Gene symbol: MPZ. Disease: Charcot-Marie-Tooth disease type 1B.
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Amino Acid Substitution↗
Biomedical subjects
K P Lin
Publications and source records attributed to K P Lin.
Brain dysfunction explored by long latency reflex: a study of adrenomyeloneuropathy.
OBJECTIVES: We can assess brain function by measuring the cortical relay time (CRT) of long latency reflex (LLR) of hand muscle. We would study if measurement of CRT of LLR can explore the brain involvement of adrenomyeloneuropathy (AMN). METHODS: Two AMN patients were included in the study. Both of them had spastic gait and mild sensory deficits but normal mental function. The LLRs were provoked at the first dorsal interosseous muscle by electrical stimulation of the middle finger. We measured the latency of LLR and its CRT. RESULTS: Delayed LLR and prolonged CRT were noted in AMN patients, even though the magnetic resonance imaging of brain did not show any significant abnormalities. CONCLUSIONS: Measuring CRT of LLR reveals brain involvement of AMN patients, and it is an adjunct in the assessment of brain function though without specific anatomic diagnosis.
Adrenoleukodystrophy↗
Optic neuropathy in a patient with chronic inflammatory demyelinating polyneuropathy.
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Adult↗
Problem-based, small-group tutorial learning in clinical neurology for second-year medical students.
BACKGROUND: Problem-based learning (PBL) in small-group tutorials has been a trend in medical education. Chinese students are known to be reserved and passive; thus, they may not be adaptable to PBL. Neuroanatomy, important to clinical neurology, is difficult to learn. We incorporated clinical neurology with PBL, complementary to the traditional neuroanatomy curriculum, to evaluate the feasibility of PBL for Chinese students in Taiwan. METHODS: Forty-two second-year medical students and seven tutors participated in the clinical neurology PBL small-group tutorials. Twelve case reports were discussed weekly beginning in February, 1999. Each case was designed to meet the progressive curriculum of the neuroanatomy course. The tutors evaluated the students by the degree of their preparation, participation, key-point comprehension and interaction. All tutors and students filled out questionnaires at the end of each session. RESULTS: The majority of the students and tutors agreed that the case materials were clearly written. Ninety percent of the students agreed that the case materials matched the traditional content of neuroanatomy. Eighty-five percent of students and 71% of tutors were satisfied and found the class rewarding. Ninety-one percent of students and 74% of tutors were in favor of PBL being continued. CONCLUSIONS: This preliminary PBL, small-group tutorial learning in clinical neurology showed satisfactory results and was, indeed, complementary to a traditional neuroanatomy course. The students, as early as during the second year of their medical school education, were able to learn through the PBL. More integration of basic and clinical sciences by PBL may be considered in future curricula designs.
Education, Medical↗
Measurement of the urinary lactate:creatinine ratio for the early identification of newborn infants at risk for hypoxic-ischemic encephalopathy.
BACKGROUND: Newborn infants with perinatal asphyxia are prone to the development of hypoxic-ischemic encephalopathy. There are no reliable methods for identifying infants at risk for this disorder. METHODS: We measured the ratio of lactate to creatinine in urine by proton nuclear magnetic resonance spectroscopy within 6 hours and again 48 to 72 hours after birth in 58 normal infants and 40 infants with asphyxia. The results were correlated with the subsequent presence or absence of hypoxic-ischemic encephalopathy. RESULTS: Hypoxic-ischemic encephalopathy did not develop in any of the normal newborns but did develop in 16 of the 40 newborns with asphyxia. Within six hours after birth, the mean (+/-SD) ratio of urinary lactate to creatinine was 16.75+/-27.38 in the infants who subsequently had hypoxic-ischemic encephalopathy, as compared with 0.09+/-0.02 in the normal infants (P<0.001) and 0.19+/-0.12 in the infants with asphyxia in whom hypoxic-ischemic encephalopathy did not develop (P<0.001). A ratio of 0.64 or higher within six hours after birth had a sensitivity of 94 percent and a specificity of 100 percent for predicting the development of hypoxic-ischemic encephalopathy. The sensitivity and specificity of measurements obtained 48 to 72 hours after birth were much lower. The mean ratio of urinary lactate to creatinine was significantly higher in the infants who had adverse outcomes at one year (25.36+/-32.02) than in the infants with favorable outcomes (0.63+/-1.50) (P<0.001). CONCLUSIONS: Measurement of the urinary lactate: creatinine ratio soon after birth may help identify infants at high risk for hypoxic-ischemic encephalopathy.
Asphyxia Neonatorum↗
Electronic structure of Ni3Al and Ni3Ga alloys.
