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Biomedical subjects

C S Lu

Publications and source records attributed to C S Lu.

At least 37 records · Page 2Linked to original sources

Decreased dopamine transporter binding in Machado-Joseph disease.

UNLABELLED: The aim of this study was to use 99mTc-TRODAT-1 brain SPECT for investigation of the binding of dopamine transporter (DAT) in the nigrostriatal dopaminergic pathway of symptomatic Machado-Joseph disease (MJD) and to compare the results with the abnormal cytidylate, adenylate, and guanylate (CAG) expansion in the MJD1 gene and other clinical factors. METHODS: Ten symptomatic MJD patients (8 women, 2 men; age range, 20-71 y; mean age +/- SD, 36.4 +/- 10.6 y; mean duration of illness, 9.8 +/- 5.4 y) and 21 healthy volunteers (age range, 24-71 y; mean age, 47.6 +/- 20.1 y) were examined. Brain SPECT images were acquired 4 h after injection. The ratio of specific to nonspecific nigrostriatal 99mTc-TRODAT-1 binding was measured and compared with the clinical symptoms, duration of illness, and size of abnormal expanded CAG repeats. RESULTS: All nigrostriatal 99mTc-TRODAT-1 ratios were significantly lower in MJD patients than in healthy volunteers (P < 0.05). Discriminant function analysis of all MJD patients showed that the decreased binding of 99mTc-TRODAT-1 in the putamen was not significantly different from that in the caudate nucleus. Eight of 10 MJD patients had significantly decreased 99mTc-TRODAT-1 uptake. Of these 8, 2 had extrapyramidal signs and 6 had no obvious extrapyramidal signs. The other 2 patients, who had normal 99mTc-TRODAT-1 uptake, had no obvious extrapyramidal signs. CONCLUSION: Our findings indicate that 99mTc-TRODAT-1 brain SPECT is an appropriate method for evaluating damage to the nigrostriatal DAT in symptomatic MJD patients with and without extrapyramidal signs. The decreased binding of 99mTc-TRODAT-1 in the nigrostriatal dopaminergic pathway in symptomatic MJD patients correlates with the phenotype of extrapyramidal signs but not with the abnormal CAG repeat length, age at disease onset, or disease duration.

Adolescent↗

Dopamine deficiency in rubral tremor caused by midbrain hemangioma: case report.

To elucidate the nigrostriatal involvement in rubral tremor, we studied single photon emission computed tomography (SPECT) imaging with [2-[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3.2.1]oct-2- yl]methyl](2-mercaptoethyl)amino]ethyl]amino]ethanethiolato(3-)- N2,N2',S2,S2']oxo-[1R-(exo-exo)]-[99mTc]technetium ([99mTc]TRODAT-1) in a 70-year-old woman with a midbrain hemangioma. She had developed a slow tremor in her right arm and leg after an episode of hemorrhage at the age of 28. The tremor was 3 to 5 Hz in frequency at rest, which was enhanced by outstretching the limb and action. There was no rigidity or bradykinesia. Neurological examination also revealed mild palsy of the left oculomotor nerve. The magnetic resonance imaging (MRI) of the brain showed a small hemangioma in the left midbrain localized mainly in the substantia nigra. The [99mTc]TRODAT-1 SPECT imaging revealed significantly reduced [99mTc]TRODAT-1 uptake in the left caudate and putamen, but it was only mildly reduced in the right striatum. This reduction in uptake was even more severe than that of patients with Parkinson's disease, and indicated that the dopamine function was markedly impaired in the left nigrostriatal system. The tremor had not progressed over the years, and she responded moderately to treatment with levodopa. We concluded that the rubral tremor in the right extremities was probably caused by a dopamine deficiency in the left nigrostriatal system. This suggests that a dopamine deficiency secondary to the midbrain hemangioma might have contributed to the development of the rubral tremor in this patient.

Aged↗

Limb myorhythmia in association with hypertrophy of the inferior olive: report of two cases.

