Demographics, Risk Factors, and Etiology of Posterior Cerebral Artery Stroke Causing Homonymous Hemianopia.

BACKGROUND: Posterior cerebral artery (PCA) strokes account for up to 10% of all ischemic strokes, often presenting with homonymous hemianopia. The proportion of these strokes attributed to various etiologies varies widely in previously published studies, owing largely to differing patient populations, definitions of stroke pathogenesis, and vascular territories involved. The Causative Classification System (CCS), an automated version of the Stop Stroke Study (SSS) Trial of Org 10,172 in Acute Stroke Treatment (TOAST) system, allows for a more rigorous assignment of stroke etiology. METHODS: We excerpted clinical and imaging data on 85 patients who had PCA stroke with homonymous hemianopia examined at the University of Michigan. We compared the stroke risk factor profile of our PCA cohort with that of 135 patients with stroke in the distribution of the internal carotid artery (ICA) and middle cerebral artery (MCA) in an unpublished University of Michigan registry. We applied the CCS web-based calculator to our PCA cohort to determine stroke etiology. RESULTS: In our PCA cohort, 80.0% had at least 2 conventional stroke risk factors and 30.6% had 4 risk factors, most commonly systemic hypertension. The risk factor profile of our PCA cohort resembled that of our ICA/MCA cohort except that the mean age of our PCA cohort was more than a decade younger and had a significantly lower frequency of atrial fibrillation (AF) than our ICA/MCA cohort. In nearly half of the patients with AF in our PCA cohort, AF was diagnosed after the stroke. Among stroke etiologies in our PCA cohort, 40.0% were of undetermined cause, 30.6% were from cardioaortic embolism, 17.6% were from other determined causes, and only 11.8% were from supra-aortic large artery atherosclerosis. Strokes after endovascular or surgical interventions were prominent among other determined causes. CONCLUSIONS: Most patients in our PCA cohort had multiple conventional stroke risk factors, a finding not previously documented. Mean age at stroke onset and AF frequency were lower than in our ICA/MCA cohort, in agreement with previous studies. As some other studies have found, nearly 1/3 of strokes were attributed to cardioaortic embolism. Within that group, AF was often a poststroke diagnosis, a finding not previously highlighted. Compared with earlier studies, a relatively high portion of strokes were of undetermined etiology and of other determined etiologies, including stroke after endovascular or surgical interventions. Supra-aortic large artery atherosclerosis was a relatively uncommon explanation for stroke.

Characteristics, Risk Factors And Outcome Of Early-Onset Delirium In Elderly Patients With First Ever Acute Ischemic Stroke - A Prospective Observational Cohort Study.

INTRODUCTION: Post-stroke delirium is a common clinical problem, occurring in 10% to 48% of patients. It has been associated with longer hospitalization times, increased mortality and worse functional outcome. In early phase of stroke, it may be regarded as particularly difficult to differentiate from other neurological symptoms. For practical purposes, there is a need to identify simple pre-operative laboratory parameters that may aid delirium diagnosis early after stroke. Our aim was to identify the incidence of early-onset (first 24 hrs) post-stroke delirium, its risk factors and outcomes (complications and mortality) in patients with first-ever acute ischemic stroke (AIS). MATERIAL AND METHODS: A retrospective analysis of a prospective observational study (NCT03944694) was performed. Patients were screened for delirium using CAM-ICU method. Clinical and laboratory data were collected, including baseline inflammatory parameters. RESULTS: Final analysis included 760 patients, 121 (15.9%) developed delirium. Patients with delirium were older (75.9±13.5 years, p<0.001). Most common complications in the delirium group were pulmonary (57.8% vs 21.4%, p<0.001), cardiac (38.8% vs 13.6%, p<0.001) and renal (13.2% vs 7.5%, p=0.038). Neutrophil-to-lymphocyte ratio (NLR) (6.71±9.65 vs 4.55±5.51, p<0.001), C-reactive protein level (32.59±65.94 vs 15.70±38.56, p<0.001) and troponin T level (72.59±180.15 vs 26.85±77.62, p<0.001) were higher in delirious patients and platelet-to white blood cell count ratio (PWR) (23.42±9.51 vs 27.13±10.58, p<0.001) was lower. Multivariable logistic regression showed that atrial fibrillation (OR 1.651, p=0.049), higher Rankin score on admission (OR 1.689, p<0.001), hemianopia (OR 2.422, p=0.003) and PWR <20.22 (OR 2.197, p=0.002) were independently associated with delirium. Kaplan-Meier curves indicated that mortality increased for patients with delirium at 3 months (p<0.001) and 1 year (p<0.001) after AIS. CONCLUSION: Atrial fibrillation, higher Rankin score, hemianopia and lower PWR were independently associated with early onset delirium in patients with first ever AIS. This confirms that deprivation of senses and early generalized inflammatory response are critical for delirium development.

