ARTICLE: Micturitional disturbance in pure autonomic failure

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Neurology

Volume 54 . Number 2 . January 25, 2000

Copyright © 2000 American Academy of Neurology

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Brief Communications

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Micturitional disturbance in pure autonomic failure

R. Sakakibara MD, T. Hattori MD, T. Uchiyama MD, M. Asahina MD, T. Yamanishi

MD

From the Departments of Neurology (Drs. Sakakibara, Hattori, Uchiyama, and

Asahina) and Urology (Dr. Yamanishi), Chiba University, Chiba, Japan.

Received February 2, 1999.

Accepted in final form September 11, 1999.

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Address correspondence and reprint requests to Dr. Ryuji Sakakibara,

Department of Neurology, Chiba University, 1-8-1 Inohana Chuo-ku, Chiba

260-8670 Japan.

Article abstract We obtained micturitional histories and performed

urodynamic studies in six patients with pure autonomic failure. All patients

had urinary symptoms. Urodynamic studies showed postmicturition residuals in

two, small bladder capacities in two, detrusor hyperreflexia in four, low

bladder compliance in two, detrusor-external sphincter dyssynergia in one,

neurogenic sphincter electromyography in three, and denervation

supersensitivity of the bladder in two. Micturitional disturbance is a

common feature in pure autonomic failure because of peripheral and central

types of abnormalities.

Key words: Pure autonomic failure; Neurogenic urinary incontinence;

Urodynamic study; Detrusor hyperreflexia

Introduction

Pure autonomic failure (PAF) is an idiopathic, sporadic disorder

characterized by orthostatic hypotension usually with evidence of more

widespread autonomic failure. [1] [2] No other neurologic features are

present, such as apparent parkinsonism or cerebellar or pyramidal signs,

distinguishing this disorder from autonomic failure with multiple system

atrophy (AF with MSA) or autonomic failure with PD (AF with PD). [1] [2]

Micturitional disturbance is described as one of the features in PAF.

However, few reports are available of urodynamic studies in PAF that show

detrusor hyperreflexia [3] and detrusor areflexia with denervation

supersensitivity. [4] We describe the results of our detailed micturitional

histories and urodynamic studies in PAF.

Methods.

We reviewed the records of six patients with PAF according to the published

criteria. [1] [2] They included five men and one woman, with mean age at

onset of 59 years (range 46 to 66 years) and mean duration of 8 years (range

4 to 18 years) (table). None of the patients had cerebellar or pyramidal

signs. Four of six patients had increased rigidity of the wrist and ankle in

response to contralateral muscle activation, which appeared 8.8 years (range

3 to 16 years) after the onset of autonomic symptoms. However, they lacked

resting tremor, postural instability, or other evidence of PD. None of the

patients were bedridden. All six patients had orthostatic hypotension, and

mean systolic pressure decrease was 44 mm Hg (34 to 73 mm Hg) (normal <20 mm

Hg) with minimal or no heart rate increase. Mean supine plasma noradrenaline

level was 55.6 pg/mL (10 to 87 pg/mL) (normal >100 pg/mL). Mean coefficient

of variation of the R-R interval was 0.7 (0.34 to 1.77) (normal >1.5). Mean

increment of blood pressure was 76 mm Hg (range 53 to 118) (normal 30 mm Hg)

in response to noradrenaline infusion at the rate of 0.1 mg/kg/min for 6

minutes, suggestive of denervation supersensitivity of the vessels. Brain CT

scan and MRI showed no evidence of atrophy of the cerebellum or the

brainstem, nor abnormal intensity in the putamen suggesting MSA. There was

no abnormality in the EKG, chest roentgenogram, blood chemistry (including

blood sugar), or urinalysis. None of the men had apparent prostate

hypertrophy by ultrasound echography.

Table 1. Patients and micturitional symptoms Characteristic Patient no.

