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No abstract available.
Brain Injuries ; Neuroimaging

No abstract available.
Humans ; Interferon-beta ; Interferons ; Multiple Sclerosis ; Porphyria Cutanea Tarda ; Porphyrias

BACKGROUND: Acute transient obstructive hydrocephalus is rare in adults. We describe a patient with intraventricular hemorrhage (IVH) who experienced the delayed development of acute transient hydrocephalus. CASE REPORT: A 33-year-old man with a previously diagnosed Spetzler-Martin Grade 5 arteriovenous malformation presented with severe headache, which was found to be due to IVH. Forty hours after presentation he developed significant obstructive hydrocephalus due to the thrombus migrating to the cerebral aqueduct, and a ventriculostomy placement was planned. However, shortly thereafter his headache began to improve spontaneously. Within 4 hours after onset the headache had completely resolved, and an interval head CT scan revealed resolution of hydrocephalus. CONCLUSIONS: In patients with IVH, acute obstructive hydrocephalus can develop at any time after the ictus. Though a delayed presentation of acute but transient obstructive hydrocephalus is unusual, it is important to be aware of this scenario and ensure that deterioration secondary to thrombus migration and subsequent obstructive hydrocephalus do not occur.
Adult ; Arteriovenous Malformations ; Cerebral Aqueduct ; Dietary Sucrose ; Head ; Headache ; Hemorrhage ; Humans ; Hydrocephalus ; Thrombosis ; Ventriculostomy

BACKGROUND AND PURPOSE: Sudden cardiac death is one of the leading causes of death in patients with myotonic dystrophy type 1 (DM1). It has been proposed that a prolonged QT interval is associated with sudden cardiac death in several neurological diseases, including multiple system atrophy, idiopathic Parkinson's disease, and diabetic autonomic neuropathy. However, analyses of the corrected QT (QTc) interval in DM1 patients are rare in the literature. The purposes of this study were to determine the association between the QT interval and DM1, and the affecting factors. METHODS: Thirty-nine patients diagnosed with DM1 through genetic testing were enrolled. The QTc interval (calculated using Bazett's formula: QTc=QT/radicalRR) was compared between these patients and 39 normal healthy controls. The clinical and laboratory factors affecting QTc interval in the patient group were investigated. RESULTS: The QTc interval was significantly longer in the DM1 group (411.2+/-44.7 msec, mean+/-SD) than in the normal control group (355.6+/-20.6 msec). Intragroup analysis revealed that a prolonged QTc interval in DM1 patients was associated with being female and older, having a longer disease duration, and exhibiting abnormal electrocardiography findings. CONCLUSIONS: The higher incidence of sudden cardiac death in the DM1 population is associated with the observed prolonged QTc interval in those patients.
Cause of Death ; Death, Sudden, Cardiac ; Diabetic Neuropathies ; Electrocardiography ; Female ; Genetic Testing ; Humans ; Incidence ; Multiple System Atrophy ; Myotonic Dystrophy ; Parkinson Disease

BACKGROUND AND PURPOSE: To estimate clinical roles of 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) versus angiography and ultrasonography in carotid plaque characterization. METHODS: We characterized two groups of patients with recently (<1 month) symptomatic (n=14; age=71.8+/-8.6 years, mean+/-SD) or chronic (n=13, age=68.9+/-9.0 years) carotid stenosis using a battery of imaging tests: diffusion magnetic resonance (MR) imaging, MR or transfemoral angiography, duplex ultrasonography (DUS), and carotid FDG-PET/computed tomography. RESULTS: The degree of angiographic stenosis was greater in patients with recently symptomatic carotid plaques (67.5+/-21.5%) than in patients with chronic carotid plaques (32.4+/-26.8%, p=0.001). Despite the significant difference in the degree of stenosis, lesional maximum standardized uptake values (maxSUVs) on the carotid FDG-PET did not differ between the recently symptomatic (1.56+/-0.53) and chronic (1.56+/-0.34, p=0.65) stenosis groups. However, lesional-to-contralesional maxSUV ratios were higher in the recently symptomatic stenosis group (113+/-17%) than in the chronic stenosis group (98+/-10%, p=0.017). The grayscale median value of the lesional DUS echodensities was lower in the recently symptomatic stenosis group (28.2+/-10.0, n=9) than in the chronic stenosis group (53.9+/-14.0, n=8; p=0.001). Overall, there were no significant correlations between angiographic stenosis, DUS echodensity, and FDG-PET maxSUV. Case/subgroup analyses suggested complementarity between imaging modalities. CONCLUSIONS: There were both correspondences and discrepancies between the carotid FDG-PET images and DUS or angiography data. Further studies are required to determine whether FDG-PET could improve the clinical management of carotid stenosis.
Angiography ; Atherosclerosis ; Carotid Stenosis ; Constriction, Pathologic ; Diffusion ; Fluorodeoxyglucose F18 ; Humans ; Magnetic Resonance Spectroscopy ; Molecular Imaging ; Positron-Emission Tomography

