Cerebellar Degeneration Associated with Sjogren's Syndrome

BACKGROUND: Neurologic manifestations of primary Sjogren's syndrome (PSS) have been reported to vary from sensory polyneuropathy to encephalopathy or psychiatric problems. However, marked cerebellar degeneration associated with PSS has rarely been reported. CASE REPORT: We describe a patient with Sjogren's syndrome who exhibited rapidly progressive cerebellar ataxia, nystagmus, cognitive decline, and psychiatric problems. Brain magnetic resonance imaging revealed marked atrophy of the cerebellum, and 18F-fluorodeoxyglucose positron-emission tomography demonstrated glucose hypometabolism of the cerebellum. CONCLUSIONS: Our PSS patient exhibited a progressive course of cerebellar syndrome, as evidenced by cerebellar atrophy on serial brain images.

Evaluation of Balance Control in Patients with Idiopathic Parkinson's Disease Using Tetra-ataxiometric Posturography

OBJECTIVE: To investigate the postural stability of patients with mild to moderate stage of idiopathic parkinson's disease (IPD) using Tetra-ataxiometric posturography (Tetrax) and to evaluate the correlation between balance and activity of daily living (ADL). METHOD: Twenty patients with IPD at mild to moderate stage, twenty young controls and twenty elderly controls were enrolled in this study. In order to evaluate postural stability, we checked stability index, fourier index, weight distribution index, synchronization index, and fall index at eight positions using Tetrax. We also assessed Berg Balance Scale, Postural Instability and Gait Difficulty (PIGD) score of Unified Parkinson Disease Rating Scale (UPDRS). In order to evaluate the correlation between balance and functional performance, the ADL score of UPDRS was checked in the patients with IPD. RESULTS: The patients with IPD showed a significantly higher fall index, weight distribution indices in five positions than the elderly controls. The stability indices of the patients with IPD were higher in all positions, but significantly higher in only one position than the elderly controls. The fallers in the IPD patients had a higher fall index and lower synchronization indices than the non-fallers. The balance and functional performance showed significant correlations in the IPD patients. CONCLUSION: We could identify a high falling risk and uneven weight distribution in the IPD patients and a close correlation between balance and functional performance. Therefore the evaluation of postural stability should be considered from mild stage because postural instability might impair activity of daily living and increase fall risk.

Determining Factors for the Reduction of Dopaminergic Drugs after Bilateral Subthalamic Nucleus Deep Brain Stimulation

BACKGROUND: We aimed to investigate the possible factors determining reduction of dopaminergic drugs in patients who received bilateral subthalamic nucleus (STN) deep brain stimulation (DBS). METHODS: We studied 33 consecutive Parkinson's disease (PD) patients who underwent bilateral STN DBS between March 2002 and April 2006. Patients were assessed at baseline and 6 months and 1 year after surgery. RESULTS: The dose of dopaminergic drugs was significantly decreased by 16.5% (p<0.05) and 14.9% (p<0.05), at post op 6 months and 12 months respectively. The reduction rate of dopaminergic drugs after bilateral STN DBS was positively correlated with the dopaminergic drug dosage at baseline (p<0.05) and was negatively correlated with the scores of "off"-period UPDRS II (p<0.05), III (p<0.001), and total UPDRS (p<0.001) at baseline. The difference of UPDRS scores between "on" and "off" periods on the levodopa challenge test was negatively correlated with the reduction rate of dopaminergic drug dosage after bilateral STN DBS (p<0.001). CONCLUSIONS: It is likely that the main determining factors for the reduction of dopaminergic drug dosages after bilateral STN DBS in advanced PD are the UPDRS III score during "off" periods, difference of the UPDRS III score between "on" and "off" periods on the levodopa challenge test and dose of dopaminergic drugs at baseline.

