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Chin-Chang Huang

Publications and source records attributed to Chin-Chang Huang.

32 records · Page 2Linked to original sources
PubMed2004

Delayed encephalopathy after carbon monoxide intoxication--long-term prognosis and correlation of clinical manifestations and neuroimages.

In order to understand the correlation between the clinical and neuroimaging manifestations and the long-term prognosis in delayed encephalopathy after carbon monoxide (CO) intoxication, we retrospectively reviewed 12 patients who had delayed encephalopathy from 89 patients with CO intoxication. There were 8 men and 4 women, with a mean age of 54.4 +/- 17.2 years (range: 11-79 years). All patients had prominent consciousness disturbance in the acute stage and received high flow of O2 or hyperbaric oxygen therapy. All of them regained consciousness within 1-7 days, but subsequently developed delayed encephalopathy. The delayed encephalopathy occurred from 14 to 45 days after recovery from the acute stage. The clinical manifestations included cognitive impairment, akinetic mutism, sphincter incontinence, gait ataxia and extrapyramidal syndromes such as chorea, dystonia, and parkinsonism. Brain MRI revealed multiple lesions in the subcortical white matter and basal ganglia, mostly in the globus pallidus, and to a lesser degree in the putamen, and caudate. In the follow-up period, sphincter incontinence first disappeared. The cognitive impairment improved greatly in the following few months, but the involuntary movements were improved only slightly. Some patients had persistent neurological sequelae, such as dystonia. Similary, the follow-up brain MRI showed a steady improvement. In conclusion, the delayed encephalopathy usually developed 2 weeks to 1.5 months after the acute phase of CO intoxication. Globus pallidus and subcortical white matter were commonly involved. The neurological manifestations improved and correlated roughly with the neuroimaging changes.

Adolescent↗
PubMed2004

Clinical and electrophysiological studies of a family with probable X-linked dominant Charcot-Marie-Tooth neuropathy and ptosis.

BACKGROUND: The X-linked dominant Charcot-Marie-Tooth neuropathy (CMTX) is a hereditary motor and sensory neuropathy linked to a variety of mutations in the connexin32 (Cx32) gene. Clinical and genetic features of CMTX have not previously been reported in Taiwanese. METHODS: Clinical evaluations and electrophysiological studies were carried out on 25 family members of a Taiwanese family group. Molecular genetic analysis of the Cx32 gene was performed. A sural nerve biopsy was obtained from 1 patient. RESULTS: Nine patients had clinical features of X-linked dominant inheritance and a moderate Charcot-Marie-Tooth (CMT) neuropathy phenotype. Molecular genetic analysis showed no mutation of the Cx32 coding region, but revealed a G-to-A transition at position -215 of the nerve-specific promoter P2 of the Cx32 gene. Ptosis is 1 clinical manifestation of neuropathy in this probable CMTX family. Familial hyperthyroidism is an additional independent feature of the family. Electrophysiological and histological studies showed features of axonal neuropathy. Multimodality evoked potential studies revealed normal central motor and sensory conduction velocities. CONCLUSIONS: The presence of ptosis in this family illustrates the existence of clinical heterogeneity among related family members with CMTX similar to that in CMT of autosomal inheritance. Electrophysiological and histological findings revealed normal central conduction and axonal neuropathy.

Acute Disease↗
PubMed2003

Dopamine transporter binding in chronic manganese intoxication.

