Drager Syndrome Research Articles

A Case of Shy Drager Syndrome in a Patient Following Orthotopic Liver Transplantation

A Case of Shy Drager Syndrome in a Patient Following Orthotopic Liver Transplantation

Shy –Drager Syndrome In Two Nigerian Male Adults

Baroreceptor sensitivity decreases with advancing age and systemic hypertension. In consequence, rapid adjustment of the circulation to changes in posture may be impaired so that postural hypotension occurs. One such condition where this is well demonstrated is the Shy-Drager Syndrome, multiple system atrophy with autonomic failure characterized by postural hypotension. Though a rare disease, a lot of interest has recently been focused on it by the National Institute of Neurological Disease and Stroke (NINDS) because of difficulties in diagnosis and treatment. Typical cases of Shy-Drager Syndrome have not been reported in the Nigerian literature although related cases abound. The following are case reports of two adult Nigerian males. The first is that of a 70-year-old Bini man, a known hypertensive who was being managed for benign prostatic hypertrophy (BPH), parapareisis and occasional disorientation. He was later diagnosed as a case of Shy-Drager Syndrome. The second is that of a 52-year-old Bini man who became incapacitated by recurrent syncopal attacks. Both responded well to treatment with fludrocortisone acetate. A very rare condition in our environment. The need for its awareness is hereby emphasized Keywords: Shy-Drager Syndrome; Nigerian, Male, Adult. Annals of Biomedical Science Vol. 2 (1) 2003: pp. 54-58

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.

Sleep disordered breathing may not be an independent risk factor for diabetes, but diabetes may contribute to the occurrence of periodic breathing in sleep

Sleep disordered breathing may not be an independent risk factor for diabetes, but diabetes may contribute to the occurrence of periodic breathing in sleep

Comparison of the blood pressure response to food in two clinical subgroups of multiple system atrophy (Shy–Drager syndrome)

Multiple system atrophy (MSA) is a progressive disorder in which autonomic features vary at different stages. Abnormal cardiovascular responses are common and it is now recognized that factors in daily life, such as exercise or food ingestion, can lower blood pressure (BP) substantially. The BP response to exercise differs in the cerebellar (MSA-C) and parkinsonian (MSA-P) forms of MSA. Whether this also occurs after food ingestion has not been addressed. We, therefore, compared the BP response following a liquid meal in MSA-C (n=10) and MSA-P (n=7) subjects. In both forms, after head-up tilt while fasted, there was a similar fall in mean arterial blood pressure (MAP) in MSA-C (23+/-4mmHg) and MSA-P (23+/-4mmHg). After the liquid meal there was a fall in supine BP in both forms, but the fall in MAP was greater in MSA-C (19+/-5mmHg) than in MSA-P (8+/-2mmHg, p<0.05). On re-tilt, 45min after the meal, BP fell to a lower level in both subgroups, with a similar MAP fall in MSA-C (20+/-5mmHg) and MSA-P (23+/-7mmHg). The larger supine BP fall in response to a liquid meal in MSA-C than in MSA-P was similar to the responses found with exercise and suggests that cerebellar and/or brain stem autonomic pathways are impaired to a greater extent in MSA-C than in MSA-P. Pooling MSA subgroups, therefore, may obscure patho-physiological differences to certain stimuli. The response to upright posture in MSA-C and MSA-P was similar post meal as it was after exercise. Clinically, therefore, when postural hypotension is being assessed, separation of the subgroups may not be essential.

