Muscle pain in myophosphorylase deficiency (McArdle's disease): the role of gender, genotype, and pain-related coping.
Pain characteristics were examined in 24 patients with myophosphorylase deficiency (McArdle's disease). Pain parameters were related to mutation analyses as well as psychosocial data using a pain questionnaire including an assessment of psychosocial distress and coping measures (Beck Depression Inventory BDI; Kiel Pain Inventory KPI, Multidimensional Fatique Inventory MFI). Twenty-three patients complained of pain, which was intermittent and exercise-induced in 15 patients. Eight patients complained of permanent pain, which was superimposed by exercise-induced pain in 7 patients. Patients reported 3-7 different pain characters and various localisations. Patients with permanent pain were significantly more frequently female, experienced higher impact on general activities and sleep as well as higher scores on the MFI. Furthermore, these patients revealed higher scores regarding several psychosocial risk factors including avoidance behavior whereas patients with intermittent pain predominantly showed endurance coping. There was no correlation between age or disease duration, pain intensity as well as mutation type and development of permanent or intermittent pain. In addition, severity of the clinical phenotype did not correlate with ACE polymorphism. Although McArdle's disease is a muscle glycogenosis with marked biochemical homogeneity, the clinical presentation can be quite heterogeneous. A substantial number of patients revealed permanent pain as a major clinical symptom. As permanent pain is not related to age or disease duration, it might be a clinically important subgroup of McArdle's disease. Gender-related genetic factors as well as maladaptive pain-related coping may contribute to the development of such a chronic pain symptom.
Rommel
Oliver
O
Department of Neurology, Neuromuscular Center Ruhrgebiet, Kliniken Bergmannsheil, Ruhr-University, Bochum, Germany Department of Human Genetics, Ruhr-University, Bochum, Germany Department of Medical Psychology and Medical Sociology, Ruhr-University, Bochum, Germany Department of Neurology and Pain Therapy, Rommel-Klinik GMBH, Bad Wildbad, Germany.
Kley
Rudolf A
RA
Dekomien
Gabriele
G
Epplen
Jörg T
JT
Vorgerd
Matthias
M
Hasenbring
Monika
M
D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000342:Affective Symptoms; D000368:Aged; D000369:Aged, 80 and over; D015444:Exercise; D005260:Female; D005783:Gender Identity; D005838:Genotype; D024982:Glycogen Phosphorylase, Muscle Form / Q000172:deficiency / Q000235:genetics; D006012:Glycogen Storage Disease Type V / Q000453:epidemiology / Q000235:genetics* / Q000523:psychology*; D015438:Health Behavior; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000378:metabolism; D010146:Pain / Q000453:epidemiology / Q000235:genetics* / Q000523:psychology*; D010147:Pain Measurement; D010641:Phenotype; D012307:Risk Factors; D012720:Severity of Illness Index; D017678:Sex Distribution
D024982:Glycogen Phosphorylase, Muscle Form