1/20. Clinical and physiological characteristics of autonomic failure with Parkinson's disease.We analyzed the clinical and physiological features of autonomic failure with Parkinson's disease (AF-PD) in seven patients and compared them with those of autonomic failure with multiple system atrophy (AF-MSA). In AF-PD, parkinsonism was more gradually progressive than in AF-MSA, and symptoms were responsive to L-dopa. All seven patients with AF-PD had orthostatic hypotension, postprandial hypotension, and constipation, but no urinary retention. Of these, three had hypohidrosis and five had frequent urination; five patients had subnormal plasma norepinephrine (NE) concentrations. Supersensitivity to NE infusion was observed in all patients. head-up tilting (HUT) test resulted in no increase of plasma NE concentrations in both groups, but a significant increase of the plasma arginine vasopressin (AVP) concentrations in the patients with AF-PD. Urodynamic studies revealed that urinary bladder function was relatively well preserved in AF-PD in contrast to AF-MSA. In conclusion, there exists some clinical and physiological differences in autonomic features between AF-PD and AF-MSA, and postganglionic involvement predominates in AF-PD.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
2/20. Brainstem-type lewy body disease presenting with progressive autonomic failure and lethargy.The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
3/20. Decreased parasympathetic activities in Malayan krait (bungarus candidus) envenoming.Three patients were bitten by the Malayan krait (bungarus candidus). The patients developed ptosis and generalized muscle weakness which later progressed to respiratory paralysis. All patients showed evidence of decreased parasympathetic activity manifested by mydriasis, hypertension and tachycardia. No specific antivenom was available. All patients received assisted ventilation and supportive treatment. The other forms of treatment included administration of neostigmine, the banded krait (bungarus fasciatus) antivenom (Thai red cross) and plasmapheresis without beneficial response. Two patients recovered. The other patient had permanent brain damage due to anoxia from two episodes of cardiac arrest. While hypertension resolved 6-60 days after admission, mydriasis and tachycardia persisted after discharge in all patients for between 7 days and 2 years. One patient had constipation and defect in micturition which still persisted 2 years after the bite. Decreased parasympathetic activities in Malayan krait bite are perhaps not uncommon and should be examined.- - - - - - - - - - ranking = 0.0014127106047811keywords = paralysis (Clic here for more details about this article) |
4/20. Clinical features of malignant syndrome in Parkinson's disease and related neurological disorders.INTRODUCTION: We elucidated the cause and clinical characteristics of malignant syndrome (MS) in patients with Parkinson's disease (PD), early-onset parkinsonism (EOP), and other neurological disorders. MATERIALS AND methods: Subjects were 260 patients with PD or EOP, and three patients with other neurological disorders associated with MS. We studied clinical symptoms before and after the onset of MS, and evaluated autonomic function particularly before the onset of MS. RESULTS: The overall incidence of MS accompanying PD and EOP in our department was eight of 260 patients (3.1%). The incidence of MS in EOP was significantly higher than that in PD. All patients with MS showed comparatively longer duration of illness and advanced stage of PD and EOP. Factors triggering MS included reduced dosage or discontinuation of anti-parkinsonian drugs, reduction of oral intake, dehydration, infectious disease, postoperative state, and treatment with major tranquilizers. Although patients demonstrated marked autonomic symptoms at the onset of MS, in many cases autonomic dysfunction developed before the onset of MS. Even EOP patients, who usually demonstrated milder autonomic dysfunction, showed abnormalities in the correlation between circadian rhythm of blood pressure and pulse rate, and/or abnormal gastric emptying test, suggesting that autonomic dysfunction plays an important role in the cause of MS. Cooling the body, fluid replacement, resumption or increasing the dosage of anti-parkinsonian drugs and administration of dantrolene sodium overcame MS in all cases. CONCLUSION: Autonomic dysfunction is related to the cause and clinical features of MS in PD, EOP and some other neurological disorders.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
5/20. association between cardiac denervation and parkinsonism caused by alpha-synuclein gene triplication.Parkinson's disease patients frequently have symptoms and signs of autonomic nervous dysfunction that are the source of considerable disability. Recent studies have revealed that most patients with Parkinson's disease, and all with Parkinson's disease-associated orthostatic hypotension, have a loss of cardiac sympathetic innervation. Familial Parkinson's disease, caused by mutation of the gene encoding alpha-synuclein, also features orthostatic hypotension, sympathetic neurocirculatory failure and cardiac sympathetic denervation. We have recently described a whole-gene triplication of alpha-synuclein causing Lewy body parkinsonism in a large, well characterized family called the 'iowa kindred'. Here we report the results of cardiac PET scanning using the sympathoneural imaging agent, 6-[18F]fluorodopamine in affected and unaffected members of this kindred. Four family members were studied, two with parkinsonism, one clinically normal and one with benign essential tremor alone. Both affected members had obvious loss of cardiac sympathetic innervation; the unaffected member had normal innervation, as did the member with isolated essential tremor. The results indicate that, in this family, where disease is caused by overexpression of normal alpha-synuclein, cardiac sympathetic denervation cosegregates with parkinsonism. Post-mortem studies have demonstrated synuclein-positive Lewy body formation in the brains of individuals with parkinsonism who were also in the family described here and who also carry this triplication. These results indicate that both parkinsonism and cardiac sympathetic denervation can result from an excess of normal synuclein.- - - - - - - - - - ranking = 9keywords = parkinsonism (Clic here for more details about this article) |
