BACKGROUND: Gerstmann-Straussler-Scheinker disease (GSS) is a type of human transmissible spongiform encephalopathy (TSE) that is determined genetically. CASE REPORT: A 46-year-old woman presented with a slowly progressive ataxic gait and cognitive decline. She was alert but did not cooperate well due to severe dementia and dysarthria. High signal intensities in the cerebral cortices were evident in MRI, especially in diffusion-weighted images (DWI). A prion protein gene (PRNP) analysis revealed a P102L (proline-to-leucine) mutation in codon 102. CONCLUSIONS: This is the first reported case of GSS (confirmed by PRNP analysis) in Korea. Distinctive MRI findings are also presented.
Cerebral Cortex ; Codon ; Dementia ; Dysarthria ; Female ; Gait ; Gerstmann-Straussler-Scheinker Disease ; Humans ; Korea ; Middle Aged ; Prion Diseases

BACKGROUND: It has recently been suggested that diffusion and perfusion MRI can identify subgroups likely to benefit or potentially be harmed by reperfusion therapies. CASE REPORT: We investigated serial MRI data of two patients with occlusion of the proximal middle cerebral artery (MCA). In both cases, acute multiple cortical infarcts evident on diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI) showed extensive areas of severe perfusion delays, indicating a malignant MRI profile. However, despite the malignant MRI profiles in these cases, no new ischemic lesions or hemorrhage evolved even in the presence of persistent arterial occlusion, and the patients recovered without sequelae. CONCLUSIONS: These two cases suggest that time-domain PWI findings should be interpreted with caution in certain scenarios of acute ischemic stroke.
Diffusion ; Hemorrhage ; Humans ; Ischemia ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Perfusion ; Reperfusion ; Stroke

BACKGROUND: While tonic pupils have been attributed to various diseases, including syphilis, herpes zoster, orbital trauma, temporal arteritis, endometriosis, and paraneoplastic syndromes, obstructive hydrocephalus has not been implicated. CASE REPORT: A 36-year-old woman visited a neurology department with a 7-day history of throbbing headache and blurred vision in both eyes. She had early dorsal midbrain syndrome mimicking an Adie's tonic pupil, and cholinergic supersensitivity was demonstrated using topical 0.125% pilocarpine. Brain MRI revealed obstructive hydrocephalus at the level of the aqueduct of Sylvius, and her symptoms resolved 4 days after surgery. CONCLUSIONS: We report a patient with early dorsal midbrain syndrome that was initially believed to represent a tonic pupil on the basis of pharmacologic testing. The findings in our patient suggested that early dorsal midbrain syndrome mimicking an Adie's tonic pupil can be caused by obstructive hydrocephalus compressing the Edinger-Westphal nucleus.
Adult ; Brain ; Cerebral Aqueduct ; Endometriosis ; Eye ; Female ; Giant Cell Arteritis ; Headache ; Herpes Zoster ; Humans ; Hydrocephalus ; Mesencephalon ; Neurology ; Orbit ; Paraneoplastic Syndromes ; Pilocarpine ; Syphilis ; Tonic Pupil ; Vision, Ocular

BACKGROUND AND PURPOSE: Multifocal seeding of the leptomeninges by malignant cells, which is usually referred to as leptomeningeal carcinomatous metastasis, produces substantial morbidity and mortality. The diagnosis of leptomeningeal metastasis is usually established by cerebrospinal fluid (CSF) investigation, including cytology, cell counts, protein, glucose, and a tumor marker such as carcinoembryonic antigen (CEA). This study examined the diagnostic value of CEA in the CSF. METHODS: We measured the CSF CEA level in 32 patients with leptomeningeal metastasis. The control group consisted of 19 cancer patients without leptomeningeal metastasis. CEA was measured by the chemiluminescent emission method. RESULTS: The CEA level was significantly higher in patients with leptomeningeal metastasis than in the control group (p<0.005). The level of CSF protein was higher and that of CSF glucose was lower in patients with leptomeningeal metastasis than in the control group (p<0.005). CONCLUSIONS: The CSF CEA level is useful for diagnosing leptomeningeal carcinomatous metastasis. The CSF levels of protein and glucose are also useful in the diagnosis.
Carcinoembryonic Antigen ; Cell Count ; Glucose ; Humans ; Neoplasm Metastasis ; Seeds