Trinucleotide Repeats Number in SCA2, SCA3, and SCA17 in Early-Onset Parkinson's Disease

BACKGROUND: Abnormal expansion of trinucleotide repeats in genes causing spinocerebellar ataxias such as SCA2, SCA3, SCA8, or SCA17 was reported in sporadic or familial Parkinson's disease. Genetic factors play an important role especially in early-onset Parkinson's disease (EOPD). To investigate mutations of ATXN2, ATXN3, and TBP as a possible cause in Korean EOPD, we analyzed mutations in these genes. We also investgated the possibility that trinucleotide repeats numbers in these genes contribute to the development of EOPD. METHODS: Mutation analysis of ATXN2, ATXN3, and TBP was done in 153 EOPD defined as age-at-onset before 51. Distribution of CAG repeats numbers were compared between EOPD and age- and sex-matched controls. RESULTS: No patients with EOPD had CAG repeats numbers in ATXN2, ATXN3, and TBP in mutation range. There was no difference in the distribution of CAG repeats between EOPD and controls, although we found a trend that CAG repeats numbers in ATXN3 appear larger in EOPD than in controls. CONCLUSIONS: Mutations of genes causing SCA2, SCA3, or SCA17 may not be a common genetic cause in Korean EOPD.

The Predictable Clinical Factors for the Levodopa Responsiveness of Resting Tremor in Patients with Parkinson's Disease

BACKGROUND: The pathophysiology of resting tremor in Parkinsons disease (PD) remains unclear. Dopaminergic treatment provides variable effects on resting tremor in PD. We aimed to evaluate the predictable clinical factors for the levodopa responsiveness of resting tremor in patients with PD. METHODS: Eighty-five PD patients with prominent resting tremor who visited Asan Medical Center between June 2004 and June 2005 were included. The prominent resting tremor was defined as tremor scoring more than 3 in at least one limb in the Unified Parkinsons Disease Rating Scale (UPDRS). Subjects were divided into the responsive group (RG) or non-responsive group (NRG) according to the responsiveness of resting tremor to dopaminergic treatment. Responsiveness was defined as a minimum 2 points reduction of UPDRS score for the resting tremor after dopaminergic treatment for more than 3 months. RESULTS: Among the 85 patients, there were 35 men and 50 women ages 34-87 years (mean age, 67 years). Thirty-six patients (42.4%) were grouped into RG and 49 (57.6%) into NRG. Mean age of RG was significantly younger than that of NRG. RG showed significantly higher initial UPDRS part III subtotal score (p=0.015) and more severe Hoehn & Yahr stage (p=0.010) than those of NRG. UPDRS subscores for rigidity (p=0.012), bradykinesia (p=0.021) and postural impairment (p=0.018) were correlated with the responsiveness of dopaminergic treatment. CONCLUSIONS: Resting tremor in PD patients more favorably responded to dopaminergic treatment when it presented in combination with bradykinesia and rigidity suggesting dopaminergic role in the genesis of resting tremor in those PD patients.

A Case of Intracranial Hypertension with Hypoglycorrhachia Caused by Bilateral Transverse Sinus Stenoses

Intracranial hypertension is a clinical syndrome of raised intracranial pressure with various etiologies. The possible pathogenic mechanisms of intracranial hypertension are excess CSF production, reduced CSF absorption and increased cerebral venous pressure. CSF glucose in intracranial hypertension is at usually normal levels and hypoglycorrhachia in intracranial hypertension has yet to be reported. We report a 23-year-old woman, who had intracranial hypertension with hypoglycorrhachia caused by a bilateral transverse sinus stenoses.

Delusion and Hallucination in Parkinson's Disease: A Pilot Study of Clinical Features and Pathogenesis

BACKGROUND: Although the clinical and epidemiological characteristics of delusions and visual hallucinations in Parkinson's disease (PD) have been described, the underlying etiology and pathogenesis of those neuropsychiatric manifestations in PD remains a matter of debate. The purpose of this study was to investigate whether or not delusion is in the same spectrum of dopamine related neuropsychiatric manifestations as visual hallucination is in patients with PD. METHODS: We studied the clinical features of 13 PD patients with delusions and 32 with hallucinations. Patients with a history of psychotic symptoms before the onset of PD were excluded. RESULTS: Age at onset of PD was younger in patients with delusions (56.3+/-8.7 years) than in those with hallucinations (63.9+/-9.2 years) (p=0.019). The level of education was higher in patients with delusions than those with hallucinations (p=0.006). Daily levodopa equivalent dosages were higher in patients with delusions (982+/-653.7 mg/day) than those with hallucinations (559+/-311.2 mg/day) (p=0.002). Dopamine agonists were more frequently used in patients with delusions than in those with hallucinations (p=0.020). CONCLUSIONS: Compared with PD patients with hallucinations, those with delusions were associated with longer disease durations, higher levodopa-equivalent daily doses, and use of dopamine agonists. To evaluate the role of dopaminergic dysfunction for the development of delusions and hallucinations in PD patients, further well- designed prospective studies are needed.

