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JMD : Journal of Movement Disorders

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12 "Chul Hyoung Lyoo"
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Asymmetric Parkinsonism With Progressive Nigrosomal Change Secondary to Kernohan’s Notch Phenomenon
Han-Kyeol Kim, Min Seok Baek, Sung Jun Ahn, Chul Hyoung Lyoo
J Mov Disord. 2022;15(2):184-186.   Published online March 16, 2022
DOI: https://doi.org/10.14802/jmd.21162
  • 613 View
  • 90 Download
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A Patient with Neuroferritinopathy Presenting with Juvenile-Onset Voice Tremor
Chan Wook Park, Nan Young Kim, Yun Joong Kim, Sook Keun Song, Chul Hyoung Lyoo
J Mov Disord. 2020;13(1):66-68.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19038
  • 4,959 View
  • 89 Download
  • 3 Citations
PDF
Novel Ferritin Light Chain Gene Mutation in a Korean Patient with Neuroferritinopathy
So Hoon Yoon, Nan Young Kim, Yun Joong Kim, Chul Hyoung Lyoo
J Mov Disord. 2019;12(1):63-65.   Published online January 30, 2019
DOI: https://doi.org/10.14802/jmd.18062
  • 4,272 View
  • 73 Download
  • 4 Citations
PDF
Review Article
Tau Positron Emission Tomography Imaging in Degenerative Parkinsonisms
Chul Hyoung Lyoo, Hanna Cho, Jae Yong Choi, Young Hoon Ryu, Myung Sik Lee
J Mov Disord. 2018;11(1):1-12.   Published online January 23, 2018
DOI: https://doi.org/10.14802/jmd.17071
  • 8,894 View
  • 411 Download
  • 6 Citations
AbstractAbstract PDF
In recent years, several radiotracers that selectively bind to pathological tau proteins have been developed. Evidence is emerging that binding patterns of in vivo tau positron emission tomography (PET) studies in Alzheimer’s disease (AD) patients closely resemble the distribution patterns of known neurofibrillary tangle pathology, with the extent of tracer binding reflecting the clinical and pathological progression of AD. In Lewy body diseases (LBD), tau PET imaging has clearly revealed cortical tau burden with a distribution pattern distinct from AD and increased cortical binding within the LBD spectrum. In progressive supranuclear palsy, the globus pallidus and midbrain have shown increased binding most prominently. Tau PET patterns in patients with corticobasal syndrome are characterized by asymmetrical uptake in the motor cortex and underlying white matter, as well as in the basal ganglia. Even in the patients with multiple system atrophy, which is basically a synucleinopathy, 18F-flortaucipir, a widely used tau PET tracer, also binds to the atrophic posterior putamen, possibly due to off-target binding. These distinct patterns of tau-selective radiotracer binding in the various degenerative parkinsonisms suggest its utility as a potential imaging biomarker for the differential diagnosis of parkinsonisms.
Case Report
A Patient with Recurrent Dyskinesia and Hyperpyrexia Syndrome
Min Seok Baek, Hyung Woo Lee, Chul Hyoung Lyoo
J Mov Disord. 2017;10(3):154-157.   Published online July 14, 2017
DOI: https://doi.org/10.14802/jmd.17022
  • 6,150 View
  • 173 Download
  • 5 Citations
AbstractAbstract PDF
Dyskinesia hyperpyrexia syndrome is a rare medical emergency in Parkinson’s disease. It is characterized by continuous dyskinesia associated with hyperthermia, rhabdomyolysis, and alteration of the mental state. We present the case of a 74-year-old woman who presented with recurrent dyskinesia hyperpyrexia syndrome. Although some provocation factors and clinical manifestations seem to be shared with parkinsonism hyperpyrexia syndrome, a clear distinction in management should be considered.
Original Articles
Clinical Features Indicating Nigrostriatal Dopaminergic Degeneration in Drug-Induced Parkinsonism
Seung Ha Lee, Han Kyeol Kim, Young Gun Lee, Chul Hyoung Lyoo, Sung Jun Ahn, Myung Sik Lee
J Mov Disord. 2017;10(1):35-39.   Published online December 27, 2016
DOI: https://doi.org/10.14802/jmd.16045
  • 8,121 View
  • 148 Download
  • 3 Citations
AbstractAbstract PDFSupplementary Material
Objective