We report on 2 patients who uncommonly developed isolated limb myorhythmia in association with inferior olive hypertrophy (IOH) after an acute stroke in the brain stem. A slow tremor presented in the proximal upper limbs predominantly when at rest. It was aggravated by outstretched arms and by active hand movements. The surface electromyogram (EMG) recorded simultaneous activities over the agonist and antagonist muscles with a rate of 3.5 Hz and 2.5 Hz in 2 patients respectively. In the first patient, bilateral limb myorhythmia presented 12 months after the brain stem stroke, and both inferior olives were hypertrophic. In the second patient, unilateral limb myorhythmia developed in the left hand 7 months after right pontine hemorrhage, and only the right inferior olive was hypertrophic. These findings indicate that limb myorhythmia commencing after brain stem insult is anatomically and temporally related to hypertrophy of the contralateral inferior olive. Based on our 2 patients and previously reported cases, we propose that a possible causal relationship exists between limb myorhythmia and contralateral IOH, although its pathophysiological mechanisms remain to be established. We suggest that, similar to palatal myoclonus, isolated limb myorhythmia is within the clinical spectrum of IOH.

Brain Stem↗

Emission characteristics of VOCs from athletic tracks.

Dynamic and flow-through flux chambers are convenient tools for field measurements of gas or VOC emission flux from solid surfaces in the field. This study was undertaken to collect on site and quantify the emissions of volatile organic compounds (VOCs) released from athletic running tracks. Three typical types of tracks, one synthetic rubber and two tracks (types I and II) consisting mainly of polyurethane, were studied. They were all installed with adhesives and backings, both of which contributed significant amount of VOCs. VOCs released from the track surface were collected with a flux chamber and subsequently analyzed by a gas chromatograph/mass spectrometer (GC/MS). Also, for each track and at each selected time the emission flux and mass emission were measured on site under outdoor conditions over a period of 40 min. GC/MS analyses show that the VOCs emitted include 2-methyl furan, butanal, methyl ethyl ketone, benzene, heptane, methyl isobutyl ketone, toluene+octane, hexanal, nonane+ethylbenzene, xylenes+styrene, propyl benzene, decane, 1,3,5-trimethyl benzene, 1,2,4-trimethyl benzene, 1,2, 3-trimethyl benzene and undecane. Of these, hexanal was the common and principal compound for all three types of tracks. 2-Methyl furan and methyl isobutyl ketone were the characteristic compounds for the synthetic rubber and the type II of polyurethane tracks, respectively. In the field studies, no unique compounds were found in the type I of polyurethane tracks. For each of these three types of tracks the total-VOCs emission flux was correlated to the track age and track surface temperature. The results of multiple regression analysis showed good correlation. The type II polyurethane track had the highest decay rate, while the synthetic rubber track had the lowest decay rate. Two years after the track installation, the VOC concentrations measured at 1.5 m above the track, the breathing height of school children, were not significantly higher than the background levels.

Air Pollutants↗

Serial magnetic resonance imaging in patients with Balò's concentric sclerosis: natural history of lesion development.

We reviewed the magnetic resonance imaging scans from 22 serial studies of 5 patients with Balò's concentric sclerosis collected during the past 3 years. The data showed the concentric lesions did not occur simultaneously but developed step by step in a centrifugal direction. The development of lesions was preceded by an enhancing ring relatively devoid of demyelination and was followed by progressive demyelination occurring mainly at the inner aspect of the enhancement. The same process recurred on the edge of the previous enhanced zone. Thus, an appearance of concentric rings with alternating demyelinated and relatively myelin-preserved bands was formed.

Adult↗

Progressive myoclonic epilepsies syndrome (Ramsay Hunt syndrome) with mental disorder: report of two cases.

Ramsay Hunt syndrome (RHS) is a rare condition within the progressive myoclonic epilepsies syndrome (PME), with a triad of action myoclonus, grand mal seizure and severe cerebellar ataxia. There are few reports about the psychiatric disturbances associated with PME or RHS. The present study examines the evidence that RHS may accompany an organic mental syndrome, ethanol's effective suppression of myoclonus, and the possible resultant problem of alcohol dependence in RHS patients. Two brothers with the previous long-standing diagnosis of RHS and their mental symptoms of persecutory delusion and depression are reported, as well as the additional problem of alcohol dependence in one of them. The cerebellar dysfunction found in RHS may be associated with an underlying organic condition. Determination of the relationship between cerebellar dysfunction and psychosis in RHS will require further study. Although the mechanism of the suppression of myoclonus by alcohol remains unclear, patients should be allowed to drink socially, and alcohol consumption should not be totally prohibited. However, effective treatment of the problems of alcohol tolerance, abuse, or dependence requires the cooperation of both neurologists and psychiatrists.

