The dropped head syndrome resulting from neck extensor myopathy seems to be a heterogeneic condition with different causes. In most reported cases an unspecific, restricted, noninflammatory myopathy has been found,2,3 but there are also reports about inclusion body myositis,13 focal myositis,14 congenital myopathy,15 nemaline myopathy,16 and hyperparathyroidism17 presenting with severe neck weakness. The origin of the myopathy in our patients is unknown. It has been suggested that mechanical stretching produces the injury in isolated neck extensor myopathy.3 Kyphotic postural changes and loss of tissue elastic associated with aging18 may place increasing workloads on cervical spinal muscles that leave some individuals susceptible to injury. Patients with parkinsonism, especially those with MSA, may be more vulnerable because of their posture. If this hypothesis would be correct, it is, however, difficult to explain why the neck weakness may precede the parkinsonism by years, as in our patients 4 and 5. All 7 patients described herein had autonomic dysfunction and 6 responded poorly to levodopa therapy, making a diagnosis of MSA probable.11 It cannot be excluded that higher levodopa doses might have induced a better response, but because of orthostatic hypotension and psychiatric side effects, it was impossible to increase the doses further. "Disproportionate antecollis" has been reported to be much more frequent in MSA than in Parkinson disease.6 It is possible that a neck extensor myopathy may be overlooked in a patient with pronounced rigidity in the neck. Yoshiyama et al9 reported that the muscle strength in the neck was almost normal in all of their patients who had parkinsonism and the dropped head sign, but conventional needle EMG was only performed in 2 of their 7 patients. Our patients 2, 5, 6, and 7 illustrate that pronounced rigidity and myopathy in the neck can occur in the same patient. In patient 2, the neck rigidity increased over time to such an extent that at the latest physicial examination it was impossible to assess the patient's muscle strength. It is essential to differentiate neck muscle weakness from hyperactive neck flexors. In our patient there were, however, no signs of hyperactivity of the neck flexors whether on palpation or on the needle EMG. As the biopsy findings confirmed myopathy in all 5 cases in which biopsies were performed, we do not think that we have overdiagnosed myopathy on needle EMG. For most limb muscles, as well as facial muscles, reference values for motor unit potential size have been collected. However, for paraspinal and neck muscles, reference studies are difficult for practical and ethical reasons. Therefore, needle EMG findings from these muscles are not evaluated as myopathic unless they are convincing enough. Around 20 motor unit potentials have been collected from each examined muscle to participate in analysis of amplitude, duration, and the number of phases. At maximal voluntary activation of a muscle a dense interference pattern was obtained in congruence with myopathy. No denervation activity was seen in any of our patients, indicating that the myopathies found had had a longer course.