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Journal Article↗
Use of botulinum toxin A in the treatment of hemifacial spasm and blepharospasm.
BACKGROUND: Hemifacial spasm and blepharospasm are both dystonic disorders. They may seriously affect individuals' lifestyle and social activities. In 1990, the Food and Drug Administration of the USA approved botulinum toxin A as a therapeutic agent in the treatment of hemifacial spasm and blepharospasm. We present a therapeutic review of botulinum toxin A in 80 patients in Taiwan. METHODS: Fifty-eight patients with hemifacial spasm and 22 with blepharospasm. Botulinum toxin A was prepared and injected into the facial and eyelid muscles. Patients were monitored every two weeks and classified into four groups (excellent, moderate, mild and no improvement) according to the clinical improvement scale. Complications were also recorded. RESULTS: A total of 86.2% of hemifacial spasm patients and 81.8% of blepharospasm patients had excellent improvement on the spasm intensity scale, while 6.8% of hemifacial spasm and 9.0% of blepharospasm patients had moderate improvement. The complication rate was low and included transient mild facial weakness (5%), ptosis (3.8%), eyelid swelling and/or ecchymosis (3.8%), nausea/vomiting (2.5%) and transient severe facial weakness (1.3%). CONCLUSION: Botulinum toxin A is an excellent therapeutic agent to improve spasm intensity and has a low complication rate.
Adult↗
Botulism: heart rate variation, sympathetic skin responses, and plasma norepinephrine.
BACKGROUND: Botulism may involve the autonomic nervous system. METHODS: We assessed the autonomic function of 6 botulism patients with heart rate variations, sympathetic skin responses, and plasma norepinephrine. RESULTS: Two weeks after onset, all the patients had absent sympathetic skin response in the palm and sole. Compared with controls, the heart rate variation of botulism patients was significantly decreased at rest (3.1 +/- 1.2% vs. 20.9 +/- 2.0%, p = 0.0018) and during deep breathing (4.3 +/- 2.3% vs. 29.7 +/- 2.6%, p = 0.0018). The botulism patients had significantly lower plasma norepinephrine levels (supine 29.2 +/- 10.1 pg/ml vs. 257.5 +/- 65.8 pg/ml, p = 0.0018; standing 40.3 +/- 13.1 pg/ml vs. 498.5 +/- 85.6 pg/ml, p = 0.0018). The heart rate variation and sympathetic skin response was greatly improved 6 months after onset. CONCLUSIONS: Heart rate variation, absence of sympathetic skin response, and low plasma norepinephrine are all manifestations of autonomic dysfunction in botulism patients.
Adult↗
Transient syndrome of continuous muscle fiber activity associated with staphylococcal infection.
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Abscess↗
Estimation of myocardial glucose utilisation with PET using the left ventricular time-activity curve as a non-invasive input function.
The validation study is described of a new modelling method that has been developed, using tracer kinetic modelling with positron emission tomography (PET) to achieve non-invasive measurement of myocardial metabolic rate of glucose (MMRGlc). Eight data sets obtained from dynamic cardiac PET 2-[18F]fluoro-2-deoxy-D-glucose (FDG) studies on human subjects are employed, and the estimation of MMRGlc using both the new and traditional methods is compared. The results from all eight human FDG studies are consistent with those from previous computer simulations. With the new method, the estimated mean of K (a parameter directly proportional to MMRGlc) increases by about 8%, and that of k 4 (the rate constant of FDG dephosphorylation) decreases by about 48%. The approach should be more suitable for use in dynamic cardiac PET studies when non-invasive means are used to obtain the plasma time-activity curve from left-ventricle PET images.
Computational Biology↗
Correlation of peripheral nerve fiber loss and trinucleotide repeats in Machado-Joseph disease.
BACKGROUND: Machado-Joseph disease (MJD) is a dominantly inherited cerebellar ataxia associated with spasticity, ophthalmoplegia and dystonia. There has been no report of electrophysiological or histological alterations of the peripheral nervous system in patients with MJD. METHODS: Four patients with MJD were identified by polymerase chain reaction. The peripheral nerves of these patients were subjected to electrophysiological testing and histological study. Correlation analyses were made between various clinical parameters and the electrophysiological and histological changes. RESULTS: Electrophysiological studies demonstrated a marked reduction of sensory action potential, acute denervation changes on needle EMG, as well as mild decrease in the compound motor action potential. Light microscopy of the sural nerves revealed clear loss of myelinated fibers, and morphometry studies showed a loss of large myelinated fibers. Moreover, the severity of these pathological changes was found to be related to the CAG repeat length in the MJD gene. CONCLUSION: Our findings indicated that the peripheral nervous system was frequently affected in patients with MJD. These findings were similar to those seen in Friedreich's ataxia, suggesting a loss of sensory and motor fibers probably following a lesion of the dorsal root ganglion and the anterior horns in the spinal cord. Furthermore, the number of CAG repeats seems to have an inverse relationship to the extent of pathological changes of the peripheral nerves.
