Photographic Assessment of Intermittent Exotropia Using a Translucent Cover.

PURPOSE: To evaluate the validity of the photographic assessment with a translucent cover that is clinically available for the evaluation of intermittent exotropia. METHODS: Photographs of 270 patients who cooperated for the prism and alternate cover test (PACT) were reviewed. Full-face images were obtained with a digital camera while a translucent cover was placed in front of either eye. The change in distance from the medial canthus to the nasal limbus with occlusion was measured and the photographic angle was estimated by three independent ophthalmologists based on representative photographs. These two measurements were correlated with the angle measured using the PACT, and clinical features related to the discrepancy between the photographic angle and the angle measured with the PACT were determined. RESULTS: Patients with intermittent exotropia of 27.0 ± 6.1 prism diopters (PD) showed a 4.5 ± 3.3 mm exodrift and an estimated angle of 29.0 ± 4.3 PD on the photographs with occlusion. The exodrift distance and photographic angle were positively correlated with the angle of PACT (r = 0.256, P < .001 and r = 0.546, P < .001, respectively). Of the 47 patients with a discrepancy of greater than 8 PD, 38 patients (80.9%) were regarded as having a photographic angle that was larger than the angle of PACT, which was more common in older patients, in those with a small distance angle, and when taking off the spectacles. CONCLUSIONS: Photographs with a translucent cover can reveal the latent components of intermittent exotropia. However, the photographic angle might differ from the real angle, particularly in older patients with a small angle and with the spectacles off. [J Pediatr Ophthalmol Strabismus. 2021;58(5):331-338.].

Sternocleidomastoid muscle asymmetry in unilateral congenital superior oblique palsy.

BACKGROUND: To determine whether there is an asymmetry in bilateral sternocleidomastoid muscle (SCM) thickness in patients with unilateral congenital superior oblique palsy (SOP) and its association with surgical results. METHODS: The medical records of 186 patients with head tilt secondary to unilateral SOP, who were evaluated for the status of the SCM with neck ultrasound or magnetic resonance imaging, were reviewed. The SCM asymmetry index was calculated as a bilateral difference in the maximal muscle thickness divided by each tilted-side SCM thickness. The presence of SCM asymmetry, defined as an index of >10%, and its relationship to residual torticollis ≥5° after SOP surgery were assessed. RESULTS: Of 186 patients with a median age of 1.2 years, SCM asymmetry was present in 102 (54.8%) patients (6.8 ± 1.9 mm for the SOP side vs. 6.6 ± 2.1 mm for the tilted side). The SCM asymmetry did not differ according to age, amount of head tilt or hypertropia. In the patients with SCM asymmetry, more patients (87.3%) underwent physiotherapy than those without asymmetry (61.9%) (P = 0.021). In 99 patients who underwent surgery for SOP, the resolution of torticollis was not significantly different between patients with and without SCM asymmetry (87.2% vs. 76.9%, P = 0.184). CONCLUSIONS: Nearly half of the patients with congenital SOP had SCM thickness asymmetry that was already determined at a young age. However, the surgical results did not differ significantly with respect to SCM asymmetry when physiotherapy was combined. Thus, SOP surgery can be considered despite preoperative SCM asymmetry.

Binocular discrepancy in lateral rectus muscle attachment in intermittent exotropia with eye dominance.

