Dong-Seok Oh

Two different clinical entities of small vessel occlusion in TOAST classification.

BACKGROUND Small deep infarcts might be classified into 2 types: lacunar and branchatheromatous infarcts. However, since their initial description, small deep infarcts were still regarded as the same category of the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification, small vessel occlusion (SVO). We hypothesized that the 2 types of small deep infarcts would be distinct clinical entities. This study was conducted to investigate the clinical characteristics in the 2 groups of patients according to lesion pattern and combined atherosclerotic diseases. METHODS We included patients with small deep infarcts in the subcortical area. The patients were divided into 2 groups: (1) island lesions and (2) linear lesions on coronal diffusion weighted imaging. The status of the relevant artery was categorized as no stenosis, non-significant (<50% of luminal narrowing) and significant (≥ 50% of luminal narrowing). We compared the clinical and imaging characteristics of two lesion types according to various arterial status. RESULTS This study analyzed a total of 248 patients. Independent factors for island lesions on coronal DWI were male, severe leukoaraiosis, microbleeds, abnormal glycated hemoglobin (HbA1C), and abnormal estimated glomerular filtration ratio (eGFR) adjusted by age, sex, and initial National Institutes of Health Stroke Scale. In addition, in patients without significant relevant arterial stenosis, island lesion patterns were more frequently associated with severe periventricular white matter hyperintensity, diabetes mellitus, abnormal eGFR and abnormal HbA1C than linear lesion patterns. CONCLUSION This study demonstrated that SVO of TOAST classifications had different imaging and clinical characteristics according to the lesion patterns of coronal imaging. It suggests that two types of SVO should be regarded as the different categories of stroke classification.

Rotational vertebral artery syndrome in moyamoya disease: A sign of unilateral vertebral artery stenosis

Rotational vertebral artery syndrome (RVAS), also known as ow hunter’s syndrome (BHS), causes a transient hemodynamic schemia due to reversible compromises of the posterior circulation 1]. It is characterized by recurrent attacks of vertigo, nystagmus, nd ataxia induced by head rotation [1,2]. In RVAS, the hemodyamic ischemia is caused by mechanical steno-occlusion of the ominant vertebral artery (VA) against a fibrous band or bony rominence at the C1–C2 level by the head rotation under the conition that the blood supply through remained VA is insufficient 1–3]. The exact incidence of RVAS is unknown but thought to be rare. n literature reviews, we have found about 70 cases consistent with he diagnosis of RVAS, and it is suggested that the contralateral VA ends to be hypoplastic, stenotic or occluded [1,2]. However, RVAS s a sign of impending steno-occlusion of unilateral VA in moymoya disease has not yet been reported. We report a patient with oyamoya disease whose initially normal unilateral VA became tenotic during follow-up period and the other dominant VA was otentially compromised by head rotation that manifested as tranient vertigo and nystagmus.

Zebrafish as a tool for molecular diagnosis of Alexander disease

WCN 2013 No: 316 Topic: 6 — MS & Demyelinating Diseases Zebrafish as a tool for molecular diagnosis of Alexander disease T.-S. Nam, D.-S. Oh, G.-J. Yoon, I.-G. Kim, C.-S. Shin, S.-J. Park, K.-H. Cho. Chonnam National University Medical School and Hwasun Hospital, Gwangju, Republic of Korea; Chonnam National University Hospital, Gwangju, Republic of Korea; Gwang-Ju Hospital, Gwangju, Republic of Korea; Suhgwang Hospital, Gwangju, Republic of Korea; Cheomdan Medical Center, Gwangju, Republic of Korea; Chonnam National University Hospital Medical School, Gwangju,

The association of infarct pattern on coronal diffusion-weighted MR image and early neurologic deterioration in deep subcortical infarction

