Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis. Pharmacotherapy for control of involuntary movements has generally been of limited benefit. Deep brain stimulation (DBS) has recently been used for treatment of some refractory cases of ChAc. We report here on the effect of bilateral high-frequency DBS of globus pallidus interna in a patient with ChAc.
Abetalipoproteinemia ; Chorea ; Deep Brain Stimulation* ; Drug Therapy ; Dyskinesias ; Globus Pallidus* ; Humans ; Neuroacanthocytosis*

OBJECTIVE: To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. METHODS: Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment. RESULTS: On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction. CONCLUSION: Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.
Adult* ; Cerebral Palsy* ; Deep Brain Stimulation* ; Dystonia ; Dystonic Disorders ; Globus Pallidus ; Humans

OBJECTIVE: To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. METHODS: Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment. RESULTS: On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction. CONCLUSION: Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.
Adult* ; Cerebral Palsy* ; Deep Brain Stimulation* ; Dystonia ; Dystonic Disorders ; Globus Pallidus ; Humans

Hemiballism is a ballistic, involuntary movement disorder, usually of a sudden onset, an often occurs due to encephalomalasic lesion, mostly an infarction, of deep structure of contralateral cerebral hemisphere, rarely by a small hemorrhage. The lesion site is thought to be mainly subthalamic nucleus contralateral to the abnormal movement. But there have been several reports on hemiballism without involvement of subthalmic nucleus throughout the world. We report a case of acute vascular hemiballism occurred due to a small hemorrhage with involvement of left putamen and globus pallidus, which was demonstrated by computed tomography and magnetic resonance imaging study of brain.
Brain ; Cerebrum ; Dyskinesias* ; Globus Pallidus ; Hemorrhage ; Infarction ; Magnetic Resonance Imaging ; Putamen ; Subthalamic Nucleus

OBJECTIVE: The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). METHODS: Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for 18.7 +/- 11.1 months. The mean duration of the CD was 5.8 +/- 3.4 years. The mean age at time of surgery was 54.2 +/- 10.2 years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. RESULTS: The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p = 0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged 81.7 +/- 6.8% at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. Conclusions: Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.
Deep Brain Stimulation ; Dysarthria ; Follow-Up Studies ; Globus Pallidus ; Humans ; Microelectrodes ; Torticollis

ABSTRACT OBJECTIVES: We report event-related functional magnetic resonance imaging(fMRI) methodology to investigate human brain activity during motor imagery. METHODS: A 1.5 Tesla clinical MR scanner was used in the acquisition of a series of T2* weighted MR images covering the whole brain. Blood oxygenation level-dependent(BOLD) signal changes associated with the imagery event were subsequently detected while healthy right-handed subjects imagined clenching of a right hand cued by auditory stimulus. RESULTS: Group analysis across nine right-handed subjects revealed activations in the medial and superior frontal gyri, cuneus, insula, middle/superior temporal gyri, and anterior cingulate gyri. Bilateral primary motor, premotor and supplementary motor areas exhibited event-related MR signal changes. Although unilateral hand clenching was imagined, bilateral activation of eloquent motor areas was observed. The proposed method also allowed for the visualization of subcortical areas, such as putamen, globus pallidus and thalamus, responsive to the event of motor imagery. CONCLUSION: The major cortical and subcortical areas in the motor pathways were identified and visualized during motor imagery event. Our results suggest that motor imagery and actual movement share common neural substrates.
Brain ; Brain Mapping ; Efferent Pathways ; Globus Pallidus ; Hand ; Humans ; Magnetic Resonance Imaging* ; Oxygen ; Putamen ; Thalamus

Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests various symptoms and signs of extrapyramidal and pyramidal involvement. Authors report a 28-year-old female patient with suspected Hallervorden-Spatz disease in the aspects of clinical and MRI findings suggesting metal deposition in the globus pallidus, substantia nigra, and red nucleus on both side.
Adult ; Female ; Globus Pallidus ; Humans ; Magnetic Resonance Imaging ; Pantothenate Kinase-Associated Neurodegeneration* ; Red Nucleus ; Substantia Nigra

OBJECTIVES: The purpose of this study was to investigate the regional differences of the basal ganglia volume between the boy subjects who have Tourette's disorder and the control group who don't have this disorder by using Brain MRI. METHODS: Sixteen subjects who were diagnosed as Tourette's disorder using DSM-IV criteria, and sixteen healthy subjects were selected. For analysis the coronal slice images of the basal ganglia were collected and collected MR images were analyzed with NIH Image (version 6.1 ppc). RESULTS: The total brain volume in the subjects with Tourette's disorder were smaller than control group by 6.2% (F=6.2, d.f.=1, p=0.019). The right putamen volume in boys with Tourette's disorder were significant smaller than control group statistically (F=3.2, d.f.=3, p=0.040). The asymmetry (left>right) in the globus pallidus decreased significantly in the boys with Tourette's disorder (p=0.06) were found from this experiment. CONCLUSION: These findings suggest that boys with Tourette's disorder may have neuropathological abnormalities in the basal ganglia. Moreover, the present findings point out the important role of basal ganglia, especially putamen, in pathophysiology of Tourette's disorder. More studies about the structural and functional analysis of the basal ganglia in Tourette's disorder are highly required.
Basal Ganglia ; Brain ; Diagnostic and Statistical Manual of Mental Disorders ; Globus Pallidus ; Putamen ; Tourette Syndrome

Manganese (Mn) intoxication is known to induce parkinsonism. Mn-induced parkinsonism preferentially affects the globus pallidus in contrast to idiopathic parkinsonism where the degeneration predominantly involves the nigral pars compacta. We present three patients who had been occupationally exposed to Mn and developed parkinsonism, and one patient who had been exposed to long-term Mn and was proven to have idiopathic parkinsonism. All the manganism patients had a particular propensity to fall in a backward gait and a prominent postural instability compared with the severity of other parkinsonian features. Case 1 also showed a cock walk and dystonic features, quite characteristic findings of manganism. The rigidity, another important parkinsonian sign, was not found in all three manganism patients. There was no sustained therapeutic response to levodopa. A [18F]6-fluoro-L-dopa (6FD) PET scan performed in case 1 was normal in contrast to a reduced uptake in the striatum of the patient with idiopathic parkinsonism. These cases indicate that Mn-induced parkinsonism can be differentiated from idiopathic parkinsonism in that the former has unique clinical features and a normal 6FD PET scan.
Gait ; Globus Pallidus ; Humans ; Levodopa ; Manganese* ; Occupations ; Parkinsonian Disorders* ; Positron-Emission Tomography

Few studies have investigated cognitive impairment associated with globus pallidal lesion after acute mountain sickness (AMS). A 54-year-old mountaineer presented with a slowed response, social withdrawal, and apathy after exposure to high altitude. Neuropsychological evaluation revealed impairment in memory and frontal function. MRI revealed bilateral globus pallidal lesions and SPECT revealed decreased blood flow in the left temporal cortex. Symptoms and imaging findings were still impaired after 3 years. We speculate that globus pallidal lesions can cause cognitive dysfunction in AMS.
Altitude ; Altitude Sickness ; Apathy ; Globus Pallidus ; Humans ; Memory ; Middle Aged ; Tomography, Emission-Computed, Single-Photon