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It is difficult to differentiate multiple system atrophy (MSA) from Parkinson's disease (PD) at least in the early stage. Urodynamic study (UDS) is useful in differentiating MSA from PD. We aimed to clarify which UDS parameter was useful in differentiating MSA from PD.We retrospectively reviewed 273 cases and performed UDS and external anal sphincter electromyography (EAS-EMG) in patients with MSA (n=182) and PD (n=91). We analyzed the utility of UDS parameters, including postvoid residuals (PVR), detrusor overactivity (DO), degree of bladder contraction, and mean duration of motor unit potentials (MUPs) in EAS-EMG, for differentiating MSA from PD.PVR150ml during free-flow study strongly indicated MSA rather than PD (OR 8.723, 95% CI 2.612-29.130, p0.001). 'Weak detrusor' also suggested MSA, but it was not a statistically significant indicator (OR 10.598, 95% CI 0.359-312.473, p=0.172). DO and neurogenic changes in EAS-EMG (mean duration of MUPs10ms) did not significantly contribute to the differentiation of MSA from PD.PVR150ml during free-flow study might be more useful than other UDS parameters in clinically differentiating MSA from PD.

Objective Although it is well known that patients with multiple system atrophy (MSA) cerebellar dominant type (MSA-C) show severe autonomic dysfunction, the relationship between autonomic and motor dysfunction remains uncertain. Previously we reported that severe urinary voiding dysfunction is useful in differential diagnosis of MSA and other diseases. Herein, we aimed to clarify the relationship between the severity of motor dysfunctions and urinary dysfunction. Method This study is a retrospective review of 46 patients with MSA-C diagnosed according to Gilman's second consensus criteria. The severity of motor dysfunctions was evaluated using International Cooperative Ataxia Rating Scale (ICARS). Urinary voiding dysfunction was evaluated by measuring post-void residual (PVR). The mean duration of motor unit potentials in external anal sphincter muscles on electromyography, which represents the severity of neurodegeneration in Onuf's nucleus, was also examined. Results The mean age of patients was 63.8 ± 8.2 years and mean disease duration was 3.0 ± 1.9 years. The mean ICARS score was 40.1 ± 14.7. The mean PVR was 119.1 ± 102 ml and the mean duration of motor unit potentials (MUPs) in anal sphincter electromyography was 9.2 ± 2.2 ms. The correlation coefficient between ICARS and PVR was 0.093 (p = .539), and between ICARS and mean duration of MUPs was 0.105 (p = .811). A significant positive correlation (r = 0.296, p = .005) was noted between PVR and the mean duration of MUP. Conclusion Motor and urinary dysfunctions were not correlated in MSA-C.

Background Limited attention has been paid to the relationship between bladder dysfunction and motor disorder in multiple system atrophy (MSA). Objective We aimed to correlate pressure-flow urodynamic parameters with video-gait analysis parameters in MSA. Methods We recruited 34 patients with MSA: 20 men, 14 women; age 64.0 + − 7.9 years; disease duration 2 years (1–4 years). Nineteen cases had the cerebellar form, and 15 had the parkinsonian form; the patients were taking levodopa 300 mg/day (100–400 mg). All patients underwent pressure-flow urodynamics (parameters: detrusor overactivity [noted in 72%] and Watts factor) and video-gait analysis (parameters: time and the number of strides taken to walk 5 m (simple task) and time for timed up and go (complex task). Statistical analysis was done using Student's t-test to analyze the relation between detrusor overactivity and gait, and Spearman's rank correlation coefficient test to analyze the relation between the remaining parameters and gait. Results We found no relation between filling-phase urodynamics (detrusor overactivity) and video-gait analysis parameters. Also, we found no relation between voiding-phase urodynamics (Watts factor, reflecting detrusor power) and all three video-gait analysis parameters in our MSA patients. Conclusion The fact that neither detrusor overactivity nor the Watts factor was related with motor disorders in the present study suggests that bladder dysfunction occurs independently from motor disorder in MSA.

