Background and Purpose Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the pyramidal motor system. However, recent studies have suggested that degeneration of the extramotor system plays a role in the disability experienced by patients with ALS. We investigated the local shape changes and mean volumes of the subcortical nuclei in sporadic ALS patients with preserved cognition. Methods The participants comprised 32 patients with ALS and 43 age-and sex-matched healthy controls. Three-dimensional T1-weighted structural images were acquired. Surfacebased vertex analysis was performed with fully automated segmentation of both amygdalae, hippocampi, caudate nuclei, nuclei accumbens, putamina, pallida, and thalami, and the brainstem. The scalar distances from the mean surfaces of the individual subcortical nuclei were compared between groups, and correlations of the local shape distances with initial Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALS-FRS-R) scores and the delta FRS-R and with the disease duration were analyzed. Results ALS patients showed regional shape contractions on the lateral surfaces of both pallida, the lateroposterior surface of the right putamen, and the anterior basal surface of the right accumbens. Delta FRS-R scores were negatively correlated with local shape distances in the right hippocampus and the putamina. However, the initial ALS-FRS-R score and disease duration were not correlated with local shape distances. Conclusions Subcortical gray-matter structures are involved in the neurodegenerative process of ALS before cognitive impairment becomes evident.

Stereopsis or depth perception is an awareness of the distances of objects from the observer, and binocular disparity is a necessary component of recognizing objects through stereopsis. In the past studies, patients with neurodegenerative disease (Alzheimer dementia, AD; Parkinson’s disease IPD) have problems of stereopsis but they did not have actual stimulation of stereopsis. Therefore in this study, we used a 3-dimensional (3D) movie on 3D television (TV) for actual stereopsis stimulation. We propose research through analyzing differences between the three groups (AD, IPD, and Controls), and identified relations between the results from the Titmus Stereo Fly Test, and the 3D TV test. The study also looked into factors that affect the 3D TV test. Before allowing the patients to watch TV, we examined Titmus stereo Fly Test and cognitive test. We used the 3D version of a movie, of 17 minutes 1 second duration, and carried out a questionnaire about stereopsis. The scores of the stereopsis questionnaire were decreased in AD patients, compared with in IPD and controls, although they did not have any difference of Titmus Stereo Fly Test scores. In IPD patients, cognitive function (Montreal cognitive assessment, MoCA) scores were correlated with the scores of the stereopsis questionnaire. We could conclude that Titmus fly test could not distinguish between the three groups and cognitive dysfunction contributes to actual stereopsis perception in IPD patients. Therefore the 3D TV test of AD and IPD patients was more effective than Titmus fly test.

Background: Recently, several studies suggested potential involvements of α-synuclein in Alzheimer's disease (AD) pathophysiology. Higher concentrations of α-synuclein were reported in cerebrospinal fluid (CSF) of AD patients with a positive correlation towards CSF tau, indicating its possible role in AD. We analyzed the CSF biomarkers to verify whether α-synuclein could be an additional supported biomarker in AD diagnosis. Methods: In this cross-sectional study, CSF samples of 71 early-onset AD, 34 late-onset AD, 11 mild cognitive impairment, 17 subjective cognitive decline, 45 Parkinson's disease, and 32 healthy control (HC) were collected. CSF amyloid-β1-42 (A), total tau (N), and phosphorylated tau181 (T) were measured by commercial ELISA kits, and inhouse ELISA kit was developed to quantify α-synuclein. The cognitive assessments and amyloid-PET imaging were also performed.

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Cerebellar ataxia (hereinafter referred to as CA) designate a group of neurodegenerative disorders. CA is distinguished into a group of hereditary and non-hereditary disorders. CA shows clinically progressive features and accompanies various neurological abnormalities. However, there are very few studies and case reports in Korean patients. To estimate the prevalence rate and current status of the CA patients in Korea, we used data from the Health Insurance Review and Assessment Service (HIRAS) and from the National Health Insurance Corporation. To evaluate the functional status of CA patient in Korea, we conducted a simple random sampling among the 500 members of Korea Ataxia Society registered on its homepage. We evaluated the functional status and degree of disturbance to their everyday life with modified Rankin scales and Barthel ADL index. Using the data from HIRAS, we could estimate the prevalence rate of CA patients in Korea as 8.29 patients/100,000 persons. The prevalence rate of hereditary and non-hereditary cerebellar ataxia was 4.99 patients/100,000 persons and 3.30 patients/100,000 persons, respectively. Data on rare intractable diseases reported by the Korean Centers for Disease Control and Prevention in 2006 suggested that the number of CA patients who have visited medical institutes was almost doubled for the 2-year period. The medical expense and hospital stay also increased 4.5- and 3-fold, respectively. After severity evaluation with modified Rankin scales and Barthel ADL index, we found that most CA patients in Korea have ataxia-related difficulties in their everyday life.