Clinical Characteristics of Non-Amnestic Mild Cognitive Impairment: A Single-Center Experience

BACKGROUND: To evaluate the clinical characteristics of patients with non-amnestic mild cognitive impairment (naMCI) in a memory disorder clinic at a single center. METHODS: A retrospective study was conducted involving 312 patients with naMCI from May 2011 to July 2018. Brain magnetic resonance imaging and detailed neuropsychological tests were performed in all patients. We used the proposed criteria for naMCI to classify the patients into single- and multiple-domain groups. We compared the baseline clinical characteristics, neuroimaging findings, and the rate of progression to dementia between these two groups. RESULTS: The 312 patients comprised 210 in the single-domain group (67.3%) and 102 in the multiple-domain group (32.7%). The mean age was significantly higher in the multiple-domain group than in the single-domain group. The years of education, mean Mini Mental State Examination score, and mean Clinical Dementia Rating Scale Sum of Boxes score were significantly lower in the multiple-domain group than in the single-domain group. The Z-scores of neuropsychological tests in most cognitive domains were significantly lower in the multiple-domain group than in the single-domain group. Compared to the single-domain group, the multiple-domain group showed more-severe medial temporal atrophy and contained a higher proportion of patients with moderate white-matter hyperintensities. Thirteen (8.4%) patients with naMCI progressed to dementia, most of who were diagnosed with Alzheimer's disease. CONCLUSIONS: We present a single-center experience of clinical characteristics in patients with naMCI. Close observation of the clinical profiles of patients with naMCI may help identify individuals at the greatest risk of dementia.

Nonmotor and Dopamine Transporter Change in REM Sleep Behavior Disorder by Olfactory Impairment

OBJECTIVE: It is unclear whether the decline in dopamine transporters (DAT) differs among idiopathic rapid eye movement sleep behavior disorder (iRBD) patients with different levels of olfactory impairment. This study aimed to characterize DAT changes in relation to nonmotor features in iRBD patients by olfactory loss. METHODS: This prospective cohort study consisted of three age-matched groups: 30 polysomnography-confirmed iRBD patients, 30 drug-naïve Parkinson's disease patients, and 19 healthy controls without olfactory impairment. The iRBD group was divided into two groups based on olfactory testing results. Participants were evaluated for reported prodromal markers and then underwent 18F-FP-CIT positron emission tomography and 3T MRI. Tracer uptakes were analyzed in the caudate, anterior and posterior putamen, substantia nigra, and raphe nuclei. RESULTS: Olfactory impairment was defined in 38.5% of iRBD patients. Mild parkinsonian signs and cognitive functions were not different between the two iRBD subgroups; however, additional prodromal features, constipation, and urinary and sexual dysfunctions were found in iRBD patients with olfactory impairment but not in those without. Tracer uptake showed significant group differences in all brain regions, except the raphe nuclei. The iRBD patients with olfactory impairment had uptake reductions in the anterior and posterior putamen, caudate, and substantia nigra (p < 0.016 in all, adjusted for age), which ranged from 0.6 to 0.8 of age-normative values. In contrast, those without olfactory impairment had insignificant changes in all regions ranging above 0.8. CONCLUSION: There was a clear distinction in DAT loss and nonmotor profiles by olfactory status in iRBD.

Amantadine and the Risk of Dyskinesia in Patients with Early Parkinson's Disease: An Open-Label, Pragmatic Trial

OBJECTIVE: We examined whether amantadine can prevent the development of dyskinesia. METHODS: Patients with drug-naïve Parkinson's disease (PD), younger than 70 years of age and in the early stage of PD (Hoehn and Yahr scale < 3), were recruited from April 2011 to December 2014. The exclusion criteria included the previous use of antiparkinsonian medication, the presence of dyskinesia, significant psychological disorders, and previous history of a hypersensitivity reaction. Patients were consecutively assigned to one of 3 treatment groups in an open label fashion: Group A-1, amantadine first and then levodopa when needed; Group A-2, amantadine first, dopamine agonist when needed, and then levodopa; and Group B, dopamine agonist first and then levodopa when needed. The primary endpoint was the development of dyskinesia, which was analyzed by the Kaplan-Meier survival rate. RESULTS: A total of 80 patients were enrolled: Group A-1 (n = 27), Group A-2 (n = 27), and Group B (n = 26). Twenty-four patients were excluded from the analysis due to the following: withdrawal of amantadine or dopamine agonist (n = 9), alternative diagnosis (n = 2), withdrawal of consent (n = 1), and breach in the protocol (n = 12). After exclusion, 5 of the 56 (8.93%) patients developed dyskinesia. Patients in Group A-1 and A-2 tended to develop dyskinesia less often than those in Group B (cumulative survival rates of 0.933, 0.929, and 0.700 for A-1, A-2, and B, respectively; p = 0.453). CONCLUSION: Amantadine as an initial treatment may decrease the incidence of dyskinesia in patients with drug-naïve PD.

Pregnancy and Delivery in a Generalized Dystonia Patient Treated with Internal Globus Pallidal Deep Brain Stimulation: a Case Report

Internal globus pallidus (GPi) deep brain stimulation (DBS) has been widely accepted as an effective treatment modality of medically refractory dystonia. However, there have been few studies regarding the safety issue of pregnancy and childbirth related with DBS. This report describes a female patient who was pregnant and delivered a baby after GPi DBS surgery. A 33-year-old female patient with acquired generalized dystonia underwent bilateral GPi DBS implantation. She obtained considerable improvement in both movement and disability after DBS implantation. Four years later, she was pregnant and the obstetricians consulted us about the safety of the delivery. At 38-weeks into pregnancy, a scheduled caesarian section was carried out under general anesthesia. After induction using thiopental and succinylcholine, intubation was done quickly, followed by DBS turn off. For hemostasis, only bipolar electrocautery was used. Before awakening from the anesthesia, DBS was turned on as the same parameters previously adjusted. After delivery, she could feed her baby by herself, because the dystonia of left upper extremity and hand was improved. Until now, she has been showing continual improvement and being good at housework, carrying for children, with no trouble in daily life. This observation indicates that the patients who underwent DBS could safely be pregnant and deliver a baby.