Thalamic infarction presenting with heat anesthesia is rare. A 62-year-old man developed acute heat anesthesia and deep sensory disturbance in the right half of his body, but sensation for cold and pain was preserved. The resolution of these symptoms was accompanied by the gradual development of central dysesthesia. Magnetic resonance imaging (MRI) and computed tomography (CT) findings showed a small infarction in the left thalamic principal somatosensory nucleus (ventral caudal) and pulvinar. Single-photon emission CT showed hypoperfusion in the mid-cingulate cortex (mid-CC) and supplementary motor area (SMA), however, the primary and secondary somatosensory cortices were spared. Somatosensory-evoked potential findings were normal. The disruption of spinothalamocortical projection to the mid-CC and SMA is attributable to the development of central dysesthesia in the present case.

Objectives: Machado–Joseph disease (MJD), or hereditary spinocerebellar ataxia type 3, is the most common dominantly inherited ataxia. However, lower urinary tract (LUT) dysfunction in MJD has not been fully delineated. We investigated LUT dysfunction in MJD by clinical-urodynamic observations. Methods: In 24 genetically diagnosed MJD, we recruited all 11 patients with LUT symptoms (six men, five women; age, 18–61 [mean 48] years; disease duration, 2–24 [mean 9] years; voiding difficulty, 7, urinary incontinence, 4). Urodynamic studies consisted of uroflowmetry, measurement of post-void residuals and electromyography (EMG)-cystometry. Neurophysiology tests consisted of motor unit potential (MUP) analysis of the sphincter and extremity muscles, tibial nerve somatosensory evoked potentials (SEP) and nerve conduction studies (NCS) of the extremities. Results: Urodynamic abnormalities were seen in all 11 patients studied. Maximum or average flow rate was decreased in five. Post-void residual was noted in three but residual urine volume >100 ml was noted in only one patient. Maximum urethral closure pressure was low in one and high in one of five patients studied. EMG-cystometry during filling showed detrusor overactivity in five, impaired bladder sensation in four, low compliance detrusor in one, uninhibited sphincter relaxation in one and incompetent urethra in one. Voiding phase abnormalities included detrusor areflexia in three and detrusor-sphincter dyssynergia in two. Bethanechol supersensitivity of the bladder was noted in one of three patients studied. Bulbocavernosus reflex was absent in two of five patients studied. MUP analysis showed neurogenic changes in six of nine sphincter muscles and in all six extremity muscles studied. Five patients had prolonged or absent cortical response in SEP and four had sensory axonal neuropathy in NCS, which were relevant to the impaired bladder sensation. Conclusion: In the present study, a half of MJD patients had LUT symptoms and they showed various urodynamic abnormalities. Detrusor overactivity, impaired bladder sensation, and neurogenic sphincter EMG were common findings, and large post-void residuals were rare. These findings are relevant to central and peripheral nervous system pathology of MJD.

The effects of combined treatment with low-density lipoprotein (LDL)-apheresis, chenodeoxycholic acid (CDCA) and 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase inhibitor were studied in 2 patients with cerebrotendinous xanthomatosis. Patient 1 was initially treated with LDL-apheresis alone: serum cholestanol levels decreased by 50% after each apheresis, but returned to their initial levels within 2 weeks. After an addition of CDCA administration, the serum cholestanol levels steadily decreased, resulting in slight improvement of neurological symptoms. Patient 2 received a combined treatment with LDL-apheresis, CDCA and HMG-CoA reductase inhibitor. This combination showed less LDL-apheresis-dependent fluctuation and more rapid decrease of serum cholestanol levels than those in Patient 1, resulting in improvement and stabilization of the symptoms. Our results suggest that LDL-apheresis in combination with CDCA and HMG-CoA reductase inhibitor may have beneficial effects and can be one of the treatment options.