WCN 2013 No: 289 Topic: 3 — Stroke Clinico-epidemiological features of cerebral venous thrombosis in Algarve H. Nzwalo, F. Rodrigues, P. Carneiro, L. Arez, F. Ferreira, C. Basilio. Neurology, Faro Hospital, Faro, Portugal; Internal Medicine, Centro Hospitalar do Barlavento Algarvio E.P.E., Portimao, Faro, Portugal; Universidade do Algarve, Faro, Portugal Background: Cerebral vein thrombosis (CVT) is a very uncommon disorder with a wide variety of clinical manifestations. Little information exists about CVT, particularly from peripheral regions. Objective: To describe the epidemiology and clinical characteristics of CVT in a relatively small region. Material and methods: Hospital based retrospective study of CVT occurred between January 2001 and December 2012, in the public hospitals serving a population of 395,218–451,005 habitants. Results: Thirty-five cases of CVT were identified. Children (4) and non-residents (3) were excluded from the analysis. Gross incidence was approximately 0.56–0.64/100,000 cases/year. The mean and median ages were 40.4 and 35 years respectively [18–71 years]. The majority (21/75%) were woman. The mean interval between the onset of symptoms and the diagnosis was 8 days [8–41 days]. Patients presented with isolated intracranial hypertension (17/61%), focal syndrome (4/14%), and encephalopathy (7/25%). Brain TC was diagnostic in 10 (42%) cases. Brain MRI and MR venography was performed in all. Use of oral contraceptives was found in 14/66 % of females, with/without other risk factors. In 6 (21%), no risk factor was found. All cases received heparin or LMWH followed by warfarin. Complete recovery occurred in 24 (85 %); death in 1 (3.6%); important sequels in 3 (11%): epilepsy, severe visual loss and motor deficit. Conclusion: Our findings are comparable to those described in similar case series. However, in comparison to what has been found in our country (0.22/100,000 cases/year), our incidence is high. Diagnosis improvement, availability of MRI, and use of oral contraceptives may explain this. doi:10.1016/j.jns.2013.07.670 Abstract — WCN 2013 No: 325 Topic: 3 — Stroke The value of apparent diffusion coefficient maps in subcortical infarct lesion D.S. Oh, M.H. Park, D.E. Kim, K.H. Choi, Y.J. Choi, T.S. Nam, J.T. Kim, S.H. Lee. Department of Neurology, ChonnamNational University Hospital, Gwang-Ju, Republic of Korea Background: Prediction of the early neurologic deterioration (END) in patients with acute ischemic stroke is one of great clinical interests. Objective: This study was designed to analyze whether apparent diffusion coefficient (ADC) values provide reliable quantitative information for the prediction of the neurologic deficits in stroke patients. Patient and methods: We reviewed 36 patients not treated by thrombolytics who had a diffusion restricted lesion in deep subcortical area within 24 h after stroke onset. Serial National Institutes of Health Stroke Scale (NIHSS) scores were checked from admission. We compared the ADC values in the initial diffusion weighted image (DWI). We tested the value of the ADC to predict tissue outcome and development of END. All images were processed on an independent workstation by using custom software (MIDAS 1.11). Results: Among 36 patients, 14 patients developed END and 22 patients did not develop END. Univariate analysis showed that the ADC values in diffusion restricted lesion were significantly decreased in patient that developed to END compared with those that remained without END. (p value = 0.037). Conclusions: Initial ADC values can predict the development of END in patient with deep subcortical infarct. doi:10.1016/j.jns.2013.07.671 Abstract — WCN 2013 No: 266 Topic: 3 — Stroke The association of infarct pattern on coronal diffusion-weighted MR image and early neurologic deterioration in deep subcortical infarction WCN 2013 No: 266 Topic: 3 — Stroke The association of infarct pattern on coronal diffusion-weighted MR image and early neurologic deterioration in deep subcortical infarction D.S. Oh, D.E. Kim, K.H. Choi, Y.J. Choi, T.S. Nam, J.T. Kim, S.H. Lee, B.C. Kim, M.K. Kim. Department of Neurology, Chonnam National University Hospital, Gwang-Ju, Republic of Korea Background: Early neurological deterioration (END) is frequently observed in patients with small deep infarction of subcortical area. Objective: In this study, we investigated the association between the END and the patterns of small deep infarction in coronal diffusionweighted imaging (DWI). Patients and methods: Consecutive 95 patients diagnosed with acute ischemic stroke in subcortical area, whowere admitted to stroke center betweenMarch 2008 and June 2009, were included. Brain coronal DWI and MR angiography (MRA) were carried out. The patterns of infarct lesions are divided into 3 categories: (1) lacunar pattern; isolated lesion in the subcortical area, (2) skipped pattern: lesions that presented with disconnected shape, separated by unaffected white matter of internal capsule, on coronal DWI, (3) linear pattern; linear lesion extending to the basal surface on coronal DWI. ENDwas defined as an increase of 2 or more points of the National Institutes of Health Stroke Scale (NIHSS) within 7 days of stroke onset. Results: 23 patients were presented with lacunar pattern, 20 with skipped patterns, and 52 patients with linear pattern. END was observed in 36 (45.8%). Patients with skipped and lacunar patterns on coronal DWI experienced END less frequently than those with linear patterns (25% vs. 55.8% and 8.7% vs. 55.8%, p = 0.034 and 0.000, respectively). Abstracts / Journal of the Neurological Sciences 333 (2013) e152–e214 e160

