PMID- 19101700
OWN - NLM
STAT- MEDLINE
DCOM- 20090504
LR  - 20230815
IS  - 0022-510X (Print)
IS  - 0022-510X (Linking)
VI  - 278
IP  - 1-2
DP  - 2009 Mar 15
TI  - Inflammatory myopathies with mitochondrial pathology and protein aggregates.
PG  - 25-9
LID - 10.1016/j.jns.2008.11.010 [doi]
AB  - OBJECTIVES: To compare the clinical course and muscle biopsy features of 
      polymyositis with mitochondrial pathology (PM-Mito) to inclusion body myositis 
      (IBM) and steroid-responsive inflammatory myopathies (polymyositis). METHODS: We 
      compared clinical, laboratory and myopathologic features in a retrospective study 
      of patients with PM-Mito (23), IBM (26) and polymyositis (12). RESULTS: Selective 
      weakness in the quadriceps or finger flexors was common in PM-Mito (62%) and IBM 
      (87%). Weakness progressed more slowly in PM-Mito than in IBM. PM-Mito patients 
      with more rapidly progressive weakness had more cytochrome oxidase negative 
      muscle fibers. There was no history of benefit from corticosteroid treatment in 
      any PM-Mito or IBM patients. B-cell foci were absent in IBM and PM-Mito. LC3, an 
      autophagy marker, and alphaB-crystallin were common in aggregates in PM-Mito and 
      IBM, but not polymyositis. SMI-31 and TDP-43 positive aggregates were common in 
      IBM but not in PM-Mito or polymyositis. beta-amyloid showed no differences in 
      aggregates among the three groups. CONCLUSIONS: PM-Mito and IBM may be part of 
      the same disease spectrum. PM-Mito has more slowly progressive weakness than IBM 
      and rarely has TDP-43 or SMI-31 staining aggregates in muscle fibers. The most 
      frequent proteins in aggregates in both PM-Mito and IBM are LC3, an autophagy 
      marker, and alphaB-crystallin. Alterations in autophagic degradation pathways may 
      be a common pathogenic mechanism in PM-Mito and IBM. In pathologically typical 
      polymyositis, staining for mitochondrial enzyme activity, aggregates and B-cells 
      helps to distinguish PM-Mito from inflammatory myopathy syndromes that are more 
      likely to respond to corticosteroid treatment.
FAU - Temiz, Peyker
AU  - Temiz P
AD  - Celal Bayar University School of Medicine, Department of Pathology, Manisa, 
      45010, Turkey.
FAU - Weihl, Conrad C
AU  - Weihl CC
FAU - Pestronk, Alan
AU  - Pestronk A
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20081220
PL  - Netherlands
TA  - J Neurol Sci
JT  - Journal of the neurological sciences
JID - 0375403
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Crystallins)
RN  - 0 (MAP1LC3A protein, human)
RN  - 0 (Microtubule-Associated Proteins)
RN  - EC 1.9.3.1 (Electron Transport Complex IV)
SB  - IM
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Autophagy
MH  - B-Lymphocytes/pathology
MH  - Biopsy
MH  - Crystallins/metabolism
MH  - Disease Progression
MH  - Electron Transport Complex IV/metabolism
MH  - Humans
MH  - Inclusion Bodies/pathology
MH  - Microtubule-Associated Proteins/metabolism
MH  - Mitochondria, Muscle/*pathology
MH  - Muscle Weakness
MH  - Muscle, Skeletal/pathology/physiopathology
MH  - Myositis/drug therapy/*pathology/*physiopathology
MH  - Retrospective Studies
EDAT- 2008/12/23 09:00
MHDA- 2009/05/05 09:00
CRDT- 2008/12/23 09:00
PHST- 2008/07/16 00:00 [received]
PHST- 2008/11/04 00:00 [revised]
PHST- 2008/11/07 00:00 [accepted]
PHST- 2008/12/23 09:00 [entrez]
PHST- 2008/12/23 09:00 [pubmed]
PHST- 2009/05/05 09:00 [medline]
AID - S0022-510X(08)00566-2 [pii]
AID - 10.1016/j.jns.2008.11.010 [doi]
PST - ppublish
SO  - J Neurol Sci. 2009 Mar 15;278(1-2):25-9. doi: 10.1016/j.jns.2008.11.010. Epub 
      2008 Dec 20.