Chronic exposure to manganese may induce parkinsonism similar to idiopathic Parkinson's disease (PD). However, clinical manifestations of manganism also have some features different from PD. The mechanisms of manganese-induced parkinsonism remain not fully understood. (99m)Tc-TRODAT-1 is a cocaine analogue that can bind to the dopamine transporter (DAT) site reflecting the function of presynaptic dopaminergic terminals. The purpose of this study was to evaluate DAT function using (99m)Tc-TRODAT-1 to investigate the integrity of the presynaptic dopaminergic terminals in manganese-induced parkinsonism. Brain (99m)Tc-TRODAT-1 single photon emission computed tomography was performed in 4 patients with chronic manganese intoxication in a ferromanganese smelting plant in Taiwan. Twelve PD patients and 12 healthy volunteers served as abnormal and normal controls, respectively. Clinically, all manganism patients had a bradykinetic-rigid syndrome. The scores of the Unified Parkinson's Disease Rating Scale ranged between 19 and 64. The uptake values of the (99m)Tc-TRODAT-1 were 0.868+/-0.136 in the right corpus striatum and 0.865+/-0.118 in the left, as compared with 0.951+/-0.059 and 0.956+/-0.058, respectively for the normal controls. The data were significantly higher than 0.250+/-0.070 and 0.317+/-0.066 respectively for the PD patients. Interestingly, there was a mild decrease in the uptake of (99m)Tc-TRODAT-1 in the putamen and the ratio of putamen and caudate when compared with the normal controls. Although the DAT shows a slight decrease in the putamen of manganism patients as compared with that of the normal controls, the data indicate that the presynaptic dopaminergic terminals are not the main target of chronic manganese intoxication. In addition (99m)Tc-TRODAT-1 SPECT can provide a useful, convenient and inexpensive tool for differentiation between chronic manganism and PD.

Brain↗
PubMed2003

Dopamine transporter binding in Wilson's disease.

INTRODUCTION: In Wilson's disease (WD), brain magnetic resonance images (MRI) show increased signal intensity in T2 weighted images in the lenticular nuclei, thalamus and brainstem, including the substantia nigra. A poor therapeutic response to levodopa in WD suggests the mechanism of a postsynaptic abnormality. However positron emission tomography studies show an involvement of the nigrostriatal presynaptic dopaminergic pathway. CASE REPORT: We report the clinical manifestations in a case of WD with akinetic-rigid syndrome and initial hesitation. The brain MRI showed an increased signal intensity lesion in the substantia nigra region, in addition to basal ganglion and thalamic lesions. However, dopamine transporter (DAT) imaging with 99mTc-TRODAT-1 revealed a nonsignificantly increased DAT uptake, suggesting a normal presynaptic nigrostriatal dopaminergic terminal. CONCLUSION: We suggest that significant heterogeneity can be found in WD patients and a normal presynaptic dopaminergic pathway may occur in some patients, even those with typical akinetic-rigid syndrome and evidence of substantia nigra involvement in the brain on MRI.

Adult↗
PubMed2003

Elevated triglyceride and decreased high density lipoprotein level in carbon disulfide workers in Taiwan.

Carbon disulfide (CS2) is a man-made product utilized primarily in the manufacture of viscose rayon. Overexposure to CS2 has been associated with an increase in coronary heart disease. The aims of this study were to examine the dose-response relationship of CS2 exposure and elevated lipid profile tests among CS2-exposed workers in Taiwan. A total of 132 workers were recruited from two viscose rayon plants. Air sampling was performed to determine the CS2 exposure of workers. Demographic data and work history were gathered by a standard self-administered questionnaire. Lipid profile tests were also performed by routine methods. The average CS2 exposure concentration was 50.6 +/- 25.6 ppm (range: 24-127 ppm) in the high-exposure group, 12.9 +/- 5 ppm (range: 5.2-22.3 ppm) in the mid-exposure group, and 3.5 +/- 1.2 ppm (range 0.97-5.2 ppm) in the low-exposure group. There were 21 out of 33 (63.7%) elevated triglyceride levels among high-CS2-exposure workers, 27 out of 64 (42.2%) among the middle-CS2-exposure, and 14 out of 35 (40%) among low-CS2-exposure workers, respectively. Compared to the low-CS2-exposure workers, the age- and weight-adjusted odds ratios (and 95% confidence intervals) of the prevalence of elevated triglyceride value were 1.12 (0.5, 2.7) for middle-CS2-exposure workers, and 2.81 (1.02, 7.8) for high-CS2-exposure workers. There was a significant linear trend between CS2 exposure and the prevalence of elevated triglyceride value (P = 0.046) after adjusting for other factors. There was also a lower prevalence of elevated HDL level in high-CS2-exposure workers than low-CS2-exposure workers (15.2% versus 31.4%). Compared to the low-CS2-exposure workers, the age- and weight-adjusted odds ratio (and 95% confidence intervals) of elevated HDL level were 0.34 (0.1, 1.18) for high-CS2-exposure workers, which was borderline significant. In conclusion, this study suggests that elevated triglyceride level and decreased HDL level are associated with CS2 exposure. The study also suggests that exposure to CS2 (concentration > 23.2 ppm, which was above the current TLV-TWA of 10 ppm) are significantly associated with increased risk and decreased protective effect for coronary heart disease.