High prevalence of strabismic visual field expansion in pediatric homonymous hemianopia.

If homonymous hemianopia develops in childhood it is frequently accompanied by strabismus. In some of these cases the strabismus increases the size of the binocular visual field. We determined how prevalent visual-field-expanding strabismus is in children who have homonymous hemianopia. Medical records were examined from 103 hemianopic patients with exotropia (XT) or esotropia (ET). For each participant, we determined whether their strabismus was in a direction that resulted in visual field expansion (i.e. left exotropia with left homonymous hemianopia). Ages at which hemianopia and strabismus were first noted were compared to determine which developed first. The prevalence of XT (24%) and ET (9%) with homonymous hemianopia were both much higher than in the general population (1.5% and 5%, respectively). More strabismic eyes pointed to the blind than seeing side (62 vs 41, 60% vs. 40%, p = 0.02). Exotropic eyes were five times more likely to point to the blind side than esotropic eyes (85% vs 15%). Strabismus, especially exotropia, is much more common in pediatric homonymous hemianopia than in the general population. The strabismus is significantly more often in a visual field-expanding direction. These results support an adaptive role for the strabismus. Patients with HH and exotropia or esotropia should be aware that their visual field could be reduced by strabismus surgery.

Comparison of Clinical Characteristics among Subtypes of Visual Symptoms in Patients with Transient Ischemic Attack: Analysis of the PROspective Multicenter registry to Identify Subsequent cardiovascular Events after TIA (PROMISE-TIA) Registry.

BACKGROUND: A transient visual symptom (TVS) is a clinical manifestation of transient ischemic attack (TIA). The aim of this study was to investigate differences in clinical characteristics among subtypes of TVS using multicenter TIA registry data. MATERIALS AND METHODS: Patients with TIA visiting within 7 days of onset were prospectively enrolled from 57 hospitals between June 2011 and December 2013. Clinical characteristics were compared between patients with 3 major subtypes of TVS (transient monocular blindness [TMB], homonymous lateral hemianopia [HLH], and diplopia). RESULTS: Of 1365 patients, 106 (7.8%) had TVS, including 40 TMB (38%), 34 HLH (32%), 17 diplopia (16%), and 15 others/unknown (14%). Ninety-one patients with 1 of the 3 major subtypes of TVS were included. Symptoms persisted on arrival in 12 (13%) patients. Isolated TVS was significantly more common in TMB than in HLH and diplopia (88%, 62%, and 0%, respectively; P < .001). Duration of symptoms was shorter in patients with TMB than those with HLH (P = .004). The ABCD2 score was significantly lower in patients with TMB compared with those with HLH and diplopia (median 2 [interquartile range 2-3] versus 3 [2-4] and 4 [2-5], respectively; P = .005). Symptomatic extracranial internal carotid artery stenosis or occlusion was seen in 14 (16%) patients, and was more frequent in TMB than in HLH and diplopia (28%, 9%, and 0%, respectively; P = .015). CONCLUSIONS: TVS was an uncommon symptom in our TIA multicenter cohort. Some differences in clinical characteristics were found among subtypes of TVS.

Amygdalohippocampotomy for mesial temporal lobe sclerosis: Epilepsy outcome 5 years after surgery.

