Cases reported "Shy-Drager Syndrome"

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1/68. Management of impaired vocal fold movement during sleep in a patient with shy-drager syndrome.

    A 46-year-old woman with shy-drager syndrome is presented. She has impaired vocal fold abduction during sleep, but has no laryngeal dysfunction while she is awake. In order to reduce laryngeal obstruction during sleep, she initially underwent laterofixation of 1 vocal fold (Ejnell's method) with little lasting success because of accidental slipping of the ligature. Later, she successively underwent arytenoidectomy with the use of CO2 laser. Her noctural breathing improved markedly after arytenoidectomy. ( info)

2/68. pure autonomic failure in association with human alpha-synucleinopathy.

    We studied an autopsy case with pure autonomic failure, using anti-alpha-synuclein antibody. Until now there has been no report about the immunohistochemical properties of alpha-synuclein in pure autonomic failure. In conventional stainings, both pre- and post-ganglionic lesions of the sympathetic and parasympathetic nervous systems were found. lewy bodies and Lewy neurites were abundant especially in the sympathetic nervous system. These inclusions were immunoreactive to anti-alpha-synuclein antibody. The intensity of alpha-synuclein immunoreactivity was stronger in the halos than in the cores of the lewy bodies. The edges of the swollen neurites had strong immunoreactivity. The substantia nigra was well preserved, and no cortical lewy bodies were seen. These findings indicate that pure autonomic failure is one of the Lewy body type alpha-synucleinopathies, such as Parkinson's disease and dementia with lewy bodies, targeting the peripheral autonomic nervous system. ( info)

3/68. Familial mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis.

    OBJECTIVES: To describe a family with some sort of progressive autonomic failure in one generation (2 affected of a sibship of 7 sisters). The main features were: mydriasis, cardiac arrhythmia, cardiomegaly, hypohidrosis, respiratory failure, and muscular weakness. methods: Pupillometry, evaporimetry, and isokinetic power measurements were carried out. RESULTS: The autonomic dysfunction pattern (mainly cardiac abnormalities, mydriasis) seems to differ somewhat from that of progressive autonomic failure (shy-drager syndrome). "Lewy body-like" inclusions were present, in particular in substantia nigra, but also in locus ceruleus and raphe nuclei (cell loss only in locus ceruleus). There were no oligodendroglial, cytoplasmatic inclusions, apparently a marker in multiple system atrophy. Proper lewy bodies were also present. Differences seemed to prevail vs the shy-drager syndrome. Various traits: muscular weakness pattern (e.g. preferential peroneal distribution), minor elbow contractures, and arrhythmia were reminiscent of Emery-Dreifuss muscle dystrophy (E-D). Distinguishing features included: hereditary pattern, mydriasis, and hypohidrosis. CONCLUSION: Conceivably, this disorder is close to, but still not identical with E-D. ( info)

4/68. shy-drager syndrome and severe unexplained intraoperative hypotension responsive to vasopressin.

    IMPLICATIONS: We describe the first case of shy-drager syndrome diagnosed on the basis of intraoperative hemodynamic changes. The initial hypertension in the supine position followed by severe hypotension after hydralazine administration, ultimately responsive to vasopressin, led to a diagnosis of shy-drager syndrome. We suggest that vasopressin may be the drug of choice in patients with shy-drager syndrome with refractory hypotension. ( info)

5/68. Duplication of distal part of the basilar artery.

    We report a case of duplication or fenestration of the distal part of the basilar artery with shy-drager syndrome. Duplication and fenestration of the basilar artery are discussed, and a review of embryologic development is presented. ( info)

6/68. Clinical effects of elastic bandage on neurogenic orthostatic hypotension.

    Neurogenic orthostatic hypotension (OH) often causes troublesome symptoms such as dizziness, syncope and falling, interfering active daily life or various therapies in rehabilitation. Nonpharmacologic measures for treating patients with OH include wearing elastic leotard, head-up tilting at night, etc. Elastic garment or antigravity suits is certainly effective, but it may be uncomfortable and not practical. Although elastic bandage (EB) bound on the lower limbs has been thought to be useful, there is few clinical report about its beneficial evidence. We investigated short-term clinical effects of commercially available EB on OH, and estimated the mechanism of its effectiveness by measuring some blood pressure-related humoral variables in neurodegenerative patients with OH. ( info)

7/68. A remarkable patient--40 years with probable multiple system atrophy (MSA).

    A 58-year-old woman with a 40-year history of very slowly progressive multiple system atrophy is described. ( info)

8/68. Effect of xamoterol in shy-drager syndrome.

    BACKGROUND. xamoterol, a cardioselective beta 1-adrenoceptor partial agonist, has been reported to be effective on postural hypotension. We investigated the effect of xamoterol in five patients with shy-drager syndrome (SDS) in relation to their prevailing sympathetic nerve activity and sensitivity of beta-adrenoceptors and the change in circadian variation of blood pressure. methods AND RESULTS. Ambulatory blood pressure over 24 hours was monitored by noninvasive sphygmomanometer (model 5200, Spacelab). plasma norepinephrine levels of SDS patients were significantly lower than that of normal subjects (n = 5) both at rest (54 /- 15 versus 178 /- 83 pg/ml) and after 10-minute standing (74 /- 24 versus 318 /- 143 pg/ml). Infusion of isoproterenol (0.02 micrograms/kg/min) produced a mild rise of systolic blood pressure and tachycardia in normal subjects but resulted in marked hypotension and tachycardia in SDS subjects. After xamoterol administration (200 mg b.i.d.), systolic blood pressure and heart rate were significantly increased in the averages during the day; however, increases were more pronounced at night. In two of the five patients, the improvement in dizziness was large enough to enable them to increase their daily activities. CONCLUSIONS. Our observations suggest that 1) beta 1-selective, high intrinsic sympathomimetic activity of xamoterol increases blood pressure and heart rate in patients with SDS as a consequence of their prevailing beta 1-adrenoceptor hypersensitive state, and 2) blood pressure monitoring over 24 hours appears to have important advantages in evaluating the therapeutic effects on postural hypotension. ( info)

9/68. Primary sjogren's syndrome presenting with generalized autonomic failure.

    A 64 year-old woman developed Raynaud's phenomenon and dry eyes/mouth. Laboratory examination revealed positive Schirmer's test, rheumatoid factor and anti-nuclear antibody, and lymphocytic sialoadenitis on salivary gland biopsy. These features strongly suggested the diagnosis of primary sjogren's syndrome. Three years later, she gradually developed generalized autonomic failure without apparent sensory neuropathy on nerve conduction study. She had systolic pressure fall of 51 mmHg on head-up tilt test, cardiovascular supersensitivity to diluted norepinephrine infusion, cardiac denervation in [123I]-MIBG scintigraphy, impaired R-R variability, decreased sweating and prolonged colonic transit time. Autoimmune autonomic ganglionopathy was mostly responsible for her autonomic failure. ( info)

10/68. The PINK1 phenotype can be indistinguishable from idiopathic parkinson disease.

    Mutations in the PINK1 gene cause autosomal recessive parkinsonism characterized by early onset and a variable phenotypic presentation. A patient homozygous for the Ala168Pro mutation has been fully characterized clinically. Apart from onset at age 39 years and the excellent and sustained response to levodopa, all clinical and laboratory features, including SPECT and assessment of autonomic function, were indistinguishable from typical idiopathic parkinson disease. ( info)
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