We studied a correlation of urinary dysfunction with nigrostriatal dopaminergic deficit in Parkinson's disease (PD) by single-photon emission computed tomography (SPECT) imaging of dopamine transporter with [123I]-2beta-carbomethoxy-3beta-(4-iodophenyl)tropane (beta-CIT). Eleven patients were enrolled in the study, including four men and seven women, with a mean age of 64 years. Seven patients had urinary symptoms 1-5 years after the onset of motor disorder, which included nighttime frequency in six, urinary retardation in four, daytime frequency in one and urge urinary incontinence in one. Using a SPECT camera, the ratio specific to nondisplaceable [123I]-beta-CIT uptake, designated as "striatal V3" was obtained in the caudate, anterior and posterior putamen 24 h after the tracer injection. The striatal V3 was compared in patients with and without urinary dysfunction, and between men and women, using unpaired Student's t-test. Correlation of motor dysfunction and duration of illness with urinary dysfunction, was also analyzed. In the patients, there was a reduction of [123I]-beta-CIT binding in the striatum on both sides, particularly in the putamen contralateral to the affected body side. The striatal V3 of the caudate (p

Isolated facio-lingual hypoesthesia and weakness is rare. We describe a case of isolated facio-lingual hypoesthesia and weakness after a hemorrhagic infarct localized at the contralateral operculum. A 66-year-old woman developed acute onset of facio-lingual hypoalgesia, hypoesthesia, and weakness, with no such symptoms being observed in other parts of the body. Brain magnetic resonance imaging showed a subacute hemorrhagic infarct in the right frontal operculum, which spread slightly to the right temporo-parietal operculum. 123 IMP-SPECT showed hypoperfusion in the right fronto-temporo-parietal operculum, as detected by MRI, without apparent diaschisis within the brain. Neuroimaging findings for our patient suggested the involvement of the primary somatosensory-motor cortices (S1 and M1) and the secondary somatosensory cortex (S2), which receive trigemino-thalamo-cortical pathways.

Micturitional histories and urodynamic studies were performed in 14 patients with syringomyelia. Eleven patients were revealed to have urinary symptoms including difficulty of voiding in 8, urinary retention in 3, nocturnal and diurnal urinary frequency in 3, urinary incontinence in 2, and sense of urgency and enuresis in one. These urinary symptoms appeared after 5.3 years (ranging from 2 months to 13 years) from the occurrence of the neurological symptoms. Urodynamic studies revealed detrusor hyperreflexia in 7, detrusor areflexia in 4, detrusor-sphincter dyssynergia in 4 and uninhibited sphincter relaxation in 2 patients. Analysis of the motor unit potentials of the external sphincter revealed 5 of 6 patients had high amplitude or polyphasic neurogenic changes. Supranuclear as well as nuclear types of parasympathetic and somatic nerve dysfunctions seemed to be responsible for micturitional disturbance in our patients with syringomyelia. During the follow-up period of 2 to 63 months, urinary symptoms gradually improved in 4 of 6 patients after syringosubarachnoid shunts and in 3 of 4 after alpha-adrenoreceptor blocking agents.

We identified factors that predict the disappearance of the triad of symptoms (gait disturbance, cognitive impairment and urinary incontinence) of idiopathic normal pressure hydrocephalus (iNPH) following shunt surgery in this study. We classified 71 patients with iNPH into those whose objective symptoms disappeared (disappearance group) or remained (residual group), for each of the triad symptoms 12 months after shunt surgery. Logistic regression analyses were used to identify the predictors of the disappearance of symptoms among 10 variables before shunt surgery (e.g., age, sex, severity of symptoms, Evans index, cerebrospinal fluid (CSF) pressure, CSF stasis on computerized tomographic cisternography, regional cerebral blood flow on single photon emission computed tomography, three kinds of prior diseases). For each of the triad symptoms, mild symptoms before shunt surgery were predictors of the disappearance of the symptom. Young age was also a predictor of the disappearance of gait disturbance. When the analysis was conducted using subscores of the Mini Mental State Examination, a successful visuoconstruction subtest and an absence of hypertension were predictors of the disappearance of cognitive impairment. None of the neuroimaging examinations predicted the disappearance of symptoms after shunt surgery in this study.