BACKGROUND: The goal of the present study is the evaluation of the long-term clinical outcome of epilepsy in patients with mesial temporal lobe sclerosis (MTLS) submitted to amygdalohippocampotomy (AHCo). AHCo consists of the lateral ablation of the amygdala and the peri-hippocampal disconnection instead of amygdalohippocampectomy (AHC), which involves the removal of both structures. We previously reported the short-term results of AHCo, so we here present the long-term results (> 5 years of follow-up) of the patients operated on with AHCo. METHOD: Since 2007, 35 patients (22 females) aged 20-61 years (mean: 42 years) were operated on with the AHCo technique, 17 patients on the left side and 18 on the right. Of these patients, 21 (14 females) have been followed up > 5 years (5 to 7.5 years, mean 6.5 years). We compare the present results with those observed shortly after surgery and with the patients operated on with AHC. FINDINGS: In all 21 cases, the diagnosis was mesial temporal lobe sclerosis (histology confirmed in 20), 11 on the left side and 10 on the right. Epilepsy results after 5 years were good/very good in 18 patients (85.7%), with Engel class IA-B in 15 (71.4%) and II in 3 (14.3%), and bad in 3 patients, with Engel Class III in 2 (9.5%) and class IV in 1 (4.8%). Concerning morbidity, one patient had hemiparesis (hypertensive capsular hemorrhage 24 h after surgery), two verbal memory worsening, two quadrantanopia and three late depression that was reversed with medication. Comparatively, the AHC long-term results were 87% Engel class I, 8% Engel class II and 5% Engel class III-IV. The morbidity was equally small. CONCLUSIONS: The good/very good results of AHCo 5 years after surgery are 86%, which is not distinct from the AHC results. So AHCo seems to be effective and potentially safer than AHC in long-term follow-up.

Visual concerns that interfere with daily activities in patients on rehabilitation units: a descriptive study.

The purpose of this study was to estimate the prevalence of patients with visual concerns that interfere with their activities of daily living (ADL) performance in physical rehabilitation units through occupational therapy assessment. Over the two-month study period, 215 adult inpatients from a physical rehabilitation hospital were evaluated using the Brief Vision Screen (BVS) through ADL. The BVS assessed four areas of visual concerns, namely left visual field, focusing, and near- and low-contrast acuity, while patients engaged in ADL. The occupational therapists identified 33% of patients who had at least one area of visual concern, with the largest proportion diagnosed with stroke (55%), followed by pulmonary disease (40%) and joint replacement (35%). When comparing the four areas of visual concerns in the BVS between the two major diagnostic groups (acquired brain injury, ABI and non-acquired brain injury, non-ABI), a significantly higher proportion of patients with ABI were identified as having left hemianopsia concerns compared to patients with non-ABI. No significant difference was observed in other areas of visual concern between the two groups. Findings indicated that visual concerns that interfere with ADL performance among older patients in rehabilitation units are common. The high proportion of patients with pulmonary disease identified as having visual concerns warranted further confirmation and investigation. Preliminary evidence to support the psychometric properties of the BVS for identifying visual concerns in patients on rehabilitation units was established.

Visual field findings after a ruptured intracranial aneurysm.

BACKGROUND: Visual field defects (VFDs) negatively affect activities of daily living and rehabilitation following aneurysmal subarachnoid haemorrhage (aSAH). The aim here was to assess VFDs in patients with aSAH and their associations with age, gender, aSAH severity, and clinical outcome. METHODS: Patients admitted to Helsinki University Central Hospital and treated during 2011 were participants in this prospective study. Findings obtained with the Octopus 900 perimeter (Haag-Streit Inc, Koenic, Switzerland), the Goldmann perimeter (Haag-Streit Inc, Bern, Switzerland), or the confrontation visual field test on admission and 3 days, 14 days, 2 to 4 months, and 6 months postoperatively were assigned to 16 classes. Associations between post-chiasmal VFDs and relevant clinical, radiological, and demographic data were analysed with uni- and multivariate logistic regression. RESULTS: Of 105 survivors at 6 months, 20 (19 %) had VFDs occurring for aneurysm- or operation-related reasons; homonymous hemianopias or quadrantanopias were the most common finding, occurring in 16 patients (15 %). Posterior ischaemic optic neuropathy presented in two patients (2 %). Ten survivors (10 %) no longer fulfilled visual field requirements for driving licences. Significant associations emerged between VFDs at 6 months and the Hunt and Hess (H&H), World Federation of Neurosurgical Societies (WFNS), and Fisher grades on admission, presence of intracerebral haemorrhage (ICH), hydrocephalus, or postoperative infarction, and higher modified Rankin Scale scores at 6 months. Multivariate logistic regression showed the H&H grade and presence of ICH to independently predict VFDs. CONCLUSIONS: Assessing VFDs is advisable, especially among patients with poor-grade aSAH (H&H grade IV or V) and ICH.