Malignant Neuroleptic Syndrome following Deep Brain Stimulation Surgery of Globus Pallidus Pars Internus in Cerebral Palsy / 대한중환자의학회지

Neuroleptic malignant syndrome (NMS) is a rare but potentially lethal outcome caused by sudden discontinuation or dose reduction of dopaminergic agents. We report an extremely rare case of NMS after deep brain stimulation (DBS) surgery in a cerebral palsy (CP) patient without the withdrawal of dopaminergic agents. A 19-year-old girl with CP was admitted for DBS due to medically refractory dystonia and rigidity. Dopaminergic agents were not stopped preoperatively. DBS was performed uneventfully under monitored anesthesia. Dopaminergic medication was continued during the postoperative period. She manifested spasticity and muscle rigidity, and was high fever resistant to anti-pyretic drugs at 2 h postoperative. At postoperative 20 h, she suffered cardiac arrest and expired, despite vigorous cardiopulmonary resuscitation. NMS should be considered for hyperthermia and severe spasticity in CP patients after DBS surgery, irrespective of continued dopaminergic medication.

Malignant Neuroleptic Syndrome following Deep Brain Stimulation Surgery of Globus Pallidus Pars Internus in Cerebral Palsy / 대한구급학회지

Neuroleptic malignant syndrome (NMS) is a rare but potentially lethal outcome caused by sudden discontinuation or dose reduction of dopaminergic agents. We report an extremely rare case of NMS after deep brain stimulation (DBS) surgery in a cerebral palsy (CP) patient without the withdrawal of dopaminergic agents. A 19-year-old girl with CP was admitted for DBS due to medically refractory dystonia and rigidity. Dopaminergic agents were not stopped preoperatively. DBS was performed uneventfully under monitored anesthesia. Dopaminergic medication was continued during the postoperative period. She manifested spasticity and muscle rigidity, and was high fever resistant to anti-pyretic drugs at 2 h postoperative. At postoperative 20 h, she suffered cardiac arrest and expired, despite vigorous cardiopulmonary resuscitation. NMS should be considered for hyperthermia and severe spasticity in CP patients after DBS surgery, irrespective of continued dopaminergic medication.

Corticobasal Syndrome Initially Presenting with Apraxia of Speech

Apraxia of speech is a disorder of speech programming and planning. We report a 64-year-old woman presenting with progressive apraxia of speech that evolved into right-side-dominant akinesia-rigidity syndrome over a 7-year period. Her motor symptoms were highly consistent with corticobasal syndrome, and neuroimaging revealed atrophy and hypometabolism in the left premotor and supplementary motor areas, which are relevant to apraxia of speech. This case illustrates that apraxia of speech may be an initial manifestation of corticobasal syndrome.

Survival of Korean Huntington's Disease Patients

OBJECTIVE: The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported. METHODS: Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study. RESULTS: The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset. CONCLUSION: This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

Steroid Unresponsive Neuromyelitis Optica Improved With Plasmapheresis

Plasmapheresis is an emerging treatment for intravenous steroid-resistant neuromyelitis optica (NMO). We present the case of a 16-year-old girl who suffered from intravenous steroid-resistant NMO and whose neurological status improved markedly after treatment with plasmapheresis. This is the first report on the effectiveness of plasmapheresis in NMO in Korea. kwoo@plaza.snu.ac.kr

Long-term Prognosis of Patients With Acute Intracranial Large Artery Occlusion and its Determining Factors

BACKGROUND: Acute intracranial large-artery occlusions (AILAOs) are detected frequently and cause severe neurological disabilities. Most studies in this field do not focus on the natural history of AILAOs, but on the individual prognosis for each intervention. The aim of this study was to elucidate the clinical profiles and outcomes of AILAOs. METHODS: A consecutive series of patients hospitalized between January 2004 and October 2007 due to AILAO within 24 hours from onset were recruited. Based on a prospective stroke registry, their clinical profiles were collected. AILAO was defined as an intracranial internal carotid artery (ICA), middle cerebral artery (MCA), or basilar artery (BA) occlusion that could be confirmed by angiography, with relevant lesions on diffusion-weighted imaging (DWI). The modified Rankin Scale (MRS) score at 3 months and the recanalization rate within 14 days were recorded as outcomes. RESULTS: Among 1,047 patients with acute ischemic stroke who were examined within 24 hours of onset, 189 [18.1%; 101 men, 88 women; age 68.6+/-13.0 years (mean+/-SD); median National Institutes of Health Stroke Scale (NIHSS) score=11]. Occlusion sites were MCA M1, MCA M2, distal ICA, and BA in 99 (52.4%), 50 (26.5%), 20 (10.6%), and 20 (10.6%) of cases,respectively. Embolic sources were found in 103 (54.5%) cases. MRS scores were available for 184 (97.4%) of the patients, of which 78 had a favorable outcome (MRS: 0.2). Follow-up angiography was performed in 122 (64.6%) cases, with recanalization observed in 88 (72.1%) of these. Thrombolysis, occlusion site, presence of an embolic source, and initial glucose level were predictors of early recanalization (p<0.001). Recanalization status and initial NIHSS score were strongly correlated with a favorable outcome (p<0.005). CONCLUSIONS: This is the first report of the clinical profiles, outcomes, and their predictors in a cohort of Korean patients with AILAO.