Visceral orthostatic hypotension in patients with severe autonomic dysfunction

Although changes in the blood flow of the cerebral vessels and the peripheral vessels in the extremities after changing body postures have been well examined in patients with orthostatic hypotension (OH), such changes in visceral vessels have not been well investigated. To elucidate the effect of autonomic dysfunctions on changes in the abdominal blood flow, the blood flow velocity of the portal vein was measured by Doppler ultrasonography in 11 patients with familial amyloidotic polyneuropathy (FAP) (Met30), 3 with pandysautonomia, 1 with Shy–Drager syndrome, and 10 healthy controls, in the supine and at the upright position. Among the 15 patients with the above-mentioned autonomic disorders, 5 of the patients showed a marked decrease in blood flow after standing, and one of these 5 patients exhibited transient hepatic and intestinal ischemia during intensive rehabilitation because of a severe decrease in visceral blood flow. Another 7 patients exhibited moderate decreases in the blood flow after standing. In contrast, no such change was observed in the 10 healthy controls. The FAP patients with l- threo-3,4-dihydroxyphenylserine ( l-threo-DOPS) administration showed no significant correlation between the degree of OH and the decrease in the blood flow of the portal vein, and the patients without the drug exhibited a weak correlation. On the contrary, the pandysautonomia and Shy–Drager syndrome patients exhibited a linear positive correlation. These results suggest that FAP is a disease for which this kind of ultrasonographic examination should be applied, and that Doppler ultrasonography may be a helpful tool to evaluate visceral OH.

Sleep‐related breathing disorders in the Shy–Drager syndrome. Observations on investigation and management

We report on 18 patients with the Shy-Drager syndrome who were referred for assessment of a sleep-related breathing disorder. The main symptoms of snoring, apnoea, stridor and daytime hypersomnolence were detailed and vocal cord movement graded by laryngoscopy as normal, mild weakness of abduction or near paralysis of vocal cord movement Sleep studies involving oximetry and observations were performed. Obstructive and central apnoeas were detected in six patients. Many patients with near paralysis of vocal cord abduction had normal sleep studies. Successful management strategies of obstructive cases included CPAP, nasal surgery and tracheostomy or arytenoidectomy alone or in combination. Sleep-related breathing disorders in the Shy-Drager syndrome may involve significant nocturnal desaturation; investigation with suitable medical or surgical treatment should be employed in these patients.

Cholinergic dysfunction in Shy-Drager syndrome: effect of the parasympathomimetic agent, bethanechol.

To determine the frequency, severity and organ distribution of cholinergic dysfunction in the Shy-Drager syndrome, eleven patients were prospectively studied. In addition to documenting adrenergic insufficiency, a battery of twelve tests was employed to assess cholinergic function. Six tests demonstrated pupillary, lacrimal, salivary, urinary bladder, sexual and sudomotor dysfunction in the majority of patients. Cardiac vagal function as studied by the heart rate response to deep breathing, the Valsalva manoeuvre, cold face test, apnoeic facial immersion and atropine test was affected in all patients. Oesophageal motility was abnormal in six patients. Cholinergic dysfunction in patients with the Shy-Drager syndrome was widespread but of variable severity and distribution. Subcutaneous administration of the parasympathomimetic agent bethanechol demonstrated hyperresponsiveness of lacrimal, salivary, oesophageal, bowel, bladder and sudomotor functions. It is suggested that the Shy-Drager syndrome is primarily a preganglionic cholinergic disorder with transsynaptic degeneration accounting for the development of postganglionic cholinergic as well as adrenergic dysfunction.

Case Report of Epidural Block for A Patient with The Shy - Drager Syndrome

Case Report of Epidural Block for A Patient with The Shy - Drager Syndrome

Olfactory dysfunction in the Shy-Drager syndrome.

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.