Spectrum of transient visual symptoms in a transient ischemic attack cohort.

BACKGROUND AND PURPOSE: Transient visual symptoms (TVS) are common complaints. They can be related to transient ischemic attacks, but the nature of the symptoms often remains uncertain, and data on prognosis are scarce. We studied the prevalence, presentation, and effect of different types of TVS, paying particular attention to the association with high-risk pathology of embolism. METHODS: A total of 2398 patients with suspected transient ischemic attack admitted to the SOS-TIA clinic between January 2003 and December 2008 underwent immediate evaluation and treatment. RESULTS: Eight hundred twenty-six (34.5%) patients had TVS, including 422 (17.6%) patients with isolated TVS. Transient monocular blindness was the most frequent TVS (36.3%), followed by diplopia (13.4%), homonymous lateral hemianopia (12.3%), bilateral positive visual phenomena (10.8%), and lone bilateral blindness (4.5%). Positive diffusion-weighted imaging was found in 11.8%, 8.1%, 8.1%, and 5.0% of patients with homonymous lateral hemianopia, diplopia, lone bilateral blindness, and transient monocular blindness, respectively. Among 1850 patients (595 patients with TVS) with definite/possible transient ischemic attack or minor stroke, a major source of embolism of cardiac or arterial origin was found less frequently in patients with isolated or nonisolated TVS than in patients without TVS (19.6%; 19.7% versus 28.1%, respectively; P<0.001). However, we found a higher rate of atrial fibrillation in patients with homonymous lateral hemianopia (23.2%) than in patients with other TVS (4.0%; adjusted odds ratio, 6.71; 95% confidence interval, 2.99-15.06) or nonvisual symptoms (9.1%; adjusted odds ratio, 4.39; 95% confidence interval, 2.26-8.50). CONCLUSIONS: Approximately 20% of patients with TVS had a major source of embolism detected, requiring urgent management. Atrial fibrillation was particularly frequent in patients with transient homonymous lateral hemianopia.

Intravenous thrombolysis in ischemic stroke patients with isolated homonymous hemianopia: analysis of Safe Implementation of Thrombolysis in Stroke-International Stroke Thrombolysis Register (SITS-ISTR).

BACKGROUND AND PURPOSE: Hemianopia can cause considerable disability. Only scarce data are available for ischemic stroke patients presenting with isolated homonymous hemianopia and being treated with intravenous thrombolysis. We analyzed outcome of such patients registered in the Safe Implementation of Thrombolysis in Stroke-International Stroke Thrombolysis Register (SITS-ISTR). METHODS: The SITS-ISTR (n=45 079) was searched for patients who presented with isolated homonymous hemianopia. We identified 72 such patients. We report their baseline and demographic characteristics, radiological data, change in their National Institutes of Health Stroke Scale score within 7 days after thrombolysis, and their 3-month modified Rankin Scale (mRS) score. Univariate analysis of parameters associated with any improvement was performed. Hemianopia was assessed with perimetric examination. RESULTS: Of 72 patients, 40 (56%) improved within 7 days after thrombolysis; 19 (26%) had full recovery. Those who improved had significantly lower systolic blood pressure before thrombolysis and were less often administered antiplatelet agents before index stroke. Infarction was visible on 24-hour computed tomography scan in 65% of patients who improved compared with 81.2% of those without improvement (P=0.32). No symptomatic intracranial hemorrhage occurred in patients who improved compared with 1 (3.1%) patient in the nonimproved group (P=0.08). Seventy-one percent of all patients had 3-month mRS score 0 to 2, and 51% had mRS score 0 to 1. Those who improved within 7 days had a significantly better 3-month outcome (median mRS [interquartile range], 0 [0-1] vs 2 [1-2]). CONCLUSIONS: Relatively few ischemic stroke patients received thrombolysis because of isolated homonymous hemianopia. Thrombolysis seems to be safe in these patients. Of those treated, more than half improved and more than two-thirds had good outcome.