Adult↗

Congenital hepatic arterioportal fistula complicated with gastrointestinal bleeding treated with transcatheter embolization: case report.

Congenital hepatic arterioportal fistula (HAVF) is extremely rare in children. We present a patient with congenital hepaticoportal arteriovenous fistula complicated with gastrointestinal bleeding treated using transcatheter arterial embolization. Our patient was the youngest (2 days old) case ever reported with congenital HAVF and the first one to receive arterial embolization for HAVF during childhood. The 3-year-old girl was suggested of having congenital HAVF using Doppler ultrasonography. However, her family refused further investigation, and she was lost to follow-up. Three years later, she was sent to our hospital due to melaena. Repeated ultrasonography revealed dilated intrahepatic portal vein with arterial flow demonstrated using Doppler imaging. No esophageal varices or gastric or duodenal ulcer was seen during endoscopy. Angiography showed a HAVF and transcatheter embolization was done simultaneously. Follow-up at one and two weeks post-embolization revealed no more shunt flow within the portal vein, though cystic like dilatation of the portal vein persisted, and no thrombosis was observed. This case emphasizes that transcatheter arterial embolization can be easily and successfully used for treating childhood congenital HAVF. Abnormal dilatation of the portal vein in children needs doppler evaluation and possibly angiography.

Arteriovenous Fistula↗

Intussusception in a child infected with enterovirus: case report.

Most intussusceptions in childhood are idiopathic. The association of viral infection has been suspected for decades. We report a case of ileocolonic intussusception in a child during a course of enterovirus infection. A 20-month-old girl with vomiting and irritable crying of 4 days' duration was found to have intussusception by sonography. She had suffered from herpangina one week earlier. Due to her peritoneal signs, she underwent surgical manual reduction. A cluster of enlarged lymph nodes in the intussuscipiens was noted preoperatively by high resolution ultrasound. Two swollen lymph nodes were removed for pathologic evaluation and examined for a viral genome by polymerase chain reaction (PCR) amplification. Panenterovirus base pairs were confirmed on the electrophoresis print. During postoperative sonographic follow-up, significant shrinkage of previous mesenteric lymphoid hyperplasia was observed. In this article, we present the first case of childhood intussusception in associated with enterovirus infection, in which the panenterovirus genome was identified in the patient's mesenteric lymph nodes. This was also the first evaluation of the role of mesenteric lymph nodes in intussusception by high resolution sonography.

Enterovirus Infections↗

Electrophysiological studies of early stage corticobasal degeneration.

We conducted electrophysiological studies in two Asian patients with probable corticobasal degeneration (CBD). The duration of illness from onset was 16 and 20 months, respectively. The clinical manifestations were markedly asymmetric and characterized by cortical sensory loss, apraxia, action myoclonus, action tremor, and akinetic-rigid parkinsonism. Neither patient responded to levodopa therapy. Simple photon-emission computed tomography (SPECT) study showed significantly decreased regional cerebral blood flow in the frontoparietal areas and thalamus opposite to the predominantly affected limb. A series of electrophysiological studies failed to identify giant somatosensory evoked potentials (SEPs), enhanced long latency electromyography (EMG) reflex, and cortical spikes preceding myoclonic jerk. However, the earliest cortical component of the median nerve SEP was exclusively enlarged in one patient and preserved with depression of the subsequent components in the other patient. Significantly shorter postmotor-evoked potential (MEP) silent period was found after the transcranial magnetic stimulation of the motor cortex in both patients. CBD is a unique clinical entity characterized by action myoclonus probably the result of the pathologic hyperexcitability of the motor cortex, based on a loss of inhibitory input from the sensory cortex.

Aged↗

Emotional symptoms are secondary to the voice disorder in patients with spasmodic dysphonia.

