1/16. 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 = diabetes (Clic here for more details about this article) |
2/16. A case of hereditary ceruloplasmin deficiency with iron deposition in the brain associated with chorea, dementia, diabetes mellitus and retinal pigmentation: administration of fresh-frozen human plasma.We report a familial case of hereditary ceruloplasmin deficiency (HCD) showing an A-G transition in intron 6 of the ceruloplasmin gene. Clinical features consisted of chorea, cerebellar ataxia, dementia, diabetes mellitus, retinal pigmentation and iron deposition in the liver and brain without copper overload in those organs. The patient's children and siblings had similar laboratory results, but did not show any neurological abnormalities. She was medicated for diabetes mellitus at 43 years of age, and neurological signs appeared when she was 52 years old. The laboratory findings were anemia, low concentrations of iron and copper in serum and of copper in urine. ceruloplasmin was not detected in the serum. The iron and copper contents in the liver were 3,580 and 10 microg/g wet tissue, respectively. MRI of the brain showed iron deposition in the basal ganglia, dentate nucleus and thalamus. This case did not show any abnormal increase in copper in the blood and urine following CuSO(4)5H(2)O oral overloading test. Following the intravenous administration of commercially available fresh-frozen human plasma (FFP) containing ceruloplasmin, the serum iron content increased for several hours due to ferroxidase activity of ceruloplasmin. In the liver, the iron content decreased more with the combined intravenous administration of FFP and deferoxamine than with FFP administration alone. Her neurological symptoms improved following repetitive FFP treatment.- - - - - - - - - - ranking = 5.9867246310982keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
3/16. Persistent chorea triggered by hyperglycemic crisis in diabetics.Five female patients developed chorea concurrent with, or shortly after a hyperglycemic episode (admission glucose values 500-1,000 mg/dL). In four of these five patients, there was no prior history of diabetes mellitus. The chorea continued despite correction of blood glucose and persisted to the time of last follow-up, 6 months to 5 years later. The chorea developed subacutely over 2 days to 1 month and was generalized in one, unilateral in three, and involved right > left lower extremity in the other; the severity initially reached ballistic proportions in two. Associated clinical features were nil in four of these patients, but cognitive impairment and personality change occurred in one. The histories and laboratory studies identified no predisposing factors other than the hyperglycemia. The chorea was sufficiently troublesome to require administration of neuroleptic medication in all five cases. Four of the five cases had high signal intensity within basal ganglia on T1-weighted magnetic resonance (MR) imaging, as has previously been described; however, this was not seen in one case (who had the most severe clinical condition). Most previously described cases have involved a reversible clinical syndrome, in contrast to our patients. The pathogenic mechanisms remain uncertain.- - - - - - - - - - ranking = 0.99778743851637keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
4/16. Transient hemichorea/hemiballismus associated with new onset hyperglycemia.OBJECTIVE: To describe three patients suffering from transient hemichorea/hemiballismus associated with hyperglycemia, review previous reports and propose a possible pathophysiological explanation for this phenomenon. RESULTS: Our original cases and previously reported ones reveal a uniform syndrome: mostly female patients (F/M ratio of 11/2), 50-80 years old, usually with no previous history of diabetes mellitus (9/13), develop choreic or ballistic movements on one side of the body over a period of hours. serum glucose levels are elevated. In most of the patients, a lowering of the blood sugar level reverses the movement disorder within 24-48 hours. CONCLUSIONS: We believe that the combination of a recent or old striatal lesion (causing increased inhibition of the subthalamic nucleus) and hyperglycemia (causing decreased GABAergic inhibition of the thalamus) may be responsible for the appearance of this unilateral hyperkinetic movement disorder. Undiagnosed diabetes mellitus should always be suspected in patients who develop hemiballistic or hemichoreic movements. When hyperglycemia is detected and corrected, the movement disorder usually resolves within two days and may not require symptomatic therapy with dopamine receptor antagonists.- - - - - - - - - - ranking = 1.9955748770327keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
5/16. 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 = 0.2keywords = diabetes (Clic here for more details about this article) |
6/16. chorea due to nonketotic hyperglycemia.A 62 year old diabetic and hypertensive male presented with sudden onset generalized chorea. Investigations revealed uncontrolled diabetes with absent ketones and normal serum osmolality. achievement of euglycemia with insulin therapy abolished the involuntary movements completely within a day. The direct effect of hyperglycemia causing striatal neuronal dysfunction could be the pathogenesis of the chorea in our patient.- - - - - - - - - - ranking = 0.2keywords = diabetes (Clic here for more details about this article) |
7/16. 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 = 0.99778743851637keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
8/16. hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study.hyperglycemia-induced unilateral basal ganglion lesions occur mostly in Asian patients. A signal abnormality in the basal ganglion region is evident on these patients' neuroimaging. Despite characteristic imaging findings and clinical manifestations, the underlying mechanism is still unclear. To clarify the underlying pathophysiology of unilateral basal ganglion lesions, we examined the [18F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) findings in 3 patients with hyperglycemia. The PET studies were performed at 3 weeks, 5 weeks, and 7 months after clinical onset. The markedly reduced rates of cerebral glucose metabolism in the corresponding lesions on T1-weighted magnetic resonance images provided direct evidence of regional metabolic failure. We suggest that the metabolic derangements associated with hyperglycemia and vascular insufficiency contribute to regional metabolic failure in patients with poorly controlled diabetes mellitus.- - - - - - - - - - ranking = 0.99778743851637keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
9/16. 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 = 1.1977874385164keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
10/16. chorea induced by non-ketotic hyperglycaemia: a case report.We describe an 81-year-old woman presenting with sudden onset of generalised chorea. She was unaware of suffering from diabetes. Laboratory screening revealed non-ketotic hyperglycaemia. brain magnetic resonance imaging (MRI) failed to show basal ganglia abnormalities. Monotherapy with subcutaneous regular insulin induced a progressive normalisation of glycaemia as well as a parallel improvement of the abnormal involuntary movement scale on a nine-day sequential observation. This correlation strongly supports the hypothesis that non-ketotic hyperglycaemia itself might play a major pathogenetic role in chorea associated with non-ketotic hyperglycaemia. diabetes mellitus should be suspected in patients who develop sudden onset of chorea even in the absence of putaminal abnormalities on MRI.- - - - - - - - - - ranking = 0.34546678061073keywords = diabetes, mellitus (Clic here for more details about this article) |
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