Read-Right: a "web app" that improves reading speeds in patients with hemianopia.

Effective behavioral therapies exist for patients with brain injury. The main issue is one of access. Can the internet be used as a resource so that suitable patients can build up enough practice to improve? We tested this hypothesis using a web-based application for patients with a right-sided hemianopia causing slow text reading. We studied 33 patients aged 26-81 years who fitted the entry criteria and accessed the therapy website between May 2010 and December 2011, in a longitudinal cohort study. The therapy consisted of reading animated, laterally scrolling text whose content and form was selected by the patients. Reading speeds on static text (main outcome) were assessed after every 5-h period of practice had been accrued. Statistical analysis was carried out using a repeated measures ANOVA. Read-Right therapy produced significant improvements in text reading speeds at all time points with a clear dose effect: 10 % at 5 h, 20 % at 10 h, 39 % at 15 h and 46 % at 20 h. Sub-analyses demonstrated that this was unlikely to be due to either multiple exposure to the testing materials (familiarity) or to the simple passage of time. This is the first example of a clinically proven therapy being delivered effectively to stroke patients over the internet. As therapists' time is more limited than patients' capacity to improve, carefully designed, web-based resources like Read-Right represent a realistic way of delivering a sufficient therapy dose to patients so they can obtain clinically meaningful improvements.

Visual field defects of optic neuritis in neuromyelitis optica compared with multiple sclerosis.

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that predominantly affects the optic nerves and the spinal cord, and is possibly mediated by an immune mechanism distinct from that of multiple sclerosis (MS). Central scotoma is recognized as a characteristic visual field defect pattern of optic neuritis (ON), however, the differing pathogenic mechanisms of NMO and MS may result in different patterns of visual field defects for ON. METHODS: Medical records of 15 patients with NMO and 20 patients with MS having ON were retrospectively analyzed. A thorough systemic and neurological examination was performed for evaluating ON. The total number of relapses of ON and visual fields was investigated. Visual fields were obtained by Goldmann perimeter with each ON relapse. RESULTS: All MS patients experienced central scotoma, with 90% of them showing central scotoma with every ON relapse. However, 53% of NMO patients showed central scotoma with every ON relapse (p = 0.022), and the remaining 47% of patients experienced non-central scotoma (altitudinal, quadrant, three quadrant, hemianopia, and bitemporal hemianopia). Thirteen percent of NMO patients did not experience central scotoma during their disease course. Altitudinal hemianopia was the most frequent non-central scotoma pattern in NMO. CONCLUSIONS: NMO patients showed higher incidence of non-central scotoma than MS, and altitudinal hemianopia may be characteristic of ON occurring in NMO. As altitudinal hemianopia is highly characteristic of ischemic optic neuropathy, we suggest that an ischemic mechanism mediated by anti-aquaporin-4 antibody may play a role in ON in NMO patients.

Evaluation of on-road driving in people with hemianopia and quadrantanopia.

OBJECTIVE: To examine whether some drivers with hemianopia or quadrantanopia display safe driving skills on the road compared with drivers with normal visual fields. METHOD: An occupational therapist evaluated 22 people with hemianopia, 8 with quadrantanopia, and 30 with normal vision for driving skills during naturalistic driving using six rating scales. RESULTS: Of drivers with normal vision, > 90% drove flawlessly or had minor errors. Although drivers with hemianopia were more likely to receive poorer ratings for all skills, 59.1%-81.8% performed with no or minor errors. A skill commonly problematic for them was lane keeping (40.9%). Of 8 drivers with quadrantanopia, 7 (87.5%) exhibited no or minor errors. CONCLUSION: This study of people with hemianopia or quadrantanopia with no lateral spatial neglect highlights the need to provide individual opportunities for on-road driving evaluation under natural traffic conditions if a person is motivated to return to driving after brain injury.