The aims of this study were to evaluate the emotional status and life quality of the patients with spasmodic dysphonia (SD) before and after botulinum toxin treatment, and to ascertain whether SD is a somatoform disorder. Ten patients with spasmodic dysphonia were injected unilaterally into the vocal cord with botulinum toxin. Before botulinum toxin treatment, two clinician's rating scales--Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Rating Scale (HARS), and three self-rating psychometrics--Zung's Self-Rating Depression Scale (SDS), Life Quality Scale (GHQ/QL-12), and Symptom Distress Checklist (SCL-90) were applied. Self-rating scales were also administered in 20 matched normal controls. The patients were reevaluated 1 month after botulinum toxin treatment. The Clinical Global Impression Scale (CGI) was also rated by the patients themselves and a speech pathologist. The mean scores of SD patients were significantly higher than that of controls in SDS, and subscales of somatization, obsessive-compulsive symptoms, depression, anxiety, and psychoticism in SCL-90. The mean score of GHQ/QL-12 was significantly higher in the control group. The scores of HDRS, SDS, GHQ/QL-12 and subscales of somatization, depression, and anxiety in SCL-90 showed significant improvement after botulinum treatment. In CGI, seven patients were rated as improved by patients themselves and the speech pathologist. The patients with SD had more anxiety, depression and somatization symptoms, and poor life quality than normal controls. Their emotional status and life quality improved after botulinum toxin treatment. The results suggest that the emotional symptoms of patients with SD are mainly secondary to voice disorder.

Adult↗

Clinical manifestations of 20 Taiwanese patients with paroxysmal kinesigenic dyskinesia.

INTRODUCTION: We compared the clinical manifestations and response to medications between familial and sporadic patients with paroxysmal kinesigenic dyskinesia (PKD), and also between patients with autosomal dominant (AD) and autosomal recessive (AR) inheritance. MATERIAL AND METHODS: This retrospective cohort study included 9 familial and 11 sporadic Taiwanese patients with PKD diagnosed during a 10-year period at one of two hospitals. The mean duration of follow-up was 3.8 +/- 2.7 years. Each patient was interviewed and their medical records, as well as videotape recordings of PKD attacks in 6 patients, were used for analysis. Patients were treated with either carbamazepine or phenytoin, and the efficacy of sodium valproate was tested in 5 patients. RESULTS: No single distinguishing feature in terms of clinical manifestations or therapeutic response was found to differentiate among familial, and sporadic cases, or between AD and AR inheritance. Carbamazepine and phenytoin were superior to sodium valproate in treating both familial and sporadic PKD patients, and both drugs resulted in almost complete remission of attacks. CONCLUSION: Our findings indicate that the sporadic and familiar forms of PKD, as well as the AR and AD inherited types, are similar in terms of clinical manifestations and response to treatment. The functional status and prognosis of our Taiwanese patients suggest that PKD is a relatively benign entity.

Adolescent↗

Long-term progression in chronic manganism: ten years of follow-up.

We studied the long-term clinical course of five patients with chronic manganese intoxication. The mean scores of the King's College Hospital Rating Scale for Parkinson's disease increased from 15.0 +/- 4.2 in 1987 to 28.3 +/- 6.70 in 1991 and then to 38.1 +/- 12.9 in 1995. The deterioration was most prominent in gait, rigidity, speed of foot tapping, and writing. Tissue concentrations of manganese in blood, urine, scalp hair, and pubic hair returned to normal. Follow-up MRIs did not show paramagnetic high-signal intensity on T1-weighted images. The data indicate that clinical progression in patients with manganese parkinsonism continues even 10 years after cessation of exposure.

Chronic Disease↗

Childhood-onset autosomal-dominant limb-girdle muscular dystrophy with cardiac conduction block.

We report childhood-onset autosomal-dominant limb-girdle muscular dystrophy (LGMD) in a Chinese family with complete atrioventricular conduction block in the adult members. Six patients, including 5 men and 1 woman with an age of onset from 3 to 7 years, were affected. The grandfather had exercise intolerance since childhood and complete heart block with pace-maker placement at age 52. Three siblings had proximal muscle weakness and/or wasting since age 5 and heart block in their 40s. Two grandsons at the ages 7 and 3 showed exercise intolerance, and proximal muscle weakness and wasting. Sinus bradycardia was present in the elder grandson. Muscle enzymes were elevated in 3, particularly in childhood. Muscle biopsies from the proband showed myopathic changes with fatty degeneration, whorled fibers, and rimmed vacuoles. In adult patients, muscle magnetic resonance imaging scans disclosed atrophic changes and fatty degeneration in the gluteal, quadriceps, adductors, hamstrings, gastrocnemius, and soleus muscles, while in child probands the early atrophic changes were seen in the gluteal and hamstrings muscles. We conclude that this distinct family is characterized by childhood-onset autosomal-dominant LGMD with heart block and that prevention of sudden death in these patients is important.

