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Holmes' Tremor with Shoulder Pain Treated by Deep Brain Stimulation of Unilateral Ventral Intermediate Thalamic Nucleus and Globus Pallidus Internus.

Sabri AYDIN ; Huseyin CANAZ ; Ezgi Tuna ERDOGAN ; Nazlı DURMAZ ; Barıs TOPCULAR

Journal of Movement Disorders.2017;10(2):92-95. doi:10.14802/jmd.16051

A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.
Accidents, Traffic ; Arm ; Deep Brain Stimulation* ; Felodipine ; Globus Pallidus* ; Hand ; Hemorrhage ; Humans ; Male ; Neurologic Examination ; Shoulder Pain* ; Shoulder* ; Tremor* ; Visual Analog Scale ; Young Adult

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Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos.

Zheyu XU ; Tchoyoson C C LIM ; Wing Lok AU ; Louis C S TAN

Journal of Movement Disorders.2017;10(2):87-91. doi:10.14802/jmd.16059

Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.
Ataxia ; Atrophy ; Cerebellar Ataxia* ; Cerebellar Diseases ; Diagnosis ; Disease Progression ; Humans ; Mesencephalon ; Neuroimaging ; Paralysis* ; Phenotype ; Retrospective Studies ; Supranuclear Palsy, Progressive

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Comparison of Pallidal and Subthalamic Deep Brain Stimulation in Parkinson's Disease: Therapeutic and Adverse Effects.

Ho Sung RYU ; Mi Sun KIM ; Sooyeoun YOU ; Mi Jung KIM ; Young Jin KIM ; Juyeon KIM ; Kiju KIM ; Sun Ju CHUNG

Journal of Movement Disorders.2017;10(2):80-86. doi:10.14802/jmd.17001

OBJECTIVE: To compare the therapeutic and adverse effects of globus pallidus interna (GPi) and subthalamic nucleus (STN) deep brain stimulation (DBS) for the treatment of advanced Parkinson's disease (PD). METHODS: We retrospectively analyzed the clinical data of patients with PD who underwent GPi (n = 14) or STN (n = 28) DBS surgery between April 2002 and May 2014. The subjects were matched for age at surgery and disease duration. The Unified Parkinson's Disease Rating Scale (UPDRS) scores and levodopa equivalent dose (LED) at baseline and 12 months after surgery were used to assess the therapeutic effects of DBS. Adverse effects were also compared between the two groups. RESULTS: At 12 months, the mean changes in the UPDRS total and part I–IV scores did not differ significantly between the two groups. However, the subscores for gait disturbance/postural instability and dyskinesia were significantly more improved after GPi DBS than those after STN DBS (p = 0.024 and 0.016, respectively). The LED was significantly more reduced in patients after STN DBS than that after GPi DBS (p = 0.004). Serious adverse effects did not differ between the two groups (p = 0.697). CONCLUSION: The patients with PD showed greater improvement in gait disturbance/postural instability and dyskinesia after GPi DBS compared with those after STN DBS, although the patients had a greater reduction in LED after STN DBS. These results may provide useful information for optimal target selection for DBS in PD.
Deep Brain Stimulation* ; Dyskinesias ; Gait ; Globus Pallidus ; Humans ; Levodopa ; Parkinson Disease* ; Retrospective Studies ; Subthalamic Nucleus ; Therapeutic Uses

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Structure, Distribution, and Genetic Profile of α-Synuclein and Their Potential Clinical Application in Parkinson's Disease.

Xiaoli SI ; Jiali PU ; Baorong ZHANG

Journal of Movement Disorders.2017;10(2):69-79. doi:10.14802/jmd.16061

Parkinson's disease (PD), the second most common neurodegenerative disorder after Alzheimer's disease, is characterized by the loss of nigral dopaminergic neurons. PD leads to a series of clinical symptoms, including motor and non-motor disturbances. α-synuclein, the major component of Lewy bodies, is a hallmark lesion in PD. In this review, we concentrate on presenting the latest research on the structure, distribution, and function of α-synuclein, and its interactions with PD. We also summarize the clinic applications of α-synuclein, which suggest its use as a biomarker, and the latest progress in α-synuclein therapy.
alpha-Synuclein ; Alzheimer Disease ; Dopaminergic Neurons ; Lewy Bodies ; Neurodegenerative Diseases ; Parkinson Disease*

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A Patient with Recurrent Dyskinesia and Hyperpyrexia Syndrome.

