Prognostic Performance of Current Stage III Oral Cancer Patients After Curative Intent Resection: Evidence to Support a Revision of the American Joint Committee on Cancer Staging System.

BACKGROUND: The American Joint Committee on Cancer (AJCC) stage III classification of oral cavity squamous cell carcinoma (OCSCC) represents a heterogeneous group of patients with early local disease with regional metastases (T1N1 and T2N1) and advanced local disease with or without regional metastasis (T3N0 and T3N1). OBJECTIVE: The aim of this study was to evaluate prognostic heterogeneity in the stage III category. METHODS AND PATIENTS: An international retrospective multicenter study of 1815 patients who were treated for OCSCC from 2003 to 2011. RESULTS: Kaplan-Meier survival analysis and multivariate models of stage III patients revealed better overall survival (OS; HR 2.12, 95 % CI 1.03-4.15; p = 0.01) and disease-specific survival (DSS; HR 1.7, 95 % CI 1.16-4.12; p = 0.04) rates for patients with T1-2N1/T3N0 disease than for patients with T3N1 disease. The outcomes of patients with T3N1 and stage IVa disease were similar (p = 0.89 and p = 0.78 for OS and DSS, respectively). Modifying stage classification by transferring the T3N1 category to the stage VIa group resulted in a better prognostic performance [Harrell's concordance index, C index 0.76; Akaike's Information Criterion (AIC) 4131.6] compared with the AJCC 7th edition staging system (C index 0.65; AIC 4144.9) for OS. When DSS was assessed, the suggested staging system remained the best performing model (C index 0.71; AIC 1061.3) compared with the current AJCC 7th edition staging (C index 0.64; AIC 1066.2). CONCLUSIONS: The prognosis of T3N1 and stage IVa disease are similar in OCSCC, suggesting that these categories could be combined in future revisions of the nodal staging system to enhance prognostic accuracy.

¹8F-FP-(+)-DTBZ positron emission tomography detection of monoaminergic deficient network in patients with carbon monoxide related parkinsonism.

BACKGROUND AND PURPOSE: Although parkinsonism after carbon monoxide (CO) intoxication is well known, neurotransmitter deficient networks that are responsible for the severity of parkinsonism have rarely been systemically evaluated. METHODS: Eighteen patients with CO-related parkinsonism and nine age- and sex-matched controls were enrolled for detailed neurological examinations, three-dimensional T1-weighted images, diffusion tensor imaging and (18)F-9-fluoropropyl-(+)-dihydrotetrabenzazine ((18)F-FP-(+)-DTBZ) positron emission tomography (PET). The structural analysis included voxel-based morphometry to assess grey matter atrophy and tract-based spatial statistics related to white matter involvement. For presynaptic monoaminergic assessment, volume of interest analysis in six subcortical regions and non-parametric voxel-wise comparison were performed on PET images with estimation of registration parameters from magnetic resonance images. All the imaging modalities were compared between the patients and controls. For the patients, a regression model for correlation with cognitive behaviour and Unified Parkinson's Disease Rating Scale (UPDRS) score was used. RESULTS: In the patients, monoaminergic deficit networks were found in the caudate, anterior putamen, anterior insular, thalamus and anterior cingulate cortex. The UPDRS revealed significant correlations with the prefrontal white matter fractional anisotropy values and with the (18)F-FP-(+)-DTBZ uptake values in the caudate nucleus, insular, medial prefrontal and dorsomedial thalamus. The neuropsychiatric inventory score correlated with the (18)F-FP-(+)-DTBZ uptake values in the anterior cingulate cortex and dorsolateral prefrontal cortex. CONCLUSIONS: Our study demonstrated monoaminergic deficits and white matter damage networks in CO-related parkinsonism that determined the severity of parkinsonism or behaviour changes. As the substantia nigra was spared, the monoaminergic topography of involvement suggests a different pathophysiology in CO-related parkinsonism.

Minimum nodal yield in oral squamous cell carcinoma: defining the standard of care in a multicenter international pooled validation study.