Neurobiological aspects of the pelvic floor muscles involved in defecation

Neurobiological aspects of the organization of pelvic floor musculature are reviewed. Evolutionary considerations on the origin of these muscles indicate that they develop with specific attachments and function, i.e., do not derive from preexisting muscles such as the ones from the tail. Anatomically, pelvic floor muscles can be divided into 1) true sphincters and related muscles and 2) muscles which flank the visceral outlets. While in quadrupedal mammals the EAS behaves as a fast twitch muscle, in man this muscle has slow twitch characteristics. Like some epaxial muscles the EAS has a strong connectivity with its surrounding skin. In further analogy with some epaxial muscle the EAS, although endowed with muscle spindles, is devoid of the phasic, monosynaptic component of the stretch reflex. Onuf's nucleus which innervates pelvic floor muscles receives an important group of suprasegmental afferents including, probably, direct corticospinal fibers. Pelvic floor muscles play a fundamental role in signaling arrival of feces to the perineum. While sphincteric activity is important for continence, other mechanisms such as the anorectal angle and anal cushions are also of relevance. Although emphasis has been put on motor factors, fecal incontinence can also result from impairments in sensory mechanisms of the anorectal system. In diseases like amyotrophic lateral sclerosis, Werdnig Hoffman's and others there is selective sparing of neuropathology in Onuf's nucleus. In contrast, the nucleus is affected in some autonomic visceromotor neuronal disorders, e.g., Shy Drager syndrome, Fabry's disease. It has been suggested that Onuf's nucleus occupies an intermediate position between visceral and somatic nuclei.

Urodynamic differentiation of parkinson disease and the shy drager syndrome

AbstractThe urodynamic findings of 29 patients with Parkinson disease (PD) and lower urinary tract symptoms were compared with those of nine patients with the Shy‐Drager syndrome (SD). All patients with PD had bladder contraction and normal filling pressures, and 90% had detrusor hyperreflexia. Among the patients with SD, 67% had detrusor areflexia, 33% had involuntary bladder contractions, and 45% had poor vesical compliance. In all patients with SD who had a voiding cystourethrogram, an open bladder neck at rest was demonstrated, but it was found only in those with PD who had prior prostatectomy. The electromyography of the external sphincter showed some form of lower motor neuron lesion in all patients with SD, but it was normal in 39% of PD patients and consistent with just involuntary sphincteric activity in 61%. A neurological evaluation of patients with typical and atypical Parkinsonism can assist in differentiating between these two entities.

Shy—drager syndrome with elliptocytosis. An autopsy case report

Shy—drager syndrome with elliptocytosis. An autopsy case report

Cerebral blood flow in a patient with Shy—Drager syndrome determined by positron emission tomography

Cerebral blood flow in a patient with Shy—Drager syndrome determined by positron emission tomography

Speech symptoms associated with early signs of Shy Drager syndrome.

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.

Agonist regulation of alpha-adrenergic receptor responses in man.

Summary: Plasma noradrcnalinc concentrations, platelet α-receptor prop-erties. and the blood pressure responses to intravenous noradrcnalinc were measured in 14 normal subjects. 6 patients with phaeochromoeytoma. 9 patients with multiple System atrophy and sympathetic nervous system degener-ation (MSA. Shy—Drager syndrome), and 12 tetraplegic patients (cord tran-sections C4-C8). Plasma noradrenaline concentrations at rest were about 10 times normal in phaeochromoeytoma. one-tenth normal in MSA. and about one-half normal in tetraplegics. Conversely, the platelet α-receptor number was decreased in phaeochromoeytoma and increased in MSA. i.e., platelet α-receptor numbers were inversely related to plasma noradrenaline concentrations. Platelet α-receptor numbers were normal in tetraplegics. Platelet α-receptor affinity did not change in any group. Blood pressure responses to intravenous noradrenaline were greater than normal in the 9 MSA patients, but were decreased in one patient with phaeochromoeytoma. The inverse relation-ship between noradrenaline concentrations, platelet α-receptor numbers. and blood pressure responses to intravenous. exogenous. noradrenaline may mean that endogenous adrenergic agonist concentrations; in man. may regulate α-receptor properties and so may determine, in part, α-receptor mediated biological responses. In tetraplegics. frequent but intermittent exposure to high concentrations of noradrenaline during the activation of spinal sympathetic reflexes may prevent an increase in α-receptor number even though. in the resting state, exposure of adrenergic receptors to agonist is low as refleeted by the low plasma noradrenaline concentration. In tetraplegics. the increased pressor response to noradrenaline may be due only to impairment of baro-reflexes.

