Joo-Hyuk Im

Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.

BACKGROUNDnChorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain magnetic resonance images (C-H-BG) is recognized as a unique syndrome that affects elderly women exclusively. However, its overall clinical features are unclear.nnnMATERIAL AND METHODSnThe literature describing patients with C-H-BG from 1985 to 2001 was reviewed using MEDLINE. Their clinical features and those of four patients with C-H-BG at this hospital were analyzed.nnnRESULTSnThis study included 49 patients from the literature and four patients at this hospital. Their mean age at the onset was 71.1 years (range=22-92 years). Women were affected more frequently than men (men/women=17:30). The mean serum glucose level measured after the onset of chorea was 481.5 mg/dl (ranging from 169 to 1264), HbA1c level was 14.4% (ranging from 9.9 to 19.2), and the serum osmolarity was 305.9 mmol/kg (ranging from 291 to 335). Forty-seven patients developed hemichorea. Six patients developed bilateral chorea, and magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. MRI showed that putamen was involved in all cases (isolated putamen=31 patients, additional basal ganglia lesions=22 patients). None had lesions confined to the caudate nucleus or the globus pallidus. In all, except one, the anterior limb of the internal capsule was spared. Follow-up MRI studies were performed in 22 patients. In most, hemichorea improved along with the disappearance of the lesions. In 39 patients, chorea had ameliorated completely. The remaining 14 cases showed some improvement during the follow-up period. The chorea recurred in seven patients.nnnCONCLUSIONnC-H-BG is a benign disorder affecting the elderly. It affects men much more frequently than has been reported. The high signal intensity basal ganglia lesion on the T1-weighted brain MRI study was reversible, and correlated with the clinical improvement in chorea.

Ropinirole as an adjunct to levodopa in the treatment of Parkinson's disease: a 16-week bromocriptine controlled study.

Abstract.Background: and objectives Ropinirole is a non-ergoline, selective dopamine D2 agonist. The aim of this study was to evaluate the efficacy and safety of ropinirole as an adjunct to levodopa in the treatment of Parkinsons disease (PD) complicated by motor fluctuations. Methods: A total of 76 patients with PD (Hoehn and Yahr stage II to IV) were included in this trial. Each patient was randomly allocated to receive either ropinirole (n = 37) or bromocriptine (n = 39) as an adjunct to levodopa over a 16-week period. Ropinirole and bromocriptine were titrated for optimal efficacy and tolerability. This optimal dose was then maintained for the rest of the study. Response rate was defined as the percentage of patients who achieved at least a 20 % reduction in levodopa dose. Clinical status was also assessed using the Unified Parkinsons Disease Rating Scale (UPDRS), Clinical Global Impression (CGI), and reduction in time spent ‘off’. Results: Ropinirole produced a significantly greater response rate than bromocriptine (odds ratio 2.995, 95 % C. I. (1.157, 7.751) p < 0.05). There was also a statistically significant difference between the groups in the proportion of patients who were ‘improved’ on the CGI improvement scale (91.9 % for ropinirole, 74.3 % for bromocriptine, p = 0.046). Other measures, including at least a 20 % improvement in the UPDRS motor score (70 % for ropinirole and 63.3 % for bromocriptine), and a 20 % reduction in ‘off’ duration (81 % for ropinirole and 52.4 % for bromocriptine) showed a trend in favour of ropinirole. There was no significant difference between the two groups in the overall incidence of adverse effects (ropinirole, 59.5 %; bromocriptine, 59 %). In each group, the most common side-effects were dizziness, dyskinesia and nausea/vomiting. No patients were withdrawn from the study because of side-effects. Conclusion: Ropinirole was found to be safe and well-tolerated. Ropinirole as an adjunct to levodopa in the treatment of PD with motor fluctuation was associated with more significant reduction of levodopa dose and, on one form of analysis, with significantly greater improvement in CGI ratings than bromocriptine. On the other efficacy measures the two drugs were comparable.