PURPOSE: There is evidence to suggest that a nodal yield <18 is an independent prognostic factor in patients with clinically node negative (cN0) oral squamous cell carcinoma (SCC) treated with elective neck dissection (END). We sought to evaluate this hypothesis with external validation and to investigate for heterogeneity between institutions. PATIENTS AND METHODS: We analyzed pooled individual data from 1,567 patients treated at nine comprehensive cancer centers worldwide between 1970 and 2011. Nodal yield was assessed with Cox proportional hazard models, stratified by study center, and adjusted for age, sex, pathological T and N stage, margin status, extracapsular nodal spread, time period of primary treatment, and adjuvant therapy. Two-stage random-effects meta-analyses were used to investigate for heterogeneity between institutions. RESULTS: In multivariable analyses of patients undergoing selective neck dissection, nodal yield <18 was associated with reduced overall survival [hazard ratio (HR) 1.69; 95 % confidence interval (CI) 1.22-2.34; p = 0.002] and disease-specific survival (HR 1.88; 95 % CI 1.21-2.91; p = 0.005), and increased risk of locoregional recurrence (HR 1.53; 95 % CI 1.04-2.26; p = 0.032). Despite significant differences between institutions in terms of patient clinicopathological factors, nodal yield, and outcomes, random-effects meta-analysis demonstrated no evidence of heterogeneity between centers in regards to the impact of nodal yield on disease-specific survival (p = 0.663; I (2) statistic = 0). CONCLUSION: Our data confirm that nodal yield is a robust independent prognostic factor in patients undergoing END for cN0 oral SCC, and may be applied irrespective of the underlying patient population and treating institution. A minimum adequate lymphadenectomy in this setting should include at least 18 nodes.

Lymph node density in oral cavity cancer: results of the International Consortium for Outcomes Research.

BACKGROUND: Lymph node density (LND) has previously been reported to reliably predict recurrence risk and survival in oral cavity squamous cell carcinoma (OSCC). This multicenter international study was designed to validate the concept of LND in OSCC. METHODS: The study included 4254 patients diagnosed as having OSCC. The median follow-up was 41 months. Five-year overall survival (OS), disease-specific survival (DSS), disease-free survival (DFS), locoregional control and distant metastasis rates were calculated using the Kaplan-Meier method. Lymph node density (number of positive lymph nodes/total number of excised lymph nodes) was subjected to multivariate analysis. RESULTS: The OS was 49% for patients with LND0.07 compared with 35% for patients with LND>0.07 (P<0.001). Similarly, the DSS was 60% for patients with LND0.07 compared with 41% for those with LND>0.07 (P<0.001). Lymph node density reliably stratified patients according to their risk of failure within the individual N subgroups (P=0.03). A modified TNM staging system based on LND ratio was consistently superior to the traditional system in estimating survival measures. CONCLUSION: This multi-institutional study validates the reliability and applicability of LND as a predictor of outcomes in OSCC. Lymph node density can potentially assist in identifying patients with poor outcomes and therefore for whom more aggressive adjuvant treatment is needed.

Clinical nodal stage is a significant predictor of outcome in patients with oral cavity squamous cell carcinoma and pathologically negative neck metastases: results of the international consortium for outcome research.

BACKGROUND: We aimed to study the importance of clinical N classification (cN) in a subgroup of patients with oral cavity squamous cell carcinoma (OSCC) and pathologically negative neck nodes (pN-). METHODS: A total of 2,258 patients from 11 cancer centers who underwent neck dissection for OSCC (1990-2011) had pN- disease. The median follow-up was 44 months. 5-year overall survival (OS), disease-specific survival (DSS), disease free survival, local control, locoregional control, and distant metastasis rates were calculated by the Kaplan-Meier method. cN classification and tumor, node, metastasis classification system staging variables were subjected to multivariate analysis. RESULTS: A total of 345 patients were preoperatively classified as cN+ and 1,913 were classified as cN-. The 5-year OS and DSS of cN- patients were 73.6 and 82.2 %, respectively. The 5-year OS and DSS of cN+ patients were 64.9 and 76.9 %, respectively (p < 0.0001 each). A cN+ classification was a significant predictor of worse OS (p = 0.03) and DSS (p = 0.016), regardless of treatment, depth of invasion, or extent of neck dissection. cN classification was associated with recurrence-free survival (p = 0.01) and locoregional (neck and primary tumor) control (p = 0.004), but not with local (p = 0.19) and distant (p = 0.06) recurrence rates. CONCLUSIONS: Clinical evidence of neck metastases is an independent predictor of outcome, even in patients with pN- nodes.

Peritoneal metastasis in primary cervical cancer: a case report.

