- Bassich, C. J., C. L. Ludlow, et al. (1984).
Speech symptoms associated with early signs of Shy Drager syndrome.
J Neurol Neurosurg Psychiatry 47(9): 995-1001.
Speech disturbances reflecting impaired laryngeal control were found in ten patients whose autonomic dysfunction was associated with central neurological disease (Shy Drager Syndrome). In contrast, ten patients with progressive autonomic failure without evidence of CNS involvement had no difficulties on speech function tasks in comparison with normal controls. Presence of speech symptoms may aid in the clinical differentiation between patients with pure autonomic dysfunction and those with central neurological involvement.
- Bawa, R., H. H. Ramadan, et al. (1993).
Bilateral vocal cord paralysis with Shy-Drager syndrome.
Otolaryngol Head Neck Surg 109(5): 911-4.
Shy-Drager syndrome consists of progressive autonomic nervous system failure with Parkinson's disease-like symptoms and orthostatic hypotension. It can also result in airway compromise from bilateral vocal cord paralysis. Fewer than 30 cases of severe bilateral vocal cord paresis or paralysis associated with the Shy-Drager syndrome have been reported in the English literature. We present a case of a 72-year- old man who had a 2-year history of orthostatic hypotension, neurogenic bladder, impotence, anhydrosis, and extremity weakness and paresthesias. Hoarseness and dyspnea with stridor developed as a result of bilateral vocal cord paralysis in the median position and required an emergency tracheotomy. This combination of symptoms resulted in the diagnosis of Shy-Drager syndrome. We present the case along with literature review of bilateral vocal cord paralysis with the Shy-Drager syndrome.
- Berry, E. M., J. H. Growdon, et al. (1991).
A balanced carbohydrate: protein diet in the management of Parkinson's disease.
Neurology 41(8): 1295-7.
Although restricting dietary protein is a proposed adjunct to treating Parkinson's disease (PD), the effect of carbohydrate consumption is unknown. We measured plasma levodopa and large neutral amino acid (LNAA) levels in nine PD patients treated with carbidopa/levodopa and different isocaloric meals containing high protein-low carbohydrate, low protein-high carbohydrate, and balanced 5:1 carbohydrate:protein mixtures. We found that levodopa levels increased significantly regardless of the type of diet, but that plasma LNAA levels varied less and motor performance was superior after the balanced diet than after the other two meals. We conclude that PD patients can consume nutritionally adequate meals and still maintain a stable plasma levodopa:LNAA ratio.
- Hanson, D. G., C. L. Ludlow, et al. (1983).
Vocal fold paresis in Shy-Drager syndrome.
Ann Otol Rhinol Laryngol 92(1 Pt 1): 85-90.
Twelve patients with Shy-Drager syndrome (SDS) presenting symptoms of multiple nervous system atrophy and orthostatic hypotension were examined for laryngeal movement disorders and vocal impairment in speech. Vocal fold abductor paresis was found in 11 patients and was bilateral in 10. Speech task performance was recorded in SDS patients, Parkinson patients and age- and sex-matched controls. Trained listeners with inter-rated reliability greater than or equal to .85 judged each recording on 20 attributes while blind to speaker identity. SDS patients had a breathy and strained voice quality, reduced loudness, monopitch and monoloudness, imprecise consonants, variations in rate and rate-slowing, suggesting a flaccid type of dysarthria. In comparison with Parkinson patients, SDS patients had excess vocal hoarseness, intermittent glottal fry and a slow and deliberate speaking rate. Orthostatic hypotension, laryngeal stridor, hoarseness, intermittent glottal fry and slow speech rate were found to be discriminating symptoms of SDS
- Hodder, J. (1997).
Shy Drager syndrome
Axone 18(4): 75-9.
Shy Drager Syndrome (SDS) is a movement disorder which is often referred to as a parkinson plus syndrome or Multiple System Atrophy (MSA). For patients afflicted with this condition, rigidity and bradykinesia are the primary extrapyramidal symptoms which are present. The "plus" refers to autonomic nervous system dysfunction which leads to much of the disability seen in this disorder. Syncope, urinary incontinence, impotence, constipation, fecal incontinence, cardiac arrythmias as well as other symptoms occur as a result of widespread pathological changes in multiple areas of the central and autonomic nervous system. The goal of this paper is to provide an overview of the pathophysiology, signs and symptoms of and treatment for SDS. Nursing Care of the patient and family coping with Shy Drager Syndrome and the challenges it presents to the Movement Disorder Nurse are discussed. A coordinate, multidisciplinary team approach is suggested.
- Imaoka, K., S. Kobayashi, et al. (1990).
[An association of Shy-Drager syndrome with moyamoya disease--a case report].
Rinsho Shinkeigaku 30(5): 560-2.