article i nfo Objectives: Whereas delayed gastric emptying is believed to be a causative factor for producing delayed-on and motor fluctuation in Parkinson's disease (PD), few studies have directly measured levodopa pharmaco- dynamics and gastric emptying together. In order to determine the relationship, we measured these two pa- rameters in a single PD patients cohort. Methods: Thirty-one patients with PD were enrolled in the study. They were 11 men and 20 women; age, 68.1±7.8 years; disease duration, 4.2±3.8 years; Unified Parkinson's Disease Rating Scale Part 3 Motor Score18.37±8.60;bowelmovementb3timesaweekin20;alltaking301 mg±94 mg/daylevodopa/carbidopa. All patients underwent levodopa pharmacokinetic study and the gastric emptying study using 13 C-octanoic acid expiration breath test. Statistical analysis was performed by Student's t-test and Mann-Whitney's U test. Results: Pharmacokinetic study showed that the plasma levodopa peak was at 2 hours in 42% (13/31 patients) whereas at 1 hour in 58% (18/31 patients), total of 50.7±16.4 min (mean±standard deviation) in all 31 pa- tients. The gastric emptying study showed that Tmax ( 13 C)>60 min was more common in patients with a plas- ma levodopa peak at 2 hours (14/18, 69%) than in those with a plasma levodopa peak at 1 hour (4/13, 22%) (pb0.05), total of 50.7±16.4 min in all 31 patients. Conclusion: We found a significant relationship between levodopa pharmacokinetics and gastric emptying in PD patients, suggesting that delayed gastric emptying is a causative factor for producing delayed-on in PD. There- fore, studies of improved gastric emptying in order to ameliorate delayed-on in PD are warranted.

Urinary retention is a frequent feature in patients with acute myelitis (AM). We here describe the result of a survey of 32 consecutive AM patients as to what extent of AM patients initially present with micturition disturbance. Among those, 3 patients (9%) were shown to have urinary retention as the initial sole manifestation, which was followed by dysesthesia of foot in 3 and leg weakness in 1; and the average interval between micturition disturbance and these second-appearing symptoms/signs was 11 days (8-15 days). Although initial presentation of urinary retention was not common, occurring in only 9% of all the AM patients, clinically it has relevance because patients who suffer urinary retention but have no obvious neurological symptoms may first see general physicians or urologists. In those patients, when obstructive disease has been excluded, AM should be considered.

Putaminal hemorrhage presenting pure sensory stroke is rare. We describe a case of left putaminal hemorrhage presenting contralateral hemisensory disturbance without hemiparesis. A 52-year-old man developed analgesia and thermoanesthesia in the right half of his body, but deep sensation was relatively well preserved. Neuroradiological and somatosensory evoked potential findings suggested that thalamocortical sensory pathways to the secondary somatosensory cortex (S2) were involved, whereas those to the primary somatosensory cortex (S1) were spared. In experimental animals, spinothalamic projections from the thalamic nucleus input directly to S2. In humans, thalamocortical pathways are still a subject of debate, but results of recent functional imaging studies suggest that the pathway of pain inputs directly to S2 and that of tactile sensation to S2 via S1. Our findings support these reports.

Micturitional histories were taken from 39 patients with acute brainstem stroke. Within 3 months from onset, 49% had irritative as well as obstructive urinary symptoms, the most common being voiding difficulty and nocturnal urinary frequency in 28%, followed by urinary retention in 21%. Urodynamic studies of 11 symptomatic patients revealed detrusor hyperreflexia in 73%, low compliance bladder in 9%, atonic cystometrogram in 27%, detrusor-sphincter dyssynergia in 45% and uninhibited sphincter relaxation in 27%. Three asymptomatic patients had normal urodynamic findings. Brain magnetic resonance images of the lesions of the symptomatic patients were concentrated in the dorsolateral pons including pontine reticular nucleus and the reticular formation adjacent to the medial parabrachial nucleus and the locus coeruleus. These regions seem to be mainly responsible for supranuclear types of pelvic and pudendal nerve dysfunction in our patients with brainstem stroke, corresponding to the pontine urinary storage and micturation center reported in animal studies.

Micturitional histories and urodynamic studies were performed in 72 acute hemispheric stroke patients. Within 3 months from the onset, 53% of the patients had urinary symptoms including irritative as well as obstructive, and the most common symptom was nocturnal urinary frequency in 36%, which was followed by urge urinary incontinence in 29% and difficulty of voiding in 25% of the patients. We found a correlation between micturitional disturbance with hemiparesis (p