lesion and the condition of the patient 
      have remained stable for 24 months now. This is the first description of cerebral 
      LCDD developing without systemic disease in conjunction with the diagnosis of a 
      cerebral low-grade B cell lymphoma. We present the clinical, laboratory and 
      radiological findings and discuss the pathogenesis of this unusual LCDD 
      manifestation.
FAU - Fischer, L
AU  - Fischer L
AD  - Department of Hematology, Oncology and Transfusion Medicine, Klinikum Benjamin 
      Franklin, Freie Universitat, Berlin, Germany.
FAU - Korfel, A
AU  - Korfel A
FAU - Stoltenburg-Didinger, G
AU  - Stoltenburg-Didinger G
FAU - Ransco, C
AU  - Ransco C
FAU - Thiel, E
AU  - Thiel E
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Switzerland
TA  - Brain Pathol
JT  - Brain pathology (Zurich, Switzerland)
JID - 9216781
RN  - 0 (Anticonvulsants)
RN  - 0 (Antimetabolites, Antineoplastic)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 33CM23913M (Carbamazepine)
RN  - YL5FZ2Y5U1 (Methotrexate)
SB  - IM
MH  - Adult
MH  - Anticonvulsants/therapeutic use
MH  - Antimetabolites, Antineoplastic/therapeutic use
MH  - Brain Diseases/complications/drug therapy/metabolism/*pathology
MH  - Brain Edema/etiology/*pathology
MH  - Brain Neoplasms/complications/drug therapy/metabolism/*pathology
MH  - Carbamazepine/therapeutic use
MH  - Humans
MH  - Immunoglobulin Light Chains/*adverse effects/*metabolism
MH  - Lymphoma, B-Cell/complications/drug therapy/metabolism/*pathology
MH  - Male
MH  - Methotrexate/therapeutic use
MH  - Ocular Motility Disorders/etiology
MH  - Paraproteinemias/complications/drug therapy/metabolism/*pathology
MH  - Seizures/drug therapy/etiology/pathology
MH  - Treatment Outcome
MH  - Unconsciousness/etiology/pathology
PMC - PMC8095819
EDAT- 2006/06/14 09:00
MHDA- 2006/09/22 09:00
PMCR- 2006/06/08
CRDT- 2006/06/14 09:00
PHST- 2006/06/14 09:00 [pubmed]
PHST- 2006/09/22 09:00 [medline]
PHST- 2006/06/14 09:00 [entrez]
PHST- 2006/06/08 00:00 [pmc-release]
AID - BPA003_3 [pii]
AID - 10.1111/j.1750-3639.2006.00003_3.x [doi]
PST - ppublish
SO  - Brain Pathol. 2006 Apr;16(2):185-6, 187. doi: 10.1111/j.1750-3639.2006.00003_3.x.