Carbon Disulfide↗
PubMed2003

Diffuse white matter lesions in carbon disulfide intoxication: microangiopathy or demyelination.

Long-term exposure to carbon disulfide (CS(2)) may induce diffuse encephalopathy with parkinsonism, pyramidal signs, cerebellar ataxia, and cognitive impairments, as well as axonal polyneuropathy. The pathogenic mechanisms of diffuse encephalopathy are unclear, although vasculopathy and toxic demyelination have been proposed. Recently, we have encountered a patient who developed headache, limb tremors, gait disturbance, dysarthria, memory impairment, and emotional lability after long-term exposure to CS(2). The brain magnetic resonance images (MRI) showed diffuse hyperintensity lesions in T(2)-weighted images in the subcortical white matter, basal ganglia, and brain stem. The brain computed tomography perfusion study revealed a diffusely decreased regional cerebral blood flow and prolonged regional mean transit time in the subcortical white matter and basal ganglion. To our knowledge, there have been few reports demonstrating diffuse white matter lesions in chronic CS(2) encephalopathy using brain MRI. In addition, the (99m)Tc-TRODAT-1 single photon emission computed tomography showed a normal uptake of the dopamine transporter, indicating a normal presynaptic dopaminergic pathway. We conclude that diffuse white matter lesions may develop after chronic exposure to CS(2), possibly through microangiopathy. In addition, CS(2) poisoning can be considered as one of the causes of chronic leukoencephalopathy.

Brain↗
PubMed2003

Clinical features of Leber's hereditary optic neuropathy with the 11778 mitochondrial DNA mutation in Taiwanese patients.

BACKGROUND: To characterize the clinical features of Leber's hereditary optic neuropathy (LHON) in Taiwanese patients with the 11778 mutation of mitochondrial DNA (mtDNA). METHODS: A retrospective review of the clinical manifestation was undertaken in 13 LHON patients with the 11778 mtDNA mutation from 1994 to 2001 in Chang Gung Memorial Hospital. RESULTS: The male-to-female ratio among patients was 12:1 (92% male). The age at onset of visual loss for the first eye ranged from 7 to 30 years old, with a mean of 19.4 years. The time interval between when the second eye was affected ranged from simultaneous onset to 7 months (mean, 2.2 months). The final visual acuity was 0.1 or worse in 24 eyes (92.3%). A suspect fundus was present in 14 of 26 eyes (54%). Patients with later onset of visual loss (< 20 years) tended to have better final visual acuities. Abnormal visual evoked potentials (VEPs) were recorded after acute onset of visual loss in all of our patients and even before onset of visual symptoms in 1 patient. CONCLUSIONS: The clinical characteristics of our patients harboring the 11778 mitochondrial mutation are mostly similar to those previously reported from other countries. In addition to the common features of LHON, we also noted that there was a relationship between age of onset and visual prognosis. We also suggest that VEP is a good indicator for predicting visual loss.

Adolescent↗
PubMed2003

Low antioxidant content and mutation load in mitochondrial DNA A3243G mutation-related diabetes mellitus.

