Chaewon Shin

Ischemic Stroke During Sleep: Its Association With Worse Early Functional Outcome

Background and Purpose— Approximately one fourth of stroke occur during sleep. Despite the clinical and radiological similarities between wake-up stroke (WUS) and non-WUS, the functional outcomes of WUS are largely unknown. Methods— This retrospective analysis reviewed 2289 consecutive patients with acute ischemic stroke who were admitted between November 2002 and December 2009. We used 3 end-point analytic techniques to evaluate the association between WUS and functional outcomes: dichotomized analysis for “functional dependency” (a discharge modified Rankin Scale [mRS] score ≥2 regardless of initial stroke severity), severity-adjusted responder analysis for “unfavorable outcome” (a discharge mRS ≥1 for an admission National Institutes of Health Stroke Scale score 0 to 7; mRS ≥2 for National Institutes of Health Stroke Scale 8 to 14; or mRS ≥3 for National Institutes of Health Stroke Scale ≥15), and shift analysis for changes in overall distributions of discharge mRS scores. Results— The initial National Institutes of Health Stroke Scale score of patients with WUS was significantly higher than that of their non-WUS counterparts (median [interquartile range]; 4 [2 to 7] versus 3 [1 to 6]; P<0.01). The dichotomized analysis strategy failed to detect a significant association between WUS and functional dependency at discharge (adjusted OR, 0.99; 95% CI, 0.76 to 1.28). However, the responder analysis showed that patients with WUS were more likely to have “unfavorable outcomes” (adjusted OR, 1.33; 95% CI, 1.02 to 1.72), and the shift analysis also detected significant effect of WUS on the mRS score distributions toward increased dependency (adjusted OR, 1.22; 95% CI, 1.01 to 1.48). Conclusions— From our study, we documented that WUS was associated with worse short-term outcomes after ischemic stroke. Careful selection of appropriate analytic techniques may help to detect modest associations in observational studies.

Survival of Korean Huntington's Disease Patients.

Objective The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported. Methods Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study. Results The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset. Conclusion This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

Combined focal myoclonus and dystonia secondary to a cerebellar hemorrhage: a case report

BackgroundMyoclonus is a clinical sign characterized by sudden, brief jerky, shock-like involuntary movements of a muscle or group of muscles. Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. Cases of myoclonus or dystonia secondary to a structural lesion in the cerebellum have been reported. However, there has never been a reported case of combined myoclonus and dystonia secondary to a cerebellar lesion.Case presentationHerein, we report a 22-year-old female patient with sudden-onset myoclonic jerks, dystonic posture and mild ataxia in the right upper extremity. At age 19, she experienced sudden headache with vomiting. The neurological examination showed ataxia, myoclonus and dystonia in the right upper extremity. Brain images demonstrated a hemorrhage in the right cerebellar hemisphere secondary to a cavernous malformation. After resection of the hemorrhagic mass, headache with vomiting disappeared and ataxia improved, but myoclonus and dystonia persisted.ConclusionsIt is the first report of combined focal myoclonus and dystonia secondary to a cerebellar lesion.

Submandibular gland is a suitable site for alpha synuclein pathology in Parkinson disease

OBJECTIVE To validate the role of α-synuclein (AS) pathology in submandibular gland (SMG) as a biomarker for Parkinson disease (PD). METHODS We performed ultrasonography (USG) guided core needle biopsy of SMG in PD patients and procured SMG biopsy tissues or surgical excision specimens from non-PD patients as controls. Then, we compared AS deposition in the SMG tissues between the PD patients and the controls. We recruited 16 PD patients in this study. In each individual, two core needle biopsy tissues were obtained from the left submandibular gland under USG guidance. Fourteen sex and age-matched controls who did not have PD and dementia but received a core needle biopsy or surgical resection of the SMG due to SMG diseases were procured from the pathology archive. Biopsy tissues and surgical specimens were immuno-stained with serine 129 phosphorylated AS (pAS) antibody for microscopic examination. pAS deposition in neural structures such as ganglion cells and neurites was considered as positive. RESULTS No serious complication occurred during and after the SMG biopsy. We found glandular parenchyma and neural structures in all biopsied SMG tissues from the patients and the controls. Nine out of 16 PD patients (56.2%) were positive for pAS staining, while none of the controls were positive (0%). CONCLUSIONS SMG core needle biopsy can reliably and safely obtain sufficient glandular parenchyma and neural structures to evaluate the α-synuclein pathology. AS pathology in SMG has high specificity and good sensitivity as a biomarker for PD.

