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Clinical and Genetic Spectrum of ATP1A3-Related Disorders in a Korean Pediatric Population

Kim WJ, Shim YK, Choi SA, Kim SY, Kim H, Hwang H, Choi J, Kim KJ, Chae JH, Lim BC

BACKGROUND AND PURPOSE: The aim of this study was to expand the understanding of the genotype-phenotype spectrum of ATP1A3-related disorders and to evaluate the therapeutic effect of a ketogenic diet...
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Intrafamilial variability and clinical heterogeneity in a family with PLA2G6-associated neurodegeneration

Park JK, Youn J, Cho JW

Phospholipase A2 group VI (PLA2G6)-associated neurodegeneration (PLAN) is an autosomal recessive neurodegenerative disease with a wide clinical spectrum; however, the genotype-phenotype correlation is unknown. Here, we report different phenotypes in...
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Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study

Wiblin L, Durcan R, Galna B, Lee M, Burn D

OBJECTIVE: Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead...
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Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy

Kataoka H, Nishimori , Kiriyama T, Nanaura H, Izumi T, Eura N, Iwasa N, Sugie K

OBJECTIVE: The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain....
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Oro-Pharyngeal Dysphagia in Parkinson's Disease and Related Movement Disorders

Kwon M, Lee JH

Oro-pharyngeal dysphagia is a common symptom in patients with Parkinson's disease (PD) and related disorders, even in their early stage of diseases. Dysphagia in these patients has been underdiagnosed, probably...
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Urodynamic Mechanisms Underlying Overactive Bladder Symptoms in Patients With Parkinson Disease

Vurture G, Peyronnet , Palma JA, Sussman RD, Malacarne , Feigin A, Palmerola , Rosenblum , Frucht S, Kaufmann H, Nitti VW, Brucker BM

PURPOSE: To assess the urodynamic findings in patients with Parkinson disease (PD) with overactive bladder symptoms. METHODS: We performed a retrospective chart review of all PD patients who were seen in...
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Spatiotemporal Gait Parameter Comparison for Parkinson's Disease, Multiple System Atrophy, and Other Parkinsonism Diseases

Bae CH, Jeong HW, Yun JY, Lee JE, Han SJ

The aim of this study was to compare and quantify the spatiotemporal and gait parameters obtained by foot pressure analysis during the gait in a group of Parkinson's disease (PD)...
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Comparative analysis of dose-dependent neurotoxic response to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine in C57BL/6 N mice derived from three different sources

Hwang DJ, Kwon KC, Song HK, Kim KS, Jung YS, Hwang DY, Cho JY

MPTP, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine is commonly used to induce nigrostriatal defects to induce parkinsonism and/or parkinsonian syndrome, to replicate the lesions seen in Parkinson's disease (PD), with use in numerous PD models...
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Trends in the Prevalence of Drug-Induced Parkinsonism in Korea

Byun JH, Cho H, Kim YJ, Kim JS, Baik JS, Jang S, Ma HI

PURPOSE: Discontinuation of offending drugs can prevent drug-induced parkinsonism (DIP) before it occurs and reverse or cure it afterwards. The aim of this study was to investigate the prevalence of...
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Reduced Uptake on Dopamine Transporter Imaging by Methylphenidate

Ha J, Ko J, Song JT, Hong JY

No abstract available.
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Anti-Ma2-Associated Encephalitis with Axonal Sensorimotor Polyneuropathy

Song JM, Kwon S, Kim J, Kim BJ, Min JH

Anti-Ma2-associated encephalitis is one of the paraneoplastic limbic and brainstem encephalitis characterized by decreased consciousness, parkinsonism and the limitation of vertical eye movement. It is usually associated with non-small cell...
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Manganism without Parkinsonism: Isolated Unilateral Upper Limb Tremor in a Welder

Prasad S, Shamim U, Minj A, Faruq M, Pal PK

No abstract available.
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The History of Parkinson's Disease and Famous Patients

Kim KT, You S

BACKGROUND: Parkinson's disease (PD) is one of the most common neurodegenerative diseases. However, the history of PD and famous persons with PD have not been described in detail yet. METHODS: We...
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An Autopsy Proven Case of CSF1R-mutant Adult-onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia (ALSP) with Premature Ovarian Failure

Kim SI, Jeon B, Bae J, Won JK, Kim HJ, Yim J, Kim YJ, Park SH

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder caused by mutations in the tyrosine kinase domain of the CSF1R gene. ALSP is...
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A Case of Creutzfeldt-Jakob Disease Presented as Rapid Progressive Parkinsonism

Park Y, Lee CN

No abstract available.
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Transient Hypersomnolence Provoked by Metoclopramide in a Patient with Degenerative Parkinsonism

Yoo SW, Choi KE, Kim JS

No abstract available.
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Clinical Characteristics of Involuntary Movement in Hospitalized Patients

Kwon KY, Lee HM, Lee SM, Koh SB

OBJECTIVE: Neurological symptoms in hospitalized patients are not rare, and neurological consultation for movement disorders is especially important in evaluating or managing those with various movement disorders. Therefore, we investigated...
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The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases

Way C, Pettersson D, Hiller A

OBJECTIVE: To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism. METHODS: The radiologic information systems at an academic...
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Pseudobulbar Affect in Parkinsonian Disorders: A Review

Hakimi M, Maurer CW

Pseudobulbar affect (PBA) is a neurological symptom of inappropriate and uncontrollable laughter or crying that occurs secondary to a variety of neurological conditions, including parkinsonian disorders. PBA is a socially...
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Abnormal Eye Movements in Parkinsonism and Movement Disorders

Jung I, Kim JS

Abnormal eye movements are commonly observed in movement disorders. Ocular motility examination should include bedside evaluation and laboratory recording of ocular misalignment, involuntary eye movements, including nystagmus and saccadic intrusions/oscillations,...
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