Parkinsonism and Neck Extensor Myopathy

Abstract

Parkinsonism may be associated with isolated neck extensor myopathy resulting in dropped head, and this condition should be suggestive of multiple system atrophy.

Myofibrillar disorganization characterizes myopathy of camptocormia in Parkinson’s disease

“…Only Wharton et al [43] investigated cervical paraspinal muscles in 23 consecutive necropsies and found ragged red fibers in 60–70%, and also core-targetoid fibers in some patients over 60 years of age as well as mild focal endomysial chronic inflammation in 13%. Although it is known that the number of ragged red fibers increases with age [28], we and others [4] found a higher number of ragged red fibers in paraspinal muscles than in the limb muscles usually biopsied. We speculate that this physiologically high number of ragged red fibers in paraspinal muscles is the reason why the diagnosis of a mitochondriopathy was made in several camptocormia patients [17, 34, 46].…”

Myofibrillar disorganization characterizes myopathy of camptocormia in Parkinson’s disease

“…Only Wharton et al [43] investigated cervical paraspinal muscles in 23 consecutive necropsies and found ragged red fibers in 60–70%, and also core-targetoid fibers in some patients over 60 years of age as well as mild focal endomysial chronic inflammation in 13%. Although it is known that the number of ragged red fibers increases with age [28], we and others [4] found a higher number of ragged red fibers in paraspinal muscles than in the limb muscles usually biopsied. We speculate that this physiologically high number of ragged red fibers in paraspinal muscles is the reason why the diagnosis of a mitochondriopathy was made in several camptocormia patients [17, 34, 46].…”

Dropped Head Syndrome Preceding the Onset of Dementia with Lewy Bodies

“…Therefore, although there have been no reports of similar patients, we assumed that the pre-ceding dropped head syndrome was associated with DLB in our patient. In contrast, focal neck extensor myopathy has been proposed as another mechanism of dropped head syndrome in patients with PD (10,11). However, myopathy may be ruled out in this case because the patient did not exhibit neck muscle weakness.…”

An 18F-FDG PET study of cervical muscle in parkinsonian anterocollis

“…The median modified Hoehn and Yahr scale was 3 [1–3] for PD and 4 for both MSA patients. The median duration of primary diagnosis from onset of symptoms to the date of the study evaluation was 10 years [5–13] for PD and 6 years [4–8] for MSA. The median duration of anterocollis from onset of symptoms to the study evaluation was 3.5 years [2–5] for MSA and 2 (0.2–13) years for PD.…”

Myofibrillar disorganization characterizes myopathy of camptocormia in Parkinson’s disease

“…Only Wharton et al [43] investigated cervical paraspinal muscles in 23 consecutive necropsies and found ragged red fibers in 60–70%, and also core-targetoid fibers in some patients over 60 years of age as well as mild focal endomysial chronic inflammation in 13%. Although it is known that the number of ragged red fibers increases with age [28], we and others [4] found a higher number of ragged red fibers in paraspinal muscles than in the limb muscles usually biopsied. We speculate that this physiologically high number of ragged red fibers in paraspinal muscles is the reason why the diagnosis of a mitochondriopathy was made in several camptocormia patients [17, 34, 46].…”

Dropped Head Syndrome Preceding the Onset of Dementia with Lewy Bodies

“…Therefore, although there have been no reports of similar patients, we assumed that the pre-ceding dropped head syndrome was associated with DLB in our patient. In contrast, focal neck extensor myopathy has been proposed as another mechanism of dropped head syndrome in patients with PD (10,11). However, myopathy may be ruled out in this case because the patient did not exhibit neck muscle weakness.…”

An 18F-FDG PET study of cervical muscle in parkinsonian anterocollis

“…The median modified Hoehn and Yahr scale was 3 [1–3] for PD and 4 for both MSA patients. The median duration of primary diagnosis from onset of symptoms to the date of the study evaluation was 10 years [5–13] for PD and 6 years [4–8] for MSA. The median duration of anterocollis from onset of symptoms to the study evaluation was 3.5 years [2–5] for MSA and 2 (0.2–13) years for PD.…”

Myofibrillar disorganization characterizes myopathy of camptocormia in Parkinson’s disease

“…Only Wharton et al [43] investigated cervical paraspinal muscles in 23 consecutive necropsies and found ragged red fibers in 60–70%, and also core-targetoid fibers in some patients over 60 years of age as well as mild focal endomysial chronic inflammation in 13%. Although it is known that the number of ragged red fibers increases with age [28], we and others [4] found a higher number of ragged red fibers in paraspinal muscles than in the limb muscles usually biopsied. We speculate that this physiologically high number of ragged red fibers in paraspinal muscles is the reason why the diagnosis of a mitochondriopathy was made in several camptocormia patients [17, 34, 46].…”

Dropped Head Syndrome Preceding the Onset of Dementia with Lewy Bodies

“…Therefore, although there have been no reports of similar patients, we assumed that the pre-ceding dropped head syndrome was associated with DLB in our patient. In contrast, focal neck extensor myopathy has been proposed as another mechanism of dropped head syndrome in patients with PD (10,11). However, myopathy may be ruled out in this case because the patient did not exhibit neck muscle weakness.…”

An 18F-FDG PET study of cervical muscle in parkinsonian anterocollis

“…The median modified Hoehn and Yahr scale was 3 [1–3] for PD and 4 for both MSA patients. The median duration of primary diagnosis from onset of symptoms to the date of the study evaluation was 10 years [5–13] for PD and 6 years [4–8] for MSA. The median duration of anterocollis from onset of symptoms to the study evaluation was 3.5 years [2–5] for MSA and 2 (0.2–13) years for PD.…”