One-Year Open-Label Study of Entacapone in Patients with Advanced Parkinson Disease

BACKGROUND AND PURPOSE: A carboxy-O-methyl transferase inhibitor entacapone has been introduced as an adjuvant drug for Parkinson disease (PD) patients. Although clinical trials reported beneficial role of entacapone, a long-term trial over 3 years failed to show significant effect. The goals of this study were to evaluate the clinical benefit and the efficacy of entacapone in an open clinical practice. METHODS: After the completion of a double-blind placebo-controlled entacapone study, 149 patients from 4 centers were included. Antiparkinsonian medications were optimized by the judgment of the neurologists in charge. The clinical global impression (CGI) scale was obtained at 6 months and 1 year after the initiation of entacapone treatment. RESULTS: Of the 149 patients, 117 patients chose to try entacapone in an open-label fashion. Sixty-nine (59%) patients completed the 1-year trial. Twenty-nine patients discontinued entacpaone before 6 months, and 19 between 6 months and 1 year during trial. Twelve patients out of 48 patients discontinued entacapone because of its poor efficacy. The CGI scale was 3.9 (+/-1.5) at the beginning of the trial, 4.3 (+/-1.1) at 6 month, and 3.8 (+/-1.3) at 1 year, respectively. The CGI scale of those who discontinued between 6 month and 1 year was 3.4 (+/-1.7), which was worse, but insignificantly, than that of the continuer. CONCLUSIONS: The dropout at 1 year of our study was very high at 41%. Even though entacapone is indicated for advanced PD patients with motor fluctuation, the fluctuators commonly have dyskinesia and mental symptoms, which can become more troublesome with entacapone. In the patients with advanced PD, the clinical efficacy and side effects should be carefully considered in a long-term use of entacapone.

The clinical characteristics of lower back pain in Parkinson's Disease

BACKGROUND: Lower back pain (LBP) in Parkinson's disease (PD) is common but frequently overlooked, due to the tendencies of focusing on the management of the motor symptoms and signs by most neurologists. Uncontrolled LBP may impact on the activities of daily living of the PD patients. However, study on the LBP in PD has been rarely reported. METHODS: Sixty three PD patients with LBP were included, between October 2004 and April 2005. We investigated the clinical characteristics of LBP and response to the management prospectively. RESULTS: Eleven male and 52 female patients were included and the mean age was 64.4 years. On lumbar MRI and/or X-ray, degenerative spondylosis was detected in 22 patients, bulging discs in 30, and fractures in 9. Fourteen patients reported improvement of LBP after levodopa therapy. Young age at onset and abnormally flexed posture correlated with the responsiveness of LBP to levodopa therapy (p<0.05, respectively). These responders were more frequently experiencing motor fluctuation and dyskinesia than the non-responders (p<0.05, respectively). CONCLUSIONS: LBP in PD has diverse etiologies and clinical features. Some PD patients with LBP show response to levodopa therapy. We suggest that the prudent evaluation and proper management of LBP are important to achieve the better activities of daily living in PD patients.

Cerebral Air Embolism Following Pneumopyelography

No abstract available.

The Caregivers' Burden in Parkinson's disease

BACKGROUND: Caring for patients with Parkinson's disease (PD) is a burden to caregivers since currently available treatment modalities for PD depend on symptomatic treatments. However, there have only been a few studies regarding the caregivers of PD patients. The authors investigated the burden, depression, and anxiety of the caregivers of PD patients. METHODS: Fifty-three main caregivers of PD patients were included. The burden, anxiety, and depression of the caregivers were evaluated using the Zarit Burden Inventory (ZBI), the Spielberger State-trait Anxiety Inventory, and the Beck Depression Inventory. RESULTS: Twenty-one male and 32 female patients had a mean age 61.4 years and a mean disease duration of 7.5 years. The caregivers included 29 men and 24 women with a mean age of 55.8 years. The sex of the patients (male) and caregivers (female), the relation to the patient (daughter-in-law), and frequency of hospital visits were all significantly associated with the caregiver's burden. Among the disease characteristics, the duration, severity of PD, presence of motor fluctuation, and levodopa-associated confusion/hallucination affected the caregivers' burden significantly. The level of depression and anxiety was positively correlated with the level of burden. On a stepwise regression analysis, the significant predictors of the caregivers' burden were ADL, UPDRS IV, and state anxiety in order of strength. CONCLUSIONS: The caregivers' burden in PD was affected by various demographic and disease characteristics, which also correlated with the level of depression and anxiety. We suggest that comprehensive treatment strategies for PD should be developed for the caregivers as well as the patients.