Patients with drug-induced parkinsonism (DIP) may have nigrostriatal dopaminergic degeneration. We studied the clinical features that may indicate nigrostriatal dopaminergic degeneration in patients with DIP.
Methods
Forty-one DIP patients were classified into normal and abnormal [18F] FP-CIT scan groups. Differences in 32 clinical features and drug withdrawal effects were studied.
Results
Twenty-eight patients had normal (Group I) and 13 patients had abnormal (Group II) scans. Eight patients of Group I, but none of Group II, had taken calcium channel blockers (p = 0.040). Three patients of Group I and six of Group II had hyposmia (p = 0.018). After drug withdrawal, Group I showed greater improvement in Unified Parkinson’s Disease Rating Scale total motor scores and subscores for bradykinesia and tremors than Group II. Only hyposmia was an independent factor associated with abnormal scans, but it had suboptimal sensitivity.
Conclusion
None of the clinical features were practical indicators of nigrostriatal dopaminergic degeneration in patients with DIP.
Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans
Jae-Hyeok Lee, Jongkyu Park, Ho-Sung Ryu, Hyeyoung Park, Young Eun Kim, Jin Yong Hong, Sang Ook Nam, Young-Hee Sung, Seung-Hwan Lee, Jee-Young Lee, Myung Jun Lee, Tae-Hyoung Kim, Chul Hyoung Lyoo, Sun Ju Chung, Seong Beom Koh, Phil Hyu Lee, Jin Whan Cho, Mee Young Park, Yun Joong Kim, Young H. Sohn, Beom Seok Jeon, Myung Sik Lee
J Mov Disord. 2016;9(1):20-27.   Published online January 25, 2016
DOI: https://doi.org/10.14802/jmd.15058
  • 18,447 View
  • 211 Download
  • 13 Citations
AbstractAbstract PDFSupplementary Material
Objective
Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea.
Methods
We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN).
Results
Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN.
Conclusions
We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.
Current Status of Huntington’s Disease in Korea: A Nationwide Survey and National Registry Analysis
Hyun Sook Kim, Chul Hyoung Lyoo, Phil Hyu Lee, Sang Jin Kim, Mee Young Park, Hyeo-Il Ma, Jae Hyeok Lee, Sook Kun Song, Jong Sam Baik, Jin Ho Kim, Myung Sik Lee
J Mov Disord. 2015;8(1):14-20.   Published online January 31, 2015
DOI: https://doi.org/10.14802/jmd.14038
  • 14,633 View
  • 109 Download
  • 12 Citations
AbstractAbstract PDF
Objective Huntington’s disease (HD) is a rare neurological disorder, and its current status in Korea is not well investigated. This study aims to determine the prevalence and incidence of HD and to investigate the clinical features of HD patients in Korea.
Methods We estimated the crude prevalence and annual incidence of HD based on the databases of the Rare Diseases Registry (RDR) and the National Health Insurance (NHI). The clinical data of genetically confirmed HD patients was collected from 10 referral hospitals and analyzed.
Results The mean calculated annual incidence was 0.06 cases per 100,000 persons, and the mean calculated prevalence was 0.38 based on the NHI database. The estimated crude prevalence based on the RDR was 0.41. Of the sixty-eight HD patients recruited, the mean age of onset was 44.16 ± 14.08 years and chorea was most frequently reported as the initial symptom and chief complaint. The mean CAG repeat number of the expanded allele was 44.7 ± 4.8 and correlated inversely with the age of onset (p < 0.001). About two-thirds of the patients have a positive family history, and HD patients without positive family history showed a delay in onset of initial symptoms, a prolonged interval between initial symptom onset and genetic diagnosis and a delay in the age of genetic diagnosis.
Conclusions To the best of our knowledge, this is the first study to estimate the prevalence and incidence of HD in Korea and the largest HD series in the Asian population. Our analyses might be useful for further studies and large-scale investigations in HD patients.
Case Reports
Neuroleptic Malignant Syndrome in a Patient with Corticobasal Degeneration
Myung Jun Lee, Chul Hyoung Lyoo, Myung Sik Lee
J Mov Disord. 2011;4(2):73-74.
DOI: https://doi.org/10.14802/jmd.11015
  • 8,988 View
  • 49 Download
AbstractAbstract PDF

