We compared heavily T2W high field MR images of 18 Parkinson's disease (PD group), 8 Parkinsonian syndromes (PS group: progressive supranuclear palsy, OPCA, Shy-Drager syndrome, atypical' parkinsonism), and 20 control patients and exaimined the reported abnormalities (putaminal hypointensity, restoration of the signal intensity of the substantia nigra, narrowing of the pars compacta, brain atrophy) in our patients by 2.0 Tesla MRI. In this study, the narrowing of the signal band from the pars compacta of the substantia nigra was the most valuable index differentiating PD group or PS group from control group and the signal restoration of the substantia nirga was more common in PD than PS or control group. The frequency of putaminal hypointensity and brain atrophy increased with aging and brainstem atrophy was observed in only PS group.
Aging ; Atrophy ; Brain ; Brain Stem ; Humans ; Magnetic Resonance Imaging* ; Parkinson Disease* ; Parkinsonian Disorders* ; Shy-Drager Syndrome ; Substantia Nigra ; Supranuclear Palsy, Progressive

Hallervorden-Spatz disease (HSD) is a rare, progressive, autosomal recessive hereditary disorder characterized by pyramidal and extrapyramidal signs, speech disturbances, mental deterioration and retinal degeneration during childhood or adolescence. In late-onset form after the age of 20, parkinsonian features may be the predominant clinical manifestation. Meanwhile, involuntary tongue movements are rare and poorly understood, which have been reported in tardive dyskinesia, neuroacanthocytosis, chronic epilepsy, and after head trauma. We report a case of a patient with 'fly catcher's tongue' as a major clinical presentation, accompanied with mild parkinsonism, and typical MR findings of HSD.
Adolescent ; Craniocerebral Trauma ; Diptera* ; Epilepsy ; Humans ; Movement Disorders ; Neuroacanthocytosis ; Pantothenate Kinase-Associated Neurodegeneration* ; Parkinsonian Disorders ; Retinal Degeneration ; Tongue*

BACKGROUND: Akathisia is defined as inner feeling or restlessness and can be associated with restless movement. It is a frequent and disabling complication of neuroleptics and was reportedly common in postencephalitic parkinsonism. Akathisia has been reported and appears to be common in Parkinson's disease(PD). OBJECTIVE: To determine the frequency and clinical features of akathisia and dexamine the relationship between presence of akathisia and various clinical parameters of PD, Method: we evaluated 137 PD patients from movement disorder clinics in Samsung Medical Center and Seoul National University Hospital, using a modified akathisia questionnare. We compared two groups between those with akathisia and those without akathisia. We analysed the pattern, location and chronology of akathisia, and the correlation between akathisia and clinical parameters of PD. RESULTS: 1. Of the 137 patients (76 women, 61 men), 43(22 women, 21 men) (31.4%) had akathisia. 2. Patients with akathisia had more advanced disease than those without akathisia as determined by Hoehn and Yahr stage (p<0.05). 3. Patients with akathisia were more often akinetic-rigid than tremor predominent (p<0.05). 4. Patients with akathisia were more frequently treated with levodopa than those without akathisia (p<0.05). 5. There was no difference between two groups for age, sex or disease duration(p<0.05). 6. Of the 43 patients with akathisia, 35 patients had motor restlessness and 27 patients had associated sensory complaints. 7. The whole body or legs were most frequently affected. 8. Akathisic symptoms occurred irregularly and mainly in the afternoon. 9. Nineteen patients had difficulty in their jobs due to akathisia. Conclusion: Akathisia is a common problem in PD especially in severe akinetic-rigid form. It can be the main cause of disability in PD. Recognition and proper management are needed.
Antipsychotic Agents ; Female ; Humans ; Leg ; Levodopa ; Movement Disorders ; Parkinson Disease* ; Parkinson Disease, Postencephalitic ; Psychomotor Agitation* ; Seoul ; Tremor

