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The diagnosis of spinocerebellar ataxia (SCA) currently depends upon genetic testing. Although genetic testing for SCA is highly specific, clinical parameters for the differentiation of SCAs are still insufficient. We aimed to assess the vestibulo-ocular reflex (VOR) parameters of various SCA subtypes to determine whether they have substantial value in differential diagnosis. We consecutively enrolled 33 genetically confirmed SCA patients (SCA2 = 8, SCA3 = 6, SCA6 = 10, SCA7 = 9). Normative data were obtained from 36 age- and gender-matched healthy controls. Quantitative indicators of VOR were measured using video head impulse test (HIT) and combined ocular motor dysfunctions were investigated using video-oculography. Compared with the control group, the VOR gains in SCA2 were relatively spared, but were markedly decreased for all six canals in SCA3. The VOR gains for the posterior canals (PCs) were significantly decreased in SCA6, and for both vertical canals were decreased in SCA7. The VOR gains for the horizontal canals in SCA3 were negatively correlated with disease severity (R = -0.900, p = 0.037). Abnormal catch-up saccades were common in SCA3 and SCA6, rare in SCA7 and absent in SCA2. Spontaneous, headshaking-induced, and positional nystagmus were only documented in SCA6. SCA3 and SCA6 commonly showed horizontal gaze-evoked nystagmus, but SCA2 and SCA7 had characteristic saccadic slowing without gaze-evoked nystagmus. VOR impairments are common in SCAs, but their patterns vary depending on subtype. In addition to ocular motor characteristics, distinctive VOR performance for each subtype using video HIT may aid the differential diagnosis of the SCA genotypes. [ABSTRACT FROM AUTHOR]

Parkinson's disease (PD) patients are at risk for developing bone health problems, and freezing of gait (FOG) in PD is associated with a high risk of falling and fracture. This study aimed to determine the association between FOG and bone mineral density (BMD) in patients with PD. We included 148 PD patients. FOG was assessed using the FOG Questionnaire (FOG-Q), and BMD was measured by dual-energy X-ray absorptiometry. Of 148 PD patients, 102 (68.9%) had FOG. PD patients with FOG were older and had longer disease duration, higher levodopa equivalent dose, higher modified Hoehn and Yahr stage, higher Unified PD Rating Scale motor score, higher FOG-Q score, higher total Non-Motor Symptom Scale score, and lower BMD scores in the femoral neck area than those without FOG. Pearson correlation analysis revealed that age, sex, body mass index, and age at onset were significantly correlated with areal BMDs in all areas. FOG-Q scores correlated negatively with areal BMDs in the total hip area and femoral neck, but not with areal BMD in the lumbar spine. Multivariate regression analysis showed that FOG-Q score was significantly correlated with areal BMD in the femoral neck, but not with areal BMDs in the lumbar spine or total hip. FOG in PD patients correlates significantly with BMD in the femoral neck area. Therefore, PD patients with FOG should be screened for osteoporosis. [ABSTRACT FROM AUTHOR]

Background: Isolated central positional vertigo (CPV) due to cerebellar infarction is often difficult to differentiate from benign paroxysmal positional vertigo (BPPV). Here, we aimed to evaluate whether vascular risk factors and serum vitamin D level can differentiate between positional vertigo types. Methods: A total of 78 consecutive patients were consecutively enrolled from January 2017. All CPV patients had a National Institutes of Health Stroke Scale score of 0 and cerebellar infarctions confirmed by brain MR imaging. Vascular risk factors and serum 25-hydroxyvitamin D levels were compared between the two groups of patients. Results: The proportion of men was higher in the CPV than in the BPPV group (p = 0.004). Atrial fibrillation was common in the CPV group on univariate analysis (p = 0.046). However, there were no independent differentiating factors between the two groups. The proportion of patients according to the number of risk factors was significantly different between the two groups (linear by linear association test, p = 0.02). The mean serum 25-hydroxyvitamin D level did not differ. Also, the proportions of vitamin D insufficiency and deficiency did not differ significantly between the two groups. Conclusions: Increased number of vascular risk factors including male sex suggested more CPV than BPPV. However, the serum vitamin D level was below the normal range in both groups. Our results demonstrate that serum vitamin D level has little value in the differential diagnosis of positional vertigo. Efforts to identify differentiating factors are warranted, and accumulating evidences including our research may lead to a diagnostic algorithm for isolated positional vertigo. [ABSTRACT FROM AUTHOR]