Action Potentials↗
Brachial plexus compression due to subclavian pseudoaneurysm from cannulation of jugular vein hemodialysis catheter.
Jugular venous cannulation is generally safer than subclavian cannulation. The traumatic complications associated with jugular vein hemodialysis catheters are rare. A jugular vein, therefore, has become the preferred site for hemodialysis catheter insertion. We describe the first case of brachial plexus compression attributable to delayed recognition of a right subclavian pseudoaneurysm as a complication of jugular venous cannulation of hemodialysis catheter. We advocate that any neck swelling, new bruit, and the symptoms of brachial plexopathy after jugular venous cannulation warrant an intensive investigation to exclude arterial injury. Because delayed diagnosis may lead to a worsened prognosis in patients with brachial plexus palsy, physicians should exercise vigilance to detect and manage early the potentially serious and fatal complications of subclavian artery pseudoaneurysm and brachial nerve injury.
Aged↗
An electrophysiologic and pathologic study of peripheral nerves in individuals with Machado-Joseph disease.
BACKGROUND: Machado-Joseph disease (MJD) is characterized by cerebellar ataxia, pyramidal signs, progressive external ophthalmoplegia, and variable degrees of bulging eyes and dystonia. Electrophysiologic and histologic alterations of the peripheral nervous system in patients with MJD have rarely been reported. METHODS: The peripheral nerves of four patients with MJD who were identified by polymerase chain reaction were subjected to electrophysiologic testing and histologic study. Correlation analyses were made between clinical parameters and the electrophysiologic as well as histologic changes. RESULTS: Electrophysiologic studies demonstrated a marked reduction of sensory action potential, as well as a decrease in the compound motor action potential. Light microscopy of the sural nerves revealed marked loss of myelinated fibers, and morphometry studies showed a loss of large myelinated fibers. The severity of these pathologic changes was not related to the duration of the disease. CONCLUSIONS: In MJD, the peripheral nervous system was frequently affected. These findings were similar to those seen in Friedreich's ataxia, suggesting a loss of sensory and motor fibers probably following a lesion of the dorsal root ganglion and the anterior horns in the spinal cord.
Adult↗
The Kinmen Neurological Disorders Survey (KINDS): a study of a Chinese population.
We conducted an epidemiological study of several neurological disorders among the Chinese aged 50 years or older on the islet of Kinmen. All participants were interviewed and examined by neurologists. From the targeted population of 5,061 individuals, 3,915 (77.4%) of them completed the evaluations. Among the 4,087 individuals with whom face-to-face contact was made, the refusal rate was 4.2%. The disorders of interest were dementia, Parkinson's disease, essential tremor, stroke, transient ischemic attacks, and migraine. Among the 3,915 participants, 366 cases were found with 1 or more of the surveyed neurological disorders on the prevalence day, August 1, 1993, yielding a prevalence of 93.5/1,000. The purpose of this study, the general methodology, and some overall findings are presented in this communication in order to provide a common background for detailed findings on each disorder to be reported separately.
Age Factors↗
Prevalence of headaches in a Chinese elderly population in Kinmen: age and gender effect and cross-cultural comparisons.
OBJECTIVE: To investigate the prevalence of headaches in a Chinese elderly population. BACKGROUND: There are few headache surveys in the elderly. Previous studies have shown a low headache prevalence in Chinese. METHODS TARGET POPULATION: eligible registered residents > or = 65 years old (N = 2,003) in two townships of Kinmen Island on August 1, 1993. All participants completed a headache questionnaire and underwent clinical evaluation and examination by a neurologist. Headache diagnoses were made according to the International Headache Society, 1988. RESULTS: 1,533 persons (77%) participated in the study, of whom 584 (38%) had at least one episode of headache in the previous year. One-year prevalence of migraine was 3.0%, and tension-type headache, 35%. The prevalence of migraine, but not tension-type headaches, continued to decline with age in the elderly. Life-time prevalence of "incapacitating headache" was 10%, and that of migraine, 5.2%. Forty-two percent of migraineurs stopped having migraine before this survey. In comparison with "10 years ago" 8% participants felt their current headaches were worse, 25% better, and 67%, no change, with a net improvement of 17%. CONCLUSIONS: We have reported the highest headache prevalence among different Chinese elderly populations, but these were still lower than those reported from Western series. More than half of the elderly life-time migraineurs still had attacks of migraine. Severe headaches, including migraine but not tension-type headaches, declined with age.