A 58-year-old female was admitted to our hospital because of orthostatic syncope, and difficulty in urination and walking for 2 years. At age 35, she suffered from cerebral hemorrhage and was diagnosed as Moyamoya disease (MD). Neurological examination on admission revealed severe orthostatic syncope so that she could not stand. At that time, her blood pressure and heart rate were 104/74 mmHg and 78/min in supine position and 52/48 mmHg and 52/min in 60 degrees head-up position. She also had severe pollakisuria, cerebellar ataxia and dysarthria. Cranial nerves, motor strength and deep reflexes were normal. ECG, chest X-ray, and EEG were normal. Aschner, Czermak, and cold pressor tests revealed no response. Urodynamic study revealed autonomic bladder. MRI showed only enlarged fourth ventricle because of atrophy of the pons. Bilateral CAG revealed "Moyamoya" vessels in the cerebral basal regions. Cerebral blood flow (CBF) was measured by 133Xe inhalation method. Mean arterial blood pressure changed from 134 mmHg to 126 mmHg in 45 degrees head-up tilting and CBF decreased from 47.5 mg/100 g/min to 37 ml/100 g/min position. Though there was no relationship between SDS and MD, each one shows dysautoregulation of CBF, it is supposed that a severe orthostatic syncope attack was resulted from synergism of both effects
- Jacob, G. and D. Robertson (1995).
Orthostatic hypotension: epidemiology, pathophysiology and management.
Curr Opin Nephrol Hypertens 4(5): 452-4.
Orthostatic hypotension is characterized by low upright blood pressure levels and symptoms of cerebral hypoperfusion. Whereas orthostatic hypotension is heterogeneous, correct pathophysiologic diagnosis is important because of therapeutic and prognostic considerations. Although therapy is not usually curative, it can be extraordinarily beneficial if it is individually tailored. Management of the Shy-Drager syndrome (multiple-system atrophy) remains a formidable challenge.
- Kakulas, B. A., N. Tan, et al. (1986).
The neuropathology of progressive autonomic failure of central origin (the Shy-Drager syndrome).
Clin Exp Neurol 22: 103-11.
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.
- Karstaedt, P. J., J. H. Pincus, et al. (1991).
Standard and controlled-release levodopa/carbidopa in patients with fluctuating Parkinson's disease on a protein redistribution diet. A preliminary report.
Arch Neurol 48(4): 402-5.
Ten patients with Parkinson's disease (PD) with motor fluctuations that responded to a protein redistribution diet were studied. All 10 patients were receiving standard Sinemet (levodopa/carbidopa). Five randomly selected patients were changed from standard Sinemet to a controlled-release form of Sinemet. The other five patients continued to receive standard Sinemet. To maintain the same degree of control of PD in the five patients switched to the controlled-release form of Sinemet, the daily levodopa intake increased. While receiving optimal therapy (standard Sinemet or controlled-release Sinemet) and a protein redistribution diet, all 10 patients then underwent hourly videotaping and blood sampling (for plasma levodopa levels) during 2 consecutive days. Videotapes were blindly reviewed for PD disability, dyskinesia, and the time required to walk a measured distance. Comparing the two groups, standard Sinemet with controlled-release Sinemet, respectively, mean levodopa requirements were 505 and 1895 mg, plasma levodopa levels were 6.1 and 17.6 mumol/L, and abnormal involuntary movement scale scores were 14 and 26. Their mean PD disability scores did not differ statistically or clinically. Also no statistically significant differences were noted in either their mean walking times or their mean daily dose frequencies.
- Lockwood, A. H. (1976).
Shy-Drager syndrome with abnormal respirations and antidiuretic hormone release.
Arch Neurol 33(4): 292-5.
A patient with Shy-Drager syndrome exhibited a partial defect in antidiuretic hormone (ADH) release, and cluster breathing, an indication of pontomedullary respiratory center damage, with a normal CO2 response curve. This extends the spectrum of abnormalities associated with the degenerative disease of the central nervous system. The presence of a pontomedullary respiratory pattern without an impaired CO2 response curve suggests that neurons that determine respiratory rhythm function independently from those that function as chemoreceptors.
- McGlashan, J. A. and D. G. Golding-Wood (1989).
Snoring as a presenting feature of the Shy-Drager syndrome.
J Laryngol Otol 103(6): 610-1.
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.
- Nee, L. E., J. Scott, et al. (1993).
Olfactory dysfunction in the Shy-Drager syndrome.
Clin Auton Res 3(4): 281-2.
The aetiology of the Shy-Drager syndrome (multiple system atrophy) is unknown. We reported previously a preliminary association between environmental-occupational risk factors and Shy-Drager syndrome. To further investigate this relationship, we evaluated olfactory function in eight patients in different stages of disease. When the eight patients' olfactory function was compared with 203 age- and sex-matched controls using a self-administered olfactory test, seven scored below the 39th percentile of this population. Five of the eight patients had total anosmia or microsmia. Additional studies will be required to elucidate the significance of this abnormal clinical observation.