Dae Soo Jung

Susceptibility-Weighted Imaging for Detection of Thrombus in Acute Cardioembolic Stroke

Background and Purpose Susceptibility-weighted imaging (SWI) can show an intravascular thrombus as a hypointense susceptibility vessel sign (SVS). In this study, we investigated the usefulness of SWI in the detection of an intravascular thrombus in acute cardioembolic stroke by comparing the SVS on SWI to the vessel status on time-of-flight magnetic resonance angiography (MRA). Methods We consecutively enrolled patients with cardioembolic stroke in the anterior circulation within 3 days from stroke onset. The frequency and location of the SVS on SWI were compared with those of occlusion on MRA. Results One hundred and twenty-two patients were conclusively enrolled in this study. The SVS was observed in 75.4% (92/122) of the enrolled patients. MRA showed occlusion in 57% (70/122) of the enrolled patients. The SVS was identified in all 70 patients with occlusion on MRA. The SVS was observed in 22 (42.3%) of 52 patients without occlusion on MRA (P<0.001), which was identified mainly in post-bifurcation segments of the middle cerebral artery: the M2 segment in 4 patients, M3 segment in 10 patients, M4 segment in 4 patients, A3 segment in 1 patient, and multiple segments in 2 patients. The mean length of the SVS in the M1 segment was 13.65 mm (median: 12.39 mm, length range: 2.70–39.50 mm). Conclusions SWI can provide useful information about the thrombus location, the presence of a single thrombus or multiple thrombi especially in distal intracranial arteries, and the thrombus burden, all in acute cardioembolic stroke.

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia linked CSF1R mutation: Report of four Korean cases.

We describe detailed clinical, biochemical, neuroimaging and neuropathological features in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), encompassing hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD), linked to colony-stimulating factor 1 receptor (CSF1R) mutations in four Korean cases. Clinical, biochemical, neuroimaging and neuropathological findings were obtained by direct evaluation and from previous medical records. The genetic analysis of the CSF1R gene was done in two autopsy-confirmed ALSP cases and two cases where ALSP was suspected based on the clinical and neuroimaging characteristics. We identified two known mutations: c.2342C>T (p.A781V) in one autopsy-proven HDLS and clinically ALSP-suspected case and c.2345G>A (p.R782H) in another autopsy-proven POLD case. We also found a novel mutation (c.2296A>G; p.M766V) in a patient presenting with hand tremor, stuttering and hesitant speech, and abnormal behavior whose father died from a possible diagnosis of spinocerebellar ataxia. To the best of our knowledge, this is the first documented ALSP-linked CSF1R mutation in Korea and supports the suggestion that HDLS and POLD, with pathological characteristics that are somewhat different but which are caused by CSF1R mutations, are the same spectrum of disease, ALSP.

Hemimacropsia after medial temporo-occipital infarction

Dysmetropsia is a disorder of visual perception characterised by an apparent modification of the size of perceived objects.1–3 Objects can appear larger (macropsia) or smaller (micropsia) than their actual size. Dysmetropsia can result from retinal oedema causing a dislocation of the receptor cells and from lesions affecting other parts of extracerebral visual pathways. Transient micropsia can also result from epileptic seizure, migraine, infectious mononucleosis, the action of mescaline and other hallucinogenic drugs, and psychopathological phenomena. Permanent dysmetropsia following focal cerebral lesions is rare. Most of the prior reports described hemimicropsia due to lesions mainly involving the lateral aspect of the visual association cortex.1–3 However, reports of hemimacropsia following focal cerebral lesions have been extremely rare1,4 and hemimacropsia following a focal vascular lesion has not been described previously. We describe a patient with left hemimacropsia due to right medial temporo-occipital infarction. A 64-year-old right-handed man with hypertension was admitted 4 days after a sudden onset of visual disturbance. He …

A Case of Lambert-Eaton Myasthenic Syndrome Associated with Atypical Bronchopulmonary Carcinoid Tumor

The Lambert-Eaton myasthenic syndrome (LEMS) is typically recognized as a paraneoplastic syndrome associated with a small cell lung carcinoma (SCLC), whereas LEMS with other neuroendocrine lung tumors, including carcinoids or large cell lung carcinoma, are highly unusual. Here, we report a rare case of LEMS with atypical bronchopulmonary carcinoid tumor: A 65-yr-old man presented with progressive leg weakness and a diagnosis of LEMS was made by serial repetitive nerve stimulation test. Chest CT revealed a lung nodule with enlargement of paratracheal lymph nodes, and surgically resected lesion showed pathological features of atypical carcinoid tumor. We concluded that LEMS could be associated with rare pulmonary neuroendocrine tumor other than SCLC, which necessitates pathologic confirmation followed by aggressive treatment for optimal management in these rare cases.