BACKGROUND AND SIGNIFICANCE: Oro-facial dyskinesia are stereotyped movements, consisting of smacking and pursing of the lips, lateral deviation and protrusion of the tongue, and occasionally lateral deviation and protrusion of the jaw. Oro-facial dyskinesia rarely occur with Huntington's disease, acquired hepatocerebral degeneration, or other choreatic disorders. Spontaneous oro-facial dyskinesias occur in the elderly and had been said to result from edentulousness. It is important to clarify the pathophysiology of senile oro-facial dyskinesia for the prevention and proper treatment. I have had a doubt on the current concept that edentulism is the main etiologic factor of senile oro-facial dyskinesia. Through the clinical experience, I have had an impression that edentulism might not be the primary underlying cause of senile oro-facial dyskinesia. CASE AND METHODS: I have recently experienced four cases of senile oro-facial dyskinesia. There were no history of being exposed to neuroleptics. I tried to clarify the role of edentulism in senile oro-facial dyskinesia through clinical observations, electrophysiologic studies, and pharmacologic trials. CONCLUSION: Two patients said that wearing of denture might be responsible for the development of oro-facial dyskinesia. EMG studies with some procedures showed somewhat consistent results between two patients who were tested. EMG activities were increased by wearing of denture and decreased by touching on the perioral area with examiner's hand. Furthermore two patients who were medicated with dopamine receptor blocker showed marked clinical improvements. These results suggested that senile degenerative changes of central nervous systems might be the primary cause of senile oro-facial dyskinesia.
Aged ; Antipsychotic Agents ; Central Nervous System ; Chorea ; Dentures ; Dyskinesias* ; Hand ; Hepatolenticular Degeneration ; Humans ; Huntington Disease ; Jaw ; Lip ; Receptors, Dopamine ; Tongue

Hand tremor is one of the most frequent movement disorders and embarrassing symptoms of a wide spectrum of diseases. Hand tremor can be classified by form in which the hand tremor occurs, the main distinction being whether the hand tremor occurs at rest or is produced by voluntary muscle contractions. Essential tremor is the most common pathologic tremor in humans. The clinical features of essential tremor are heterogeneous across patients. Parkinson's disease is a common disease in the elderly with increasing prevalence over the last decade. Rest tremor is one of the cardinal motor features of Parkinson's disease. Drug-induced hand tremor is also common and should be diagnosed accurately. Hand tremor in Wilson's disease is very important to identify because the disease can be treatable. Here, the diagnosis and treatment of hand tremor were reviewed to provide a practical guide for managing patients with hand tremor.
Aged ; Contracts ; Essential Tremor ; Hand ; Hepatolenticular Degeneration ; Humans ; Movement Disorders ; Muscle, Skeletal ; Parkinson Disease ; Prevalence ; Tremor

There are a variety of different causes of parkinsonism including PD, secondary parkinsonism, and the parkinsonism plus syndromes. Secondary parkinsonism is caused by structural, toxic, metabolic, or infectious mechanisms. Among structural causes, intracranial neoplasms are a rare cause of secondary parkinsonism. Moreover, there are almost never case reports with intracranial space-occupying lesions resulting in parkinsonism associated with rapid cognitive impairment. Therefore, we report herein a 37-year-old woman diagnosed with papillary meningioma who presented with parkinsonism associated with rapidly progressive cognitive impairment mimicking diffuse Lewy body disease.
Adult ; Brain Neoplasms ; Dementia ; Female ; Humans ; Lewy Body Disease ; Meningioma ; Parkinson Disease, Secondary ; Parkinsonian Disorders

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.
Adult ; Age of Onset ; Alleles ; Basal Ganglia ; Brain ; Dystonia ; Freezing ; Gait ; Gene Frequency ; Genetic Association Studies ; Humans ; Iron ; Korea ; Movement Disorders ; Neurodegenerative Diseases ; Pantothenate Kinase-Associated Neurodegeneration* ; Parkinsonian Disorders ; Phenotype ; Population Characteristics* ; Referral and Consultation ; Weather

