Ji Young Yun

Survival of Korean patients with multiple system atrophy

We conducted a retrospective medical record review to determine the survival of 455 Korean multiple system atrophy (MSA) patients and examined the effect of clinical factors that could possibly influence survival. The patients comprised 222 men and 233 women.

Musculoskeletal problems in Parkinson's disease: Neglected issues

BACKGROUND To identify the prevalence and clinical features of musculoskeletal problems in patients with Parkinson disease (PD) compared to controls. METHODS 400 PD patients and 138 age- and sex-matched controls were interviewed by physicians about their musculoskeletal problems. RESULTS The prevalence of musculoskeletal problems was significantly higher in the PD group than in the control group (66.3% vs. 45.7%, P < 0.001). Commonly involved body sites were the low back, knee, and shoulder in that order. The low back was more frequently involved in the PD group than in the control group (44.3% vs. 24.6%, P < 0.001), and the shoulder tended to be more involved in the PD group than in the control group (15.0% vs. 8.7%, P = 0.061). However, the knee was similarly involved in both group (12.3% vs. 18.0%, P = 0.121). Among the past diagnoses associated with musculoskeletal problems, frozen shoulder, low back pain, osteoporosis and fracture were more common in the PD group than in the control group (P < 0.05). Older age, female, and a higher score on the Unified Parkinsons Disease Rating Scale I & II were associated with musculoskeletal problems in the PD group. Only 26.8% of the PD patients and 52.5% of the controls with musculoskeletal problems answered that their musculoskeletal problems were recovering. Furthermore, musculoskeletal problems in the PD group tended to receive less treatment than that of the control group (P = 0.052). CONCLUSION Musculoskeletal problems were more common in the PD group than in the controls. Furthermore, despite PD patients having a higher prevalence, they did not receive adequate treatment.

Clinical and imaging characteristics of dementia in multiple system atrophy

BACKGROUND Recent reports show that dementia occurs in 5-26% of multiple system atrophy (MSA) patients. However, the structural or pathological correlates of dementia in MSA are unclear yet. METHODS Of 152 patients with MSA, 59 fulfilled the criteria of probable MSA and 9 (15%) had dementia. Six of those patients and 9 without dementia, in addition to 10 controls, were included. All subjects underwent clinical evaluation including UMSARS, neuropsychological examinations, 3T-MRI, and Pittsburgh Compound B (PIB) PET imaging. The cortical thickness was assessed using surface-based morphometry. RESULTS Age and disease duration were similar between MSA with dementia and without dementia, while motor disability was more severe in MSA with dementia. In neuropsychological tests, attention, visuospatial function, and language function were impaired in MSA with dementia. Mean PIB binding was similar among the three groups. Cortical thickness was reduced in precuneus/cuneus, uncus, and posterior cingulate in MSA with dementia compared to the controls, and in parahippocampal and lingual cortices compared to MSA without dementia. CONCLUSIONS Dementia was found in 15% of the probable MSA patients, which was similar to those reported in previous studies. It appears that amyloid pathology has limited role in dementia in MSA, although some patients had increased cortical amyloid burden. Cortical thinning in MSA-D was observed in areas where cortical thinning was reported in Alzheimer disease or Parkinson disease dementia, but its pathological relevance is unclear. The neuropathological processes leading to the development of dementia in MSA appears to be multifactorial and heterogenous.

Loss of Nigral Hyperintensity on 3 Tesla MRI of Parkinsonism: Comparison With 123I‐FP‐CIT SPECT

The aim of this study was to investigate whether 3 Tesla susceptibility‐weighted imaging can detect the alteration of substantia nigra hyperintensity in Parkinsons disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP) and to assess the concordance between the loss of nigral hyperintensity on 3 Tesla susceptibility‐weighted imaging and the nigrostriatal dopaminergic degeneration indicated by 123I‐2β‐carbomethoxy‐3β‐(4‐iodophenyl)‐N‐(3‐fluoropropyl)‐nortropane single photon emission computerized tomography.