Epidemiology and characteristics of occipital brain infarcts in young adults in southwestern Finland.

Occipital stroke and occipital epilepsy are possible manifestations of mitochondrial diseases. A previous study in northern Finland suggested a frequency of 10% for mitochondrial disorder in young patients with stroke. Here we studied the epidemiology of occipital brain infarcts in a defined population in southwestern Finland. Patients diagnosed with brain infarct or visual field defect with onset at the ages of 18-45 years were identified from the discharge files at the Turku University Hospital. We further ascertained those patients with an occipital brain infarct in brain imaging or homonymous hemianopia with no signs of other etiology in brain imaging. We reviewed the clinical data for known stroke risk factors and analyzed samples for the m.3243A > G and m.8344A > G mutations in mitochondrial DNA (mtDNA), and determined mtDNA haplogroups and five common mutations in the gene encoding polymerase gamma (POLG1). Migraine was more common in young patients with occipital brain infarct than in the general population, especially among women. None of the patients harboured the m.3243A > G or m.8344A > G mutation in mtDNA or any of the five common mutations in POLG1. Interestingly, 17% of the men and 33% of the women belonged to the mtDNA haplogroup Uk, while its frequency in the general population is 17%. Our results suggest that mtDNA haplogroup Uk is associated with increased risk of occipital stroke in young women. POLG1 mutations have been associated with occipital epilepsy, but we did not find the common mutations in patients with occipital stroke.

Is visuospatial hemineglect really a determinant of postural control following stroke? An acute-phase study.

OBJECTIVE: The purpose of this study was to determine the independent contribution of visuospatial hemineglect to impaired postural control in the acute phase (<2 weeks) of stroke compared with other possible clinical and biological determinants. METHODS: This study was conducted in 4 hospitals in the mid-east region of the Netherlands. A total of 78 consecutive patients with a first-ever acute supratentorial stroke was included. Functional balance was measured with the Trunk Impairment Scale, the Trunk Control Test, the Berg Balance Scale, and the Functional Ambulation Categories. Visuospatial hemineglect was assessed by means of an asymmetry index obtained from the Behavioral Inattention Test. The Motricity Index, vibration threshold, sustained attention, and the presence of hemianopia were registered as other possible clinical determinants. Stepwise backward multiple linear regression analysis was performed introducing all selected clinical determinants as well as age and poststroke time as possible biological determinants. RESULTS: Hemineglect was present in 17 patients (21.8%). The groups with and without hemineglect were different for gender and the proportion of right hemisphere strokes, but not for age, type of stroke, or poststroke time. Neglect patients had on average lower scores on all functional balance tests as well as on the clinical assessments. Multivariate linear regression showed that, besides hemineglect, only muscle strength and age independently contributed to impaired balance explaining 65% to 72% of variance of the selected outcomes. CONCLUSION: This study showed that hemineglect independently contributes to impaired postural control in the acute phase of stroke.

Comparing consequences of right and left unilateral neglect in a stroke rehabilitation population.

OBJECTIVE: This article details right and left unilateral neglect (UN) in a stroke rehabilitation population. DESIGN: This prospective observational cohort study documented hemipersonal neglect and hemispatial neglect in 309 of 325 stroke rehabilitation patients consecutively admitted over a 28-month period. Shoulder-hand complications, safety concerns, length of stay, discharge function, and discharge destination were documented. RESULTS: Of the 85 with right UN and 113 with left UN, 17.7% had expressive aphasia, and 17.7% had mixed or receptive aphasia. Hemispatial neglect was associated with hemianopsia (29.2% and 31.8% for right and left, respectively). Having both hemipersonal neglect and hemispatial neglect was related to greater safety risk (46.9% vs. 24.3%), greater incidence of shoulder-hand complications (28.3% vs. 9.9%), lower FIM scores (>10 points lower), longer length of stay (8 days), and less likelihood of discharge to home (67.3% vs. 87.4%) than subjects without UN. Results were similar for those with right and left UN. CONCLUSIONS: Right and left UN occur after stroke, can be detected even in the presence of aphasia, and are associated with shoulder-hand problems, lower discharge function, and lower likelihood of discharge home. Having both hemispatial neglect and hemipersonal neglect impacts people more than having either type of UN alone.