Min Seok BAEK ; Hyung Woo LEE ; Chul Hyoung LYOO

Journal of Movement Disorders.2017;10(3):154-157. doi:10.14802/jmd.17022

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.
Aged ; Dyskinesias* ; Emergencies ; Female ; Fever ; Humans ; Parkinson Disease ; Parkinsonian Disorders ; Rhabdomyolysis

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Oculodentodigital Dysplasia Presenting as Spastic Paraparesis: The First Genetically Confirmed Korean Case and a Literature Review.

Kye Won PARK ; Ho Sung RYU ; Juyeon KIM ; Sun Ju CHUNG

Journal of Movement Disorders.2017;10(3):149-153. doi:10.14802/jmd.17050

Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant inherited disease caused by mutations of the human gap junction alpha 1 gene, which encodes the protein Connexin-43. Patients with ODDD may present with neurological deficits with a typical pleiotropic combination of characteristic craniofacial, ophthalmological, phalangeal, and dental anomalies. In this report, we describe the first genetically confirmed Korean ODDD patient, who presented with spastic paraparesis. We will also review the neurological aspects of ODDD as reported in the literature.
Gap Junctions ; Humans ; Muscle Spasticity* ; Paraparesis, Spastic*

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‘Hummingbird’ Sign in a Patient with Guam Parkinsonism-Dementia Complex.

Tianrong YEO ; Louis CS TAN

Journal of Movement Disorders.2017;10(3):145-148. doi:10.14802/jmd.17025

We present a case of a 71-year-old male Chamorro patient from Guam who presented with progressive supranuclear palsy (PSP)-Richardson’s syndrome. Considering his strong family history of parkinsonism and a PSP phenotype, he was clinically diagnosed with Guam parkinsonism-dementia complex (PDC). Magnetic resonance imaging (MRI) of the brain revealed prominent midbrain atrophy with preserved pontine volume, forming the ‘hummingbird’ sign, which has not been described before in Guam PDC. Molecular analysis of the chromosome 9 open reading frame 72 gene (C9orf72) showed only 6 GGGGCC repeats. We discuss the clinico-pathological similarities and differences between PSP and Guam PDC, and highlight the topography of neuropathological changes seen in Guam PDC to explain the appearance of the ‘hummingbird’ sign on MRI.
Aged ; Atrophy ; Brain ; Chromosomes, Human, Pair 9 ; Guam* ; Humans ; Magnetic Resonance Imaging ; Male ; Mesencephalon ; Open Reading Frames ; Parkinsonian Disorders ; Phenotype ; Supranuclear Palsy, Progressive

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Quantitative Gait Analysis in Patients with Huntington's Disease.

Seon Jong PYO ; Hanjun KIM ; Il Soo KIM ; Young Min PARK ; Mi Jung KIM ; Hye Mi LEE ; Seong Beom KOH

Journal of Movement Disorders.2017;10(3):140-144. doi:10.14802/jmd.17041

OBJECTIVE: Gait disturbance is the main factor contributing to a negative impact on quality of life in patients with Huntington’s disease (HD). Understanding gait features in patients with HD is essential for planning a successful gait strategy. The aim of this study was to investigate temporospatial gait parameters in patients with HD compared with healthy controls. METHODS: We investigated 7 patients with HD. Diagnosis was confirmed by genetic analysis, and patients were evaluated with the Unified Huntington’s Disease Rating Scale (UHDRS). Gait features were assessed with a gait analyzer. We compared the results of patients with HD to those of 7 age- and sex-matched normal controls. RESULTS: Step length and stride length were decreased and base of support was increased in the HD group compared to the control group. In addition, coefficients of variability for step and stride length were increased in the HD group. The HD group showed slower walking velocity, an increased stance/swing phase in the gait cycle and a decreased proportion of single support time compared to the control group. Cadence did not differ significantly between groups. Among the UHDRS subscores, total motor score and total behavior score were positively correlated with step length, and total behavior score was positively correlated with walking velocity in patients with HD. CONCLUSION: Increased variability in step and stride length, slower walking velocity, increased stance phase, and decreased swing phase and single support time with preserved cadence suggest that HD gait patterns are slow, ataxic and ineffective. This study suggests that quantitative gait analysis is needed to assess gait problems in HD.
Diagnosis ; Gait* ; Humans ; Huntington Disease* ; Quality of Life ; Walking