Adolescent↗

Mechanical properties of acrylic bone cement containing PMMA-SiO2 hybrid sol-gel material.

An organic-inorganic hybrid material, poly(methyl methacrylate) (PMMA)-SiO2 (SiO2 content of 72 wt%), was prepared by incorporating PMMA structure units covalently into an SiO2 glass network via the sol-gel approach. The hybrid sol-gel material PMMA-SiO2 was subsequently used as the solid powder component of bone cement and its mechanical properties were evaluated. The effects of the addition of tricalcium phosphate (TCP), hydroxyethyl methacrylate (HEMA), and ethylene glycol dimethacrylate (EGDMA) on the properties of the sol-gel hybrid bone cement were also investigated. The influence of these components on the temperature rise during polymerization was discussed. It was found that the new bone cement containing PMMA-SiO2 hybrid sol-gel material had higher modulus than that of Simplex-P bone cement. The addition of TCP in the new bone cement increased the Young's modulus and the polymerization time; the inverse was observed for the tensile, bending, and compressive strengths, and the polymerization temperature. The addition of HEMA and EGDMA in the new bone cement had the opposite effect of TCP. The comparison between the new sol-gel bone cement and the commercial Simplex P bone cement was discussed.

Biocompatible Materials↗

Comparative wear ranking of dental restorative materials utilizing different wear simulation modes.

The aim of this study was to compare the wear of seven different restorative materials using two different wear simulation modes. This included a non-impact sliding wear test (rotary pin and disc) and an impact-cum-sliding wear test (masticatory simulator). The difference in wear ranking between the two wear tests was compared as well as the correlation of wear to the hardness of the materials. Hardness ranking in the order of decreasing hardness was as follows: Dispersalloy (DA), P50 (P50), Hi-Dense (HD), TPH (TPH), Fuji II LC (FJ), Dyract (DR) and Vitremer (VM). For volumetric wear using the non-impact sliding test, the following ranking in the order of decreasing wear resistance was obtained: DA, TPH, DR, HD, VM, FJ, P50. The results for volumetric wear with impact-cum-sliding wear testing in the order of decreasing wear resistance were: TPH, DR, P50, AR, FJ, VM, HD. Results showed that there is no correlation between hardness and wear resistance. There is also no correlation between impact-cum-sliding wear and non-impact sliding wear. Impact wear should be considered in future two-body wear assessment of materials.

Composite Resins↗

Reciprocal inhibition in Parkinson's disease.

We studied the inhibition of median H-reflex by conditioning stimuli on the radial nerve in 14 normal controls, 6 patients with unilateral and 1 patient with predominantly left-sided Parkinson's disease. In normal controls, the electrophysiological studies were performed on their right hands, yet both hands were examined in patient group. In the controls, we identified three inhibitory phases, with maximal inhibition at conditioning-test intervals of 0 ms (41.66 +/- 4.73%), 20 ms (45.19 +/- 4.33%), and 100 ms (44.55 +/- 6.84%), respectively. In the less- or a- symptomatic side of the patient group, the inhibitory patterns are similar to those of the controls. However, in the symptomatic arms, loss of inhibition, or even mild potentiation, was observed in the third inhibitory phase. When the symptomatic and asymptomatic sides of patients were compared, in contrast to the striking phenomenon found between symptomatic side and the controls, no difference was observed in the third phase. The current results imply that, although no obvious rigidity can be detected on the asymptomatic sides, subtle functional corruption may have occurred within the contralateral basal ganglia in patients with unilateral Parkinson's disease. The remarkable change of the third phase on the symptomatic sides of patients suggests the perturbation of the polysynaptic long latency reflex pathway may somehow play a role in the rigidity pathogenesis.

Adolescent↗