Patients with Parkinson's disease (PD) are liable to experience impairment in their activities of daily living (ADL), which include ambulating, eating, dressing, bathing, and personal hygiene. The aim of this study is to assess which clinical characteristics contribute significantly to instrumental ADL (IADL) in PD patients without dementia. We included 106 PD patients in our study, and each patient's motor and non-motor status and basic and instrumental ADL were assessed using the appropriate scales. Of the 106 PD patients, 31 (29.2%) had abnormal Korean IADL (K-IADL) scores. These patients were older and had higher scores in terms of the modified Hoehn and Yahr (mHY) staging scale, Unified Parkinson's Disease Rating Scale (UPDRS) parts II and III, UPDRS part IV motor fluctuation, Beck Depression Inventory (BDI), and total Non-Motor Symptoms assessment scale for PD (NMSS), as well as lower scores in the Mini-Mental State Examination (MMSE). Pearson's correlation analysis showed significant associations between the scores of K-IADL and each of the following characteristics of the patients: age, mHY stage, UPDRS parts II and III, UPDRS part IV motor fluctuation, BDI, total NMSS, and MMSE. Multivariate linear regression analysis showed that the significant clinical characteristics associated with the K-IADL scores were determined to be the UPDRS part II, MMSE, and BDI scores. The results of our study revealed that the cognitive, depression, and motor symptoms were the significant predictors of IADL in PD patients without dementia. [ABSTRACT FROM AUTHOR]

Background: Freezing of gait (FOG) is a common and debilitating problem in patients with Parkinson's disease (PD). The aim of this study was to estimate the prevalence of FOG, and to identify factors that independently contribute to FOG in patients with PD.Method: We included 157 PD patients. FOG was assessed using the FOG Questionnaire (FOG-Q). Patients with or without FOG were defined as item 3 in the FOG-Q.Results: One hundred eleven (70.7%) out of 157 PD patients presented with FOG. Patients with FOG were older, had long disease duration, were taking higher doses of dopaminergic agents, and had higher motor and non-motor scores than those without FOG. Multivariate linear regression analysis showed that high modified Hoehn and Yahr (mHY) stage, Unified PD Rating Scale (UPDRS) part II score, and non-motor symptom assessment scale for PD (NMSS) total score were significant predictors of a high FOG-Q score. Patients with FOG had significantly higher scores for cardiovascular, gastrointestinal tract, urinary, and miscellaneous NMSS domains than those without FOG.Conclusions: FOG in PD was associated with higher mHY stage, UPDRS part II score, and total NMSS score. Therefore, clinicians should consider non-motor, motor features and activities of daily living states for the proper management of FOG. [ABSTRACT FROM AUTHOR]

Magnetic resonance (MR) measurements of brainstem structures have been reported to be useful in differentiating patients with progressive supranuclear palsy (PSP) from those with Parkinson's disease (PD). The aim of this study was to determine whether quantitative measurements of brainstem structures on MR images can help differentiate vascular parkinsonism (VaP) from degenerative parkinsonism (PD and PSP). Areas of the midbrain and pons, and widths of the superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) were measured in 62 patients with PD, 25 patients with PSP (11 probable and 14 possible), and 24 patients with VaP on T 1-weighted MR images. The midbrain-to-pons area ratio (M/P ratio), MCP-to-SCP width ratio (MCP/SCP ratio), and MR parkinsonism index (MRPI; P/M × MCP/SCP) were calculated. The midbrain area and M/P ratio of patients with VaP (104 and 0.22 mm2, respectively) were smaller than those in patients with PD (121 and 0.24 mm2, respectively) and larger than those in patients with PSP (90 and 0.19 mm2, respectively). The MRPI was significantly larger in patients with PSP (13.6) in comparison with those with PD (10.1) and VaP (10.7). However, the MRPI of patients with VaP was not significantly different from patients with PD. Our study showed that MRPI was useful in differentiating PSP from VaP or PD. Thus, MR imaging measurements of brainstem structures may help differentiate patients with VaP from those with PD and PSP. [ABSTRACT FROM AUTHOR]