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A Comparative Study of Central Hemodynamics in Parkinson's Disease.

Joong Hyun PARK ; Sang Won HAN ; Jong Sam BAIK

Journal of Movement Disorders.2017;10(3):135-139. doi:10.14802/jmd.17035

OBJECTIVE: To explore the central aortic pressure in patients with Parkinson's disease (PD). METHODS: We investigated central arterial stiffness by measurement of the augmentation index (AIx) in PD patients. Patients were eligible for the study if they were de novo PD and 45 years of age or older. The patients’ demographics, vascular risk factors, and neurologic examinations were collected at baseline. The AIx was measured by applanation tonometry. RESULTS: A total of 147 subjects (77 in control and 70 in PD groups) were enrolled in the study. While there was no significant difference in peripheral systolic blood pressure (SBP), diastolic blood pressure (DBP), or mean arterial pressure between groups, peripheral pulse pressure (PP) was significantly lower in the PD group than in the control group (p = 0.012). Regarding central pressure, aortic DBP was significantly higher and PP was significantly lower in the PD group (p = 0.001, < 0.0001). Although there was no significant difference in the AIx between the groups, a trend toward a lower AIx was observed in the PD group (31.2% vs. 28.1%, p = 0.074). CONCLUSION: This study showed that peripheral and central PP was significantly lower in the PD group than in the control group. Our study suggests that PD patients may have a low risk of a cardiovascular event by reason of a lower PP.
Arterial Pressure ; Blood Pressure ; Demography ; Hemodynamics* ; Humans ; Manometry ; Neurologic Examination ; Parkinson Disease* ; Risk Factors ; Vascular Stiffness

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Need for Registration and Reporting of Acupuncture Trials in Parkinson's Disease in Korea.

Timothy E LEE ; Aryun KIM ; Mihee JANG ; Beomseok JEON

Journal of Movement Disorders.2017;10(3):130-134. doi:10.14802/jmd.17047

OBJECTIVE: Many people dealing with Parkinson’s disease (PD) turn to complementary and alternative medicine when searching for a cure or relief from symptoms. Acupuncture is widely used in the Korean PD population to alleviate symptoms and in hopes of curing the illness. However, acupuncture use for PD patients has only recently begun to be studied scientifically and is still considered an unproven treatment for PD. Therefore, there is an urgent need for acupuncture to be studied, validated and used for PD. Thus, our study’s aim is to examine how many acupuncture studies in PD are registered and reported in Korea. METHODS: The registries Clinicaltrials.gov and the Clinical Research Information Service (CRIS) and the search engine PubMed were searched to find relevant human clinical studies involving acupuncture therapy in PD patients. We examined the registration of trials, the posting and publication of results, and whether published articles were registered. RESULTS: In Clinicaltrials.gov, one completed trial was found with published results. In CRIS, one completed trial was found with published results. A total of 6 publications were found in our study: 2 articles were registered, but only 1 had the registered trial number listed in the article. CONCLUSION: Acupuncture is popular among the PD population in Korea regardless of its unproven safety and efficacy. Despite the pressing need for clinical trials, the number of studies listed in the registries was small, and only a few publications were registered. More effort and rigor are needed to validate the efficacy and safety of acupuncture for PD.
Acupuncture Therapy ; Acupuncture* ; Complementary Therapies ; Hope ; Humans ; Information Services ; Korea* ; Parkinson Disease* ; Publications ; Registries ; Search Engine

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