BACKGROUND: Chronic acquired hepatocerebral degeneration (CAHD), a slowly progressive neurologic disease characterized by chronic intermittent hepatic encephalopathy, is seen sporadically in patients with chronic hepatic disease. The spectrum of clinical presentations could include neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, Parkinsonism, myoclonus, dystonia) or both. Unfortunately only a few reports are available concerning the neurological manifestations as well as the findings of brain Magnetic resonance image (MRI) in patients with CAHD in spite of high prevalence of chronic liver disease in Korea. METHODS: We reviewed clinical or laboratory data of 3 patients with CAHD. All patients had evaluations such as neurological examination, mini-mental status examination, liver function test and brain MRI. From sagittal image of their brain MRI, we calculated pallidal signal intensity. RESULTS: The most common neurological findings were cognitive dysfunction, dysarthria, and parkinsonism including tremor, rigidity and bradykinesia. The brain MRI of all three patients showed hyperintensity signal in globus pallidus on T1 weighted image. Some patients also showed similar signal intensity on their brainstem. CONCLUSIONS: We describe three cases of CAHD with clinical, radiological feature.
Brain ; Brain Stem ; Chorea ; Dysarthria ; Globus Pallidus ; Hepatic Encephalopathy ; Hepatolenticular Degeneration* ; Humans ; Hypokinesia ; Korea ; Lethargy ; Liver Diseases ; Liver Function Tests ; Magnetic Resonance Imaging ; Movement Disorders ; Myoclonus ; Neurologic Examination ; Neurologic Manifestations ; Parkinsonian Disorders ; Prevalence ; Tremor

OBJECTIVE: Parkinsonism and other movement disorders have previously been reported in the acquired hepatocerebral degeneration associated with portosystemic shunting. However, there is no study to date about their prevalence as has been noted in general practice. METHODS: One hundred and forty-three patients with hepatic cirrhosis from the gastroenterology clinic and internal medicine wards were enrolled. Liver data included the diagnoses, etiologies, assessments of complications, and treatments for cirrhosis. Hepatic encephalopathy was classified with regard to the West Haven criteria for semi-quantitative grading for mental status. Neurological examination results and abnormal involuntary movements were recorded as primary outcomes. Neuro-radiology was used for the detection of severe brain lesions. RESULTS: Alcoholism was the most common cause of liver cirrhosis. Eighty-three patients (58%) presented with movement disorders. Asterixis was found in one of the cases. The most common movement disorder seen was an intentional tremor at 37.1%, which was followed by bradykinesia, Parkinsonism, and postural tremors at 29.4%, 10.5%, and 6.3%, respectively. The prevalence of movement disorders simultaneously increased with a high Child-Turcotte-Pugh score. The hepatic encephalopathy was grade 1 and 2. With the inclusion of age-range adjustments, we found that alcoholic cirrhosis and hepatic encephalopathy are statistically significant factors [p < 0.05, odds ratio (OR) = 6.41, 95% confidence interval (CI) 1.38-29.71 and p < 0.001, OR = 13.65, 95% CI 4.71-39.54] for the development of movement disorders in non-Wilsonian cirrhotic patients. CONCLUSIONS: Intentional tremor is a common abnormal movement. Alcoholic cirrhosis and hepatic encephalopathy are significant risk factors in the development of movement disorders in non-Wilsonian cirrhotic patients.
Alcoholism ; Brain ; Diagnosis ; Dyskinesias ; Fibrosis ; Gastroenterology ; General Practice ; Hepatic Encephalopathy ; Hepatolenticular Degeneration ; Humans ; Hypokinesia ; Internal Medicine ; Liver ; Liver Cirrhosis ; Liver Cirrhosis, Alcoholic ; Movement Disorders* ; Neurologic Examination ; Odds Ratio ; Parkinsonian Disorders ; Portasystemic Shunt, Surgical ; Prevalence* ; Risk Factors* ; Schools, Medical* ; Tremor

Dystonia is an abnormal movement characterized by sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. Torsion dystonia is characterized by torsion spasms of muscle contraction, which distorts the limbs and trunk into dystonic postures. We present a case of a patient with torsion dystonia who was recalcitrant to oral medication or even surgical operations. This patient was treated with motor point block using 5% phenol solution. Using electromyographical guidance, phenol was injected into the paraspinal and upper extremity muscles, respectively. He showed reduction of dystonia and improvement of functional abilities. Motor point block using phenol can be considered as a tool of the management for patients with torsion dystonia. MDVAQ@yumc.yonsei.ac.kr
Dyskinesias ; Dystonia ; Dystonia Musculorum Deformans* ; Extremities ; Humans ; Muscle Contraction ; Muscles ; Phenol* ; Posture ; Spasm ; Upper Extremity