PURPOSE OF INVESTIGATION: Peritoneal metastasis presenting at initial diagnosis of squamous carcinoma of the uterine cervix is extremely rare. However, one such case was treated successfully with curative treatment. CASE: A 43-year-old woman presented with FIGO Stage IVA cervical carcinoma and underwent concurrent chemoradiation (CCRT) with weekly cisplatin. During CCRT, she suffered from acute lower abdominal pain, high fever, and leukocytosis. Computed tomography (CT) favored a tubo-ovarian abscess, while [18F] fluorodeoxyglucose positron emission tomography (FDG-PET) showed a lesion midway between the umbilicus and symphysis pubis. An exploration by laparoscopy, an omental metastasis adhering to the bladder dome was excised. This patient finished CCRT encompassing the disease extent including the trochar sites and no evidence of disease was noted 50 months after initial diagnosis. CONCLUSION: Though peritoneal metastasis above the pelvis seems ominous, curative treatment may still be viable in selected patients, even in surgical Stage IVB.

Supraclavicular lymph node metastases in cervical cancer.

PURPOSE OF INVESTIGATION: To evaluate the outcome and prognostic factors of patients with supraclavicular lymph node (SCLN) involvement at primary diagnosis. METHODS: We reviewed the medical records of cervical cancer patients primarily treated at Chang Gung Memorial Hospital between 1987 and 2005. Thirty-three patients with histologically confirmed SCLN metastasis at primary diagnosis were eligible for analysis. Clinical and pathological features were analyzed for association with outcome. RESULTS: The 3- and 5-year survival rates of patients with SCLN metastasis were 16.5% and 16.5%, respectively. Multivariate analysis showed the serum level of squamous cell carcinoma antigen (SCC-Ag) < 15 ng/ml at initial diagnosis (p = 0.021) and staging/restaging including [18F] fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) (p = 0.006) to be associated with a better prognosis. CONCLUSION: Primary SCLN metastasis in cervical cancer is not incurable. The benefit from PET findings might help in selecting appropriate patients for curative primary and/or salvage treatment.

Multidetector computed tomography assessment on tumor size and nodal status in patients with locally advanced breast cancer before and after neoadjuvant chemotherapy.

AIMS: To evaluate the utility of multidetector computed tomography (MCT) in assessing tumor size and nodal status in patients with advanced breast cancers before and after the neoadjuvant chemotherapy. METHODS: Twenty-eight proven locally advanced breast cancer patients with 30 tumors were enrolled in this study. MCT was used to assess tumor size and axillary lymph nodes before and after the neoadjuvant chemotherapy. The correlation between tumor size on MCT and gross tumor size was tested. RESULTS: The MCT measurements documented complete response in 3, partial response in 18, non-response in 8 and progressed in 1. The mean tumor diameters on pathology and post-chemotherapy MCT were 3.6cm (S.D.=+/-2.9cm) and 3.1cm (S.D.=+/-2.6cm), respectively. The Pearson correlation coefficient was 0.76 (p<0.001). The sensitivity, specificity, positive predictive valve, negative predictive valve and accuracy of MCT in diagnosing the axillary lymph node metastases after pre-operative neoadjuvant chemotherapy were counted, respectively, to 72%, 40%, 85.7%, 22.2% and 66.7%. All the 5 downstaged axillary nodal statuses from node-positive to node-negative on MCT had micrometastases. CONCLUSION: MCT can be used to evaluate tumor size and nodal status in patients with advanced breast cancer. As there is a baseline MCT before chemotherapy for comparison, we are potentially aware of the possibility of false negative nodal micrometastases on the post-chemotherapy MCT.

Detection of recurrent or persistent nasopharyngeal carcinomas after radiotherapy with 18-fluoro-2-deoxyglucose positron emission tomography and comparison with computed tomography.

PURPOSE: The effectiveness of positron emission tomography (PET) with 1 8-fluoro-2-deoxyglucose (FDG) for detecting suspected recurrence of nasopharyngeal carcinomas (NPC) was evaluated and compared with computed tomography (CT). PATIENTS AND METHODS: FDG-PET studies were performed on 36 NPC patients 4 months after radiotherapy. The images were interpreted visually and quantitatively by calculating standardized uptake values (SUVs). RESULTS: The sensitivity, specificity, and accuracy of visually interpreted FDG-PET images, for differentiation of recurrent or persistent NPC from benign lesions, were 100%, 96%, and 97%, respectively. Cases with recurrent or persistent NPC (1.6 to 5.8) had significantly higher SUVs than cases with benign lesions (0.8 to 1.5). The sensitivity, specificity, and accuracy of CT for detecting recurrent or persistent NPC were 72%, 88%, and 83%, respectively. CONCLUSION: FDG-PET is a better tool than CT for the detection of recurrent or persistent NPC. Either visual interpretation or SUV can be used to differentiate benign lesions from recurrent or persistent NPC.