Impulse control and related behaviors after bilateral subthalamic stimulation in patients with Parkinson's disease.

The effect of subthalamic nucleus deep brain stimulation (STN DBS) on impulse control and related behaviors (ICRB) in patients with Parkinsons disease (PD) is conflicting. We evaluated ICRB before and after bilateral STN DBS in patients with PD. A total of 89 patients with PD treated with bilateral DBS of STN underwent retrospective assessment of ICRB before and after DBS. Of the 89 patients studied, 20 patients (22.5%) had ICRB in the preoperative period. In 13 of those 20 patients (65%), preoperative ICRB improved, including resolution in six patients. Nine patients developed de novo ICRB after DBS, thus 23 patients (25.8%) had ICRB in the postoperative period. There was no demographic difference between the patients with or without ICRB in the preoperative state. In the postoperative state, the patients with ICRB had higher levodopa equivalent daily dose (LEDD) levels and lower Mini-Mental State Examination (MMSE) scores than the patients without ICRB. However, postoperative worsening or de novo ICRB did not correlate with LEDD levels or MMSE scores. Severity of ICRB worsened more after DBS in older patients. Patients with worsened or de novo ICRB after surgery had a greater decrease in Beck Depression Index scores after surgery compared with patients whose ICRB improved. In conclusion, ICRB may resolve or improve, or new ICRB may appear, after bilateral STN DBS. The difference in risk factors for preoperative vs. postoperative ICRB suggests that the pathogenesis of those conditions is different, at least in part.

Meningitis by Toxocara canis after Ingestion of Raw Ostrich Liver

Recently reports on toxocariasis are increasing by serodiagnosis in Korea. A previously healthy 17-yr-old boy complained of headache, fever, dyspnea, and anorexia. He showed symptoms and signs of eosinophilic meningitis with involvement of the lungs and liver. Specific IgG antibody to Toxocara canis larval antigen was positive in serum and cerebrospinal fluid by ELISA. He took raw ostrich liver with his parents 4 weeks before the symptom onset. His parents were seropositive for T. canis antigen but had no symptoms or signs suggesting toxocariasis. This is the first report of toxocariasis in a family due to ingestion of raw ostrich liver in Korea.

Dysport and Botox at a ratio of 2.5:1 units in cervical dystonia: A double‐blind, randomized study

We aimed to compare Dysport (abobotulinumtoxinA, Ipsen Biopharm, Slough, UK) and Botox (onabotulinumtoxinA, Allergan, Irvine, CA, USA) at a 2.5:1 ratio in the treatment of cervical dystonia (CD). A Dysport/Botox ratio of lower than 3:1 was suggested as a more appropriate conversion ratio, considering its higher efficacy and more frequent incidence of adverse effects not only in the treatment of CD but also in other focal movement disorders. A randomized, double‐blind, multicenter, non‐inferiority, two‐period crossover study was done in CD, with a duration of at least 18 months. Patients were randomly assigned to treatment for the first period with Dysport or Botox, and they were followed up for 16 weeks after the injection. After a 4‐week washout period, they were switched to the other formulation and then followed up for 16 weeks. The primary outcome was the changes in the Tsui scale between the baseline value and that at 1 month after each injection. A total of 103 patients were enrolled, and 94 completed the study. Mean changes in the Tsui scale between baseline and 4 weeks after each injection tended to favor Botox; however, this was not statistically significant (4.0 ± 3.9 points for the Dysport treatment vs. 4.8 ± 4.1 points for Botox; 95% confidence interval, −0.1‐1.7; P = 0.091). The mean change of the Toronto western spasmodic torticollis rating scale score, the proportion of improvement in clinical global impression and patient global impression, and the incidences of adverse events were not significantly different between the two treatments. With regard to safety and efficacy, Dysport was not inferior to Botox in patients with CD at a conversion factor of 2.5:1. [clinicaltrial.gov: NCT00950664]

Intravenous Amantadine for Freezing of Gait Resistant to Dopaminergic Therapy: A Randomized, Double-Blind, Placebo-Controlled, Cross-Over Clinical Trial