BACKGROUND AND PURPOSE: Several circulating biomarkers have been implicated in carotid atherosclerotic plaque rupture and thrombosis; however, their clinical utility remains unknown. The aim of this study was to determine the role of a large biomarker panel in the discrimination of symptomatic (S) vs. asymptomatic (A/S) subjects in a contemporary population with carotid artery stenosis (CS). METHODS: Prospective sampling of circulating cytokines and blood lipids was performed in 300 unselected, consecutive patients with > or =50% CS, as assessed by duplex ultrasound (age 47-83 years; 110 with A/S and 190 with S) who were referred for potential CS revascularization. RESULTS: CS severity and pharmacotherapy did not differ between the A/S and S patients. The median values of total cholesterol, low-density lipoprotein cholesterol, and lipoprotein(a) did not differ, but high-density lipoprotein (HDL) cholesterol was significantly higher (p<0.001) and triglycerides were lower (p=0.03) in the A/S-CS group than in the S-CS group. Interleukin-6 (IL-6) and high-sensitivity C-reactive protein were higher (p=0.04 and p=0.07, respectively) in the S-CS group. Circulating visfatin, soluble CD 40 receptor ligand, soluble vascular cell adhesion molecule, leptin, adiponectin, IL-1beta, IL-8, IL-18, monocyte chemoattractant protein-1, myeloperoxidase, matrix metalloproteinases-8, -9, and -10, and fibrinogen were similar, but tissue inhibitor of matrix metalloproteinases-1 (TIMP) was reduced in S-CS compared to A/S-CS (p=0.02). Nevertheless, incorporation of TIMP and IL-6 did not improve the HDL-cholesterol receiver operating characteristics for S-CS status prediction. S-CS status was unrelated to angiographic stenosis severity or plaque burden, as assessed by intravascular ultrasound (p=0.16 and p=0.67, respectively). Multivariate logistic regression analysis revealed low HDL-cholesterol to be the only independent predictor of CS symptoms, with an odds ratio of 1.81 (95% confidence interval=1.15-2.84, p=0.01) for HDL <1.00 mmol/L (first quartile) vs. >1.37 (third quartile). In S-CS, osteoprotegerin and lipoprotein-associated phospholipase A2 (Lp-PLA2) were elevated in those with recent vs. remote symptoms (p=0.01 and p=0.02, respectively). CONCLUSIONS: In an all-comer CS population on contemporary pharmacotherapy, low HDL-cholesterol (but not other previously implicated or several novel circulating biomarkers) is an independent predictor of S-CS status. In addition, an increase in circulating osteoprotegerin and Lp-PLA2 may transiently indicate S transformation of the carotid atherosclerotic plaque.
1-Alkyl-2-acetylglycerophosphocholine Esterase ; Adiponectin ; Biomarkers ; C-Reactive Protein ; Carotid Arteries ; Carotid Stenosis ; Chemokine CCL2 ; Cholesterol ; Constriction, Pathologic ; Cytokines ; Discrimination (Psychology) ; Fibrinogen ; Humans ; Interleukin-18 ; Interleukin-6 ; Interleukin-8 ; Leptin ; Lipoprotein(a) ; Lipoproteins ; Logistic Models ; Nicotinamide Phosphoribosyltransferase ; Odds Ratio ; Osteoprotegerin ; Peroxidase ; Plaque, Atherosclerotic ; Prospective Studies ; Risk Factors ; ROC Curve ; Rupture ; Triglycerides ; Vascular Cell Adhesion Molecule-1