Short- and Long-Term Outcomes of Spontaneous CSF Hypovolemia

We undertook a study to investigate the short- and long-term outcomes of spontaneous CSF hypovolemia. Fifty-three consecutive patients with spontaneous CSF hypovolemia were included. Short-term outcome was assessed 4 weeks after the treatment in all patients. Long-term outcome after a mean follow-up of 61 months (range, 13–101 months) was evaluated in 26 patients. There were 18 men and 35 women (66%) with an age range of 22–64 years (mean 37 ± 9.5). In assessing the short-term outcome of CSF hypovolemia, we found that complete headache relief was significantly higher in 43 patients who received epidural blood patch (EBP) than in 10 patients treated with supportive measures (p < 0.05). After a mean follow-up of 61 months, 25 (96%) of the 26 patients evaluated for long-term outcome had complete headache relief. These results suggest that EBP is safe and effective, resulting in excellent short- and long-term outcomes of CSF hypovolemia.

Ropinirole as an adjunct to levodopa in the treatment of Parkinson's disease

Abstract.Background: and objectives Ropinirole is a non-ergoline, selective dopamine D2 agonist. The aim of this study was to evaluate the efficacy and safety of ropinirole as an adjunct to levodopa in the treatment of Parkinsons disease (PD) complicated by motor fluctuations. Methods: A total of 76 patients with PD (Hoehn and Yahr stage II to IV) were included in this trial. Each patient was randomly allocated to receive either ropinirole (n = 37) or bromocriptine (n = 39) as an adjunct to levodopa over a 16-week period. Ropinirole and bromocriptine were titrated for optimal efficacy and tolerability. This optimal dose was then maintained for the rest of the study. Response rate was defined as the percentage of patients who achieved at least a 20 % reduction in levodopa dose. Clinical status was also assessed using the Unified Parkinsons Disease Rating Scale (UPDRS), Clinical Global Impression (CGI), and reduction in time spent ‘off’. Results: Ropinirole produced a significantly greater response rate than bromocriptine (odds ratio 2.995, 95 % C. I. (1.157, 7.751) p < 0.05). There was also a statistically significant difference between the groups in the proportion of patients who were ‘improved’ on the CGI improvement scale (91.9 % for ropinirole, 74.3 % for bromocriptine, p = 0.046). Other measures, including at least a 20 % improvement in the UPDRS motor score (70 % for ropinirole and 63.3 % for bromocriptine), and a 20 % reduction in ‘off’ duration (81 % for ropinirole and 52.4 % for bromocriptine) showed a trend in favour of ropinirole. There was no significant difference between the two groups in the overall incidence of adverse effects (ropinirole, 59.5 %; bromocriptine, 59 %). In each group, the most common side-effects were dizziness, dyskinesia and nausea/vomiting. No patients were withdrawn from the study because of side-effects. Conclusion: Ropinirole was found to be safe and well-tolerated. Ropinirole as an adjunct to levodopa in the treatment of PD with motor fluctuation was associated with more significant reduction of levodopa dose and, on one form of analysis, with significantly greater improvement in CGI ratings than bromocriptine. On the other efficacy measures the two drugs were comparable.

Differential patterns of dopamine transporter loss in the basal ganglia of progressive supranuclear palsy and Parkinson's disease: Analysis with [123I]IPT single photon emission computed tomography

We evaluated the patterns of dopamine transporter loss in the striatum of ten controls, twenty patients with Parkinsons disease (PD), and nine with progressive supranuclear palsy (PSP) using (123)I-IPT single photon emission tomography (SPECT). Four ROIs in the striatum correspond to the head of caudate nucleus (ROI 1), a transitional region between head of caudate and putamen (ROI 2), anterior putamen (ROI 3), and posterior putamen (ROI 4). A striatal ratio of specific to nondisplaceable uptake (V3) was calculated normalizing the activity of the ROIs to that of occipital cortex. V3 values were significantly reduced in all ROIs of PD and PSP patients, compared with controls (p=0.001). V3 value in ROI 2 was significantly lower in PSP group, compared with PD group (p=0.02). The percent reductions of striatal uptake in ROI 1, ROI 2, ROI 3 and ROI 4 were 56%, 53%, 64% and 78% in PD patients, whereas 75%, 72%, 75% and 77% in PSP patients, respectively. The reduction patterns of uptake were significantly different between PD and PSP groups (p=0.001). In PD patients, the percent reductions of (123)I-IPT uptake were significantly greater in ROI 3 and 4 compared with ROI 1 or 2, whereas those were similar in all ROIs of PSP patients. In addition, PD patients showed a significantly higher posterior putamen/caudate ratio of reduced (123)I-IPT uptake than the anterior putamen/caudate ratio (p=0.005). Our results implicate that (123)I-IPT SPECT is a relatively simple and reliable technique that may be useful in differentiating PD from PSP.

