1/14. blood brain barrier destruction in hyperglycemic chorea in a patient with poorly controlled diabetes.A case of hemichorea in a patient with poorly controlled diabetes is reported. T1-weighted magnetic resonance imaging (MRI) showed an unusual homogeneous high-intensity area in the corpus striatum. Of interest in the case was the fact that the globus pallidus, which was enhanced with gadolinium at the onset of hemichorea, showed homogeneous high-intensity on a subsequent T1-weighted image. This indicated that blood brain barrier destruction preceded the signal intensity change in the basal ganglia. As far as the authors could determine, this is the first reported case showing such enhancement during the course of diabetic hemichorea.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
2/14. Neuronal activity in the globus pallidus in chorea caused by striatal lacunar infarction.pallidotomy was performed in a patient with hemichorea caused by lacunar infarction in the striatum. chorea in the lower limb was reduced after a neurosurgical lesion in the medial portion of the sensorimotor territory of the internal segment of the globus pallidus, and chorea in the upper limb disappeared after an additional lesion in the lateral portion of that same area. Intraoperative neuronal recording revealed that mean firing rates were low, and that firing was irregular in the globus pallidus compared with off-state parkinsonian patients. These results suggest that chorea with striatal infarction is driven by phasic neuronal activity with a low firing rate in the globus pallidus and that the neural pathway of chorea has a functional somatotopical organization in the globus pallidus.- - - - - - - - - - ranking = 8keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
3/14. Hemichorea-hemiballism in primary diabetic patients: MR correlation.PURPOSE: The purpose of this work was to describe the characteristic imaging findings and clinical presentations in patients with hemichorea-hemiballism (HC-HB) associated with nonketotic hyperglycemia (NKH) in primary diabetes mellitus (DM). METHOD: The MR findings from six patients with HC-HB associated with NKH in primary DM were evaluated. Their ages ranged from 43 to 81 years. CT was performed on three patients, one of whom underwent a SPECT exam and another who had follow-up MRI. RESULTS: A high-signal putaminal lesion was evident on the T1-weighted images in all cases without edema or mass effect. Three of the six cases also showed high-signal intensities in the caudate. Two cases revealed high-signal intensities in the globus pallidus, and the lesions extended to the midbrain in one patient. The T2-weighted and FLAIR images were more variable. One diffusion-weighted image showed increased signal intensity. All three patients who had postcontrast MRI showed no enhancement. Two of the three patients who had CT studies showed high attenuation and the other isodensity. The SPECT study showed decreased perfusion. In all our patients, the chorea resolved within days to weeks after correction of the underlying hyperglycemia. CONCLUSION: In patients with HC-HB with NKH in primary DM, T1-weighted MR images showed hyperintense lesions of the putamen or caudate. Early recognition of these imaging characteristics may facilitate the diagnosis of primary DM with hyperglycemia and lead to prompt and appropriate therapy.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
4/14. arm chorea secondary to an unruptured giant aneurysm.We describe the case of a 20-year-old male who developed right-arm choreic movements secondary to a giant unruptured aneurysm impinging upon the left thalamus, putamen, globus pallidus, cerebral peduncle, midbrain, and subthalamic nucleus. The aneurysm was treated successfully with coils and a supraclinoid balloon. Abnormal movements initially failed to ameliorate, but within a few months, it was possible to discontinue symptomatic haloperidol therapy, with only mild residual abnormal movements.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
5/14. Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: technical case report.OBJECTIVE AND IMPORTANCE: Huntington's disease (HD) produces debilitating motor abnormalities that are poorly responsive to medical therapy. deep brain stimulation (DBS) may offer a treatment option for afflicted patients, but its role in the management of HD remains unclear. In the present case, DBS leads were implanted bilaterally into the posteroventral globus pallidus internus (GPi) to control disabling and medically intractable choreathetosis in a severely affected HD patient. The surgical procedure, intraoperative electrophysiological findings, and 12-month postoperative course, with patient video, are presented. CLINICAL PRESENTATION: This 41-year-old man with genetically confirmed HD developed motor symptoms at age 28. He had completed multiple medical trials without alleviation of his progressive and debilitating choreathetosis. Extensive clinical assessment, including neuropsychological testing, was performed to determine surgical candidacy. INTERVENTION: DBS leads were bilaterally implanted, under stereotactic guidance, into the posteroventral GPi. disease progression and symptom control were assessed at regular postoperative intervals. Bilateral pallidal stimulation produced a dramatic reduction in choreathetoid movements and improvement in overall motor functioning. The patient also exhibited normalization of body weight, mood, and energy level, as well as improved performance of activities of daily living. These effects were sustained at 1 year after surgery. CONCLUSION: The clinical benefits of DBS observed in this HD patient were comparable to those reported in other hyperkinetic disorders and demonstrate that pallidal stimulation can provide long-term alleviation of HD-associated choreathetosis.- - - - - - - - - - ranking = 5keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
6/14. chorea induced by globus pallidus externus stimulation in a dystonic patient.Bilateral high-frequency stimulation of the internal part of the pallidum has proven its efficacy in improving motor symptoms of dystonia. In Parkinson's disease, the stimulation of the external pallidum (GPe) can induce dyskinesias. This has never been described in dystonia. We report here a case of abnormal movements induced by the stimulation of GPe in a dystonic patient and discuss the pathophysiological mechanisms.- - - - - - - - - - ranking = 4keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
7/14. ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.Five generations of a family exhibit a unique autosomal dominant neurologic disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale. The clinical and pathologic findings are closely correlated. ataxia and chorea are related to severe neuronal loss in the dentate nucleus with calcification in the globus pallidus. dementia occurs with progressive demyelination of the centrum semiovale, and loss of posterior column function occurs with neuroaxonal dystrophy of the nucleus gracilis and nucleus cuneatus.- - - - - - - - - - ranking = 2keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
8/14. A case of transient choreoathetosis with amnesic syndrome after acute monoxide poisoning.We report a case of acute CO poisoning, followed after 9 days by the onset of choreoathetosis in the upper limbs and face, in a 24 year old man. The patient also showed memory disturbances that tended to clear gradually within about six months. CT scan and NMR revealed symmetrical bilateral lesions in the globus pallidus.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
9/14. Neurochemical findings in neuroacanthocytosis.We performed a neurochemical study of the brain of two unrelated patients, living in different continents, with neuroacanthocytosis. The levels of monoamines and their metabolites, gamma-aminobutyric acid and substance p, were measured in several brain areas and the monoamine metabolites in cerebrospinal fluid. The binding of 3H-spiperone to striatal membranes and to lymphocytes was also measured. Both patients had a progressive neurological disorder with onset in the third decade of life and characterized by a complex movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder initially manifested with oromandibular dystonia and limb chorea, but at the time of death was characterized by a severe dystonic syndrome. The chemical changes were similar in the two patients. The most important neurochemical findings were a depletion of dopamine and its metabolites in most brain areas, most notably in the striatum, and elevation of norepinephrine levels in the putamen and globus pallidus. substance p was markedly reduced in the striatum and substantia nigra. Our findings may provide clues to the neurochemical mechanisms underlying dystonia.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
10/14. Delayed choreoathetosis following acute carbon monoxide poisoning.Ten days after accidental exposure to carbon monoxide, a 17-year-old youth developed transitory choreoathetosis of both arms, face, and neck, with moderate dysarthria. CT revealed symmetric bilateral infarction in the head of the caudate nucleus, the putamen, and the small parts of the anterolateral globus pallidus.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
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