Unusual primary manifestations of multiple sclerosis.

OBJECTIVES: The aim of this study is to describe patients with unusual symptoms that were primary manifestations of multiple sclerosis (MS). PATIENTS AND METHODS: We report 21 multiple sclerosis patients who presented unusual initial pictures (acute brachial pain n=4, headache n=6, ptosis n=1, oculomotor nerve palsy n=1, peripheral facial palsy n=1, throat pain n=1, hypoglossal nerve palsy n=1, visual field defect n=2, epilepsia n=2, and coma n=2) as the first manifestations in the absence of other obvious symptoms or signs. RESULTS: Investigations demonstrated changes highly suggestive of multiple sclerosis on magnetic resonance imaging, cerebrospinal fluid analysis and electrophysiological tests. All cases completely or partially recovered after high-dose corticosteroid therapy. These patients have been followed up for 5 years. CONCLUSION: In this study, we discuss possible correlations between clinical disturbances and neuroradiological abnormalities and show some rare or previously undescribed manifestations in multiple sclerosis.

Relapsing demyelinating disease affecting both the central and peripheral nervous systems.

BACKGROUND: Clinical and electromyographic findings of chronic inflammatory demyelinating polyradiculopathy (CIDP) are occasionally observed in patients with multiple sclerosis (MS). OBJECTIVE: To define a new inflammatory demyelinating disease unlike MS or CIDP. RESULTS: This study reports on five patients with a demyelinating disease affecting the central nervous system (CNS) and peripheral nervous system (PNS). Each case presented a relapsing-remitting course in which CNS involvement preceded PNS involvement. All patients fulfilled Barkhof's criteria on MRI and the McDonald criteria for MS. Two patients had grey matter lesions with typical white matter changes. No systemic inflammatory disease and no metabolic or inflammatory factor for peripheral neuropathy were found. In all cases electromyography showed a demyelinating peripheral neuropathy without conduction block. Four patients fulfilled the European Federation of Neurological Societies/PNS guideline for CIDP and Nicolas et al's criteria for CIDP, one of whom also fulfilled the Ad Hoc Subcommittee criteria for CIDP. Nerve biopsy, performed in two patients, showed histological evidence of CIDP. An improvement in clinical status and neurophysiological parameters was observed in three patients after treatment with either intravenous immunoglobulin (n = 1) or cyclophosphamide (n = 2). CONCLUSION: The CNS and PNS demyelination, the absence of oligoclonal bands and the peripheral demyelination without conduction block indicate pathogenic mechanisms different from MS and CIDP. The chronology of events suggests an entity unlike that involved in acute demyelinating encephalomyelitis. Immunological reactivity against antigens common to peripheral and central myelin may explain why the demyelinating disease affected both the CNS and PNS.

Neurological signs in relation to type of cerebrovascular disease in vascular dementia.

BACKGROUND AND PURPOSE: The aim of this study was to describe the prevalence of a number of neurological signs in a large population of patients with vascular dementia (VaD) and to compare the relative frequency of specific neurological signs dependent on type of cerebrovascular disease. METHODS: Seven hundred six patients with VaD (NINDS-AIREN) were included from a large multicenter clinical trial (registration number NCT00099216). At baseline neurological examination, the presence of 16 neurological signs was assessed. Based on MRI, patients were classified as having large vessel VaD (18%; large territorial or strategical infarcts on MRI), small vessel VaD (74%; white matter hyperintensities [WMH], multiple lacunes, bilateral thalamic lesions on MRI), or a combination of both (8%). RESULTS: A median number of 4.5 signs per patient was presented (maximum 16). Reflex asymmetry was the most prevalent symptom (49%), hemianopia was most seldom presented (10%). Measures of small vessel disease were associated with an increased prevalence of dysarthria, dysphagia, parkinsonian gait disorder, rigidity, and hypokinesia and as well to hemimotor dysfunction. By contrast, in the presence of a cerebral infarct, aphasia, hemianopia, hemimotor dysfunction, hemisensory dysfunction, reflex asymmetry, and hemiplegic gait disorder were more often observed. CONCLUSIONS: The specific neurological signs demonstrated by patients with VaD differ according to type of imaged cerebrovascular disease. Even in people who meet restrictive VaD criteria, small vessel disease is often seen with more subtle signs, including extrapyramidal signs, whereas large vessel disease is more often related to lateralized sensorimotor changes and aphasia.

