Cardiac involvement in patients with a sarcoglycanopathy (limb-girdle muscular dystrophy) has been described previously; however, this is the first cardiovascular magnetic resonance (CMR) study in such a patient demonstrating an interesting pattern of myocardial damage using late gadolinium enhancement (LGE) imaging. Moreover, the wall motion abnormality and the subepicardial pattern of LGE in this patient with a sarcoglycanopathy is in agreement with the findings in another patient with Becker muscular dystrophy. The predominance of LGE in the subepicardial layers of the left ventricular inferolateral wall suggests that such a myocardial damage pattern represents a nonspecific cardiac phenotype in response to exaggerated mechanical stress in this region, at least in patients with a sarcoglycanopathy or dystrophinopathy.
Camptocormia (CC) is defined as a trunkal flexion which worsens while standing, sitting and walking and disappears in the supine position. CC is a well-known clinical phenomenon in patients with generalized neuromuscular disorders like polymyositis, myasthenia gravis or motor neuron diseases, and it is also described in the context of movement disorders like Parkinson’s disease (PD) or multiple system atrophy. In association with PD, CC seems to be a rare symptom which occurs preferentially in late stages of the disease. Currently, there are 3 main hypotheses on the pathogenesis of CC in PD, i.e. a focal myopathy of the trunk muscles, axial dystonia and a drug-induced etiology. This review gives a synopsis and critical acclaim of these 3 etiologies and refers to the current study data and possible treatment strategies.
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