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

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

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

Parkinson's disease (PD) is a extrapyramidal movement disorder characterized by rigidity and bradykinesia. PD is one of the most common neurodegenerative disorders, affecting 1% of the population over the age of 60. Dementia is common and affects 40% of patients with PD during the course of the disease, the risk for the development of dementia being 6 times higher than in age-matched general population. In addition to motor abnormalities, there are several non-motor signs and symptoms that may create a considerable burden for patients and caregivers. Parkinsonism is a major feature of several dementing diseases. The parkinsonian disorders with dementia are Parkinson's disease dementia (PDD), parkinsonian-plus syndromes, sepcific heredodegenerative diseases, and secondary parkinsonisms. The parkinsonian-plus syndromes are neurodegenerative disorders charaterized by parkinsonism and at least one other nonparkinsonian neurological manfestation. This brief review concentrates on those disorders in which cognitive impairment/dementia and parkinsonism coexist: Parkinson's disease dementia, progressive supranuclear palsy, and corticobasal degeneration. The clinical and neuropsychological similarities and differences in these disorders are compared and contrasted along with Alzheimer's disease, Parkinson's disease, and dementia of Lewy bodies, highlighting the features critical for identifying the correct diagnosis.
Alzheimer Disease ; Caregivers ; Dementia* ; Diagnosis ; Humans ; Hypokinesia ; Lewy Bodies ; Movement Disorders ; Neurodegenerative Diseases ; Parkinson Disease ; Parkinsonian Disorders* ; Supranuclear Palsy, Progressive

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

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

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 due to poor the self-awareness of the conditions and the underuse of validated tools and objective instruments for assessment. The early detection and intervention of dysphagia are closely related to improving the quality of life and decreasing the mortality rate in these patients. The purpose of this paper is to give an overview of the characteristics of dysphagia, including the epidemiology, pathophysiology, and clinical symptomatology, in patients with PD compared with other parkinsonian disorders and movement disorders. The management of dysphagia and future research directions related to these disorders are also discussed.
Deglutition Disorders ; Dystonia ; Epidemiology ; Humans ; Mortality ; Movement Disorders ; Parkinson Disease ; Parkinsonian Disorders ; Quality of Life

Neurological complications associated with 2009 H1N1 infection in children have been reported and recognized worldwide. The most commonly reported neurological complications are seizures and encephalopathy. Secondary movement disorders are also associated with the infection, but such cases are rarely reported. Here, we describe the case of a 14-year-old boy with paroxysmal kinesigenic dyskinesia secondary to 2009 H1N1 infection, who presented with dystonia and choreic movement triggered by sudden voluntary movement.
Child ; Chorea ; Dyskinesias ; Dystonia ; Humans ; Influenza A virus ; Movement Disorders ; Seizures