Manifestaciones oftalmológicas de la enfermedad cerebrovascular / Ophthalmological manifestations of cerebrovascular disease

El ojo constituye una diana para la enfermedadvascular. El estudio de la microcirculación retinianaconstituye una oportunidad muy interesante para lavaloración del riesgo vascular al conocer la relaciónexistente entre los cambios vasculares en la retina y elriesgo de padecer un ictus. Es además una ventanaexcepcional para conocer mejor la fisiopatología de lamicrocirculación.La arteria oftálmica es la primera rama de la arteriacarótida interna. La afectación ocular puede ser uni obilateral. Los síntomas oculares o neuro-oftalmológicosson transitorios o persistentes y sus manifestaciones sonmuy heterogéneas abarcando alteraciones de la agudezavisual, trastornos de los campos visuales, síndromesoculomotores y manifestaciones clínicas corticales ysubcorticales complejas. Su conocimiento puede resultarclave para instaurar las medidas preventivas pertinenteso establecer el correcto diagnóstico y la aproximacióndiagnóstica inmediata, tan importante en la atenciónde los procesos vasculares cerebrales

Transient or persistent loss of vision in one eyeis a common and distinctive manifestation ofocclusive vascular disease. Occasionally, both eyesare involved together or sequentially, with temporaryor even permanent blindness. The internal carotidarteries supply blood to the organ of vision; thereforepathologies of those arteries caused byarteriosclerosis may have a direct influence on itsfunctioning. The most common syndromes aretemporary (amaurosis fugax) or constant reductionof visual acuity. In fundus examination central retinalartery occlusion and branch retinal artery occlusionare the most common diagnosis, while retinal veinocclusion, anterior ischemic optic neuropathy,ocular ischemic syndrome are less common. Thereare many clinical ophtlamological manifestations dueto vascular brain damage. Proper recognition anddiagnosis of the disease may protect the patientagainst serious life-threatening complications suchas stroke

Pediatric homonymous hemianopia.

INTRODUCTION: Homonymous hemianopia is a disabling condition caused by numerous lesions affecting the retrochiasmal visual pathways. Little is known about homonymous hemianopia in childhood. METHODS: All patients seen in the Neuro-Ophthalmology Unit at Emory University with homonymous hemianopia (confirmed by Goldmann, Humphrey, or confrontation visual fields) between 1989 and 2004 were included. Demographic characteristics, clinical features, and evolution of the visual field defects were compared between the pediatric group (18 years of age or younger) and the adult group (older than 18 years). RESULTS: Among 852 patients with homonymous hemianopia, there were 81 children (86 homonymous hemianopias) and 771 adults (818 homonymous hemianopias). Formal visual field testing was obtained in 93% of pediatric cases and 96% of adults; a majority of patients in both groups were evaluated with Goldmann visual field testing. Demographic and visual field characteristics were similar in both groups. Significant differences were noted for the causative lesions and their location. Traumatic brain injury (34%) and tumor (27%) were the most common causes of homonymous hemianopia in the pediatric group, whereas infarction (63%), traumatic brain injury (12%), and hemorrhage (11%) were the most common causes in the adult group. Most lesions involved the optic radiations (37%), followed by the occipital lobes (26%), in the pediatric group, whereas occipital lobes (47%), followed by optic radiations (32%), were most commonly involved in adults. Approximately one third of the pediatric patients were seen in follow-up, and one third of these homonymous hemianopias spontaneously improved over the course of time. Of the 16 pediatric homonymous hemianopias evaluated within 1 month of onset of the cerebral disorder, 8 (50%) improved. CONCLUSION: Trauma and tumors are the most common cause of homonymous hemianopia in childhood. Although spontaneous improvement can be expected in approximately one third to one half of patients within the first few months, the recognition of homonymous hemianopia frequently is delayed in this population.