Objectives-Urinary dysfunction and orthostatic hypotension are the prominent autonomic features in multiple system atrophy (MSA). A detailed questionnaire was given and autonomic function tests were performed in 121 patients with MSA concerning both urinary and cardiovascular systems. Methods-Replies to the questionnaire on autonomic symptoms were obtained from 121 patients including three clinical variants; olivopontocerebellar atrophy (OPCA) type in 48, striatonigral degeneration (SND) type in 17, and Shy-Drager type in 56. Urodynamic studies comprised measurement of postmicturition residuals, EMG cystometry, and bethanechol injection. Cardiovascular tests included head up tilt test, measurement of supine plasma noradrenaline (norepinephrine,NA), measurement of R-R variability (CV R-R), and intravenous infusions of NA and isoproterenol. Results-Urinary symptoms (96%) were found to be more common than orthostatic symptoms (43%) (p<0.01) in patients with MSA, particularly with OPCA (p<0.01) and SND (p<0.01) types. In 53 patients with both urinary and orthostatic symptoms, patients who had urinary symptoms first (48%) were more common than those who had orthostatic symptoms first (29%), and there were patients who developed both symptoms simultaneously (23%). Post-micturition residuals were noted in 74% of the patients. EMG cystometry showed detrusor hyperreflexia in 56%, low compliance in 31%, atonic curve in 5%, detrusor-sphincter dyssynergia in 45%, and neurogenic sphincter EMG in 74%. The cystometric curve tended to change from hyperreflexia to low compliance, then atonic curve in repeated tests. Bethanechol injection showed denervation supersensitivity of the bladder in 19%. Cardiovascular tests showed orthostatic hypotension below -30 mm Hg in 41%, low CV R-R below 1.5 in 57%, supine plasma NA below 100 pg/ml in 28%, and denervation supersensitivity of the vessels ( in 73%; 2 in 60%) and of the heart ( 1 in 62%). Conclusion-It is likely that urinary dysfunction is more common and often an earlier manifestation than orthostatic hypotension in patients with MSA, although subclinical cardiovascular abnormalities appear in the early stage of the disease. The responsible sites seem to be central and peripheral for both dysfunctions. (J Neurol Neurosurg Psychiatry 2000;68:65-69) Keywords: autonomic dysfunction; multiple system atrophy; orthostatic hypotension; urinary incontinence; urodynamic study Multiple system atrophy (MSA)c is known to have urinary disturbance.1 2 Some male patients with MSA have undergone urological surgery for prostatic hypertrophy before the correct diagnosis had been made. The results of the surgery are often transient or unfavourable because of the progressive nature of this disease. Previously we investigated urinary function in 86 patients with MSA considering three clinical variants; striatonigral degeneration (SND), sporadic olivopontocerebellar atrophy (OPCA), and the Shy-Drager syndrome. 3 The study showed that 4% of the patients with OPCA type, 16% with SND type, and 5...
A nationwide survey of patients in Japan with spinocerebellar degenerations (SCD), including SDS and SND, was conducted from 1988 to 1989. The survey consisted of two parts. The first revealed that the estimated total number of patients with SCD in Japan was 5,050 (range: 4,100–6,000) with an estimated prevalence of 4.53 per 100,000 in 1987. The second part investigated the neurological and functional status of patients with SCD. The percentages of those belonging to each subtype of SCD were: OPCA; 34.4%, LCCA; 15.2%, MHCA; 12.6%, HHCA; 7.5%, SDS; 7.0%, HSP; 3.9%, DRPLA; 2.5%, FA; 2.4%, MJD; 2.0% and SND; 1.5%. Compared with European epidemiological studies Japan had a higher proportion of non‐hereditary types of SCD. Various clinical features of SCD subtypes were compared grouped by pathological lesion and heredity. HHCA and LCCA: cerebellar ataxia predominated in all stages, and neurological signs other than cerebellar ataxia were rare. MHCA, DRPLA and MJD: inthe early phase ataxia was the most common symptom in MHCA, the AC form of DRPLA and MJD, but ataxia was less common and chorea or epilepsy were often observed in ME and PH forms of DRPLA. Other frequently observed clinical features were parkinsonian rigidty in MHCA, abnormal movements and posture in DRPLA and MJD, and disturbances of eye movements in MHCA, the AC form of DRPLA and MJD. OPCA, SDS and SND: dominant clinical features were cerebellar ataxia in OPCA, autonomic disturbance in SDS, and parkinsonian rigidity in SND. FA and HSP: both were rare in Japan. Clinical features related to supra‐supinal lesions were frequently observed in FA. Functional status of SCD: the severity of illness was significantly associated with the level of independence in each item of ADL. Activities not requiring dynamic balance were performed independently for a longer period than those requiring dynamic balance. Among SCD subtypes, functional prognosis was poorest in non‐hereditary, multi‐systemic types (OPCA, SDS and SND) followed by hereditary multi‐systemic types (MHCA, DRPLA and MJD), and better in spinal types (FA and HSP) and cerebellar types (HHCA and LCCA).
A micturitional history of unselected 110 patients with Parkinson's disease revealed that 66 (60%) had urinary symptoms such as irritative in 28%, obstructive in 11%, and both symptoms in 21%. The frequency of urinary symptoms statistically correlated with severity of the disease, but not with the duration of illness and no sexual difference was noted. A urodynamic study was conducted in 39 patients and 7 had residual urine of 30 ml or more, 19 had detrusor hyperreflexia, 19 had a small bladder capacity and only 1 had detrusor‐sphincter dyssynergia. The results indicate that the disturbed urine storage is more frequent and to a severer degree than that of urine evacuation in Parkinson's disease.
A 37-year-old woman with a 5-year history of multiple sclerosis is reported. She began having recurrent syncope even in the sitting position; other neurological features included hiccup, faciooro-lingual flushing and clumsiness of the hands. She had alternating Horner's syndrome, mild hypoalgesia of the right face, exaggerated deep tendon reflexes of the upper extremities, decreased deep sensation and ataxia of the upper extremities, and incomplete transverse myelopathy with a T4 sensory level. Head-up tilt testing confirmed orthostatic hypotension with relative preservation of the heart rate increase. Magnetic resonance imaging indicated abnormal intensities in the paramedian tegmentum and base of the medulla, which may have been additionally responsible for orthostatic hypotension. Steroid pulse therapy and L-threo-3,4-dihydroxyphenylserine caused regression of brainstem signs and reduced syncopal attacks.
We describe the findings of urodynamic studies, together with blood pressure and heart rate monitoring, in five patients with micturition syncope. All patients had almost normal storage and evacuation function and no evidence of prostate hypertrophy. Conventional head-up tilt testing with an empty urinary bladder caused no change in arterial blood pressure, but a moderate increase in heart rate. Urinary bladder filling caused minimal increases of the arterial pressure and heart rate. The sitting posture with a distended bladder caused mild orthostatic hypotension. Urinary bladder evacuation caused a fall in arterial pressure with a decrease in heart rate. These responses were similar to those described in vasovagal syncope. The central mechanism for the initiation of urinary evacuation, or sensory input from the lower urinary tract, may trigger micturition syncope.
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