BACKGROUND AND PURPOSE: Diabetes mellitus (DM) is a common clinical manifestation in patients harboring mitochondrial encephalomyopathy, lactic acidosis with stroke-like episodes (MELAS)-specific A3243G mitochondrial DNA (mtDNA) mutation. However, in some MELAS family members, the presence of mtDNA mutation in the blood is not always associated with DM, and the relationship between development of DM and A3243G mtDNA mutation is not fully understood. This study evaluated the relationship between A3243G mtDNA mutation and DM in a Taiwanese family. METHODS: We analyzed the relation of genotypic and phenotypic characteristics in a 2-generation DM family associated with the A3243G mtDNA mutation. The contents of mutant mtDNA in various tissue samples of 11 family members and their serum levels of antioxidants, including protein thiols and alpha-tocopherol (vitamin E) were determined and correlated with their past history and various clinical manifestations. RESULTS: DM in 4 members of the first and second generations was associated with age and decreased serum levels of antioxidant protein thiols. In a series of studies of mutant mtDNA content in various tissues, a relatively low proportion of A3243G mutant mtDNA was noted in the elderly proband and her elderly symptomatic siblings. A low proportion of mutant mtDNA was also noted in a younger family member presenting with DM. Moreover, a significantly lower average level of protein thiols was found in the symptomatic family members compared to the asymptomatic members (2.3 +/- 0.2 vs 3.5 +/- 0.54 nmol/mg protein, p < 0.05). CONCLUSIONS: The finding of relatively lower levels of mutant mtDNA in the elderly proband and her elderly symptomatic family members indicates that DM may be a late phenotypic expression in patients harboring MELAS-specific mtDNA mutation. Decreased serum protein thiols, suggestive of increased oxidative stress, also appear to be an early sign associated with subsequent development of DM.

Adult↗
PubMed2002

Hemiballism after subthalamotomy in patients with Parkinson's disease: report of 2 cases.

The occurrence of persistent hemiballism after subthalamotomy for Parkinson's disease (PD) has not been described as frequently as mild or transient dyskinesia. We report on 2 patients with advanced PD who developed hemiballism and/or dyskinesia after subthalamotomy. One patient with a small lesion confined to the subthalamic nucleus (STN) developed persistent hemiballism; the other with a larger lesion involving the STN and also the zona incerta presented with a transient dyskinesia in a single limb. We conclude that a precise STN lesion might bear a potential risk of persistent hemiballism.

Chorea↗
PubMed2002

Normal dopamine transporter binding in dopa responsive dystonia.

We report the clinical manifestations of dopa responsive dystonia (DRD) in 2 patients from the same family. The brain magnetic resonance images (MRI) were normal. The dopamine transporter (DAT) imaging with (99m)Tc-TRODAT-1 was performed in the 2 probands, 8 patients with young onset Parkinson disease (YOPD) and 16 normal controls. The ratios of (99m)Tc-TRODAT-1 brain SPECT in the striatum were 2.40 +/- 0.12 (right) and 2.30 +/- 0.17 (left) in these 2 DRD patients as compared with 1.38 +/- 0.18 (right), 1.41 +/- 0.20 (left) in YOPD patients, and 2.15 +/- 0.35 (right), 2.14 +/- 0.32 (left) in normal controls respectively. A normal DAT uptake was found in DRD suggesting a normal presynaptic nigrostriatal dopaminergic terminal. We conclude that a normal DAT in parkinsonian patients can differentiate DRD from YOPD. In addition, DAT with (99m)Tc-TRODAT-1 is a reliable and convenient tool to study the function of the presynaptic dopaminergic axonal terminals.

Adult↗
PubMed2002

Clinical course in patients with chronic carbon disulfide polyneuropathy.

The natural course of clinical manifestations and electrophysiological changes were studied in six patients with carbon disulfide (CS(2)) induced polyneuropathy. All of the six patients worked in the cutting-machine department of a viscose rayon plant. The environmental monitoring was also conducted in the initial stage and followed up 3 years later. In the 3-year follow-up period, the neurological symptoms and signs persisted. The highest concentration of CS(2) in the cutting machine where these patients worked was about 100-200 ppm. Three years later, the highest concentration was decreased to between 10 and 20 ppm in the cutting machine of the new production line after the engineering control had been improved. Nerve conduction velocity (NCV) studies revealed persistent abnormality in motor and sensory NCVs. Although, a tendency to improvement was noted, it did not reach a statistical significance except for conduction velocity of sural nerve in sensory NCV. Sural nerve biopsy from one patient, 2 years after diagnosis showed degeneration of both axon and myelin and a predominant loss of large myelinated fibers. A remyelination process was also noted. We concluded that CS(2) intoxication may induce a persistent damage to the peripheral nerves even after CS(2) exposure had ceased for 3 years.