Co-Occurrence of Seizure and Chorea in a Patient with Nonketotic Hyperglycemia

roleptic drugs. She had no family history of movement disorders. On admission, she had chorea involving the left arm and leg. The chorea was continuous and could not be suppressed voluntarily, although it disappeared during sleep. It gradually worsened over the following days, involving the left face, left arm, left leg, and the right leg to a lesser degree. The patient also showed frequent, recurrent episodes of reduced attentiveness and responsiveness, rambling speech, irritability, and visual hallucinations, which consisted of several onions painted in all the colors of the rainbow. The level of fasting blood glucose was 473 mg/dl and glycosylated hemoglobin A Ic was 18%. No ketones were detected in urinalysis. Other routine blood tests and specialized tests including thyroid test, antistreptolysin O, rheumatoid factor, lupus anticoagulant, antiphospholipid antibody, antinuclear antibody, anti-ds-DNA, and antineutrophil cytoplasmic antibody were all normal. Neurological examination revealed only mildly decreased deep tendon refl exes and sensation of vibration on both lower legs. Her gait was markedly impaired due to severe chorea. A brain CT performed 10 days after symptom onset showed slightly high densities in the bilateral basal ganglia ( fi g. 1 a). The level of hyperattenuation decreased in Dear Sir, Chorea and striatal hyperintensity on T 1 -weighted MRI associated with nonketotic hyperglycemia (NKH) have been recognized as a peculiar disorder [1, 2] . It tends to occur in elderly people. Most reported cases are in people of east Asian origin, which suggests a possible genetic disposition to the disorder [3] . The etiological relationship between chorea and striatal hyperintensity, and the pathogenesis of striatal lesions remains unknown. Seizures associated with NKH have also been reported [4, 5] . However, the coexistence of seizure and chorea in a patient with NKH has not been previously reported. We describe here a patient who concurrently developed seizure and chorea associated with NKH.

Levodopa‐responsive parkinsonism associated with hydrocephalus

We report on a patient who developed acute parkinsonian symptoms following shunt revision for hydrocephalus. Parkinsonism improved dramatically after levodopa therapy. Furthermore, there was no recurrence of Parkinsonism despite discontinuation of levodopa. This observation suggests that Parkinsonism in this patient was caused by reversible dysfunction of the presynaptic nigrostriatal dopaminergic pathway due to hydrocephalus.

A novel missense mutation of the GTP cyclohydrolase I gene in a Korean family with hereditary progressive dystonia/dopa-responsive dystonia

Hereditary progressive dystonia with marked diurnal fluctuation/dopa-responsive dystonia (HPD/DRD) shows the considerable heterogeneity of clinical phenotypic expression and a dramatic sustained response to levodopa. The autosomal dominant HPD/DRD is caused by mutations in the gene coding GTP cyclohydrolase I (GCH I), the enzyme that catalyzes the first step in the biosynthesis of tetrahydrobiopterin. Previous studies suggested that normal [18F]Dopa positron emission tomography or [123I]beta-CIT single-photon emission computed tomography (SPECT) imaging, indicating intact structural integrity of nigrostriatal neurons, may be useful for differentiating HPD/DRD from clinically similar conditions such as juvenile Parkinsons disease with dystonia that have a considerably poorer prognosis. We here report a Korean family affected with HPD/DRD due to a novel missense mutation of the GCH I gene (T-->G mutation in exon 2), Met 137 Arg, which may change the conformation of the binding site of GCH I. The clinical features are considerably variable within the family. We documented normal striatal uptake of [123I]IPT, a dopamine transporter ligand with fast washout kinetics, in our patients by using SPECT. This method can be helpful in diagnosing HPD/DRD in uncertain cases.