1/16. Diffuse lewy body disease presenting as multiple system atrophy.OBJECTIVES: The majority of patients with diffuse lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities. methods: Case report based on a retrospective chart review and neuropathological examination. RESULTS: We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord. CONCLUSIONS: In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.- - - - - - - - - - ranking = 1keywords = rigidity (Clic here for more details about this article) |
2/16. The dropped head sign in parkinsonism.We describe seven patients who exhibited the dropped head sign in parkinsonism. These included six females and one male between the ages of 53 and 74. Three patients were clinically diagnosed as probable Parkinson's disease and four were diagnosed with probable multiple system atrophy. None had weakness in the posterior neck muscles or spasms in the anterior neck muscles. When the patients attempted to extend the head voluntarily or passively muscle contraction that was not seen in the dropped-head condition appeared. Surface electromyography of the neck indicated that the anterior neck muscles had rigidity. A gamma-block of the SCM muscles reduced the muscle activity when the head was elevated and improved the dropped-head condition slightly. These findings seem to indicate that the dropped head sign in parkinsonism could be associated with anterior neck muscle rigidity. Although the severity of the dropped head condition was affected by medication or by the clinical course in three patients, there was no clear relationship between the severity of the dropped head condition and the parkinsonism. We suspected that unbalanced muscle rigidity between the anterior and the posterior neck muscles could cause the dropped head sign.- - - - - - - - - - ranking = 1.502513632137keywords = rigidity, muscle (Clic here for more details about this article) |
3/16. Postural and action myoclonus in patients with parkinsonian type multiple system atrophy.patients with a parkinsonian syndrome and features of multisystem atrophy (pMSA) may exhibit abnormal movements of the hands and fingers, which are reported in the literature either as "jerky" tremor or myoclonus. We studied clinically and electrophysiologically these movements in 11 consecutive patients with pMSA. No abnormal movements were observed when the patients were at complete rest, except for a characteristic parkinsonian "pill-rolling" tremor in one patient. Abnormal small-amplitude, nonrhythmic movements involving just one or a few fingers, or more rarely the whole hand, were observed in nine patients when holding a posture or at the beginning of an action. Accelerometric recordings showed small-amplitude irregular oscillations which, contrary to those of patients with tremor, had no predominant peak in the Fast Fourier frequency spectrum analysis. Electromyographic recordings in the forearm and hand muscles showed brief jerks of less than 100 ms duration which were synchronous in antagonist muscles of the forearm and alternated with brief periods of silence. Electrical stimulation of the digital nerves evoked consistent reflex responses in the wrist flexor and extensor muscles at a latency of 55.3 /-4.1 ms (range, 50-63 ms). Routine electroencephalographic (EEG) and somatosensory evoked potentials to median nerve stimulation were normal. back-averaging of the EEG activity time-locked to the jerks was performed in two patients with no evidence of abnormal cortical activity. Two patients had episodes of transient respiratory failure related to pneumonia. This caused a long-lasting enhancement of the abnormal hand and finger movements, which became larger and more widespread, with features of posthypoxic myoclonus. We conclude that the abnormal hand and finger movements of patients with pMSA are a form of postural and action myoclonus, and can be described as mini-polymyoclonus.- - - - - - - - - - ranking = 0.00083787737898553keywords = muscle (Clic here for more details about this article) |
4/16. electroconvulsive therapy for elderly patients with multiple system atrophy: a case series.multiple system atrophy (MSA) is a progressive neurological illness associated with parkinsonism. electroconvulsive therapy (ECT) improves motor function in Parkinson's disease and, thus, might be beneficial in MSA. Three cases of MSA treated with ECT are described. All patients improved neurologically, but none regained independent ambulation. A review, including previously reported cases, demonstrates that ECT can be safe and effective for depression associated with MSA. Reduced tremor and rigidity may occur, but substantial gait improvement cannot be expected.- - - - - - - - - - ranking = 0.5keywords = rigidity (Clic here for more details about this article) |
5/16. Morphometric study of nucleus ambiguus in multiple system atrophy presenting with vocal cord abductor paralysis.AIM: To identify lesions responsible for vocal cord abductor paralysis (VCAP) in multiple system atrophy (MSA), we performed a morphometric study of the nucleus ambiguus which innervates the intrinsic laryngeal muscles. methods: Two autopsied cases of MSA presenting with VCAP and one control were examined. Both cases of MSA showed selective neurogenic atrophy of the posterior cricoarytenoid muscles among the intrinsic laryngeal muscles, while no abnormalities were seen in the control. From a block of the medulla oblongata, sections 10 microm thickness were cut serially without spacing and stained with cresyl violet. The ambiguus neurons were counted in all the sections to make a histogram. RESULTS: In the control case, ambiguus neurons showed densely populated areas and sparsely populated areas alternately with significant difference in the mean neuronal density between two areas. In MSA, ambiguus neurons were significantly decreased in number at all levels. It indicates that the neurogenic atrophy of the posterior cricoarytenoid muscle is derived from the neuronal loss of the nucleus ambiguus. CONCLUSION: Though it has still been controversial whether or not the ambiguus neurons are decreased in number in MSA with VCAP, we speculated possible reasons for the disagreement on the involvement of the nucleus ambiguus as follows: different mechanism of VCAP are playing role, and histometric data have been disturbed by factors such as split-cell counting error and marked variation in the distribution of the ambiguus neurons.- - - - - - - - - - ranking = 0.0011171698386474keywords = muscle (Clic here for more details about this article) |