6/20. Parkinsonism, dysautonomia, and intranuclear inclusions in a fragile X carrier: a clinical-pathological study.A new tremor-ataxia syndrome, fragile X-associated tremor/ataxia syndrome (FXTAS), has been described among carriers of premutation expansions (55-200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene. The prevalence of FMR1 premutation alleles has been reported to be 1 in 813 among men. patients with FXTAS may also have features of parkinsonism. Postmortem findings have been described in eight patients with FXTAS and detailed descriptions of the pathological features of this syndrome have been published in two of these. We present a detailed description of the postmortem findings in a third patient. The patient had parkinsonism and was a carrier of a premutation expansion in the FMR1 gene. As in previous reports, the most prominent finding was the presence of eosinophilic nuclear inclusions in neurons and astrocytes, loss of purkinje cells, and regional vacuolation of the cerebral white matter. As in one previous report, nuclear inclusions were also present in ependymal and choroid plexus cells. A new finding is that of nuclear inclusions in both the adeno- and neurohypophysis. These findings confirm the diffuse nature of this pathology. Further studies of clinical-pathological correlation in a larger sample of brains would provide additional insight into the mechanisms of the tremor, ataxia, and parkinsonism in these patients.- - - - - - - - - - ranking = 3keywords = parkinsonism (Clic here for more details about this article) |
7/20. respiratory insufficiency as the primary presenting symptom of multiple-system atrophy.BACKGROUND: Respiratory stridor, sleep-disordered breathing, and respiratory insufficiency are part of the clinical spectrum of multiple-system atrophy (MSA). We have encountered cases where these were presenting symptoms, with the diagnosis of MSA being initially unrecognized. OBJECTIVE: To describe cases in which breathing difficulties were the initial and primary manifestation of MSA. DESIGN: database review from January 1, 1996, through October 31, 2005. SETTING: Mayo Clinic, Rochester, Minn. patients: All patients diagnosed as having MSA, cross-referenced for apnea, hypopnea, or hypoventilation. On review, we included only cases in which respiratory dysfunction was the primary initial clinical event in MSA, excluding equivocal cases. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Characteristics and clinical course of patients. RESULTS: Six cases were identified in which substantial respiratory insufficiency occurred as an early, presenting symptom of MSA. Three patients had been examined emergently for acute respiratory distress before the ultimate diagnosis of MSA; the other 3 patients were diagnosed as having obstructive sleep apnea unresponsive to therapy, with bilateral vocal cord paralysis found on ear, nose, and throat examination. Stridor was noted early in the course in all. All patients required tracheostomy, and all eventually developed features consistent with probable MSA. CONCLUSIONS: Multiple-system atrophy may occasionally present as primary respiratory failure or dysfunction, with initially mild motor and autonomic symptoms. Otherwise unexplained central respiratory failure, bilateral vocal cord paralysis, stridor, or refractory central sleep apnea should prompt consideration of MSA.- - - - - - - - - - ranking = 0.0028254212095622keywords = paralysis (Clic here for more details about this article) |
8/20. snoring as a presenting feature of the shy-drager syndrome.A 67-year-old man with a progressive snoring habit is presented. Fluctuant bilateral abductor vocal cord paralysis was later recognized together with autonomic features suggesting a diagnosis of shy-drager syndrome. snoring as a presenting feature of this condition has been infrequently described. This case highlights the importance of careful assessment of snorers.- - - - - - - - - - ranking = 0.0014127106047811keywords = paralysis (Clic here for more details about this article) |
9/20. Pathophysiological findings in a patient with Shy-Drager and alveolar hypoventilation syndromes.This report describes the clinical, physiologic and pathologic findings in a patient with alveolar hypoventilation, acquired autonomic dysfunction, parkinsonism, and sensorimotor polyneuropathy. While there were pathologic findings in both central and peripheral nervous systems, there were no morphologic changes in the regions of the brainstem considered responsible for respiratory rhythmogenesis. A hypothesis is developed based on the assumption that the abnormalities in respiratory control which led to the patient's death are explained by abnormalities in the feedback control of respiration.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
10/20. serum antibodies to brain proteins in a patient with parkinsonism associated with IgM paraproteinemia.We report a 58 year-old man with various neurological symptoms as parkinsonism, autonomic dysfunction, mental deterioration and lower motor neuron involvement associated with IgM gammopathy whose serum showed antibody activities to human brain proteins. His serum reacted with 156kDa protein from substantia nigra and 130kDa of cerebral cortical grey matter, cerebellar cortex, putamen, thalamus or pallidum by immunoblotting method. The serum of this patient reacted with proteins widely distributed in the central nervous system, and this antibody in his serum might be the cause of his various neurological symptoms.- - - - - - - - - - ranking = 5keywords = parkinsonism (Clic here for more details about this article) |
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