BACKGROUND AND PURPOSE: It is recommended that Botox be used within 5 hours of reconstitution, which results in substantial quantities being discarded. This is not only uneconomic, but also inconvenient for treating patients. The aim of this study was to determine the potencies of Botox used within 2 hours of reconstitution with unpreserved saline, the same Botox refrigerated (at +4degrees C) 72 hours after reconstitution, and during the next 4 consecutive weeks (weeks 1, 2, 3, and 4). This comparison was used to determine the length of refrigeration time during which reconstituted Botox will maintain the same efficacy as freshly reconstituted toxin. METHODS: Individual paralysis rates in the extensor digitorum brevis (EDB) compound muscle action potential (CMAP) amplitude and area were measured 1 week after injecting fresh reconstituted 2.5 MU of Botox on one side of the foot, and when the same quantity of Botox that had been refrigerated for a designated time (i.e., 72 h, or 1, 2, 3, or 4 weeks) into the other side of the foot. The EDB CMAP amplitude and area at 12 and 16 weeks postinjection were also measured to compare the efficacy durations in all five comparative groups. RESULTS: Ninety-four volunteers were divided into five groups according to the refrigerator storage time of the second Botox injection. The paralysis of the EDBs was significant for each injection of Botox, both fresh and refrigerated, with no statistically significant differences between them, regardless of the refrigeration time. There was a tendency toward increased CMAP amplitude and area at 12 or 16 weeks postinjection (p<0.0001). The duration of effective muscle paralysis did not differ significantly throughout the 16-week follow-up period between all five groups. CONCLUSIONS: The potency of reconstituted Botox is not degraded by subsequent refrigeration for 4 weeks. However, there are definite concerns regarding its sterility, and hence its safety, since multiple withdrawals from the same vial over long periods can introduce bacterial contamination.
Action Potentials ; Botulinum Toxins, Type A ; Follow-Up Studies ; Foot ; Humans ; Infertility ; Muscles ; Paralysis ; Refrigeration

BACKGROUND AND PURPOSE: PET scanning with fluorodeoxyglucose (FDG-PET) is a non-invasive method that measures regional glucose metabolic rate. Phenylalanine (Phe) and its metabolites appear to impair several aspects of brain energy metabolism. 1) To evaluate brain glucose metabolism with FDG-PET imaging in phenylketonuria (PKU) patients before and 4 months after sapropterin therapy; 2) to evaluate neurodevelopmental changes, blood Phe levels and dietary Phe tolerance before and after sapropterin therapy; 3) to generate pilot data to assess the feasibility of evaluating brain glucose metabolism with FDG-PET imaging and to explore potential trends resulting from the administration of sapropterin therapy. METHODS: We enrolled 5 subjects, ranged in age from 22 years to 51 years, with PKU. Subjects underwent FDG-PET brain imaging, blood tests for Phe and tyrosine levels, and neurocognitive evaluations before and 4 months after sapropterin therapy (20 mg/kg/day). All subjects' Phe and tyrosine levels were monitored once a week during the study. Subjects kept 3 day diet records that allow calculation of Phe intake. RESULTS: None of the subjects responded to sapropterin therapy based on 30% decrease in blood Phe level. The data show that glucose metabolism appeared depressed in the cerebellum and left parietal cortex while it was increased in the frontal and anterior cingulate cortices in all five subjects. In response to sapropterin therapy, relative glucose metabolism showed significant increases in left Broca's and right superior lateral temporal cortices. Interestingly, there was corresponding enhanced performance in a phonemic fluency test performed during pre- and postneurocognitive evaluation. CONCLUSIONS: Further studies with a larger sample size are needed to confirm the above changes in both sapropterin non-responsive and responsive PKU patients.
Biopterin ; Brain ; Cerebellum ; Diet Records ; Electrons ; Energy Metabolism ; Glucose ; Hematologic Tests ; Humans ; Neuroimaging ; Phenylalanine ; Phenylketonurias ; Pilot Projects ; Positron-Emission Tomography ; Sample Size ; Tyrosine