PURPOSE: To determine whether there is asymmetry in the lateral rectus (LR) muscle attachment between both eyes in patients with intermittent exotropia (IXT) with a non-dominant eye, but without amblyopia or anisometropia. METHODS: In total, 109 patients who underwent bilateral lateral rectus recession for IXT were included, 81 with and 28 without eye dominance. The limbus-insertion distance and tendon width of the LR muscle were measured intraoperatively using callipers. The insertion-equator distance (presumed arc of contact), area of contact (arc of contact × tendon width), and torque value (radius of globe × arc of contact) of the LR muscle were calculated based on intraoperative measurements and axial length measured using a partial interferometer. Parameters regarding LR muscle attachment were compared between fellow eyes and between groups. RESULTS: Mean measurements in all parameters related to LR muscle attachments other than tendon width were not different between the two eyes or between groups. The mean tendon width of the non-dominant eye was 9.2 ± 0.7 mm, narrower than the 9.4 ± 0.5 mm width in either eye of patients without dominance (p = 0.020). However, there was no difference in all parameters in 21 pairs of patients after matching. The proportion of patients who showed binocular discrepancies in attachment measurements beyond that attributable to potential measuring errors did not differ between the two groups. CONCLUSIONS: Structural parameters related to LR muscle attachments did not differ based on eye dominance, suggesting that the anatomic structure of LR muscle attachments is not responsible for eye dominance in IXT.

TWELVE-MONTH EFFICACY OF INTRAVITREAL BEVACIZUMAB INJECTION FOR CHRONIC, ATYPICAL, OR RECURRENT CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To evaluate intravitreal bevacizumab (IVB) injection efficacy for the treatment of chronic, atypical, or recurrent central serous chorioretinopathy (CSC). METHODS: Clinical data from 77 eyes of 71 patients with chronic, atypical, or recurrent CSC treated with IVB were retrospectively analyzed. After initial 6-weekly IVB administration until no subretinal fluid remained at the fovea, additional as-needed IVBs were administered, based on optical coherence tomography findings. Best-corrected visual acuity and central retinal thickness (CRT) were analyzed at baseline and 3, 6, 9, and 12 months after initial IVB. RESULTS: The significant improvement of baseline logarithm of minimum angle of resolution best-corrected visual acuity and CRT at 3 months (both P < 0.001) was maintained throughout the 12-month follow-up period. Best-corrected visual acuity improved significantly in patients with chronic and recurrent CSC, at all time points (all P < 0.05), but not in patients with atypical CSC. The CRT reduction was significant in all subgroups during the follow-up period (all P < 0.05). Definite leakage on initial fluorescein angiography correlated with improved reduction in CRT (P = 0.039). CONCLUSION: As-needed optical coherence tomography-based IVB was effective for reducing CRT in patients with chronic, atypical, or recurrent CSC, and for vision improvement in chronic and recurrent CSC over the 1-year follow-up period.

The relationship between alexithymia and headache impact: the role of somatization and pain catastrophizing.

PURPOSE: The present study compared psychological factors (i.e., alexithymia, somatization, pain catastrophizing (PC), anxiety, and depression) and QOL for headache patients and headache-free individuals, and examined whether somatization and PC mediate the relationship between alexithymia and headache impact in headache patients. METHODS: Study participants consisted of 123 headache patients from an outpatient clinic at a university hospital and 124 headache-free individuals in Daegu, Korea. The survey employed the somatization and anxiety subscales of the Symptom Checklist-90-revised, the Patient Health Questionnaire-9, Pain Catastrophizing Scale, Toronto Alexithymia Scale, Short-Form Health survey-8 (SF-8), and the Headache Impact Test-6. RESULTS: Headache patients showed a higher level of all psychological factors and lower level of two summary scores (physical and mental health) as well as the seven dimensions of the SF-8 compared with headache-free individuals. Examination employing the SPSS Process macro found that the direct effect of alexithymia on headache impact was not significant after controlling for somatization and PC. The total indirect effects of alexithymia on headache impact were significant without anxiety and depression as covariates with the significant indirect effects of alexithymia on headache impact via somatization or via PC as well as via somatization and PC. However, after controlling for anxiety and depression, PC was the only significant pathway through which alexithymia was related to headache impact. CONCLUSIONS: Headache patients may benefit from interventions aiming at improving psychological factors in order to improve the functioning and QOL of headache patients.

Spectrum of cognitive impairment in Korean ALS patients without known genetic mutations.