An ovary in luteal phase mimicking common iliac lymph node metastasis from a primary cutaneous peripheral primitive neuroectodermal tumour as revealed by 18-fluoro-2-deoxyglucose positron emission tomography.

A 30-year-old female underwent surgical removal of a primary cutaneous peripheral primitive neuroectodermal tumour (PNET) of the left thigh. A subsequent 18-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) scan showed abnormal accumulation of FDG in the left upper pelvic region, consistent with metastasis to a left common iliac node. A series of follow-up imaging studies revealed that a cyst of the corpus luteum of ovary was responsible for the abnormal FDG accumulation.

Age-dependent increase of mitochondrial DNA deletions together with lipid peroxides and superoxide dismutase in human liver mitochondria.

We previously reported an age-dependent deterioration of mitochondrial respiration as well as two age-associated mitochondrial DNA (mtDNA) deletions in the human liver. In this study, we further determined the relative quantities of the deleted mtDNAs in liver biopsies from 64 subjects of different ages. The results showed that both mtDNA deletions increase in frequency and quantity with age. Moreover, we measured hepatic lipid peroxides (malondialdehyde; MDA) of isolated mitochondria and manganese superoxide dismutase (Mn-SOD) activity of submitochondrial particles. We found a significant age-dependent increase in both MDA and Mn-SOD levels in liver mitochondria. These results confirm the previous contention that enhanced generation of lipid peroxides in the mitochondria during the aging process may damage mtDNA, and mtDNA deletions may be one of the important factors contributing to aging in humans.

Comparative radionuclide imaging of metastatic insular carcinoma of the thyroid: value of technetium-99m-(V)DMSA.

We report a case of metastatic insular carcinoma of the thyroid evaluated with 201TI, 99mTc-MIBI, 99mTc-(V)DMSA, 99mTc-MDP and 131I whole-body scans, which were obtained after total thyroidectomy. For the majority of lesions detected in the skeleton and soft tissue, 131I images were generally available, although most were visualized easier with 99mTc-(V)DMSA. Technetium-99m-MDP images were considered better than 99mTc-(V)DMSA images in showing bone lesions but not soft-tissue lesions. Both 201TI and 99mTc-MIBI scans provided sufficient advantage to exhibit neck and mediastinal metastases, but they did not surpass 99mTc-(V)DMSA in detecting abdominal or bony lesions. In this patient with various metastases from insular carcinoma of the thyroid, 99mTc-(V)DMSA seemed to be the tracer of choice for whole-body imaging.

Technetium-99m-HMPAO brain SPECT in systemic lupus erythematosus with CNS involvement.

UNLABELLED: Functional brain SPECT is playing an increasingly important role in evaluating CNS conditions in patients with systemic lupus erythematosus (SLE). However, SPECT findings varied in different studies because of their small population. Furthermore, earlier researchers, being restricted by the resolution of the camera, might not have been able to evaluate deep-seated nuclei such as the basal ganglia and thalamus. In this study, we describe the different patterns of SPECT findings in SLE patients with CNS involvement. METHODS: Seventy-two SLE patients (aged 14-67 yr; mean 33.2 yr) were divided into three groups: Group 1 with definite neuro-psychiatric disorder (including stroke, seizures and psychosis); Group 2 with minor neuropsychiatric disorders (headache, dizziness and recent memory impairment); and Group 3 without any neuropsychiatric symptoms or signs. Ninety minutes after injection of 1110 MBq 99mTc-HMPAO, brain SPECT was performed using a dual-head camera and fan-beam collimator. In addition, MRI and an electroencephalography (EEG) were also performed. RESULTS: SPECT findings were normal in 87% of the Group 3 patients and abnormal in all Group 1 patients; 84.6% of the Group 2 patients had abnormal SPECT findings. The parietal, frontal and temporal lobes were the most common areas of CNS involvement. Parietal lobes were involved in 95.6% of Group 1 patients and 80.7% in Group 2 patients. Frontal lobes were involved in 56.5% of Group 1 patients and 65.3% of Group 2 patients. Temporal lobes were involved in 56.5% of Group 1 patients and 46.1% of Group 2 patients. The basal ganglion was involved in about 30% of Group 1 patients and 11.5% of Group 2 patients, while the thalamus and cerebellum were less involved in neuropsychiatric SLE. MR images showed less sensitivity in the detection of CNS involvement than the SPECT and were normal in 27.3% of patients with definite neuropsychiatric disorders. The EEG and anticardiolipin antibody did not correlate well to the clinical diagnosis. CONCLUSION: HMPAO brain SPECT had the best correlation with the clinical diagnosis and may provide additional and objective information on SLE patients with potential CNS involvement.