Factors Related to Quality of Life in Patients with Parkinson's Disease

BACKGROUND: The goal of therapeutic interventions for Parkinson's Disease (PD) is to improve the symptoms and mitigate the effect on the Quality of Life (QOL) in the individual patient. The purpose of this study was to investigate QOL and related factors in PD patients in Korea. METHODS: Between January 1, 2004 and July 15, 2004, eighty-one PD patients were included. The patients were assessed using Parkinson's Disease Quality of Life (PDQL), Modified Beck Depression Inventory (BDI), and K-MMSE. RESULTS: Twenty-five male and 56 female patients were included in the study. The mean age was 60.7 years and the mean disease duration was 7 years. The male patients (p=0.07) and young age at onset (p=0.07) showed borderline correlation with PDQL score. Medical cost for PD showed significant correlation with PDQL score (p<0.001). The patients working in the daytime or spending the daytime with their spouse showed significantly higher PDQL score than those who did not (p=0.01). Among the disease characteristics, the disease duration, levodopa dosage, UPDRS score, ADL, Hoehn and Yahr stage, the presence of motor fluctuation and dyskinesia, showed that depression had a strong correlation with the PDQL score (p<0.001). On the stepwise regression analysis, the most important factor was presence of depression, disease duration and the UPDRS total score in order of strength. CONCLUSIONS: The QOL in PD patients was strongly associated with depression, disease duration, and the severity of PD. We suggest that the assessment and proper management of depression as well as other PD symptoms is necessary to improve QOL of the PD patients.

Penicillamine Induced Breast Enlargement in a Patient with Wilson's Disease

No abstract available.

Clinical Outcomes of Simultaneous Bilateral Pallidotomy in Advanced Parkinson's Disease

OBJECTIVE: Pallidotomy is known to improve the symptoms of idiopathic Parkinson, s disease (PD), motor fluctuations and dyskinesia related to levodopa therapy. Previous studies reported significantly higher complication rates associated with bilateral pallidotomy than unilateral pallidotomy. The authors assess the safety and clinical outcomes of bilateral pallidotomy for advanced PD. METHODS: Simultaneous bilateral pallidotomy was performed in eight patients with advanced PD between January 1, 2001 and December 31, 2001. All patients underwent lesion making in posteroventral site of internal globus pallidus. The target was localized using macroelectrode stimulation and MRI guided stereotactic technique. The lesions were made by radiofrequency currents. RESULTS: Among eight cases, seven had severe disabling dyskinesias. Compared with baseline scores, the values of dyskinesia after surgery was significantly decreased (P<0.05) for up to 1 year. The mean score changed from 4.4+/-2.1 to 0.3+/-0.5. According to Unified Parkinson's Disease Rating Scale (UPDRS), the mean motor score in off period, which was 38.3+/-13.8 was significantly decreased for 6 months (P<0.05). The ADL (on/off) scores and motor "on" scores of UPDRS were unchanged or deteriorated to 12 months after surgery. There was no apparent adverse effect after surgery in all patients. Only transient mild dysphagia happened in one patient. CONCLUSION: Simultaneous bilateral pallidotomy in advanced PD appears to be effective and safe, particularly in reducing the dyskinesia; in our experience, the side effects are not as high as reported by other groups.

Leaked CSF on Spine MRI in a Patient with Syndrome of CSF Hypovolemia

A 34-year-old woman was admitted because of severe orthostatic headache. On CSF examination, the opening pressure was below 30 mmH2O. A spine MRI showed a large collection of extradural fluid indicating that CSF leaked into the extradural space. The headache was resolved by an epidural blood patch. We present a patient with a syndrome of CSF hypovolemia, whose spine MRI showed a large amount of leaked CSF in the epidural space of the spine.