Emergent intracranial stenting for acute M2 occlusion of middle cerebral artery

OBJECTIVE Intracranial stenting is a possible option as a rescue strategy for acute secondary division (M2) occlusion of middle cerebral artery (MCA) when intravenous thrombolysis is ineffective or contraindicated. METHODS We reviewed 10 patients of acute M2 occlusion treated by intracranial stenting who were ineligible for intravenous thrombolysis or resistant to intravenous thrombolysis. All patients underwent intracranial stenting with the Wingspan stent system. We analyzed clinical and angiographic outcomes. RESULTS The mean NIHSS score on admission was 13.8 points (range 6-23). The occlusion sites were located in the superior division (n=4, left: 3, right: 1), the middle division (n=1, right) and the inferior division (n=5, all: right) of MCA. The mean time interval from stroke symptom onset to stenting was 348.9 ± 90.4 min. Successful recanalization was achieved in all patients. No intracranial hemorrhage, vessel perforations or dissections occurred in any patient. One patient developed acute thrombosis in distal ICA of the stented side at 4 days after a stent placement and was managed with mechanical thrombectomy. All patients improved on the NIHSS (mean amount: 8.8) and to the NIHSS score of 5 ± 4.6 (median 4.5, range 0-15) at 7 days. At discharge, an mRS of ≤ 3 was achieved in 8 patients (80%) and an mRS of ≤ 2 was achieved in 6 patients (60%). CONCLUSIONS Endovascular recanalization with a Wingspan stent can be a safe and feasible procedure for acute M2 occlusion when intravenous thrombolysis is ineffective or not available.

Functional outcome after recanalization for acute pure M1 occlusion of the middle cerebral artery as assessed by collateral CTA flow

OBJECTIVE Collateral flow is important for ischemic brain tissue after an acute occlusion of the cerebral artery. The purpose of this study was to evaluate baseline collateral flow, on CT angiography (CTA), as a predictor of functional outcome in patients who had endovascular recanalization, after acute pure first segment (M1) occlusion of the middle cerebral artery (MCA). METHODS Thirty patients with acute pure M1 occlusion treated by endovascular recanalization, who were ineligible for intravenous thrombolysis or resistant to intravenous thrombolysis, were reviewed. The relationship between baseline collateral flow, on CTA, and functional outcome, was analyzed. In addition, other factors affecting clinical outcome were assessed. RESULTS The mean NIHSS score on admission was 16.87±4.86 (7-24). The mean time interval between onset of stroke symptoms and recanalization was 324.37±68.38 (210-463) min. Successful recanalization (TICI 2b-3) was achieved in 18 patients (60%). Twenty-seven of 30 patients improved their NIHSS score (mean 8.4); NIHSS score 8.9±5.4 (median 10, range 0-16) at seven days. Two patients had aggravated symptoms and one patient had no change on the NIHSS score. At 90 days after recanalization, a modified Rankin Scale (mRS) of ≤3 was achieved in 15 patients (50%) and a mRS of ≤2 was achieved in nine patients (30%). Symptomatic intra-cerebral hemorrhage occurred in two patients (6.7%). Multivariate regression analysis showed an initial NIHSS score (p=0.004), grade of baseline collateral flow on CTA (p=0.025), presence of diabetes mellitus (p=0.037), and TICI scale (p=0.049) were factors associated with an improved NIHSS. For the mRS at 90 days, only the grade of the baseline collateral flow on CTA was associated with a good functional outcome (p=0.013). CONCLUSIONS The results of this study suggest that the grade of baseline collateral flow, on CTA, is an independent predictor of functional outcome for endovascular recanalization of acute pure M1 occlusion of the middle cerebral artery.

Directional and spatial motor intentional disorders in patients with right versus left hemisphere strokes.

OBJECTIVE Motor intentional disorders (MIDs) are characterized by dysfunction in the preparation, initiation, maintenance, and termination of goal-oriented actions. In this study, we investigated (1) whether patients with right hemisphere strokes (RHS) and left hemisphere strokes (LHS) differ in the frequency of delayed action initiation (hypokinesia) and motor impersistence; (2) whether there is a directional or hemispatial component of hypokinesia or motor impersistence; (3) whether there is an association between the presence of hemispatial neglect and tests for MID; and (4) the location of injury associated with MID. METHOD Thirty-two patients with acute unilateral stroke (21 with RHS and 11 with LHS) and 12 age-matched healthy controls participated in the study. To determine the presence and severity of directional and spatial hypokinesia and impersistence we used a new apparatus, the Directional Movement Tester (DMT). While being tested with the DMT, the participants held a static bar located either in the right or left hemispace using either their right or left hand and upon stimulus onset pushed the bar either leftward or rightward and maintained a given force for 10 sec. RESULTS AND CONCLUSION The frequency of hypokinesia and impersistence was higher in the RHS group than in the LHS group, but there were no hypokinetic directional or spatial asymmetries. The RHS group did demonstrate left contralesional directional impersistence, but there were no spatial asymmetries of impersistence. Signs of hemispatial neglect were not associated with these measures of MID. Participants with frontal or subcortical lesions were significantly more likely to demonstrate hypokinesia and impersistence than those with posterior lesions.

