1/23. Restricted unilateral Sydenham's chorea: reversible contralateral striatal hypermetabolism demonstrated on single photon emission computed tomographic scanning.Sydenham's chorea results from group A streptococcus infection and subsequent generation of antineuronal antibodies directed at the caudate nucleus and putamen. Predominantly bilateral, in up to 30% of cases the chorea can be unilaterally restricted. Imaging studies, both structural (magnetic resonance imaging) and functional (positron emission tomography), in patients with bilateral Sydenham's chorea have suggested reversible striatal abnormalities. Two patients with unilateral Sydenham's chorea are presented. Computed tomographic and magnetic resonance imaging were normal in both. However, hexamethylpropylenamine oxime single photon emission tomographic (HMPAO SPECT) studies demonstrated hypermetabolism in the contralateral basal ganglia. Resolution of symptoms in one of the patients coincided with normalization of the SPECT scan. Thus, unilateral striatal hypermetabolism appears to underlie the contralateral chorea observed. A SPECT scan probably should be included in the work-up of new-onset chorea.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
2/23. Cerebral arteriovenous malformations and movement disorders.A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
3/23. Non-ketotic hyperglycemia in a young woman, presenting as hemiballism-hemichorea.We report a 22-year-old girl presenting with acute onset left sided hemiballism-hemichorea (HH) and non-ketotic hyperglycemia (NKH). Initial brain CT revealed faint hyperdensities, sharply confined to the contralateral nucleus caudatus and putamen. Sequential MRI investigations yielded increasing hypersignal intensities on T1-weighted images and resolving hypodensities on T2-weighted images of the right striatum, leaving small sequelae in the head of the right caudate nucleus. NKH is an unusual cause of HH. The abnormalities seen in neuroimaging are rare, but seem to be quite specific to this syndrome. We give an update on current literature regarding the possible pathophysiological processes underlying this specific clinical entity.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
4/23. Hemidystonia and hemichoreoathetosis as an initial manifestation of moyamoya disease.OBJECTIVE: To describe hemidystonia and hemichoreoathetosis in an adult patient with moyamoya disease without a previous history of cerebrovascular accident. DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 22-year-old woman suddenly developed dystonic spasms in her left hand and left foot after a severe emotional stress. The dyskinesia gradually subsided over the next 4 months. Five months after the onset, she suddenly developed choreoathetoid movement in her right hand and right foot. MAIN OUTCOME AND RESULTS: The patient had both somatic and cortical sensory deficits in the right hand and right foot. magnetic resonance imaging of the brain showed an infarction at the right putamen and lesions involving the right frontal lobe and the left frontotemporoparietal lobe. Magnetic resonance cerebral angiography showed severe stenoses of both internal carotid arteries at the supraclinoid portion and numerous collateral vessels, compatible with moyamoya disease. Single photon emission tomography of the brain showed hypoperfused areas at the right frontal and left frontotemporoparietal lobes. The choreoathetosis of the right limbs improved markedly, along with improvement of sensory deficits. CONCLUSIONS: To our knowledge, this is the first report of an adult patient presenting with hemidystonia and hemichoreoathetosis as the initial manifestations of moyamoya disease. Arch Neurol. 2000;57:1510-1512- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
5/23. diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome?BACKGROUND: The magnetic resonance (MR) imaging findings of hemichorea-hemiballismus (HCHB) associated with hyperglycemia are characterized by hyperintensities in the striatum on T1-weighted MR images and computed tomographic scans, with a mechanism of petechial hemorrhage considered to be responsible. diffusion-weighted MR imaging (DWI) has been reported to detect early ischemic damage (cytotoxic edema) as bright areas of high signal intensity and vasogenic edema as areas of heterogeneous signal intensity. We report various DWI findings in 2 patients with hyperglycemic HCHB. OBJECTIVES: To describe the DWI and gradient echo findings and characterize the types of edema in HCHB associated with hyperglycemia. SETTING: A tertiary referral center neurology department. DESIGN AND methods: Two patients with HCHB associated with hyperglycemia underwent DWI, gradient echo imaging, and conventional MR imaging with gadolinium enhancement. The patients had an elevated serum glucose level on admission and a long history of uncontrolled diabetes, and the symptoms were controlled by dopamine receptor blocking agents. Initial DWIs were obtained 5 to 20 days after symptom onset. Apparent diffusion coefficient (ADC) values were measured in the abnormal lesions with visual inspection of DWI and T2-weighted echo planar images. RESULTS: T1- and T2-weighted MR images and brain computed tomographic scans showed high signal intensities in the right head of the caudate nucleus and the putamen. Gradient echo images were normal. The DWIs showed bright high signal intensity in the corresponding lesions (patient 1), and the ADC values were decreased. The decrease in ADC and the high signal intensity on DWI persisted despite the disappearance of HCHB, even after 70 days. CONCLUSIONS: Gradient echo MR imaging findings were normal in HCHB with hyperglycemia, whereas DWI and the ADC map showed restricted diffusion, which suggests that hyperviscosity, not petechial hemorrhage, with cytotoxic edema can cause the observed MR abnormalities.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
6/23. 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 = putamen (Clic here for more details about this article) |
7/23. 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 = putamen (Clic here for more details about this article) |
8/23. Unilateral putaminal CT, MR, and diffusion abnormalities secondary to nonketotic hyperglycemia in the setting of acute neurologic symptoms mimicking stroke.A 75-year-old Asian man presented with two episodes of chorea associated with nonketotic hyperglycemia. His chorea rapidly resolved after restitution of a normal serum glucose level, although an MR image obtained at the time of acute symptoms demonstrated high signal intensity on T1-weighted images, low signal intensity on T2-weighted images, and restricted diffusion, all involving the left putamen. A CT scan obtained 1 month later demonstrated faint hyperattenuation of the involved putamen. The reported pathophysiologic considerations for these imaging features are reviewed, and an original explanation is proposed.- - - - - - - - - - ranking = 2keywords = putamen (Clic here for more details about this article) |
9/23. chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as moyamoya disease.We here report one case of hemichorea and Broca aphasia occurred with diabetic ketoacidosis. A 20-year-old woman with type 1 diabetes mellitus had experienced diabetic ketoacidosis fourth time after the onset of diabetes. At the third ketoacidotic episode, the patient was admitted to our hospital for the first time to show hemichorea of the left extremities. brain computed tomography (CT) demonstrated a high-density area in the right caudate head and low-density area in the right putamen. magnetic resonance angiography (MRA) demonstrated a stenosis at the root of the bilateral middle and anterior cerebral arteries. The hemichorea disappeared within 3 days. At the fourth ketoacidotic episode, not hemichorea but unconsciousness was there for 2 days even after ketoacidosis disappeared. After the unconscious state, Broca aphasia was demonstrated for 15 days. The cerebral angiography showed a finding compatible to moyamoya disease. These findings support that chorea and Broca aphasia induced by diabetic ketoacidosis was developed in addition to blood vessel abnormalities such as moyamoya disease. We suggest that poorly controlled diabetic patients with hemichorea should undergo cerebral angiography.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
10/23. 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 = putamen (Clic here for more details about this article) |
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