Adult↗
PubMed2002

Clinical phenotype, prognosis and mitochondrial DNA mutation load in mitochondrial encephalomyopathies.

We studied 42 individuals, including 8 patients with either complete or partial syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), 8 patients with either complete or partial syndrome of myoclonic epilepsy with ragged-red fibers (MERRF) and 26 maternal family members who carried either the A3243G or A8344G mutation of mitochondrial DNA (mtDNA). Clinical manifestations and prognosis were followed up in the patients harboring the A3243G or A8344G mutation. The relationship between clinical features and proportions of mutant mtDNAs in muscle biopsies, blood cells and/or hair follicles was studied. In the 8 regularly followed patients with the A3243G mutation, 4 died within 1 month to 7 years due to status epilepticus and/or recurrent stroke-like episodes. Two patients developed marked mental deterioration and 2 remained stationary. All of the patients harboring the A8344G mutation were stable or deteriorated slightly, except for 1 patient who died due to brain herniation after putaminal hemorrhage. The A3243G and A8344G mtDNA mutations were heteroplasmic in the muscle biopsies, blood cells and hair follicles of both the probands and their maternal family members. The mean proportion of A3243G mutant mtDNA in the muscle biopsies of the patients with MELAS syndrome (68.5 +/- 21.3%, range 33-92%) was significantly higher than that of the asymptomatic family members (37.1 +/- 12.6%, range 0-51%). The average proportions of A8344G mutant mtDNA in the muscle biopsies (90.1 +/- 3.9%, range 89-95%) and hair follicles (93.9 +/- 6.4%, range 84-99%) of the patients with MERRF syndrome were also significantly higher than those of the asymptomatic family members (muscle: 40.3 +/- 39.5%, range 1-80%; hair follicles: 51.0 +/- 44.5%, range 0.1-82%). We concluded that measurement of the proportion of mutant mtDNA in muscle biopsies may provide useful information in the identification of symptomatic patients with mitochondrial encephalomyopathies. For patients with the A3243G mutation, the prognosis was related to status epilepticus and the number of recurrent stroke-like episodes and was much worse than for patients with the A8344G mutation of mtDNA, who had stable or slowly deteriorating clinical courses.

Adolescent↗
PubMed2002

Sensory neuropathy in X-linked recessive bulbospinal neuronopathy.

X-linked recessive bulbospinal neuronopathy (X-BSN) is an adult-onset spinal and bulbar amyotrophy. Neurophysiologic studies demonstrate subclinical involvement of sensory nerves with diminished or absent sensory nerve action potientials and denervation changes, indicating the involvement of sensory neurons. We report the clinical features, findings of electrophysiologic study, and results of morphometric analysis of sural nerve pathology in a patient with X-BSN. Molecular genetic studies were also performed in the patient and his three daughters. Electrophysiologic studies revealed decreased amplitude sensory nerve action potentials and the presence of high amplitude motor unit potentials in all muscles tested. Sural nerve biopsy demonstrated axonal degeneration with a predominant loss of large myelinated fibers. Molecular genetic studies confirmed elongation of the CAG triplet repeats in exon 1 of the androgen receptor gene. Sequence analysis of the androgen receptor gene revealed that the number of CAG triplet repeats was 45 in the patient and was 45 to 48 in the mutant allele but only 19 to 30 in the normal allele in his three daughters. These findings suggest that both motor and sensory neurons are involved in X-BSN. Sural nerve biopsy and molecular genetic analysis are helpful in differentiation between X-BSN and other motor neuron diseases.

Biopsy↗