1 2 3 4 5 6

Age at onset, y/sex 59/M 64/F 66/M 64/M 56/M 46/M

Initial symptoms Impotence Orthostatic syncope Orthostatic faintness Voiding

difficulty, constipation Impotence Orthostatic faintness

Disease duration, y 4 4 6 9 10 18

Appearance of minimum rigidity, y after onset - 3 6 - 10 16

Autonomic signs and symptoms

Orthostatic syncope + + + + + +

Horner's syndrome - - + - - -

Laryngeal stridor - - - - - -

Perspiratory abnormality + + + + - +

Erectile dysfunction + N/A ? ? + +

Constipation + + + + + +

Micturitional symptoms

Nocturnal frequency + + + + + +

Diurnal frequency - - - - + -

Urgency - + + + + +

Incontinence Stress - Urge - - -

Hesitancy/prolongation + + + + - +

N/A = not applicable.

Detailed histories of urinary symptoms were obtained from all patients.

Urodynamic studies consisted of measurement of postmicturition residuals,

urethral pressure profilometry, and water cystometry with simultaneous

external sphincter electromyography (EMG). A bethanechol test was performed

as follows. After filling with 100 mL of water, 2.5 mg of bethanechol

chloride was injected subcutaneously. Denervation supersensitivity of the

detrusor is defined as detrusor pressure increase over 15 cm H2 O during a

30- minute observation.

Results.

Results of micturitional histories.

All of the patients had urinary symptoms (see table). The most common

urinary symptom was nocturnal urinary frequency (more than twice a night) in

six patients, followed by sensation of urgency in five and voiding

difficulty (urinary hesitation, prolongation, straining, or sensation of

residual urine) in five. Urinary incontinence was found in two patients,

including urge type in one and stress type in one. The proportion of storage

symptoms (e.g., urgency, frequency) over voiding symptoms (e.g., hesitation,

prolongation) is six/five in the patients. One patient (Patient 4) was shown

to have urinary symptoms at the onset of disease, whereas the other patients

had urinary symptoms after the appearance of impotence or orthostatic

faintness. Storage and voiding symptoms emerged almost at the same time in

five patients.

Results of urodynamic studies.

Two of six patients had residual urine of 126 mL (Patient 5; 10 years'

duration) and 320 mL (18 years' duration). Urethral pressure profilometry

was performed in three patients, and maximum urethral closure pressure was

normal in all of them. Water cystometry showed decreased first sensation in

one (Patient 4; 20 mL) and decreased bladder capacity in two (Patient 3, 150

mL; Patient 4, 80 mL). Four patients (Patients 2, 3, 4, and 5) had detrusor

hyperreflexia, and two (Patients 1 and 6) had low bladder compliance. The

bethanechol test was performed in three of the patients and showed

denervation supersensitivity of the bladder in two (Patients 1 and 6 of

Patients 1, 5, and 6). External sphincter EMG showed that one patient

(Patient 6) had detrusor-sphincter dyssynergia, and none had uninhibited

sphincter relaxation. EMG of the external urethral sphincter was performed

in four patients, and neurogenic motor unit potentials were found in three

of them (Patients 1, 2, and 5).

Discussion.

PAF is clinically characterized by widespread autonomic failure without CNS

involvement. [1] [2] None of our patients had cerebellar or pyramidal signs.

Four of the patients had minimum induced rigidity that appeared 8.8 years

after the onset of autonomic symptoms. However, they lacked other evidence

suggestive of AF with PD or AF with MSA. The current study showed that all

six patients had urinary symptoms, indicating the frequent occurrence of

micturitional disturbance in patients with PAF. The most common urinary

symptom was nocturnal urinary frequency in six patients, followed by

sensation of urgency in five and voiding difficulty in five, and urinary

incontinence was found in two patients (33%). Residual urine was found in

two (25%) (both over 100 mL and noted 10 years after onset of the disease),

and none were in a state of urinary retention. Micturitional disturbance in

PAF can be compared with that in MSA. In our previous study, [5] 98% of 86

patients with MSA had micturitional disturbance, including urinary

incontinence in 56% and residual urine in 79%. Residual urine over 100 mL or

retention was found in 51% of the patients. The results indicate that

micturitional disturbance in PAF seems to be as common but less severe than

that in MSA, probably reflecting slower progression of the disease [2] and

less vulnerability of the urinary innervation in PAF.