6/16. Parkinsonism and neck extensor myopathy: a new syndrome or coincidental findings?BACKGROUND: Dropped head in parkinsonism has been attributed to dystonia or unbalanced muscle rigidity. To our knowledge, isolated neck extensor myopathy with parkinsonism has been described in only one patient. OBJECTIVES: To assess the occurrence of neck extension weakness resulting in dropped head in patients with parkinsonism and to explore whether the head drop might be the consequence of neck extensor myopathy. patients AND methods: All patients who were evaluated because of parkinsonism in the Department of neurology in our hospital between January 1, 1997, and December 31, 1999, and were found to have both parkinsonism and neck extension weakness resulting in head drop were studied. The patients underwent clinical examination, blood tests including the levels of creatine kinase and myoglobin and neurophysiological evaluation with needle electromyography and autonomic tests. Open biopsy on a neck muscle was performed in the patients who could cooperate. RESULTS: Of 459 patients evaluated because of parkinsonism, 7 were found to have neck extensor weakness resulting in head drop. Needle electromyography revealed myopathic changes in all 7 patients. Muscle biopsy, which was performed in 5 patients, disclosed myopathic changes in all 5 patients. Electron microscopy revealed mitochondrial abnormalities in 2 of these 7 patients. Three of the patients had concomitant neck rigidity that could contribute to the neck position. All 7 patients had autonomic dysfunction and 6 responded poorly to levodopa therapy, making a diagnosis of multiple system atrophy probable. CONCLUSION: Parkinsonism may be associated with isolated neck extensor myopathy resulting in dropped head, and this condition should be suggestive of multiple system atrophy.- - - - - - - - - - ranking = 1.0005585849193keywords = rigidity, muscle (Clic here for more details about this article) |
7/16. 5: movement disorders I: parkinsonism and the akinetic-rigid syndromes.The major features of akinetic-rigid syndromes are bradykinesia (small, slow movements), rigidity and tremor, often summarised as "parkinsonism". Approximately 80% of akinetic-rigid syndromes are due to Parkinson's disease. Treatment of Parkinson's disease should be determined by level of disability and handicap. The combination of levodopa and peripheral dopadecarboxylase inhibitor is the most efficacious symptomatic treatment for Parkinson's disease. Motor fluctuations appear after 2-3 years of levodopa treatment, and affect at least 50% of patients after five years. Surgery can relieve refractory tremor and dyskinesias, but does not alter disease progression or need for drug therapy.- - - - - - - - - - ranking = 0.5keywords = rigidity (Clic here for more details about this article) |
8/16. Focal myopathy as a cause of anterocollis in Parkinsonism.We present a patient with levodopa-responsive Parkinsonism who developed disproportionate anterocollis over a period of 4 to 5 weeks. A neurological evaluation, including EMG and muscle biopsy, demonstrated the presence of a focal myopathy typical of the so-called "dropped head syndrome" or "isolated neck extensor myopathy." Parkinsonian patients presenting with disproportionate anterocollis should be evaluated for focal myopathy, as this may represent one possible cause.- - - - - - - - - - ranking = 0.00027929245966184keywords = muscle (Clic here for more details about this article) |
9/16. Shift from hypermetria to hypometria in multiple system atrophy: analysis of distal and proximal movements.Dysmetria is a classical sign which designates the overshoot, also called hypermetria, and the undershoot, or hypometria, when the patient attempts to reach rapidly an aimed target. Dysmetria is typically observed in patients presenting a cerebellar dysfunction. Dysmetria of distal movements is associated with an imbalance between the timing and/or the intensity of agonist and antagonist EMG activities. So far, 1. there is only one description in human of a shift from hypermetria to hypometria for fast goal-directed single-joint movements during an aberrant recovery following a cerebellar infarction, and 2. such a shift has not been described for proximal movements. We report a patient presenting a multiple system atrophy (MSA). Initially, he exhibited a marked cerebellar syndrome. Fast wrist flexions and fast upper limb reaches in the sagittal plane were hypermetric. The distal hypermetria was associated with a delayed onset latency of the antagonist EMG activity and reduced intensities of both the agonist and the antagonist EMG activities. The proximal hypermetria was associated with a defect in the phasic spatial tuning of the EMG activities. He developed progressively severe extra-pyramidal signs. Distal hypermetria turned into hypometria, as a result of a decrease in the intensity of the agonist muscle. Proximal hypermetria turned into hypometria, as a result of the loss of directional preference of the EMG activities in proximal muscles. MSA is the second human model of a shift from hypermetria to hypometria.- - - - - - - - - - ranking = 0.00055858491932369keywords = muscle (Clic here for more details about this article) |
10/16. Severe forward flexion of the trunk in Parkinson's disease: focal myopathy of the paraspinal muscles mimicking camptocormia.Pronounced forward flexion of the trunk, often termed camptocormia, is a typical symptom of patients with Parkinson's disease. In 4 parkinsonian patients with camptocormia, paraspinal muscles were studied by electromyography (EMG) and axial computerized tomography (CT) or magnetic resonance imaging (MRI) scans and muscle biopsy. EMG of the lumbar and thoracic paravertebral muscles showed abundant fibrillations, positive sharp waves, and bizarre high-frequency discharges. Spinal CT and MRI scans revealed variable degrees of atrophy and fatty replacement of the thoracolumbar paraspinal muscles on both sides. No other signs of neuromuscular disease were found. biopsy of the paraspinal muscles revealed end-stage myopathy with autophagic vacuoles, chronic inflammatory myopathy, unspecific myopathic changes, or mitochondrial myopathy. In parkinsonian patients with pronounced forward flexion of the trunk, myopathy confined to the erector spinae muscles must be considered.- - - - - - - - - - ranking = 0.0027929245966184keywords = muscle (Clic here for more details about this article) |
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