Multimodal Imaging Findings of Cerebral Amyloid Angiopathy Related Inflammation With Unusual Clinical Manifestation: A Case Report

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare encephalopathy characterized by the coexistence of a perivascular inflammatory reaction in patients with cerebral amyloid angiopathy. CAA-RI diagnosis is challenging as its final diagnosis requires invasive procedures such as autopsy or brain biopsy. Therefore, multimodal imaging approaches with clinical considerations are essential for the probable diagnosis of CAA-RI. In particular, in the case of CAA-RI presented with uncommon clinical symptoms, the need for imaging in diagnosis is further highlighted by difficulties of clinical approaches. Herein, we report a case of CAA-RI with unusual clinical manifestation diagnosed using multimodal imaging including magnetic resonance imaging (MRI) and amyloid positron emission tomography-computed tomography (PET-CT). Multimodal imaging approaches using adequate MRI sequences and PET-CT scans could facilitate the diagnosis of CAA-RI without requiring invasive pathological confirmation.

Cortical Thickness and Brain Glucose Metabolism in Healthy Aging

Background@#and PurposeWe aimed to determine the effect of demographic factors on cortical thickness and brain glucose metabolism in healthy aging subjects. @*Methods@#The following tests were performed on 71 subjects with normal cognition: neurological examination, 3-tesla magnetic resonance imaging, 18F-fluorodeoxyglucose positron-emission tomography, and neuropsychological tests. Cortical thickness and brain metabolism were measured using vertex- and voxelwise analyses, respectively. General linear models (GLMs) were used to determine the effects of age, sex, and education on cortical thickness and brain glucose metabolism. The effects of mean lobar cortical thickness and mean lobar metabolism on neuropsychological test scores were evaluated using GLMs after controlling for age, sex, and education. The intracranial volume (ICV) was further included as a predictor or covariate for the cortical thickness analyses. @*Results@#Age was negatively correlated with the mean cortical thickness in all lobes (frontal and parietal lobes, p=0.001; temporal and occipital lobes, p<0.001) and with the mean temporal metabolism (p=0.005). Education was not associated with cortical thickness or brain metabolism in any lobe. Male subjects had a lower mean parietal metabolism than did female subjects (p<0.001), while their mean cortical thicknesses were comparable. ICV was positively correlated with mean cortical thickness in the frontal (p=0.016), temporal (p=0.009), and occipital (p=0.007) lobes. The mean lobar cortical thickness was not associated with cognition scores, while the mean temporal metabolism was positively correlated with verbal memory test scores. @*Conclusions@#Age and sex affect cortical thickness and brain glucose metabolism in different ways. Demographic factors must therefore be considered in analyses of cortical thickness and brain metabolism.

Association between Olfactory Deficit and Motor and Cognitive Function in Parkinson’s Disease

Objective@#To investigate whether baseline olfactory dysfunction in Parkinson’s disease (PD) patients is associated with baseline and longitudinal motor and cognitive function. @*Methods@#We recruited 228 drug-naïve PD patients who were followed for a mean of 6 years. Patients underwent the Cross-Cultural Smell Identification Test (CCSIT), a neuropsychological test, and N-(3-[18F]fluoropropyl)-2β-carbomethoxy-3β-(4-iodophenyl) nortropane positron emission tomography within 6 months of the baseline evaluation. Olfactory dysfunction was categorized as normosmia (CCSIT score ≥ 9), hyposmia (CCSIT score 5–8), and anosmia (CCSIT score ≤ 4). During the follow-up period, we investigated changes in the levodopa-equivalent dose (LED) and the occurrence of wearing-off, levodopa-induced dyskinesia, and dementia. @*Results@#Among the PD patients, 80.7% were hyposmic at the time of diagnosis, and 26.1% were anosmic. Baseline olfactory dysfunction was not associated with either initial parkinsonian motor symptoms or with the longitudinal LED increment and motor complications. Meanwhile, the anosmic group had lower baseline scores on the Korea version of the Boston Naming Test and Stroop color reading test than the normosmic and hyposmic groups. The anosmic group exhibited a higher rate of conversion to dementia than the normosmic [adjusted hazard ratio (HR) 3.99, 95% confidence interval (CI) 1.08–14.72] and hyposmic (adjusted HR 2.48, 95% CI 1.15–5.32) PD groups, regardless of baseline motor deficits and cognitive status. @*Conclusion@#Baseline olfactory dysfunction was not associated with motor deficits and complications, but it was associated with cognitive dysfunction and prognosis, suggesting that severe olfactory impairment may reflect early cortical involvement, probably in the frontotemporal region, and rapid spreading of Lewy body pathology.