Clinical staging of semantic dementia in an FDG-PET study using FTLD-CDR.

Background: The frontotemporal lobar degeneration-specific clinical dementia rating (FTLD-CDR), which was recently developed to measure frontotemporal dementia (FTD) severity, includes 2 items that assess language and behavior in addition to the 6 items of the conventional CDR. Methods: To investigate which of the 3 ratings, i.e. the global score of the CDR (GCDR), the behavioral domain score of the FTLD-CDR (BCDR), or the language domain score of the FTLD-CDR (LCDR), is most suitable for monitoring the progression of semantic dementia (SD), the number of hypometabolic voxels was calculated by comparing 28 SD patients in each stage of the 3 ratings with 63 age/sex-matched controls using voxel-based statistical parametric mapping. Results: The hypometabolic areas increased as a function of the LCDR score in SD patients. However, hypometabolic areas associated with the GCDR did not increase gradually as the stage increased. Furthermore, those associated with the BCDR showed the reverse pattern. Conclusion: Our findings suggest that the severity and patterning of glucose hypometabolism measured by the LCDR correspond well with the natural course of SD reported in previous clinical and neuroimaging studies, whereas the BCDR and GCDR did not reflect disease progression in SD.

Recanalization with Wingspan stent for acute middle cerebral artery occlusion in failure or contraindication to intravenous thrombolysis: a feasibility study.

BACKGROUND AND PURPOSE: Recanalization with the Wingspan stent, which can be deployed rapidly and safely, is an option for treating acute ischemic stroke when intravenous thrombolysis has failed or is contraindicated. This study was performed to evaluate feasibility, efficacy, and safety of recanalization for acute middle cerebral artery occlusion using the Wingspan stent. MATERIALS AND METHODS: We collected 10 patients with acute MCA occlusion in whom recanalization was not achieved with a standard intravenous thrombolysis, or who were ineligible for intravenous thrombolysis, or who presented after 3 hours of symptom onset and in whom the stent placement could be completed within 8 hours from symptom onset. We analyzed angiographic and clinical results. RESULTS: Successful recanalization with the Wingspan stent was achieved in all patients. The mean NIHSS score on admission was 12.7 points (range 4–21). The occlusion sites were located in the 1st segment (n = 7; 2 left, 5 right) and 2nd segment (n = 3, all right) of the MCA. The mean time interval from stroke symptom onset to stent placement was 344.8 ± 76.3 minutes. No intracranial hemorrhage, vessel perforations, or dissections occurred in any patient. Nine patients improved on the NIHSS at 7 days. One patient did not have a change in the NIHSS score, even though the occluded artery was completely recanalized. At 7 days, the NIHSS score of all patients was 4.4 ± 4.7 (median 4, range 0–13). At discharge, an mRS of ≤3 was achieved in all patients and an mRS of ≤2 was achieved in 7 patients (70%). CONCLUSIONS: This small case series demonstrates the feasibility of using the Wingspan stent safely and effectively for MCA occlusions when standard treatments are ineffective or not available.

Corneal Endothelial Changes as a Clinical Diagnostic Indicator of Dentatorubropallidoluysian Atrophy

Objective To present a rare case of patient diagnosed with dentatorubropallidoluysian atrophy (DRPLA) accompanied by corneal endothelial cell loss. Methods A 37-year-old man with choreoathetoid movement and cerebellar ataxia was diagnosed with DRPLA based on a DNA analysis compared with that of healthy control subjects. We examined the best corrected visual acuity, color vision, light reflex, topography, corneal thickness, fundus, fluorescein angiograpic findings, the visual field, ERG, specular microscopy as well as MRI and serologic tests. Results The best corrected visual acuity was 20/20 in both eyes by Snellen chart, and the other ocular findings were within normal limits except for a significantly decreased corneal endothelial cell density, 876 cells/mm2 in the right eye and 941 cells/mm2 in the left eye. Conclusions A patient with neurodegenerative disorders such as choreathetoid movement, myoclonic seizure, cerebellar ataxia, and dementia should be examined specifically by specular microscopy because corneal endothelial cell loss is the only clinical diagnostic indicator of DRPLA.