Gastric emptying in male neurologic trauma.

UNLABELLED: Prolonged gastric emptying half-time (GET1/2) has been observed in several neurological disorders. Most patients with moderate to severe neurologic trauma (NT) initially do not tolerate enteral or nasogastric feedings. However, previous findings of altered gastric emptying (GE) in patients with NT have been questionable. Quantitative measurements of GE, to determine a possible mechanism for intolerance to enteral feeding, are lacking. In this study, we measured GET1/2 sec of solid and liquid meals by radionuclide imaging in men who were neurologic trauma patients. METHODS: A prospective study was conducted to assess GET1/2 in 30 men who were patients with spinal cord injuries (SCIs) and 20 men who were patients with head injuries (HIs) using radionuclide-labeled solid and liquid meals, respectively. Meanwhile, 18 and 14 male control subjects underwent the same imaging techniques for solid and liquid meals, respectively, to evaluate the normal ranges of solid and liquid GET1/2 sec (84.5 +/- 16.7 and 29.2 +/- 3.7 min). RESULTS: In the 30 SCI patients, GET1/2 of solid meals was significantly prolonged (138.3 +/- 49.2 min, p < 0.05), and 53% (16/30) of patients had abnormal GET1/2. A more prolonged GET1/2 and a higher incidence of abnormal GET1/2 were observed in patients with high-level injury, when compared with patients with low-level injury (p < 0.05). In the 20 HI patients, GET1/2 of liquid meals was prolonged significantly (51.7 +/- 24.8 min, p < 0.05), and 65% (13/20) of patients had abnormal GET1/2. Coma, as indicated by the Glasgow Coma Scale score, was not a statistically significant factor influencing GET1/2 (p >0.05). CONCLUSION: NT can cause significantly prolonged GE, especially in patients with high-level SCI.

Differential diagnosis of Parkinson's disease and vascular parkinsonism by (99m)Tc-TRODAT-1.

UNLABELLED: The aim of this study was to use brain SPECT to differentiate vascular parkinsonism (VP) from Parkinson's disease. METHODS: Fourteen VP patients (age range, 59-87 y; mean age, 70 +/- 7.5 y), 30 Parkinson's disease patients (age range, 54-84 y; mean age, 65 +/- 8.8 y), and 26 healthy (control) individuals (age range, 50-85 y; mean age, 60 +/- 9 y) were examined. A 925-MBq (25 mCi) dose of (99m)Tc-TRODAT-1 was injected intravenously, and brain SPECT images were acquired 4 h after injection. The ratio of specific to nonspecific striatal (99m)Tc-TRODAT-1 binding was measured and compared. RESULTS: After a region-of-interest analysis of the images from VP patients, Parkinson's disease patients, and healthy volunteers was performed to obtain ratios of putamen to occipital and striatal to occipital binding as a measurement of specific binding to the dopamine transporters in these regions of the brain, where dopamine neurons are concentrated, the specific binding in the 14 VP patients was slightly lower than but not statistically different from that of the healthy individuals in both putamen and caudate areas. A significant decrease in uptake of (99m)Tc-TRODAT-1 in the striatum (P<0.01) was found in Parkinson's disease patients. Reduction of the uptake was more pronounced in the contralateral putamen of Parkinson's disease patients than that of VP patients (P<0.001). A significant bilateral striatal asymmetry was also observed in Parkinson's disease patients but not in VP patients (P< 0.01). CONCLUSION: Our findings clearly show that, for VP patients, (99m)Tc-TRODAT-1 SPECT is a reliable method to differentiate VP from Parkinson's disease. Further studies, including those to differentiate Parkinson's disease from arteriosclerotic parkinsonism and patients with both VP and Parkinson's disease, are needed to help rule out the possibility of Parkinson's disease as early as possible.

Technetium-99m-DMSA renal SPECT in diagnosing and monitoring pediatric acute pyelonephritis.

