1/14. 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.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
2/14. 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.- - - - - - - - - - ranking = 4keywords = atrophy (Clic here for more details about this article) |
3/14. The Lewis family revisited: no evidence for autosomal dominant multiple system atrophy.In 1964, Lewis reported a familial ataxia-dysautonomia syndrome reminiscent of shy-drager syndrome subsequently known as multiple system atrophy (MSA). Here we review this report and propose that the Lewis family may represent an unusual form of autosomal dominant cerebellar ataxia type I, which might be categorized either as SCA3 (machado-joseph disease) or a new SCA subtype. There remains no conclusive evidence to support the notion of hereditary MSA.- - - - - - - - - - ranking = 5keywords = atrophy (Clic here for more details about this article) |
4/14. Marked depletion of dorsal spinal cord substance p and calcitonin gene-related peptide with intact skin flare responses in multiple system atrophy.In view of the presence of neuropeptides in spinal cord autonomic pathways, their regional concentration was studied in post mortem thoracic cord from four cases of multiple system atrophy with progressive autonomic failure (MSA). A marked depletion was observed of substance p, its related peptide substance K, and of calcitonin gene-related peptide (CGRP), particularly in dorsal regions where peptide-containing sensory fibres terminate. As substance p and CGRP in primary sensory fibres are considered mediators of skin flares in Lewis' triple response, histamine-induced skin flares were measured in 12 MSA patients and were found to be preserved. These results provide a new key to the classification and aetiology of autonomic and multiple system degenerations, as well as a model to study the role of sensory neuropeptides in man.- - - - - - - - - - ranking = 5keywords = atrophy (Clic here for more details about this article) |
5/14. Abnormal cardiovascular responses to postural changes and pharmacologic agents in a case of shy-drager syndrome.A 64-year-old carpenter had an unsteady gait, severe dizziness, nocturia, and a loss of erection for more than 4 years. The neurological manifestations consisted of a wide-based ataxic gait, bilateral dysmetria with intentional tremor, staccato speech, rigidity, bradykinesia, and an iris-thinning. There was reproducible orthostatic hypotension. A sweat test revealed severe anhidrosis. nicotine and methylbenzene sensitivity was absent, whereas norepinephrine infusion test showed a significant elevation of blood pressure. The resting plasma norepinephrine level on recumbency was low and a subnormal surge was noted on standing or exercise. We conclude that the clinical features caused by a degenerative process involving both the central and peripheral autonomic systems, together with atrophy of other systems in this patient, constitute the shy-drager syndrome.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
6/14. The neuropathology of progressive autonomic failure of central origin (the shy-drager syndrome).The neuropathological features of the "shy-drager syndrome" have, in the past, been unsettled. The position was recently clarified by Oppenheimer who reviewed the 51 reported patients with progressive autonomic failure in whom neuropathological findings were given. He divided these cases into two groups. Group I included those with lesions of the pigmented nuclei of the brain stem which contained lewy bodies. These he further subdivided into subgroups IA or IB according to whether or not the patient showed clinical evidence of Parkinson's disease in addition to the common denominator of orthostatic hypotension. In group II he placed those patients in whom multiple system atrophy was found at necropsy. These were the majority. Loss of neurons from the intermediolateral columns of the thoracic spinal cord, thought to be the cause of the postural hypotension, was present in all cases. To illustrate the new classification the necropsy findings in 3 patients taken from the files of the Royal Perth Hospital are described. One of these showed the lesions of Parkinson's disease with lewy bodies, and thus conformed to Oppenheimer's type IA. The two other patients showed multiple system atrophy and thus belonged to Oppenheimer's type II. All three showed loss of neurons in the intermediolateral columns of the thoracic spinal cord. We believe that Oppenheimer's classification of progressive orthostatic hypotension improves the clinicopathological understanding of the syndrome and is thus useful as well as informative to the practising neurologist.- - - - - - - - - - ranking = 2keywords = atrophy (Clic here for more details about this article) |
7/14. The posterior crico-arytenoid muscle in two cases of shy-drager syndrome with laryngeal stridor. Comparison of the histological, histochemical and biometric findings.The histological, histochemical and biometric findings in the posterior crico-arytenoid muscle in two patients with shy-drager syndrome were compared with those found in cases of carcinoma of the larynx. In biopsy specimens from the patients with laryngeal carcinoma, neurogenic atrophy and various structural changes in the muscle fibres were the prominent features. In the two patients with shy-drager syndrome these changes were not present and the only significant finding was the more pronounced type I fibre atrophy, with type II fibre predominance in the more severely affected case. These findings do not permit the vocal cord paralysis seen in the shy-drager syndrome to be explained by motorneuron loss and denervation. It is postulated that a possible cause may be a biochemical defect in the brain.- - - - - - - - - - ranking = 2keywords = atrophy (Clic here for more details about this article) |
8/14. shy-drager syndrome: the transitional variant.The clinicopathological features of a patient with the transitional variant of the shy-drager syndrome is described. The only previously reported case of the transitional variant was reexamined and pathological similarities to the present case are reviewed. Both patients exhibited features of Parkinson's disease with lewy bodies in the substantia nigra and locus ceruleus. Striato-nigral degeneration and olivo-ponto-cerebellar atrophy were evident in both cases. They can thus be considered as transitional forms of the shy-drager syndrome with feature of both Parkinson's disease and multiple system atrophy.- - - - - - - - - - ranking = 2keywords = atrophy (Clic here for more details about this article) |
9/14. tobacco intolerance in multiple system atrophy.In a patient with multiple system atrophy, the ataxia was temporarily exacerbated by cigarette smoking. The phenomenon is attributed to a direct effect of nicotine on the central nervous system, rather than being secondary to autonomic changes. Its prevalence and specificity are currently unknown.- - - - - - - - - - ranking = 5keywords = atrophy (Clic here for more details about this article) |
10/14. brain stem auditory evoked potentials in patients with multiple system atrophy with progressive autonomic failure (shy-drager syndrome).brain stem potentials from three groups of patients, namely those with pure progressive autonomic failure, Parkinson's disease and multisystem atrophy with progressive autonomic failure (shy-drager syndrome) were compared with each other and a group of normal subjects. In virtually all the patients with multisystem atrophy with progressive autonomic failure the brain stem potentials were abnormal in contrast to normal findings with Parkinson's disease. The closely associated group of patients with progressive autonomic failure alone also revealed no abnormalities of the BAEP. This separation of the two groups, Parkinson's disease and progressive autonomic failure from multisystem atrophy with progressive autonomic failure is important clinically as multiple system atrophy of the Shy-Drager type has extra-pyramidal features closely resembling Parkinsonism or a late onset cerebellar degeneration. From the abnormalities of the brain stem response in multisystem atrophy with progressive autonomic failure, it is clear that some disruption of the auditory pathway occurs in the ponto-medullary region as in nearly all patients there is a significant delay or reduction in the amplitude of components of the response generated beyond this region. The most likely area involved is the superior olivary complex.- - - - - - - - - - ranking = 9keywords = atrophy (Clic here for more details about this article) |
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