PMID- 16511650
OWN - NLM
STAT- MEDLINE
DCOM- 20061212
LR  - 20181113
IS  - 0340-5354 (Print)
IS  - 0340-5354 (Linking)
VI  - 253
IP  - 6
DP  - 2006 Jun
TI  - Distinct neuromuscular phenotypes in myotonic dystrophy types 1 and 2 : a whole 
      body highfield MRI study.
PG  - 753-61
AB  - Myotonic Dystrophy Type 1 (DM1) and 2 (DM2) present with distinct though 
      overlapping clinical phenotypes. Comparative imaging data on skeletal muscle 
      involvement are not at present available. We used the novel technique of whole 
      body 3.0 Tesla (T) Magnetic Resonance Imaging (MRI) to further characterize 
      musculoskeletal features in DM2 and compared the results with DM1.MRI findings of 
      15 DM1 and 14 DM2 patients were evaluated with respect to patterns of skeletal 
      muscle affection and clinical data using the Muscular Impairment Rating Scale 
      (MIRS) and Medical Research Council scale (MRC). All DM1 patients had 
      pathological MRI compared with only 5 DM2 patients. In contrast to DM2, DM1 
      patients showed a characteristic distribution of muscle involvement with frequent 
      and early degeneration of the medial heads of gastrocnemius muscles, and a 
      perifemoral semilunar pattern of quadriceps muscle affection sparing the rectus 
      femoris. The most frequently affected muscles in DM1 were the medial heads of 
      gastrocnemius, soleus, and vastus medialis muscles. In DM2, however, the erector 
      spinae and gluteus maximus muscles were most vulnerable to degeneration. MRI data 
      were in line with the clinical grading in 12 DM1 and 3 DM2 patients. In 3 DM1 and 
      5 DM2 patients, MRI detected subclinical muscle involvement. 9 DM2 patients with 
      mild to moderate proximal muscle weakness and/or myalgias had normal MRI. 
      Pathological MRI changes in DM2 emerged with increasing age and were restricted 
      to women. Whole body 3.0T MRI is a sensitive imaging technique that demonstrated 
      a characteristic skeletal muscle affection in DM1. In contrast, MRI was no 
      reliable indicator for skeletal muscle involvement in mildly affected DM2 
      patients since myalgia and mild paresis were usually not reflected by MRI signal 
      alterations.
FAU - Kornblum, Cornelia
AU  - Kornblum C
AD  - Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 
      Bonn, Germany. cornelia.kornblum@ukb.uni-bonn.de
FAU - Lutterbey, Gotz
AU  - Lutterbey G
FAU - Bogdanow, Manuela
AU  - Bogdanow M
FAU - Kesper, Kristina
AU  - Kesper K
FAU - Schild, Hans
AU  - Schild H
FAU - Schroder, Rolf
AU  - Schroder R
FAU - Wattjes, Mike Peter
AU  - Wattjes MP
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20060306
PL  - Germany
TA  - J Neurol
JT  - Journal of neurology
JID - 0423161
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - Muscle, Skeletal/*pathology
MH  - *Myotonic Dystrophy/classification/pathology/physiopathology
MH  - Phenotype
MH  - Severity of Illness Index
MH  - Whole Body Imaging/methods
EDAT- 2006/03/03 09:00
MHDA- 2006/12/13 09:00
CRDT- 2006/03/03 09:00
PHST- 2005/08/08 00:00 [received]
PHST- 2005/12/15 00:00 [accepted]
PHST- 2005/11/25 00:00 [revised]
PHST- 2006/03/03 09:00 [pubmed]
PHST- 2006/12/13 09:00 [medline]
PHST- 2006/03/03 09:00 [entrez]
AID - 10.1007/s00415-006-0111-5 [doi]
PST - ppublish
SO  - J Neurol. 2006 Jun;253(6):753-61. doi: 10.1007/s00415-006-0111-5. Epub 2006 Mar 
      6.