Non-Motor Symptom Burdens Are Not Associated with Iron Accumulation in Early Parkinson's Disease: a Quantitative Susceptibility Mapping Study

Background Quantitative susceptibility mapping (QSM) has been used to measure iron accumulation in the deep nuclei of patients with Parkinsons disease (PD). This study examined the relationship between non-motor symptoms (NMSs) and iron accumulation in the deep nuclei of patients with PD. Methods The QSM data were acquired from 3-Tesla magnetic resonance imaging (MRI) in 29 patients with early PD and 19 normal controls. The Korean version of the NMS scale (K-NMSS) was used for evaluation of NMSs in patients. The patients were divided into high NMS and low NMS groups. The region-of-interest analyses were performed in the following deep nuclei: red nucleus, substantia nigra pars compacta, substantia nigra pars reticulata, dentate nucleus, globus pallidus, putamen, and head of the caudate nucleus. Results Thirteen patients had high NMS scores (total K-NMSS score, mean = 32.1), and 16 had low NMS scores (10.6). The QSM values in the deep were not different among the patients with high NMS scores, low NMS scores, and controls. The QSM values were not correlated linearly with K-NMSS total score after adjusting the age at acquisition of brain MRI. Conclusion The study demonstrated that the NMS burdens are not associated with iron accumulation in the deep nuclei of patients with PD. These results suggest that future neuroimaging studies on the pathology of NMSs in PD should use more specific and detailed clinical tools and recruit PD patients with severe NMSs.

Bilateral stereotypy after unilateral cerebellar infarction

Stereotypy is defined as repetitive and continuous non-goaldirected movement. It was reported in association with various disorders including developmental delay, autism, schizophrenia, tardive dyskinesia, and frontotemporal dementia. There were few cases with stereotypy secondary to cerebral infarctions in the putamen or cerebellum [1]. Here, we report a case with bilateral hand stereotypy after left cerebellar infarction. An 82-year-old man was admitted to the hospital with acute onset dysarthria and gait disturbance. He was hypertensive and had a 60 pack-year smoking history. A neurological examination showed normal cognition (the Mini-Mental State Examination score = 25), dysmetria, and gait ataxia. Brain magnetic resonance imaging (MRI) showed an acute infarction in the left cerebellar hemisphere (Fig. 1a). He was treated with aspirin and clopidogrel, and discharged with only minimal residual symptoms. Three weeks after the cerebellar infarction, abnormal movements of the bilateral hands developed. He continuously rubbed both thumbs against the index fingers (Supplemental Video). The movements were temporarily suppressible on demand or decreased when he was distracted. There was no urge to move his hands when he was holding the movements. A follow-up neurological examination showed asymmetric bradykinesia without postural instability in addition to stereotypic movements. Follow-up neuroimaging studies were performed which revealed multiple areas of hypometabolism including the bilateral high frontal cortex, anterior temporal cortex, anterior cingulate gyrus, and left cerebellum using 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET), and decreased uptake in both putamen and caudate nuclei using 18F-fluorinated N-3-fluoropropyl-2-beta-carboxymethoxy-3-beta-(4-iodophenyl) nortropane (18F-FP-CIT) PET (Fig. 1b). Medications did not improve his stereotypic movement. Levodopa treatment up to 600 mg/day was neither mitigating his parkinsonism nor aggravating stereotypic movements. The informed consents for the report and Supplemental Video were received from the patient. The abnormal movements were continuous, patterned, and distractible. Without sensory urge, the movements can be best classified as stereotypy. This is the second case with stereotypy secondary to cerebellar infarction. While the motor inhibitory system, consisting of the inferior frontal gyrus and pre-supplementary motor area, was underscored in the pathophysiology of stereotypy, the contribution of the cerebellum is also becoming increasingly recognized [2]. In autism, where stereotypy and repetitive behaviors are key motor symptoms, the correlation between these behaviors and the cerebellum (the anterior lobe and lobule VIII) was demonstrated [3]. We also had reported cortical hypometabolism in a patient with stereotypy after cerebellar infarction [1]. In this case, multiple areas including the motor inhibitory system were affected by a solitary lesion in the cerebellum. Therefore, these studies suggest that the cerebellum may play a leading role in the development of stereotypy via recruiting critical areas. Electronic supplementary material The online version of this article (https ://doi.org/10.1007/s1376 0-018-0958-0) contains supplementary material, which is available to authorized users.