Histopathological analysis of skeletal muscle in patients with Parkinson's disease and ‘dropped head’/‘bent spine’ syndrome

Gdynia, Hans-Jürgen; Sperfeld, Anne-Dorte; Unrath, Alexander; Ludolph, Albert C.; Sabolek, Michael; Storch, Alexander; Kassubek, Jan

doi:10.1016/j.parkreldis.2009.06.003

« Previous Next »Parkinsonism & Related Disorders
Volume 15, Issue 9 , Pages 633-639, 5 November 2009

Histopathological analysis of skeletal muscle in patients with Parkinson's disease and ‘dropped head’/‘bent spine’ syndrome

Hans-Jürgen Gdynia
- University of Ulm, Dept. of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany
Anne-Dorte Sperfeld
- University of Ulm, Dept. of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany
- Corresponding author. Dept. of Neurology, HELIOS Klinikum Bad Saarow Pieskower Straße 33, 15526 Bad Saarow, Germany. Tel.: +49 33631 7 3882; fax: +49 33631 7 3095.
Alexander Unrath
- University of Ulm, Dept. of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany
Albert C. Ludolph
- University of Ulm, Dept. of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany
Michael Sabolek
- EMA-University of Greifswald, Dept. of Neurology, Greifswald, Germany
Alexander Storch
- Dresden University of Technology, Department of Neurology, Dresden, Germany
Jan Kassubek
- University of Ulm, Dept. of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany
- Corresponding author. Tel.: +49 731 1771206; fax: +49 731 1771202.

Received 9 April 2009; received in revised form 4 June 2009; accepted 5 June 2009.

Abstract

Background

‘Dropped head’ and ‘bent spine’ phenomena are recognized clinical presentations of neuromuscular disorders. Similar symptoms are known in patients with parkinsonian syndromes, but their pathophysiology remains unclear. One hypothesis is a relation between the movement disorder and the skeletal muscle pathology.

Methods

We describe detailed histopathological data from 19 consecutive skeletal muscle biopsies in patients with idiopathic Parkinson's disease (PD) and concomitant ‘dropped head’ or ‘bent spine’ syndrome. A biochemical analysis of the respiratory chain complexes was also performed, and clinical, electrophysiological, and imaging data were analyzed.

Results

The subjects developed neuromuscular symptoms 2.7 ± 2.4 years after onset of PD. We found no correlation with the age at onset of the disease, disease duration, or severity. We found no evidence for dystonia nor did we find any relationship between their anti-parkinsonian medication, and possible drug side effects. Muscle biopsies were abnormal in all patients. Based on histopathological criteria we divided the muscle pathology into three different groups, i.e. necrotizing myopathy, inflammatory myopathy, and myopathy with mitochondrial abnormalities. Biochemical analysis of respiratory chain complexes revealed abnormalities in nine patients.

Conclusions

‘Dropped head’ and ‘bent spine’ symptoms in association with PD appear to be accompanied by a wide spectrum of histopathological abnormalities in skeletal muscle. A muscle biopsy should be performed to identify potentially treatable conditions (i.e. inflammatory myopathies).

Keywords: Bent spine syndrome, Dropped head syndrome, Mitochondriopathy, Parkinsonism

Abbreviations: BSS, Bent spine syndrome, CS, Citrate synthase, DHS, Dropped head syndrome, EMG, Electromyography, H&E, Hematoxylin–eosin, PD, Parkinson's disease, MRI, Magnetic resonance imaging