The Influence of Body Mass Index at Diagnosis on Cognitive Decline in Parkinson's Disease

BACKGROUND AND PURPOSE: Associations between alterations in body mass index (BMI) and cognitive function have been reported in Parkinson's disease (PD). We investigated whether the BMI at a PD diagnosis is associated with cognitive decline and the future development of dementia. METHODS: We recruited 70 patients with de novo PD who underwent neuropsychological testing every 3 years and were followed up for more than 6 years. We classified patients into the following three groups based on their BMI at the diagnosis: under-/normal weight (n=21), overweight (n=22), and obese (n=27). We evaluated differences in the rate of cognitive decline over time among the groups using linear mixed models and the conversion rate to dementia using survival analysis. RESULTS: The obese patients with PD showed a slower deterioration of global cognitive function as well as language and memory functions than did the under-/normal-weight group during the 6-year follow-up. The three BMI groups showed different rates of conversion to dementia (log-rank test: p=0.026). The combined overweight and obese group showed a lower risk of developing dementia compared with the under-/normal-weight group (hazard ratio= 0.36, 95% CI=0.12–0.82, p=0.046). CONCLUSIONS: We have demonstrated that a higher-than-normal BMI at the time of a PD diagnosis has a protective effect against the deterioration of cognitive function and the conversion to dementia.

Heterogeneous Patterns of Striatal Dopamine Loss in Patients with Young- versus Old-Onset Parkinson's Disease: Impact on Clinical Features

OBJECTIVE: Ample evidence has suggested that age at onset of Parkinson's disease (PD) is associated with heterogeneous clinical features in individuals. We hypothesized that this may be attributed to different patterns of nigrostriatal dopamine loss. METHODS: A total of 205 consecutive patients with de novo PD who underwent 18F-FP-CIT PET scans (mean follow-up duration, 6.31 years) were divided into three tertile groups according to their age at onset of parkinsonian motor symptoms. Striatal dopamine transporter (DAT) availability was compared between the old- (n = 73) and young-onset (n = 66) groups. In addition, the risk of developing freezing of gait (FOG) and longitudinal requirements for dopaminergic medications were examined. RESULTS: The old-onset PD group (mean age at onset, 72.66 years) exhibited more severe parkinsonian motor signs than the young-onset group (52.58 years), despite comparable DAT availability in the posterior putamen; moreover, the old-onset group exhibited more severely decreased DAT availability in the caudate than the young-onset group. A Cox regression model revealed that the old-onset PD group had a higher risk for developing FOG than the young-onset group [hazard ratio 2.523, 95% confidence interval (1.239–5.140)]. The old-onset group required higher doses of dopaminergic medications for symptom control than the young-onset group over time. CONCLUSION: The present study demonstrated that the old-onset PD group exhibited more severe dopamine loss in the caudate and were more likely to develop gait freezing, suggesting that age at onset may be one of the major determinants of the pattern of striatal dopamine depletion and progression of gait disturbance in PD.

A Novel Heterozygous ANO3 Mutation with Basal Ganglia Dysfunction in a Patient with Adult-Onset Isolated Segmental Dystonia

No abstract available.

Anti-NMDA Receptor Encephalitis Which Has Shown Clinical Symptoms of Schizophrenia

Encephalitis associated with antibodies to the N-methyl-D-aspartate (NMDA) receptor has variable clinical manifestations and treatment responses. Anti-NMDAR encephalitis is often associated with ovarian teratoma, but some cases without tumor have been reported. Here, we describe a patient who has shown psychiatric symptoms, memory impairment and been diagnosed as schizophrenia for one year and had anti-NMDA receptor antibody. The patient showed atypical clinical course compared to previous cases with anti-NMDA receptor encephalitis.

Repeated Thrombolytic Therapy in Patients with Recurrent Acute Ischemic Stroke / 대한뇌졸중학회지

BACKGROUND AND PURPOSE: Widespread use of thrombolytic treatments, along with improved chances of survival after an initial ischemic stroke, increases the possibility of repeated thrombolysis. There are few reports, however, regarding repeated thrombolysis in patients who have suffered acute ischemic stroke. We explored the number and outcome of patients with repeated thrombolytic therapy in the era of multimodal thrombolytic treatments. METHODS: We investigated patients with acute ischemic stroke who had received thrombolytic treatments for a period of 10 years. Number of thrombolysis was determined in each patient. Recanalization was defined as Thrombolysis in Cerebral Infarction grading > or =2a. Symptomatic hemorrhagic transformation was defined as any increase in the National Institutes of Health Stroke Scale score that could be attributed to intracerebral hemorrhage. A good outcome was defined as a modified Rankin scale score < or =2. RESULTS: Of the 437 patients who received thrombolytic treatments, only 7 underwent repeated thrombolysis (1.6%). The median age at the time of repeated thrombolytic therapy was 71 years old; 4 of the patients were female. All patients had 1 or more potential sources of cardiac embolism. Recanalization was achieved in all patients, in both the first and the second thrombolysis. No symptomatic intracranial hemorrhage occurred after repeated thrombolytic treatments. Five patients (71.4%) showed good outcomes at 3 months. CONCLUSIONS: Repeated thrombolysis for recurrent acute ischemic stroke appears to be safe and feasible. Among patients who experience recurrent acute ischemic stroke, thrombolytic therapy could be considered even if the patient has had previous thrombolytic treatments.