PMID- 28669250
OWN - NLM
STAT- MEDLINE
DCOM- 20180627
LR  - 20180627
IS  - 1940-2465 (Electronic)
IS  - 1066-8969 (Linking)
VI  - 25
IP  - 8
DP  - 2017 Dec
TI  - Primary CNS Nonamyloidogenic Light Chain Deposition Disease: Case Report and 
      Brief Review.
PG  - 755-760
LID - 10.1177/1066896917717338 [doi]
AB  - The true incidence of light chain deposition disease (LCDD) restricted to the 
      central nervous system (CNS) is unknown. To our knowledge only 7 cases of LCDD 
      restricted to the brain have been previously reported. We herein describe an 
      unusual example. A 44-year-old man presented with a history of ischemic 
      retinopathy in 2004 and left lower extremity hypoesthesia in 2007 that progressed 
      gradually to left-sided weakness and numbness in the 2 years prior to his 
      hospitalization in 2015. A stereotactic brain biopsy was performed, displaying 
      nonspecific hyaline deposits of amorphous "amyloid-like" material involving deep 
      brain white matter and vessels. These were Congo red negative and were 
      accompanied by a sparse lymphoplasmacytic infiltrate. Plasma cells demonstrated 
      kappa light chain class restriction by chromogenic in situ hybridization (CISH). 
      There was patchy reactivity with kappa immunohistochemistry in the amorphous 
      deposits. A diagnosis of light chain deposition disease was made. Subsequent 
      systemic myeloma and lymphoma workups were negative. Previously reported cases 
      have included men and women, spanning the ages of 19 and 72 years, often 
      presenting with hemiparesis, hypoesthesia, or seizures. Deposits have been 
      reported in the cerebrum and cerebellum. T2/FLAIR (fluid attenuation inversion 
      recovery) changes are usual, but lesions may or may not produce contrast 
      enhancement. The light chain deposition may be of kappa or lambda class. Most 
      lesions have been accompanied by local lymphoid and/or plasma cell infiltrates 
      exhibiting light chain restriction of the same class as the deposits. In summary, 
      LCDD limited to the CNS is a rare lesion consisting of deposition of 
      amyloid-like, but Congo red-negative monotypic light chain usually produced by 
      local lymphoplasmacytic infiltrates.
FAU - Mercado, Juan Jose
AU  - Mercado JJ
AD  - 1 University of Alabama, Birmingham, AL, USA.
FAU - Markert, James M
AU  - Markert JM
AD  - 1 University of Alabama, Birmingham, AL, USA.
FAU - Meador, William
AU  - Meador W
AD  - 1 University of Alabama, Birmingham, AL, USA.
FAU - Chapman, Philip
AU  - Chapman P
AD  - 1 University of Alabama, Birmingham, AL, USA.
FAU - Perry, Arie
AU  - Perry A
AD  - 2 University of California, San Francisco, CA, USA.
FAU - Hackney, James R
AU  - Hackney JR
AD  - 1 University of Alabama, Birmingham, AL, USA.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20170702
PL  - United States
TA  - Int J Surg Pathol
JT  - International journal of surgical pathology
JID - 9314927
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Brain Diseases/*immunology/pathology
MH  - Humans
MH  - Immune System Diseases/*immunology/pathology
MH  - *Immunoglobulin Light Chains
MH  - Male
OTO - NOTNLM
OT  - LCDD restricted to the brain
OT  - light chain deposition disease
EDAT- 2017/07/04 06:00
MHDA- 2018/06/28 06:00
CRDT- 2017/07/04 06:00
PHST- 2017/07/04 06:00 [pubmed]
PHST- 2018/06/28 06:00 [medline]
PHST- 2017/07/04 06:00 [entrez]
AID - 10.1177/1066896917717338 [doi]
PST - ppublish
SO  - Int J Surg Pathol. 2017 Dec;25(8):755-760. doi: 10.1177/1066896917717338. Epub 
      2017 Jul 2.