Chan Tiel

White matter hyperintensities, executive function and global cognitive performance in vascular mild cognitive impairment

UNLABELLED Vascular mild cognitive impairment (VaMCI) represents an early symptomatic stage of vascular cognitive impairment and might be associated to fronto-executive dysfunction. METHODS Twenty-six individuals (age: 73.11±7.90 years; 65.4% female; schooling: 9.84±3.61 years) were selected through neuropsychological assessment and neuroimaging. Clinical and neuroimaging data of VaMCI individuals (n=15) were compared to normal controls (NC, n=11) and correlated with Fazekas scale. RESULTS VaMCI performed significantly worse than NC in Trail-Making Test (TMT) B, errors in TMT B, difference TMT B-A and Cambridge Cognitive Examination (CAMCOG) final scores. Correlations were found among scores in modified Fazekas scale and performances in TMT B (time to complete and errors), difference TMT B-A and CAMCOG total score. CONCLUSION Extension of white matter hyperintensities might be correlated to poorer global cognition and impairments in a set of fronto-executive functions, such as cognitive speed, set shifting and inhibitory control in VaMCI.

Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

Department of Radiology and Instituto de Neurologia Deolindo Couto, University Federal of Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil: Medico Especializando em Radiologia e Diagnostico por Imagem do Instituto de Pos-Graduacao Medica Carlos Chagas (IPGMCC), Rio de Janeiro RJ, Brazil; Medico Residente em Neurologia do Instituto de Neurologia Deolindo Couto, UFRJ; Medica Residente de Radiologia e Diagnostico por Imagem do Hospital Universitario Clementino Fraga Filho, UFRJ; Medico Neurologista do Instituto de Neurologia Deolindo Couto, UFRJ; Professor Adjunto do Departamento de Radiologia, UFRJ. Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SODplus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe.

Executive testing predicts functional loss in subjects with white matter lesions

In order to assess ecological validity of executive function (EF) tests and the impact of EF dysfunction on functional status in elderly subjects with moderate and severe subcortical white matter hyperintensities (WMHs), we made a correlation analysis between EF scores and two measures of Instrumental Activities of Daily Living (IADL). Trail-making test and CLOX correlated with the ability to perform IADL in subjects with severe WMH. EF tests might present low ecological validity for those with WMH below severe stage.

Behavioral and psychological symptoms and hippocampal atrophy in subcortical ischaemic vascular disease

Background Neuropsychiatric symptoms are common in patients with cognitive impairments, mediated by both neurodegenerative processes and cerebrovascular disease. Previous studies have reported that Behavioral and Psychological Symptoms of Dementia (BPSD) might correlate with severity of cognitive decline. Thus far, the impact of the association between white-matter hyperintensities (WHM) and hippocampal atrophy (HA) on the incidence of these symptoms has been less studied. Objective This cross-sectional study aimed to describe the clinical profile of a sample with large extensions of WMH, examining the association between different degrees of HA and cognitive, functional, and behavioral status. Methods Fifty outpatients (mean age: 76.86±8.70 years; 58% female; mean schooling: 7.44±4.69 years) with large extensions of WMH (modified-Fazekas scale=3) on MRI and different degrees of hippocampal atrophy (according to de Leon Score) underwent cognitive, functional, and behavioral assessments. Results Patients with mild-moderate to severe HA had worse performance on the Mini-Mental State Examination, Cambridge Cognitive Examination, Clinical Dementia Rating and Pfeffers Functional Activities Questionnaire, compared to the group with none or questionable HA. Appetite/Eating Behavior was the only cluster of neuropsychiatric symptoms associated with presence of HA in Vascular Cognitive Impairment patients. Discussion Although HA may exhibit distinct impact on cognitive performance and functional status, it appears to have little effect on behavioral symptoms in patients with high severity WMH.