β‐receptor numbers and thermodynamics in denervation supersensitivity

1. Denervation supersensitivity to adrenergic agonists occurs after degeneration of the sympathetic nervous system in the disease called multiple system atrophy (MSA) or the Shy Drager Syndrome.2. Supersensitivity to the chronotropic effect of i.v. isoprenaline on the heart was demonstrated in eight subjects with sympathetic nervous system degeneration and MSA.3. There was an increased number of beta-receptors present in MSA as measured by [H(3)]dihydroalprenolol ([H(3)]DHA) binding to beta-receptors on lymphocytes isolated from venous blood taken from the MSA subjects compared with [H(3)]DHA binding to lymphocytes from seven normal subjects. There was no difference in the affinity of lymphocyte beta-receptors for [H(3)]DHA in MSA.4. [H(3)]DHA binding to lymphocytes from MSA subjects was decreased at lower temperatures but was unaffected by lower temperatures in lymphocytes from normal subjects.5. Equilibrium constants for [H(3)]DHA binding to normal and MSA lymphocytes were similar, indicating that the affinity of the beta-receptors was similar in both groups. The equilibrium constants were little affected by cooling from 37 to 4 degrees C suggesting that the heat of reaction (enthalpy) for [H(3)]DHA binding was low. The Gibbs free energy change on binding was negative and similar in quantity for both normal and MSA lymphocytes. There was a similar, large, increase in entropy on binding of [H(3)]DHA to both normal and MSA lymphocytes, showing that the binding reaction was entropy driven.6. If lymphocyte beta-receptors reflect the status of cardiac beta-receptors, increased numbers of cardiac beta-receptors may contribute to the denervation supersensitivity to isoprenaline in MSA with sympathetic degeneration.

Sodium homeostasis in patients with autonomic failure.

1. The renal excretion of sodium by five patients with autonomic failure (Shy—Drager syndrome) was compared with a matched control group who had normal autonomic reflexes (Parkinson's disease). For 8 or 9 days the daily sodium intake was reduced to 17 mmol, and for 5 subsequent days it was increased to 189 mmol. 2. The sodium excretion of the patients with autonomic failure was not significantly reduced during 7 days of restricted intake whereas that of the control group fell rapidly to values comparable with their sodium intake. Patients with autonomic failure had a larger fall in body body weight than the control subjects. 3. Both lying and standing values of mean blood pressure fell during salt restriction in the patients with autonomic failure, but not in the control subjects. 4. Values of plasma renin activity (PRA) were significantly depressed in patients with autonomic failure. However, PRA rose to values similar to those of control subjects while standing during the period of restricted sodium intake. At this time the patients with autonomic failure had a large orthostatic fall in blood pressure and creatinine clearance. 5. Aldosterone secretion rates were measured in three patients with autonomic failure at both levels of sodium intake and were considerably lower than the rates found in the control group. 6. A mineralocorticoid drug (9α-fludrocortisone; 2 mg/day), given on the last 2 days of restricted sodium intake, failed to correct fully the negative sodium balance of the patients with autonomic failure, since an excessive sodium excretion persisted during the period of recumbency at night. 7. These results demonstrate a severe defect in renal salt conservation in certain patients with autonomic failure. They suggest that the defect may not be entirely due to deficient secretion of mineralocorticoid hormones.

Comparison of the renin response to dopamine and noradrenaline in normal subjects and patients with autonomic insufficiency.

1. The effect on plasma renin activity (PRA) of dopamine and noradrenaline infusions was studied in three patients with Shy—Drager syndrome, three patients with Parkinson's disease and normal autonomic reflexes, and three healthy volunteers. The patients with the Shy—Drager syndrome had functional evidence of a peripheral lesion of the sympathetic nervous system and subnormal PRA on a controlled sodium intake. 2. In all subjects catecholamines were infused step-wise for 4 min until a 30% rise in systolic blood pressure occurred. 3. In each subject, PRA fell after noradrenaline but rose after dopamine. The mean fractional increase in PRA after dopamine was no less in the Shy—Drager patients than in the control groups. 4. The results suggest, first, that stimulation of dopamine receptors can release renin, and secondly, that inadequate renin stores cannot explain the low PRA found in our patients with autonomic failure.