11/45. Progressive supranuclear palsy with asymmetric tau pathology presenting with unilateral limb dystonia.We report an autopsy case of a 77-year-old Japanese man with a 7-year history of progressive unilateral left limb dystonia and arm levitation. Brain computed tomography showed fronto-temporal atrophy. The patient was diagnosed as having corticobasal degeneration. Histopathologically, the cerebral cortices, especially of the parasagittal region, and subcortical nuclei revealed numerous Gallyas/tau-positive cytoplasmic inclusions characteristic of progressive supranuclear palsy (PSP). Grumose degeneration was evident in the dentate nucleus. Astrocytic plaques were not present, but a small number of ballooned neurons were found in the fronto-temporal regions. The involvement by the PSP lesions was quite asymmetric in the affected areas, including the frontal cortices, basal ganglia, red nuclei, and inferior olivary nuclei, being more prominent on the side contralateral to the side of limb dystonia. The apparent unilateral dominance of PSP pathology may be relevant to the asymmetric clinical presentation of this patient.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
12/45. Effect of deep brain stimulation of GPI on neuronal activity of the thalamic nucleus ventralis oralis in a dystonic patient.OBJECTIVE: To record the possible effect of acute deep brain stimulation (DBS) of the globus pallidus internus (GPI) on the neuronal activity of the ventralis oralis anterior (VOA) nucleus of the thalamus. methods: Under general propofol anaesthesia, extracelullar single unit recordings were performed in VOA of a post-anoxic dystonic patient previously implanted with GPI located electrodes for chronic DBS. RESULTS: neurons recorded in the VOA could be classified in two cell subpopulations: a high firing rate (16.5 Hz) and low burst index (BI; 15.6) type and a low firing rate (5.5 Hz) and high BI (35.6) type. GPI electrical stimulation reduced the frequency and increased the BI of the high firing rate cells while leaving the other cell type unchanged. CONCLUSION: These results demonstrate that pallidal DBS is able to inhibit a subpopulation of motor thalamic cells and question the pathophysiological model of dystonia based on a low firing rate of GPI cells.- - - - - - - - - - ranking = 5keywords = nucleus (Clic here for more details about this article) |
13/45. Focal dystonia secondary to cavernous angioma of the basal ganglia: case report and review of the literature.The case of a young woman with focal dystonia of the hand due to a cavernous angioma of the basal ganglia is presented. The lesion involved the anterior third of the lentiform nucleus and a large portion of white matter anterior to this nucleus and lateral to the head of the caudate, as shown by magnetic resonance imaging; it was completely removed through a computed tomography-assisted stereotactic craniotomy by microsurgical technique, resulting in the cure of the patient. These facts support the pathophysiological hypothesis of a disruption of the striatopallidothalamic projection to the premotor cortex as the cause of symptomatic dystonia. A review of the reported cases of cavernous angiomas of the deep cerebral gray nuclei shows that this is the first case of cavernous angioma associated with movement disorder.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
14/45. deep brain stimulation of the nucleus ventralis intermedius for Holmes (rubral) tremor and associated dystonia caused by upper brainstem lesions. Report of two cases.Holmes tremor is caused by structural lesions in the perirubral area of the midbrain. patients often present with associated symptoms such as dystonia and paresis, which are usually refractory to medical therapy. Here, the authors describe two patients in whom both tremor and associated dystonia improved markedly following unilateral stimulation of the thalamic nucleus ventralis intermedius.- - - - - - - - - - ranking = 5keywords = nucleus (Clic here for more details about this article) |
15/45. Posttraumatic segmental axial dystonia.Isolated dystonia of trunk and neck muscles without involvement elsewhere has been termed segmental axial dystonia--a rare disorder. We report a 31-year-old man who developed marked dystonia of paraspinal muscles and progressive scoliosis 6 months after a closed head injury. Computed tomography (CT) disclosed three small areas of encephalomalacia, one involving the head of the caudate nucleus. Treatment with trihexyphenidyl resulted in significant improvement of the dystonia and scoliosis.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
16/45. Camptocormia: pathogenesis, classification, and response to therapy.Originally considered a psychogenic disorder, camptocormia, an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position, is becoming an increasingly recognized feature of parkinsonian and dystonic disorders. Prior reports were limited by sample size, short follow-up, and paucity of data on response to therapy. The authors reviewed 16 patients evaluated in their PD Center and movement disorders Clinic diagnosed with camptocormia. In addition to detailed neurologic assessment all patients were videotaped. The mean age was 64.9 /- 17.4 years, mean age at onset of neurologic symptoms was 51.5 /- 19.9 years, duration from onset of neurologic symptoms to development of camptocormia was 6.7 /- 7.6 years, and the mean duration of camptocormia was 4.5 /- 3.9 years. Of the 16 patients, 11 (68.8%) had Parkinson disease (PD); others had dystonia (n = 4) and tourette syndrome (n = 1). Twelve patients received levodopa, with minimal or no improvement in the camptocormia. Nine patients received botulinum toxin type A injections into the rectus abdominus, with notable improvement in their camptocormia in four. One patient underwent bilateral subthalamic nucleus deep brain stimulation for PD, but there was no improvement in camptocormia. Based on this series and a thorough review of the literature of camptocormia, head drop, and bent spine syndrome, the authors propose etiologic classification of camptocormia and conclude that this heterogeneous disorder has multiple etiologies and variable response to systemic and local therapies.- - - - - - - - - - ranking = 180.3299883015keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