A Case of Systemic Lupus Erythematosus Presenting with Chorea: Evidence for Striatal Hypermetabolism

Chorea is an uncommon clinical manifestation of Sytemic lupus erythematosus (SLE). Its pathogenic mechanism has not been clearly clarified. We report a 54-year-old woman with SLE who presented with generalized chorea as an initial manifestation. Fluorine-18-fluorodeoxyglucose (FDG) PET revealed increased metabolism in the bilateral putamen. Intravenous and oral administration of steroid markedly improved chorea. Hypermetabolism of the bilateral putamen diminished on follow-up FDG-PET after the disappearance of chorea. This study suggests that chorea in SLE is associated with striatal hypermetabolism.

The DYT1 Gene Mutation in Primary Torsion Dystonia without Familial Background

BACKGROUND: The DYT1 dystonia is primary torsion dystonia (PTD) caused by a GAG deletion in DYT1 gene on chromosome 9 and transmits as autosomal dominant trait. It usually begins as limb-onset dystonia in childhood and tends to spread to other sites and has been reported as the most common cause of early onset PTD in Ashkenazi Jews. However, the frequency of DYT1 mutation in Korean patients with sporadic PTD has not been reported. METHODS: We examined dystonia patients who visited the Neurologic Clinic of Asan Medical Center between Jan 2001 and March 2002. The sporadic PTD patients of them were screened by genotyping with their peripheral blood samples. RESULTS: The 66 patients with sporadic PTD were recruited and two of them showed DYT1 mutation on Chromosome 9. One patient had segmental dystonia with cervical onset; the other had generalized dystonia with left leg onset. None of the patients with focal dystonia showed a DYT1 mutation. CONCLUSIONS: The DYT1 dystonia comprise a small portion of PTD without familial history in Korea. The DYT1 gene test should be considered in the early-onset or spreading type dystonia despite the absence of familial background in primary dystonia patient.

A Case of Dementia related to AIDS

We report a patient with AIDS, who initially presented with progressive cognitive impairment and gait disturbance. A T2-weighted brain MRI demonstrated multiple hyperintensities in bilateral periventricular white matter, thalami, brainstem, cerebellum, and left temporo-occipital white matter. Enzyme-linked immunosorbent assay and Western blot for HIV were positive. The CD4+ T-cell count was 158 cells/mm3 and the plasma HIV RNA level was 65, 000 copies/mL. Highly active antiretroviral therapy was administered.

A Case of Progressive Supranuclear Palsy with Schizophrenic Symptoms / 신경정신의학

We have experienced a rare case of progressive supranuclear palsy with hallucination and delusion. The common manifestations of progressive supranuclear palsy are gait disturbance, mental change and sign of vertical ophthamoplegia but no efficacious therapy has been known. Neuropsychiatric symptom clusters include cognitive impairment, affective and behavioral changes, sleep disturbance, and psychotic symptoms. Though schizophrenia-like psychosis has been reported but this is certainly rare. Addressing a case of progressive supranuclear palsy, in whom parkisonian symptoms appeared with concurrent psychotic symptoms, we emphasize accurate diagnosis.

Ropinirole as an Adjunct to Levodopa in the Treatment of Parkinson's Disease

BACKGROUND: Ropinirole is a non-ergoline D2 agonist which has a highly selective affinity to D2 receptor. The aim of this study was to evaluate the efficacy and safety of ropinirole in the treatment of Parkinson's disease (PD). METHODS: Seventy-six cases 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) over a 16-week period. All subjects were not optimally controlled on levodopa due to motor fluctuations. The response rate was defined as the percentage of patients who had at least 20% reduction of levodopa doses. The clinical status was also assessed using the Unified Parkinson's Disease Rating Scale (UPDRS), Clinical Global Impression (CGI), and reduction of off durations. RESULTS: The end-point analysis, on an intention-to-treat basis, revealed significantly higher response rate in the ropinirole group compared with the bromocriptine group (odds ratio 2.995, 95% C.I. (1.157, 7.751)). A statistically significant improvement in CGI was also observed in the ropinirole group (p=0.046). The mean off duration was significantly reduced in the ropinirole group (p=0.0001). Other parameters using the UPDRS motor score or off duration did not show significant differences between the two groups. The overall incidence of adverse effects was not significantly different between the two groups. The most common side effects were dizziness, dyskinesia, and nausea/vomiting. No subjects were withdrawn from the study due to side effects. CONCLUSION: Ropinirole is a safe and well-tolerated drug and provides superior overall efficacy compared with bromocriptine as an adjunct to levodopa. (J Korean Neurol Assoc 19(2):102~109, 2001)