BACKGROUND: Cognitive impairment is associated with a negative prognosis in amyotrophic lateral sclerosis (ALS), as well as with clinical specificity. We investigate neuropsychological function in ALS patients without known genetic mutations in a Korean tertiary clinic. METHODS: Three hundred and eighteen patients were enrolled in a prospective longitudinal cohort from September 2008 to February 2012. At the time of diagnosis of sporadic ALS, we carried out genetic and comprehensive neuropsychological tests on all patients, and collected demographic and clinical characteristics. Six cognitive domains, namely executive function, attention, language, calculation, visuospatial function and memory were evaluated. ANOVA and t-tests were used to assess differences in clinical characteristics and neuropsychological parameters between sporadic ALS patients. The Kaplan-Meier method and Cox proportional hazard model were used for survival analysis. RESULTS: One hundred and sixty-six patients were categorized into five subtypes: normal cognition (ALS pure), cognitive impairment (ALSci), behavioral impairment (ALSbi), frontotemporal dementia (ALS-FTD), and other types of dementia. Seventy patients (70/166, 42.2%) were cognitively or behaviorally impaired. Among the impaired patients, eight (8/166, 4.8%) had FTD-type dementia and one (1/166, 0.6%) was Alzheimer's disease-type. The ALS patients with cognitive impairment (ALSci) and with FTD (ALS-FTD) were more severely impaired in executive function, attention, language and memory than the cognitively intact ALS patients (ALS pure). In a survival analysis, ALSci (ß = 1.925, p = 0.025) and ALS-FTD groups (ß = 4.150, p = 0.019) tended to have shorter survival than the ALS pure group. CONCLUSIONS: About half of ALS patients without known genetic variation have cognitive or behavioral impairment. ALS patients with cognitive abnormalities, especially FTD, have a poorer prognosis than those without cognitive impairment. In neuropsychological profiling, executive tasks were effective in identifying cognitive impairment in the ALS patients. It would be useful for clinicians to classify ALS according to neuropsychological profiles, and screen for subtle cognitive impairment.

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration in association with CADASIL.

Amyotrophic lateral sclerosis (ALS) can present with heterogeneous symptoms resulting from the involvement of multiple brain systems including extramotor cortical areas. Involvement of other brain areas can cause diverse clinical symptoms including cognitive impairment and extrapyramidal symptoms. We report the case of a 50-year-old woman with bulbar onset ALS and frontotemporal lobar degeneration (FTLD), confirmed as cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). The patient and her first-degree relatives harbored a mutation (R75P) in the NOTCH3 gene, indicative of vascular deficits. The details of this case add plausibility to the idea that ALS, FTLD, and CADASIL are different aspects of a spectrum of disorders with overlapping pathologic mechanisms.

Neurobehavioral dysfunction in patients with subcortical vascular mild cognitive impairment and subcortical vascular dementia.

Amnestic mild cognitive impairment (MCI) is considered to be a prodromal stage of Alzheimer's disease. Likewise, subcortical vascular MCI (svMCI) is considered as a prodromal stage of subcortical vascular dementia (SVaD). The objective of this study was to investigate neuropsychiatric features in patients with svMCI compared to healthy controls and patients with SVaD. We evaluated 31 patients with svMCI, 42 with SVaD, and 28 healthy controls who underwent neuropsychiatric assessments using the Neuropsychiatric Inventory (NPI) and the Frontal Behavioral Inventory (FBI). On both the NPI and FBI, SVaD patients had the most severe neuropsychiatric symptoms, followed by svMCI patients and then healthy controls, suggesting that svMCI might be a prodromal stage of SVaD in terms of neuropsychiatric abnormalities. When we compared the differences of mean scores between negative and positive symptoms in FBI, negative symptoms tended to be more predominant than positive symptoms in both svMCI and SVaD patients, but the tendency was stronger in SVaD patients than in svMCI patients. These results suggest that vascular cognitive impairment with small vessel disease would start with both negative and positive neuropsychiatric symptoms and progress to present more severe negative symptoms. These behavioral ratings may be useful for early detection of vascular cognitive impairment associated with small vessel disease.

Cortical thinning in verbal, visual, and both memory-predominant mild cognitive impairment.