It remains controversial whether APOE E4 polymorphism is related to cognitive function in general population. We aimed to evaluate an association between the APOE E4 genotype and cognitive function, and whether this association may differ by age. Cognitive function was assessed using the Korean version of modified Mini-Mental State Examination (K-mMMSE) in 10,371 Koreans aged 45-74 years in Namwon City. According to the APOE E4 status, all participants were classified as non-carriers, heterozygotes, or homozygotes. Multiple linear and logistic regression models were used to evaluate the association between APOE genotypes and cognition. The frequency of APOE genotypes in the study population was 0.4, 10.1, 1.1, 72.9, 14.7 and 0.8 % for E2 E2, E2 E3, E2 E4, E3 E3, E3 E4, and E4 E4, respectively. Compared to the APOE E4 non-carriers, the heterozygotes and homozygotes showed 1.3 and 7.3 % lower K-mMMSE scores at 65-74 years and 0.8 and 4.6 % higher scores at 45-55 years, respectively. Educational attainment modified the effect of APOE E4 on cognitive function in the 45-54 age group ( p for interaction =0.003), showing that the E4 carriers with no-formal education showed significantly higher cognitive function than those with formal education. The present study demonstrates that the effect of APOE E4 on cognitive function depends on age and education. [ABSTRACT FROM AUTHOR]

Non-contrast enhanced computed tomography (NCCT) is usually performed to estimate bleeding complications immediately after procedures. However, hyperdense areas on NCCT have not yet been understood; different interpretations have been reported in the literature. It remains unclear whether NCCT performed immediately after intra-arterial revascularization (IAR) could be useful for predicting hemorrhagic transformation (HT) or clinical outcomes. Therefore, we investigated the diagnostic values of hyperdense areas on NCCT images obtained immediately after IAR. This was a retrospective study of acute ischemic stroke patients who underwent IAR between October 2007 and December 2010. NCCT scans were routinely obtained immediately after IAR and additional follow-up imaging protocols included diffusion weighted imaging (DWI)/gradient echo imaging (GRE) 24 h after IAR. HT was assessed by means of GRE obtained 24 h after IAR. Hounsfield Unit (HU) of the hyperdensity was measured in the manually drawn regions of interest. A total of 68 patients were analyzed in this study. Twenty-nine patients (42.6%) developed HT on follow-up images. Thirty-eight patients had hyperdense areas on NCCT immediately after IAR. Hyperdensity on NCCT performed immediately after IAR revealed 23 (60.5%) of the 38 patients with six false negative areas. NCCT performed immediately after IAR showed a sensitivity of 79.3%, a specificity of 61.5%, a positive predictive value of 60.5% and a negative predictive value of 80% for HT. The HU value was a predictor of HT without statistical significance (area under curve of 0.629; 95% CI: 0.49-0.76; p = 0.068). In addition, an HU of >90 poorly predicted HT with a low sensitivity (23%) and a high specificity (94%). In conclusion, our results showed that although hyperdensity on NCCT images obtained immediately after IAR had a moderate predictive value for HT, there were limitations to the prediction of subsequent parenchymal hematoma and symptomatic intracranial hemorrhage, with a low specificity and a low positive predictive value. [ABSTRACT FROM AUTHOR]