Cystometry showed that two patients with PAF had low bladder compliance,

indicating a preganglionic lesion of the pelvic nerves. The bethanechol test

showed that two of three patients studied had denervation supersensitivity

of the detrusor, indicating postganglionic lesion of the pelvic nerve. [4]

These findings match the pathologic reports of neuronal cell loss in the

intermediolateral (IML) columns of the spinal cord, [4] [6] [7] Lewy bodies

in the IML cells [7] and in the bladder wall, [6] and the physiologic

studies suggesting postganglionic abnormalities in PAF. [2] EMG of the

external urethral sphincter showed neurogenic findings in three of four

patients. This is evidence of pudendal nerve dysfunction. [8] This finding

suggests the involvement of the sacral Onuf's nucleus or its fibers with the

external sphincter in PAF. The urodynamic study also showed detrusor

hyperreflexia in four of six (66%) patients, indicating the frequent

occurrence of supranuclear parasympathetic lesions in this disorder. [3]

External sphincter EMG showed that one patient had detrusor-sphincter

dyssynergia, indicating a supranuclear pudendal lesion. The locus ceruleus,

known as the pontine micturition center, [9] [10] and nigrostriatal

dopaminergic system, both having a role in regulating micturition in

experimental animals and in humans, [9] [10] are known to be involved in

this disorder. [3] [6] Lesions in these sites may contribute to the

supranuclear type of micturitional disturbance in PAF. From these results,

the responsible sites of micturitional disturbance in PAF seem to be both

peripheral and central nervous system regulating the lower urinary tract.

Our previous study in MSA [5] showed detrusor hyperreflexia in 62%, low

bladder compliance in 28%, bethanechol supersensitivity in 75%,

detrusor-sphincter dyssynergia in 32%, and neurogenic sphincter EMG in 65%

of the patients, which resemble those in PAF. However, involvement of the

evacuating function seems to be less severe in patients with PAF.

References

1. The Consensus Committee of the American Autonomic Society and the

American Academy of Neurology. Consensus statement on the definition of

orthostatic hypotension, pure autonomic failure, and multiple system

atrophy. Neurology 1996; 46: 1470. full text

2. Low PA, Bannister R. Multiple system atrophy and pure autonomic failure.

Low PA, Clinical autonomic disorders. 2nd ed. Philadelphia:

Lippincott-Raven, 1997: 555-575.

3. Terao Y, Takeda K, Sakuta M, Nemoto T, Takemura T, Kawai M. Pure

progressive autonomic failure: a clinicopathological study. Eur Neurol 1993;

33: 409-415. abstract

4. Santangelo L, Mandarino G, de Biase L, Pesce F, Giacomini P. Multisystem

evaluation in one case of pure autonomic failure. Funct Neurol 1991; 6:

275-278. abstract

5. Sakakibara R, Hattori T, Tojo M, Yamanishi T, Yasuda K, Hirayama K.

Micturitional disturbance in multiple system atrophy. Jpn J Psychiatr Neurol

1993; 47: 591-598.

6. Hague K, Lento P, Morgello S, Caro S, Kaufmann H. The distribution of

Lewy bodies in pure autonomic failure: autopsy findings and review of the

literature. Acta Neuropathol 1997; 94: 192-196. abstract

7. RH, Lee de J, Oppenheimer DR, Spalding JMK. Autonomic failure

with orthostatic hypotension due to intermediolateral column degeneration: a

report of two cases with autopsies. Q J Med 1966; 35: 276-292. citation

8. Fowler CJ. Pelvic floor neurophysiology. Binnie C, Clinical

neurophysiology. Oxford: Butterworth-Heinemann, 1995: 233-250.

9. Blok BFM, Holstege G. A PET study on brain control of micturition in

humans. Brain 1997; 120: 111-121. abstract

10. Sakakibara R, Fowler CJ, Hattori T. Voiding and MRI analysis of the

brain. Int Urogynecol J 1999; 10: 192-199.

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