The amnestic form of mild cognitive impairment (aMCI) is likely a precursor of Alzheimer disease (AD). Both verbal and visual memory tests are used in the diagnosis of aMCI; however, it is unknown which type of test is superior at predicting the underlying pathologic changes associated with AD. In this study, we compared the topography of cortical thinning among 3 subtypes of patients with aMCI: 33 patients with predominant verbal memory impairment (verbal-aMCI), 35 with predominant visual memory impairment (visual-aMCI), and 56 with both verbal and visual memory-predominant impairment (both-aMCI), and 143 patients with normal cognition. As a result, patients with verbal-aMCI showed cortical thinning in the left anterior and medial temporal regions compared with individuals with normal cognition, while those with visual-aMCI did not show significant cortical thinning. The cortical thinning areas of both-aMCI group overlapped those of verbal-aMCI but were more widespread involving the bilateral temporal regions. These findings suggest that the verbal-aMCI and both-aMCI are more likely to be a precursor of AD than visual-aMCI, and that both-aMCI may be more advanced subtype than verbal-aMCI on the spectrum from MCI to AD.

Seoul Neuropsychological Screening Battery-dementia version (SNSB-D): a useful tool for assessing and monitoring cognitive impairments in dementia patients.

The Seoul Neuropsychological Screening Battery (SNSB) is one of the standardized neuropsychological test batteries widely used in Korea. However, it may be a bit too lengthy for patients with decreased attention span; and it does not provide the score of global cognitive function (GCF), which is useful for monitoring patients longitudinally. We sought to validate a dementia version of SNSB (SNSB-D) that was shorter than the original SNSB and contained only scorable tests with a GCF score of 300. We administered SNSB-D to patients with mild cognitive impairment (MCI) (n=43) and Alzheimer's disease (AD) (n=93), and normal controls (NC) (n=77). MCI and AD groups had GCF scores significantly different from NC group, and GCF scores were able to distinguish patients with Clinical Dementia Rating of 0.5 and 1. Test-retest reliability was high, with a correlation coefficient of 0.918 for AD, 0.999 for MCI, and 0.960 for NC. The GCF score significantly correlated with the Mini-Mental State Examination (MMSE). Through ROC-curve analysis, GCF scores were found to yield more accurate diagnoses than the MMSE. The SNSB-D is a valid, reliable tool for assessing the overall cognitive function, and can be used to monitor cognitive changes in patients with dementia.

Subcortical vascular versus amnestic mild cognitive impairment: comparison of cerebral glucose metabolism.

BACKGROUND AND PURPOSE: Most studies on mild cognitive impairment (MCI) have been focused on amnestic MCI (aMCI) that is the preclinical stage of Alzheimer's disease (AD). In contrast, only a few studies have involved patients in the preclinical stages of subcortical vascular dementia (subcortical vascular MCI, svMCI). We tried to compare the overall glucose metabolism in patients with svMCI with that of patients with aMCI. METHODS: We compared the regional metabolic patterns shown on 18 F-FDG (fluro deoxy glucose) positron emission tomography (PET) images from 18 patients with svMCI with those from 25 aMCI patients matched for age, sex, education, and mini-mental state examination (MMSE) score and with those from 35 healthy subjects using a voxel-wise analysis. SPM 2 was used for statistical analysis. RESULTS: Relative to normal controls, the hypometabolic regions in the aMCI patients were in the bilateral parahippocampal, bilateral posterior cingulate, left superior temporal gyri, left inferior parietal lobule, and right inferior frontal gyrus while those in the svMCI patients were located in the thalamus, insula, superior temporal gyrus, anterior cingulate gyrus, cingulum, right basal ganglia, cerebellum, and brainstem. A direct comparison of glucose metabolism between svMCI and aMCI showed that the glucose hypometabolism in patients with svMCI was more severe in the thalamus, brainstem, and cerebellum. CONCLUSION: Our study suggested that svMCI was distinct from aMCI in terms of neuropsychological and PET findings, which may explain their clinical manifestations.