Although high-density lipoprotein (HDL) infusion therapy now seems to be a promising therapy for the treatment of several diseases, including atherosclerosis, myocardial infarction, sepsis, and diabetes, there is a real need for more information on the production, quality, and safety of reconstituted HDL (rHDL). In this context, we described a production-scale preparation of rHDL and examined the reproducibility of the process and product, and its shelf stability over a 24-month period. Apolipoprotein A-I (apoA-I) was isolated from precipitates IV (by plasma fractionation) using polyethylene glycol, ethanol, pH precipitation, and ion-exchange chromatography. This manufacturing process included 3 virus-elimination steps consisting of ethanol precipitation, pasteurization, and nanofiltration. HDLs were then reconstituted through cholate dialysis using soybean phosphatidylcholine. The product released data from 3 separate rHDL production were adequate to fulfill the required specification and admitted range. Following lyophilization, the products were stable in the presence of sucrose for at least 24 months. Redissolved rHDLs were disc-shaped and had sizes ranging from 10 to 20 nm. Studies on structure-function relationships provided evidence that these rHDLs could be used as potential therapeutic agents for acute coronary syndrome and inflammatory diseases. [ABSTRACT FROM AUTHOR]

Kikuchi's disease (KD) is a histiocytic necrotizing lymphadenopathy. KD is an enigmatic, benign, and self-limiting disease characterized by regional lymphadenopathy. It usually affects young Asian women and an involvement of central nervous system is rare in KD and only few cases of aseptic meningitis associated with KD have been reported. Aseptic meningitis in KD might have very close clinical and laboratory findings with other etiologies of meningitis such as viral and tuberculous meningitis and it should be differentiated from those meningitis. Here, we report a case of KD manifested as meningitis with elevated cerebrospinal fluid and serum adenosine deaminase that is common and suggestive feature of tuberculous meningitis. A close observation of the different clinical course and excisional biopsy of regional lymphadenopathy might be critical points for distinguishing meningitis associated with KD from tuberculous meningitis. [ABSTRACT FROM AUTHOR]

The article provides information on the association of cerebral microbleeds (CMBs) with neurological symptoms causing intracerebral hemorrhage (ICH). It states that deposition of amyloid in cerebral blood vessels increases the susceptibility of patient to cerebral infraction, CMB and ICH. MRI study reveals that the CMBs are associated with neurological symptoms which increase during the acute period of ischemic stroke.

We investigated the association between retinal changes measured using optical coherence tomography (OCT) and diverse clinical grading scales in patients with Parkinson's disease (PD). Seventy-four eyes of 74 patients with de novo PD and 53 eyes of age-matched control subjects were included. The thickness of the peripapillary retinal nerve fiber layer (pRNFL) and macular ganglion cell-inner plexiform layer (mGCIPL) were measured. We analyzed the correlations between the clinical PD grading scales and OCT parameters, and between the OCT parameters and volumetric data in the cerebral cortical and subcortical structures. The area under the receiver operating characteristic curve (AUC) was calculated for diagnosing cognitive impairment in patients with PD. Statistically significant reductions in the thickness of average, temporal, and inferior pRNFL and overall mGCIPL were observed in patients with PD. The Montreal Cognitive Assessment score was significantly associated with mGCIPL thinning. The AUC of the mGCIPL parameters for diagnosing cognitive impairment in patients with PD ranged from 0.651 to 0.760. Moreover, thinning of the mGCIPL was significantly associated with the volumetric parameters of associated brain structures. Our findings highlight the clinical implications of OCT measurements as a potential biomarker for early detection of cognitive impairment in patients with PD. [ABSTRACT FROM AUTHOR]

