Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach

Simone Spuler*, Henriette Krug, Christine Klein, Isabel Chaure Medialdea, Wibke Jakob, Georg Ebersbach, Doreen Gruber, Karl Titus Hoffmann, Thomas Trottenberg, Andreas Kupsch

*Corresponding author for this work
51 Citations (Scopus)


Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD-associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13-14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC.

Original languageEnglish
JournalMovement Disorders
Issue number5
Pages (from-to)552-599
Number of pages48
Publication statusPublished - 15.04.2010


Dive into the research topics of 'Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach'. Together they form a unique fingerprint.

Cite this