Aged↗
A door-to-door survey of Parkinson's disease in a Chinese population in Kinmen.
BACKGROUND: Most published studies have shown lower prevalence rates of Parkinson's disease (PD) in Asian and black African than in Western countries, leading to the hypothesis that Asians and blacks might be protected from PD. OBJECTIVE: To investigate the prevalence of PD in a Chinese population. DESIGN: Community-based survey. SETTING: Registered residents 50 years of age or older (N = 5061) on the islet of Kinmen located off the southeastern coast of China [corrected]. METHOD: Single-phase door-to-door survey by neurologists. All participants were administered a questionnaire and received motor examinations of the Unified Parkinson's Disease Rating Scale. RESULTS: The participation rate was 96% (N = 3915) among 4158 contacted individuals. Twenty-three cases of PD were identified, including three cases with dementia. The crude prevalence rate of PD was 587 (95% confidence interval (CI), 373 to 884) per 100,000 persons 50 years of age or older. Assuming no case of PD among individuals under 50 years of age, the prevalence rate was 119 (95% CI, 80 to 169) per 100,000 for the total population. CONCLUSIONS: The prevalence rates of PD in Kinmen were much higher than those reported from mainland China, but slightly lower than those reported from more developed countries. The present findings suggest that, instead of genetic factors, differences in case-ascertainment, life expectancy, and the length of survival with PD may be more important contributors to the variations in observed PD prevalence rates.
Aged↗
6-[18F]fluoro-L-DOPA-PET studies show partial reversibility of long-term effects of chronic amphetamine in monkeys.
The acute and long-term effects of chronic amphetamine administration on the striatal dopamine system in monkeys were assessed with 6-[18F]fluoro-L-DOPA (FDOPA) and positron emission tomography (PET). Vervet monkeys (Cerecopithecus aethiops) were administered amphetamine doses, i.m., that increased from 4 mg/kg/d to 18 mg/kg/d over a 10 day period. Post-amphetamine FDOPA-PET scans at 1-2, 3-4, and 6 week time points in individual subjects showed persistent decrements in dopamine synthesis capacity as reflected by FDOPA influx rate constant (Ki) values being approximately 30% that of pre-drug assessment. In other animals that were administered the same drug regimen, biochemical analysis of striatal regions at 1-2 weeks post-drug indicated that dopamine concentrations were decreased by approximately 95% throughout caudate and putamen regions, while the homovanillic acid/dopamine level ratio was increased 3-10-fold. Post-drug FDOPA-PET Ki values remained consistently low up to 6 weeks; however, at the 5-6 month time point, relative increases in FDOPA-Ki values (approximately 53% of pre-drug values) were observed for all subjects, indicative of partial recovery of striatal dopamine synthesis capacity. These results demonstrate that FDOPA-PET can reveal temporal activity changes within the striatal dopamine system of individual subjects. The apparent, partial reversibility of amphetamine's neurotoxic effects suggests a plasticity of dopaminergic function that may include regeneration of dopaminergic terminals and compensatory increases in residual dopamine synthesis rates. The persistence of the partial decrement in dopamine synthesis capacity, however, may indicate a long term component of amphetamine's toxic effects.
Amphetamine↗
Automated image registration for FDOPA PET studies.
In this study, various image registration methods are investigated for their suitability for registration of L-6-[18F]-fluoro-DOPA (FDOPA) PET images. Five different optimization criteria including sum of absolute difference (SAD), mean square difference (MSD), cross-correlation coefficient (CC), standard deviation of pixel ratio (SDPR), and stochastic sign change (SSC) were implemented and Powell's algorithm was used to optimize the criteria. The optimization criteria were calculated either unidirectionally (i.e. only evaluating the criteria for comparing the resliced image 1 with the original image 2) or bidirectionally (i.e. averaging the criteria for comparing the resliced image 1 with the original image 2 and those for the sliced image 2 with the original image 1). Monkey FDOPA images taken at various known orientations were used to evaluate the accuracy of different methods. A set of human FDOPA dynamic images was used to investigate the ability of the methods for correcting subject movement. It was found that a large improvement in performance resulted when bidirectional rather than unidirectional criteria were used. Overall, the SAD, MSD and SDPR methods were found to be comparable in performance and were suitable for registering FDOPA images. The MSD method gave more adequate results for frame-to-frame image registration for correcting subject movement during a dynamic FDOPA study. The utility of the registration method is further demonstrated by registering FDOPA images in monkeys before and after amphetamine injection to reveal more clearly the changes in spatial distribution of FDOPA due to the drug intervention.
Animals↗