Background Freezing of gait (FOG) is one of the most disabling symptoms in Parkinsonism. Open-label studies have suggested that intravenous (IV) amantadine is effective against FOG resistant to dopaminergic therapy in Parkinsons disease (PD). We evaluated the efficacy of IV amantadine on FOG resistant to dopaminergic therapy. Methodology/Principal Findings This was a randomized, double-blind, placebo-controlled, cross-over study on IV amantadine. The placebo (normal saline) and amantadine (400 mg/day) were injected for 2 days with a 52-hour washout period. The instruments for the outcome measures were the Freezing of Gait Questionnaire (FOGQ), Unified Parkinsons disease rating Scale (UPDRS), and the duration of the 4×10 m walking test. The placebo arm was compared to the amantadine arm. Ten patients were enrolled but two patients withdrew, one from each arm. The FOGQ and UPDRS scores and the duration of the 4×10 m walking test improved in both arms compared to the baseline (P<0.05 in all). However, there were no differences in these values between the amantadine arm and placebo arm (P = 0.368, P = 0.583, P = 0.206, respectively). Follow-up measures 2weeks after discharge in an open-label study showed the beneficial effects of an amantadine tablet on FOG (FOGQ, P = 0.018; UPDRS, P = 0.012 respectively). Conclusions/Significance This double blind, placebo-controlled study did not show the efficacy of IV amantadine on FOG when compared with the placebo. This study provides Class II evidence due to small sample size for the lack of benefit of IV amantadine on FOG resistant to dopaminergic therapy Trial Registration Clinicaltrials.gov NCT01313819

The clinical impact of precise electrode positioning in STN DBS on three-year outcomes

Few studies have analyzed the clinical impact of subthalamic nucleus (STN) deep brain stimulation (DBS) as a function of the positioning of the inserted electrode. We investigated retrospectively the three-year outcomes in Parkinsons disease (PD) patients following bilateral STN DBS in terms of the electrode positions. Forty-one advanced PD patients were followed up for over three years following bilateral STN DBS. Patients were evaluated with the Unified Parkinsons Disease Rating Scale (UPDRS), Hoehn and Yahr staging, Schwab and England Activities of Daily Living (ADL), and the Short Form-36 Health Survey (SF-36) before surgery and one, two, and three years after surgery. The patients were divided into two groups according to the electrode position based on the fused preoperative MRI and postoperative CT images: group I included patients who had both electrodes in the STN (n=30) while group II included patients who had one of the electrodes in the STN (n=11). The UPDRS, the Hoehn & Yahr staging, the Schwab and England ADL, and the SF-36 scores showed significant improvements with decreased l-dopa equivalent daily doses (LEDDs) in both groups as well as in the group as a whole for up to three years following bilateral STN DBS. However, the off-medication UPDRS total and motor (part III) scores significantly deteriorated with increased LEDDs for patients in group II three years after STN DBS compared to that of the group I patients. We conclude that more accurate electrode positioning in the STN leads to better long-term outcomes in advanced PD patients following DBS.

Young-onset multiple system atrophy

BACKGROUND Multiple system atrophy (MSA) rarely begins before the age of 40 and detailed descriptions of young-onset MSA are lacking. METHODS Among 455 patients included in our MSA cohort, four developed disease before the age of 40. We reviewed the medical records of these patients. RESULTS Case 1 and 2 presented with cerebellar symptoms. Case 1 had clinical features and a course typical of MSA. Case 2 had a rapid course and died 3 years after onset. Case 3 and Case 4 presented with levodopa-responsive parkinsonism. Both developed motor fluctuations and peak-dose limb dyskinesias. Subthalamic deep brain stimulation (DBS) resulted in some improvements in motor symptoms, but they became totally dependent within a few years. DISCUSSION Young-onset MSA is rare but does exist. Young-onset MSA with predominant parkinsonism may closely resemble Parkinson disease at onset and is likely to develop motor complications. Attention should be given to the possibility of young-onset MSA in selecting DBS candidates.