Parkinson’s disease is a principal underlying disease of neuroleptic malignant syndrome (NMS) occurring in parkinsonian disorders, but NMS may occur in patients with progressive supranuclear palsy and multiple system atrophy. We report first patient with corticobasal degeneration (CBD) who developed NMS after abrupt reduction of antiparkinsonian medication and concurrent infection. It should be kept in mind that the prevention of infectious illness, which is common complication in parkinson-plus syndrome, is important, and dose reduction or withdrawal of anti-parkinsonian medications should be carefully performed even in the patients with CBD who are expected to be unresponsive to levodopa treatment.

Oculogyric Crisis Associated with Disulfiram-Induced Pallidonigral Lesion
Jae Hyeok Lee, Chul Hyoung Lyoo, Jin Goo Lee, Myung Sik Lee
J Mov Disord. 2009;2(1):48-49.
DOI: https://doi.org/10.14802/jmd.09013
  • 7,994 View
  • 62 Download
AbstractAbstract PDF

We report a man who developed oculogyric crisis one month after disulfiram intoxication. Brain MRI showed lesions involving bilateral globus pallidus and left substantia nigra. In our patient, neuronal discharges from pathologically reorganized basal ganglia circuit to the mid-brain ocular motor center might lead to tonic deviation of the eyes.

Original Articles
Relationship Between the Striatal and Cerebellar Glucose Metabolism and the Response to Levodopa Treatment in Patients With Multiple System Atrophy
Chul Hyoung Lyoo, Seung Hun Oh, Ki Ook Lee, Seung Yeob Lee, Young Hoon Ryu, Myung Sik Lee
J Mov Disord. 2008;1(1):26-32.
DOI: https://doi.org/10.14802/jmd.08005
  • 9,334 View
  • 53 Download
AbstractAbstract PDF
Introduction:

About two thirds of the patients with multiple system atrophy (MSA) do not respond to levodopa treatment. Postmortem pathological studies and one retrospective [18F]-deoxyglucose positron emission tomography (FDGPET) study attributed such poor response to the striatal degeneration. We prospectively investigated the relationship between levodopa responsiveness and the metabolic activities of the striatum and cerebellum in MSA patients.

Methods:

In 39 patients with MSA, the UPDRS motor score was assessed and two sets of timed motor tests were perform ed before and after the levodopa treatment. After quantitative FDG PET and baseline evaluation, treatment w as started with 3 tablets of Sinemet® 25/250 mg a day. Clinical assessments were performed monthly for three months. Metabolic activities of the caudate, anterior putamen, posterior putamen, cerebellar cortex and cerebellar vermis were measured. We compared the measurements with mean percentage changes of motor function. Also, using statistical parametric mapping (SPM) analysis, we tried to find brain areas in which metabolism correlated with the clinical changes.

Results:

Mean percentage improvements of UPDRS motor scores w ere correlated with glucose metabolism in the posterior putamen and cerebellar vermis. The mean percentage improvements of performance in Purdue peg board test correlated with the glucose metabolism in the cerebellar cortex and vermis. In SPM analysis, cerebellar glucose metabolism correlated with the improvement of UPDRS motor score and the performance of two timed motor tests.

Conclusion:

The integrity of cerebellum, as well as posterior putamen, may be an important factor for showing the response to levodopa.

Falls and Physical Injuries in Patients With Parkinson’s Disease
Tae-Jin Song, Do Hyun Kim, Kwon-Duk Seo, Seung-Hun Oh, Chul Hyoung Lyoo, Myung Sik Lee
J Mov Disord. 2008;1(1):13-18.
DOI: https://doi.org/10.14802/jmd.08003
  • 10,179 View
  • 160 Download
  • 1 Citations
AbstractAbstract PDF
Background:

In Parkinson’s disease (PD), falls and subsequent physical injuries are frequent causes of morbidity and mortality. We investigated the characteristics of falls and physical injuries in Korean patients with PD.

Methods:

This study included 239 patients with PD. Using the medical records and interviews, we studied the characteristics of fall and its consequences retrospectively.

Results:

Among the 239 patients with PD, 129 (54.0%) patients had a history of fall. The mean interval between the disease onset and the first fall was 15.3 months. Among them, 83 patients (64.3%) fell more than twice. Eighty-six patients (66.7%) had physical injuries and 21 patients (15.3%) had fractures including 7 with hip fracture and 7 with arm fracture. Patients with physical injuries fell earlier and repetitively. They tended to fall during the night, toward lateral or posterior direction, and were unable to make protective hand movements.

Conclusions:

Elderly PD patients with long duration have a high risk of fall. To prevent the physical injuries, the clinicians should try to reduce the off time and advice the patients and caregivers to limit physical activities during the night.


JMD : Journal of Movement Disorders