17/45. acupuncture-induced cerebral blood flow responses in dystonia.OBJECTIVE: The effect of acupuncture (ACP) on regional cerebral blood flow (rCBF) is unclear. Single-photon emission computed tomography studies on three patients with dystonia were performed before and after ACP treatment to test the contention that ACP affects rCBF. methods: Pre-ACP and post-ACP CBF study were performed on the same day; 99mTc ethyl cysteinate dimer was injected for each study. rCBF images were analyzed using a three-dimensional stereotaxic ROI template (3DSRT) to objectively measure rCBF. We evaluated rCBF bilaterally in five segments related to the pathophysiology of dystonia (1, superior frontal; 2, middle and inferior frontal; 3, primary sensorimotor; 4, lenticular nucleus; and 5, thalamus). More than 10% left-right asymmetry in rCBF over three continuous slices was defined as significant laterality. Post-ACP rCBF and laterality were evaluated with the pre-ACP rCBF study acting as a control in each subject. RESULTS: The clinical effect of ACP was remarkable in all patients and rCBF increased in most segments. Pre-ACP rCBF exhibited significant laterality in eight segments of the three patients. Laterality reversed in seven of these segments and resolved in the remaining segment after ACP. Pre-ACP rCBF laterality was not preserved in any segment after ACP. The remaining five segments exhibited laterality only after ACP. In total, after ACP, 13 of 15 segments demonstrated a change in CBF that was greater unilaterally. CONCLUSIONS: ACP results in an increase in CBF that is greater unilaterally. We think that unilateral change in CBF may be correlated with the action of ACP on the central nervous system in patients with dystonia.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
18/45. Hemidystonia due to porencephalic cyst. A case report.We present a 28-year old patient with left hemidystonia since early childhood after head trauma. This is the first report of a patient who had hemidystonia due to CT proven porencephalic cyst involving the caudate nucleus.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
19/45. Effective treatment with levodopa and carbidopa for hypomyelination with atrophy of the basal ganglia and cerebellum.Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare leukoencephalopathy presenting in the infantile period and characterized by diffuse cerebral hypomyelination, and atrophy of the basal ganglia and cerebellum. As patients with H-ABC lack remarkable laboratory findings, the diagnosis is based on brain magnetic resonance imaging findings alone. Only eight cases have been reported in the literature, and thus the natural course and treatment of this disease are not fully understood. We report a 35-month-old boy with H-ABC who had hemidystonia, hypomyelination, and cerebellar ataxia. We diagnosed H-ABC after considering a thorough differential diagnosis, excluding other diseases involving hemidystonia, hypomyelination, and cerebellar ataxia. Furthermore, technetium-99m ethyl cysteinate dimmer-single-photon emission computerized tomography (Tc-ECD-SPECT) and positron emission tomography with fluorodeoxyglucose (18)F (FDG-PET) revealed decreased blood flow and glucose metabolism in the bilateral lenticular nucleus, thalamus, and cerebellum. A peroral levodopa preparation containing carbidopa (levodopa-carbidopa) was effective at ameliorating and stopping the progression of the patient's dystonia (final effective doses: levodopa, 200 mg/day and carbidopa, 20 mg/day). This is the first case report of a Japanese patient with H-ABC and treatment for this disease. levodopa-carbidopa may be an effective treatment for H-ABC.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
20/45. Functional MRI reveals activation of a subcortical network in a 5-year-old girl with genetically confirmed myoclonus-dystonia.We investigated a five-year-old girl suffering from genetically confirmed, action-induced myoclonus-dystonia (M-D) with functional magnetic resonance imaging (MRI). We compared the activation pattern by movements of her right hand as if drawing a picture, which elicited M-D, with simple snapping movements (without overt M-D). The drawing and snapping conditions resulted in activation of a motor network including the motor cortex, the putamen, and the cerebellar hemispheres. The direct comparison of the drawing condition with snapping as control revealed specific activations within the thalamus and the dentate nucleus. An age matched healthy control did not show significant activation within the thalamus or dentate nucleus.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
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