We investigated the relationship between the mean blood pressure (BP) at 24–72 h and the clinical outcomes after acute ischemic stroke (AIS) in patients treated with reperfusion therapy. The primary outcome was measured using the modified Rankin Scale (mRS) at 3 months after AIS, and was based on the mean systolic BP at 24–72 h post-AIS. Favorable outcome was defined as mRS scores of 0–2. A total of 1,540 patients treated with reperfusion therapy were enrolled in the study. Favorable outcomes occurred more frequently in patients with BP ≤ 130/80 mmHg, and the risks of symptomatic intracranial hemorrhage and early neurological deterioration were lower in this optimal BP group. Multivariable analysis showed a significant association between mean BP ≤ 130/80 mmHg at 24–72 h and favorable outcomes at 3 months after AIS (odds ratio 2.95, 95% confidence interval 2.32–3.77, p < 0.001). Prespecified subgroup analyses showed that BP ≤ 130/80 mmHg had a more significant impact on clinical outcome in patients with recanalization than in those without recanalization. These data indicate that a mean BP of ≤ 130/80 mmHg at 24–72 h post-AIS is independently associated with favorable outcomes in patients treated with reperfusion therapy, particularly in those with recanalization. [ABSTRACT FROM AUTHOR]

Background: Freezing of gait (FOG) is a common and debilitating problem in patients with Parkinson's disease (PD). The aim of this study was to estimate the prevalence of FOG, and to identify factors that independently contribute to FOG in patients with PD.Method: We included 157 PD patients. FOG was assessed using the FOG Questionnaire (FOG-Q). Patients with or without FOG were defined as item 3 in the FOG-Q.Results: One hundred eleven (70.7%) out of 157 PD patients presented with FOG. Patients with FOG were older, had long disease duration, were taking higher doses of dopaminergic agents, and had higher motor and non-motor scores than those without FOG. Multivariate linear regression analysis showed that high modified Hoehn and Yahr (mHY) stage, Unified PD Rating Scale (UPDRS) part II score, and non-motor symptom assessment scale for PD (NMSS) total score were significant predictors of a high FOG-Q score. Patients with FOG had significantly higher scores for cardiovascular, gastrointestinal tract, urinary, and miscellaneous NMSS domains than those without FOG.Conclusions: FOG in PD was associated with higher mHY stage, UPDRS part II score, and total NMSS score. Therefore, clinicians should consider non-motor, motor features and activities of daily living states for the proper management of FOG. [ABSTRACT FROM AUTHOR]

This multicentre, prospective, single-arm study evaluated safinamide as add-on therapy to levodopa in Korean patients with Parkinson’s disease (PD) with motor fluctuations with ≥ 1.5 h of “off” time daily, who took levodopa ≥ 3 times/day (n = 199). Baseline levodopa and dopamine agonist doses were maintained without escalation during the 18-week treatment period. Participants received safinamide 50 mg/day for 2 weeks and 100 mg/day thereafter. PD diaries and questionnaires (Parkinson’s Disease Questionnaire, PDQ-39; Movement Disorder Society-Sponsored Revision of the Unified Parkinson’s Disease Rating Scale, MDS–UPDRS part 3 and part 4; King’s Parkinson’s Disease Pain Scale, KPPS; Mini-Mental State Examination, MMSE) were assessed at baseline and at week 18. Treatment-emergent adverse events (TEAEs) were recorded. Mean disease duration was 6.6 years, and mean levodopa equivalent daily dose was 721.1 mg/day. At week 18, significant improvements from baseline were seen for the co-primary endpoints, mean daily “off” time (− 1.3 ± 2.4 h, p < 0.001) and quality of life (QoL) based on PDQ-39 summary index (− 2.7 ± 10.3, p < 0.001), Moreover, significant improvements were seen in motor symptoms and motor complications (MDS-UPDRS part 3 and 4), daily “on” time without dyskinesia (all p < 0.001) and pain (KPPS; p = 0.013). TEAEs occurred in 40.2% of patients, with most being mild in severity. In conclusion, safinamide at a dosage of 100 mg/day significantly improved motor symptoms, QoL, and pain, and demonstrated a favourable safety profile without levodopa dosage escalation during the 18-week treatment period in Korean patients with PD.Trial registration number and date: NCT05312632, First Posted: April 5, 2022 [ABSTRACT FROM AUTHOR]