PMID- 22499107
OWN - NLM
STAT- MEDLINE
DCOM- 20120604
LR  - 20211021
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Print)
IS  - 0148-639X (Linking)
VI  - 45
IP  - 5
DP  - 2012 May
TI  - Expanding the spectrum of monoclonal light chain deposition disease in muscle.
PG  - 755-61
LID - 10.1002/mus.23287 [doi]
AB  - INTRODUCTION: The diagnosis of amyloid myopathy is delayed when monoclonal 
      gammopathies are not detected on initial testing and muscle biopsies are 
      nondiagnostic, and the EMG and symptoms can mimic an inflammatory myopathy. 
      METHODS: Case report of a patient presenting with severe progressive muscle 
      weakness of unclear etiology despite an extensive workup including two 
      nondiagnostic muscle biopsies. RESULTS: Directed by MRI, a third biopsy revealed 
      amyloid angiopathy and noncongophilic kappa light chain deposition in scattered 
      subsarcolemmal rings and perimysial regions. A serum free light chain (FLC) assay 
      revealed a kappa monoclonal gammopathy, which was not detected by multiple 
      immunofixations. CONCLUSIONS: The spectrum of immunoglobulin deposition in muscle 
      is similar to other organs. It comprises a continuum that includes parenchymal 
      amyloid deposition, amyloid angiopathy, and noncongophilic Light Chain Deposition 
      Disease (LCDD). We recommend including the FLC assay in the routine investigation 
      for monoclonal gammopathies. This case also highlights the value of MRI-guided 
      muscle biopsy.
CI  - Copyright (c) 2012 Wiley Periodicals, Inc.
FAU - Ostrow, Lyle W
AU  - Ostrow LW
AD  - Department of Neurology, Johns Hopkins School of Medicine, 733 N. Broadway, 
      Baltimore, Maryland 21205, USA.
FAU - Corse, Andrea M
AU  - Corse AM
FAU - Morrison, Brett M
AU  - Morrison BM
FAU - Huff, Carol A
AU  - Huff CA
FAU - Carrino, John A
AU  - Carrino JA
FAU - Hoke, Ahmet
AU  - Hoke A
FAU - Mammen, Andrew L
AU  - Mammen AL
LA  - eng
GR  - P30 CA006973/CA/NCI NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin kappa-Chains)
RN  - 3U05FHG59S (Congo Red)
RN  - EC 3.6.1.- (Adenosine Triphosphatases)
SB  - IM
MH  - Adenosine Triphosphatases/metabolism
MH  - Aged
MH  - Amyloidosis/blood/etiology
MH  - Biopsy
MH  - Congo Red
MH  - Humans
MH  - Immunoglobulin Light Chains/*blood
MH  - Immunoglobulin kappa-Chains/metabolism
MH  - Male
MH  - Muscle, Skeletal/*metabolism/pathology
MH  - Paraproteinemias/*blood/complications/*pathology
PMC - PMC4133774
MID - NIHMS442579
EDAT- 2012/04/14 06:00
MHDA- 2012/06/05 06:00
PMCR- 2014/08/15
CRDT- 2012/04/14 06:00
PHST- 2012/04/14 06:00 [entrez]
PHST- 2012/04/14 06:00 [pubmed]
PHST- 2012/06/05 06:00 [medline]
PHST- 2014/08/15 00:00 [pmc-release]
AID - 10.1002/mus.23287 [doi]
PST - ppublish
SO  - Muscle Nerve. 2012 May;45(5):755-61. doi: 10.1002/mus.23287.