1/8. 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) |
2/8. 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 = 1.5keywords = rigidity (Clic here for more details about this article) |
3/8. 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) |
4/8. 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 = 1keywords = rigidity (Clic here for more details about this article) |
5/8. 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) |
6/8. Longitudinal change in REM sleep components in a patient with multiple system atrophy associated with rem sleep behavior disorder: paradoxical improvement of nocturnal behaviors in a progressive neurodegenerative disease.A 60-year-old patient with multiple system atrophy (MSA) who presented with rapid eye movement (REM) sleep behavior disorder was investigated longitudinally by all-night polysomnography. REM sleep components, i.e. rapid eye movements and chin muscle activity, were analyzed together with the frequency of behaviors/movements on the videorecording. Decreased frequency of elaborate motor activity during REM sleep with time in this patient was compatible with the observation by his wife, and this change seemed to correlate with predominant tonic chin electromyogram with relatively suppressed phasic chin muscle activity, but the reduction of the rem sleep behavior disorder (RBD) episodes could be interpreted as being due to the increased rigidity along with MSA progression. The chronological change in REM sleep components in RBD with neurological disorders is worth studying in large follow-up series to enlarge our knowledge about the mechanism of behavioral manifestation of RBD in humans.- - - - - - - - - - ranking = 0.5keywords = rigidity (Clic here for more details about this article) |
7/8. A differential diagnosis of Parkinsonism.Parkinsonism is a common, age-related syndrome, characterized by resting tremor, bradykinesias, rigidity, and postural reflex impairment. Though Parkinsonism is not very difficult to recognize, all Parkinsonism is not created equal and it is important to distinguish among the most common identifiable syndromes. This review discusses the key clinical features of these various syndromes, including Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, corticobasal ganglionic degeneration, lewy body disease, vascular Parkinsonism, and Parkinsonism with no clear etiology. Symptomatology and diagnostic testing for each syndrome are discussed and 4 typical cases are analyzed to offer clinicians guidance in making a differential diagnosis for Parkinsonism.- - - - - - - - - - ranking = 0.5keywords = rigidity (Clic here for more details about this article) |
8/8. multiple system atrophy following chronic carbon disulfide exposure.carbon disulfide toxicity is well characterized. The principal target organ is the nervous system, although cardiovascular, reproductive, ophthalmologic, and other effects are also recognized. The neurotoxicity manifests in three ways: encephalopathy, peripheral and cranial nerve dysfunction, and movement abnormalities. This report describes a case of olivopontocerebellar atrophy, a form of multiple system atrophy, developing in an adult after over 30 years of occupational exposure to carbon disulfide. The patient presented with the insidious onset of balance problems, impotence, and irritability, without tremor, cogwheel rigidity, bradykinesia, or changes in facial expression. Over the next few years severe ataxia developed, and the clinical diagnosis was confirmed with computed tomography and magnetic resonance imaging scans. The patient experienced multiple medical complications and died approximately 9 years after diagnosis. This case is consistent with a large body of clinical and experimental literature, much of it 50 years old, showing that carbon disulfide can cause movement disorders. It also serves as a reminder that movement disorders, ranging from parkinsonism to dystonia, are associated with a variety of toxic exposures such as manganese, carbon monoxide, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, and medications.- - - - - - - - - - ranking = 0.5keywords = rigidity (Clic here for more details about this article) |