BACKGROUND AND PURPOSE: Frovatriptan is a selective 5-HT1B/1D agonist with a long duration of action and a low incidence of side effects. Although several placebo-controlled trials have documented the clinical efficacy and safety of frovatriptan in adults with migraine, this drug has not previously been studied in Asian including Korean patients. METHODS: In this double-blind multicenter trial, 229 patients with migraine were randomized to receive frovatriptan 2.5 mg or placebo upon the occurrence of a moderate-to-severe migraine. The primary outcome was the 2-hour headache response rate. RESULTS: Frovatriptan significantly increased the 2-hour headache response rate compared with placebo (52.9% vs. 34.0%, p=0.004). The headache response rates at 4, 6, and 12 hours were significantly higher in the frovatriptan group than in the placebo group, as was the pain-free rate at 2 hours (19.0% vs. 5.7%, p=0.004), 4 hours (40.7% vs. 23.0%, p=0.006), and 6 hours (56.1% vs. 34.0%, p=0.002). The median time to a headache response was significantly shorter in the frovatriptan group than in the placebo group (2.00 hours vs. 3.50 hours, p<0.001). The use of rescue medications was more common in the placebo group (p=0.005). Chest tightness associated with triptan was infrequent (2.5%), mild, and transient. CONCLUSIONS: These results demonstrate that 2.5-mg frovatriptan is effective and well tolerated in Korean migraineurs for acute treatment of migraine attacks.
Adult ; Asian Continental Ancestry Group ; Carbazoles ; Headache ; Humans ; Incidence ; Migraine Disorders ; Oxalates ; Thorax ; Tryptamines

BACKGROUND AND PURPOSE: Ischemic lesion recurrence on diffusion-weighted imaging (DWI-LR) is a frequently observed phenomenon after acute ischemic stroke. However, no study has elucidated the impact of DWI-LR on functional outcome. METHODS: Among a consecutive series of patients who presented with focal symptoms or signs compatible with stroke within 48 hours from the onset over a 50-month period, those who had relevant ischemic lesions on initial DWI and underwent follow-up DWI within 14 days after the onset were enrolled in this study. As outcome variables, the scores on the modified Rankin Disability Scale (mRDS) at 3 months and 1 year were measured prospectively and dichotomized into good (0-2) vs. poor (3-6). When calculating odds ratios (ORs), adjustment was performed for age, previous stroke, initial score on the NIH Stroke Scale, stroke subtype, and IV thrombolysis. RESULTS: Among those 786 patients finally enrolled in this study, 221 (28.1%) had DWI-LR. For a poor outcome at 3 months, the crude ORs of any, symptomatic, and asymptomatic DWI-LR were 2.70 [95% confidence interval (CI), 1.96 to 3.72], 10.03 (95% CI, 4.39 to 22.96), and 2.04 (95% CI, 1.44 to 2.88), respectively. With adjustment, the OR of symptomatic DWI-LR was 6.44 (95% CI, 2.50 to 16.57), whereas those of any and asymptomatic DWI-LR lost their statistical significance: 1.44 (95% CI, 0.94 to 2.20) and 1.04 (95% CI, 0.65 to 1.65), respectively. Analyzing with the 1-year outcome produced similar results. CONCLUSIONS: This study shows that symptomatic early lesion recurrence can affect functional outcome after acute ischemic stroke, whereas an asymptomatic one may not.
Cerebral Infarction ; Diffusion Magnetic Resonance Imaging ; Electrolytes ; Follow-Up Studies ; Humans ; Odds Ratio ; Prospective Studies ; Recurrence ; Stroke

Molecular imaging is a novel technology to visualize biological processes at the cellular and molecular levels, which is reshaping both biomedical research and clinical practice. By providing molecular information to supplement and augment conventional anatomy-based imaging, molecular imaging is expected to allow 1) the earlier detection of diseases, 2) precise evaluation of disease stages, and 3) both diagnostic and therapeutic monitoring of disease progression in a quantitative manner. In this brief review, we present our view on the prospects of molecular optical imaging in the field of stroke practice, focusing on the imaging vulnerability of atherosclerotic plaques, thrombolytic resistance, real-time cerebral perfusion, and penumbra.
Biological Processes ; Disease Progression ; Molecular Imaging ; Optical Imaging ; Perfusion ; Plaque, Atherosclerotic ; Stroke

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Brain Injuries ; Neuroimaging