UNLABELLED: This study compares the sensitivity of 99mTc-dimercaptosuccinic acid (DMSA) renal SPECT with planar scintigraphy, concluding the importance of 99mTc-DMSA renal SPECT for the early diagnosis of acute pyelonephritis (APN) in patients under 3 yr of age. METHODS: Twenty-seven children under 3 yr of age, with clinical and/or laboratory suspicion of APN, were investigated. All 99mTc-DMSA renal SPECT and planar images and voiding cystoureterogram (VCUG) were obtained within 3 days of hospitalization. RESULTS: In the first examination, renal cortical defects were detected in 23 patients (42 kidneys) with SPECT and in 9 patients (11 kidneys) with planar scintigraphy. One year after treatment, constant renal cortical lesions were observed in 11 patients (14 kidneys) with SPECT and 4 patients (4 kidneys) with planar scintigraphy. The high grades of vesicoureteral reflux (VUR) (grade >or= 3) correlate better with APN diagnosed by SPECT (34 kidneys) than by planar scintigraphy (8 kidneys). Multiple renal cortical defects (number of lesions >or= 4) were only seen in patients under 1.5 yr old and none of those with a negative 99mTc-DMSA renal SPECT had a positive 99mTc-DMSA renal planar scintigraphy at any time. There is a significant difference (p < 0.05) between the diagnostic ability of these two methods of examination. CONCLUSION: Our results suggest that 99mTc-DMSA renal SPECT should be used, where possible, instead of planar DMSA in routine examination of children with clinical suspicion of APN, especially for those under 3 yr of age.

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.

Mitochondrial DNA mutation in Leber's hereditary optic neuropathy.

Leber's hereditary optic neuropathy (LHON) causes acute or subacute central visual loss in healthy young males. Recently, it has been thought to be caused by a single nucleotide change in the ND4 gene in the mitochondrial genome. Mitochondrial DNA (mtDNA) of leukocytes and hair follicle cells from five patients in four families with LHON and nine relatives were analyzed by Sfa NI and Mae III enzyme digestion and DNA sequencing. Loss of Sfa NI site was found in all patients and maternal lineages but not in nonmaternal lineages and normal controls. Mae III digested all the mtDNAs that lost the Sfa NI site. The restriction fragment pattern of polymerase chain reaction (PCR) products exhibited mtDNA heteroplasmy in the hair follicle cells but not in blood cells of the proband in one family. Direct sequencing of PCR-amplified mtDNA fragments encompassing the ND4 gene of the patients disclosed a transition from guanine to adenine at nucleotide position 11778. These results confirm previous reports that a G to A point mutation is associated with LHON and that tissue variability and heteroplasmy of mtDNA exist in some, but not all, LHON patients.

Pedal (99m)Tc-sulfur colloid lymphoscintigraphy in primary isolated chylopericardium.

Primary isolated chylopericardium is a rare disorder in which chylous fluid accumulates in the pericardial space. In this case report of a 61-year-old man with chylopericardium, pedal (99m)Tc-sulfur colloid (SC) lymphoscintigraphy was performed after emergent pericardiocentesis, and when there was a recurrent massive pericardial effusion. The results showed that (99m)Tc-SC lymphoscintigraphy can clearly reveal the lymphodynamics in patients with primary isolated chylopericardium. This noninvasive investigation is valuable and can be easily performed either before or after pericardiocentesis.

A comprehensive study on the blockage of thyroid and gastric uptakes of 188Re-perrhenate in endovascular irradiation using liquid-filled balloon to prevent restenosis.

188Re-perrhenate has been reported effective in preventing restenosis after percutaneous transluminal coronary angioplasty. However, if the balloon ruptures, 188Re-perrhenate is released into the circulation, causing high radiation dosing to the thyroid and stomach. In this study, we evaluated the effects of perchlorate or iodide given at different times and in different ways for blocking the uptake of 188Re-perrhenate in the thyroid glands and the stomach to find the best method to apply clinically to reduce the radiation dose in case of balloon rupture. Sodium perchlorate, sodium iodide, or potassium iodide was given orally or intravenously to rats before, during, and after the injection of 188Re-perrhenate. The rats were sacrificed and we calculated the concentration of 188Re-perrhenate in various organs to evaluate the preblocking, mixed formula, and postblocking effects of perchlorate or iodide. Our data showed that the preblocking method effectively reduced the uptake of 188Re-perrhenate in both the thyroid and the stomach. The mixed formula method also demonstrated good blocking effect. The postblocking method showed obvious depression of thyroid uptake of perrhenate but its blocking effect on the stomach was not satisfactory.