The value of apparent diffusion coefficient maps in subcortical infarct lesion

WCN 2013 No: 289 Topic: 3 — Stroke Clinico-epidemiological features of cerebral venous thrombosis in Algarve H. Nzwalo, F. Rodrigues, P. Carneiro, L. Arez, F. Ferreira, C. Basilio. Neurology, Faro Hospital, Faro, Portugal; Internal Medicine, Centro Hospitalar do Barlavento Algarvio E.P.E., Portimao, Faro, Portugal; Universidade do Algarve, Faro, Portugal Background: Cerebral vein thrombosis (CVT) is a very uncommon disorder with a wide variety of clinical manifestations. Little information exists about CVT, particularly from peripheral regions. Objective: To describe the epidemiology and clinical characteristics of CVT in a relatively small region. Material and methods: Hospital based retrospective study of CVT occurred between January 2001 and December 2012, in the public hospitals serving a population of 395,218–451,005 habitants. Results: Thirty-five cases of CVT were identified. Children (4) and non-residents (3) were excluded from the analysis. Gross incidence was approximately 0.56–0.64/100,000 cases/year. The mean and median ages were 40.4 and 35 years respectively [18–71 years]. The majority (21/75%) were woman. The mean interval between the onset of symptoms and the diagnosis was 8 days [8–41 days]. Patients presented with isolated intracranial hypertension (17/61%), focal syndrome (4/14%), and encephalopathy (7/25%). Brain TC was diagnostic in 10 (42%) cases. Brain MRI and MR venography was performed in all. Use of oral contraceptives was found in 14/66 % of females, with/without other risk factors. In 6 (21%), no risk factor was found. All cases received heparin or LMWH followed by warfarin. Complete recovery occurred in 24 (85 %); death in 1 (3.6%); important sequels in 3 (11%): epilepsy, severe visual loss and motor deficit. Conclusion: Our findings are comparable to those described in similar case series. However, in comparison to what has been found in our country (0.22/100,000 cases/year), our incidence is high. Diagnosis improvement, availability of MRI, and use of oral contraceptives may explain this. doi:10.1016/j.jns.2013.07.670 Abstract — WCN 2013 No: 325 Topic: 3 — Stroke The value of apparent diffusion coefficient maps in subcortical infarct lesion D.S. Oh, M.H. Park, D.E. Kim, K.H. Choi, Y.J. Choi, T.S. Nam, J.T. Kim, S.H. Lee. Department of Neurology, ChonnamNational University Hospital, Gwang-Ju, Republic of Korea Background: Prediction of the early neurologic deterioration (END) in patients with acute ischemic stroke is one of great clinical interests. Objective: This study was designed to analyze whether apparent diffusion coefficient (ADC) values provide reliable quantitative information for the prediction of the neurologic deficits in stroke patients. Patient and methods: We reviewed 36 patients not treated by thrombolytics who had a diffusion restricted lesion in deep subcortical area within 24 h after stroke onset. Serial National Institutes of Health Stroke Scale (NIHSS) scores were checked from admission. We compared the ADC values in the initial diffusion weighted image (DWI). We tested the value of the ADC to predict tissue outcome and development of END. All images were processed on an independent workstation by using custom software (MIDAS 1.11). Results: Among 36 patients, 14 patients developed END and 22 patients did not develop END. Univariate analysis showed that the ADC values in diffusion restricted lesion were significantly decreased in patient that developed to END compared with those that remained without END. (p value = 0.037). Conclusions: Initial ADC values can predict the development of END in patient with deep subcortical infarct. doi:10.1016/j.jns.2013.07.671 Abstract — WCN 2013 No: 266 Topic: 3 — Stroke The association of infarct pattern on coronal diffusion-weighted MR image and early neurologic deterioration in deep subcortical infarction WCN 2013 No: 266 Topic: 3 — Stroke The association of infarct pattern on coronal diffusion-weighted MR image and early neurologic deterioration in deep subcortical infarction D.S. Oh, D.E. Kim, K.H. Choi, Y.J. Choi, T.S. Nam, J.T. Kim, S.H. Lee, B.C. Kim, M.K. Kim. Department of Neurology, Chonnam National University Hospital, Gwang-Ju, Republic of Korea Background: Early neurological deterioration (END) is frequently observed in patients with small deep infarction of subcortical area. Objective: In this study, we investigated the association between the END and the patterns of small deep infarction in coronal diffusionweighted imaging (DWI). Patients and methods: Consecutive 95 patients diagnosed with acute ischemic stroke in subcortical area, whowere admitted to stroke center betweenMarch 2008 and June 2009, were included. Brain coronal DWI and MR angiography (MRA) were carried out. The patterns of infarct lesions are divided into 3 categories: (1) lacunar pattern; isolated lesion in the subcortical area, (2) skipped pattern: lesions that presented with disconnected shape, separated by unaffected white matter of internal capsule, on coronal DWI, (3) linear pattern; linear lesion extending to the basal surface on coronal DWI. ENDwas defined as an increase of 2 or more points of the National Institutes of Health Stroke Scale (NIHSS) within 7 days of stroke onset. Results: 23 patients were presented with lacunar pattern, 20 with skipped patterns, and 52 patients with linear pattern. END was observed in 36 (45.8%). Patients with skipped and lacunar patterns on coronal DWI experienced END less frequently than those with linear patterns (25% vs. 55.8% and 8.7% vs. 55.8%, p = 0.034 and 0.000, respectively). Abstracts / Journal of the Neurological Sciences 333 (2013) e152–e214 e160

Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease

Huntington’s disease (HD) is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene. Typical age at onset of symptoms is in the 40s, but the disorder can manifest at any time. Late-onset (≥ 60 years) HD is clinically different from other adult or juvenile onset HD and characterized by mild motor problem as the initial symptoms, shorter disease duration, frequent lack of family history, and relatively low CAG repeats expansion. We report a case of an 80-year-old female with oromandibular dyskinesia as an initial manifestation of HD and 40 CAG repeats.