Rescue of Neglect and Language Impairment After Stroke Thrombectomy.

Background and Purpose: Although National Institutes of Health Stroke Scale scores provide an objective measure of clinical deficits, data regarding the impact of neglect or language impairment on outcomes after mechanical thrombectomy (MT) is lacking. We assessed the frequency of neglect and language impairment, rate of their rescue by MT, and impact of rescue on clinical outcomes. Methods: This is a retrospective analysis of a prospectively collected database from a comprehensive stroke center. We assessed right (RHS) and left hemispheric strokes (LHS) patients with anterior circulation large vessel occlusion undergoing MT to assess the impact of neglect and language impairment on clinical outcomes, respectively. Safety and efficacy outcomes were compared between patients with and without rescue of neglect or language impairment. Results: Among 324 RHS and 210 LHS patients, 71% of patients presented with neglect whereas 93% of patients had language impairment, respectively. Mean age was 71±15, 56% were females, and median National Institutes of Health Stroke Scale score was 16 (12­20). At 24 hours, MT resulted in rescue of neglect in 31% of RHS and rescue of language impairment in 23% of LHS patients, respectively. RHS patients with rescue of neglect (56% versus 34%, P<0.001) and LHS patients with rescue of language impairment (64 % versus 25%, P<0.01) were observed to have a higher rate of functional independence compared to patients without rescue. After adjusting for confounders including 24-hour National Institutes of Health Stroke Scale, rescue of neglect among RHS patients was associated with functional independence (P=0.01) and lower mortality (P=0.01). Similarly, rescue of language impairment among LHS patients was associated with functional independence (P=0.02) and lower mortality (P=0.001). Conclusions: Majority of LHS-anterior circulation large vessel occlusion and of RHS-anterior circulation large vessel occlusion patients present with the impairment of language and neglect, respectively. In comparison to 24-hour National Institutes of Health Stroke Scale, rescue of these deficits by MT is an independent and a better predictor of functional independence and lower mortality.

The visuospatial functions in children after cerebellar low-grade astrocytoma surgery: A contribution to the pediatric neuropsychology of the cerebellum.

The aim of this study was to specify whether cerebellar lesions cause visuospatial impairments in children. The study sample consisted of 40 children with low-grade cerebellar astrocytoma, who underwent surgical treatment and 40 healthy controls matched with regard to age and sex. Visuospatial abilities were tested using the spatial WISC-R subtests (Block Design and Object Assembly), Rey-Osterrieth Complex Figure, Benton Judgment of Line Orientation Test, PEBL Mental Rotation Task, and Benton Visual Retention Test. To exclude general diffuse intellectual dysfunction, the WISC-R Verbal Intelligence IQ, Performance IQ, and Full-Scale IQ scores were analysed. Post-surgical medical consequences were measured with the International Cooperative Ataxia Rating Scale. Compared to controls, the cerebellar group manifested problems with mental rotation of objects, visuospatial organization, planning, and spatial construction processes which could not be explained by medical complications or general intellectual retardation. The intensity of visuospatial syndrome highly depends on cerebellar lesion side. Patients with left-sided cerebellar lesions display more severe spatial problems than those with right-sided cerebellar lesions. In conclusion, focal cerebellar lesions in children affect their visuospatial ability. The impairments profile is characterized by deficits in complex spatial processes such as visuospatial organization and mental rotation, requiring reconstruction of visual stimuli using the imagination, while elementary sensory analysis and perception as well as spatial processes requiring direct manipulation of objects are relatively better preserved. This pattern is analogous to the one previously observed in adult population and appears to be typical for cerebellar pathology in general, regardless of age.

Amelioration of non-motor dysfunctions after transplantation of human dopamine neurons in a model of Parkinson's disease.

BACKGROUND: Patients suffering from Parkinson's disease (PD) display cognitive and neuropsychiatric dysfunctions, especially with disease progression. Although these impairments have been reported to impact more heavily upon a patient's quality of life than any motor dysfunctions, there are currently no interventions capable of adequately targeting these non-motor deficits. OBJECTIVES: Utilizing a rodent model of PD, we investigated whether cell replacement therapy, using intrastriatal transplants of human-derived ventral mesencephalic (hVM) grafts, could alleviate cognitive and neuropsychiatric, as well as motor, dysfunctions. METHODS: Rats with unilateral 6-hydroxydopamine lesions to the medial forebrain bundle were tested on a complex operant task that dissociates motivational, visuospatial and motor impairments sensitive to the loss of dopamine. A subset of lesioned rats received intrastriatal hVM grafts of ~9 weeks gestation. Post-graft, rats underwent repeated drug-induced rotation tests and were tested on two versions of the complex operant task, before post-mortem analysis of the hVM tissue grafts. RESULTS: Post-graft behavioural testing revealed that hVM grafts improved non-motor aspects of task performance, specifically visuospatial function and motivational processing, as well as alleviating motor dysfunctions. CONCLUSIONS: We report the first evidence of human VM cell grafts alleviating both non-motor and motor dysfunctions in an animal model of PD. This intervention, therefore, is the first to improve cognitive and neuropsychiatric symptoms long-term in a model of PD.

The stability of the monofixation syndrome.

PURPOSE: To determine etiology, characteristics, and stability of the monofixation syndrome (MFS) in a chart review of 63 consecutive patients in a private practice of pediatric ophthalmology. DESIGN: Retrospective, consecutive chart review. METHODS: The charts of 63 consecutive patients with MFS, encountered from 2007-2012 followed for a minimum of 3 years were included. Best visual acuity, motor angle deviation at near, fusion, and stereoacuity as recorded on the most recent visit during the follow-up period were examined. Stability of the MFS was determined by comparing the dates of diagnosis with the last examination for those patients who remained stable. Decompensated patients were also studied for characteristics and results of secondary surgery. RESULTS: The etiology of the MFS was esotropia in 58 (92.1%), anisometropia in 2 (3.2%), and exotropia in 3 patients (4.8%). The majority of patients, 57 of 63 (92%), had stable MFS for a mean of 13.9 years. Six esotropic patients decompensated after a mean of 6 years; 5 of these patients were restored to MFS by secondary surgery and 1 spontaneously recovered. Five patients were found to have improved stereoacuity to 60 arc seconds or better by the end of the study. CONCLUSIONS: The MFS is a relatively stable binocular status during the first 2 decades of follow-up. A small percentage of patients decompensated, requiring secondary surgery to restore the MFS binocular status. A similar small percentage of MFS patients developed a higher grade of stereoacuity when followed for a sufficient interval of time.

Visuoperceptual and visuospatial abilities prior to and after anterior temporal lobectomy: a case study.

We describe a patient who presented with temporal lobe epilepsy and a seizure onset pattern in the right temporo-occipital area. Structural MRI revealed sclerosis in the right hippocampus. A comprehensive presurgical neuropsychological assessment allowed us to disentangle deficits in visual object recognition and visual imagery from well-preserved spatial capacities. Following a right temporal lobectomy, the patient remained seizure free, and 1 year postsurgery, the patient's scores on object recognition and imagery were in the normal range. Our findings suggest that visual object recognition and visual imagery are sustained by cortical areas located in proximity to the temporo-occipital ventral pathway and that perceptual and imagery spatial processing is subserved as well by anatomically close mechanisms. Furthermore, the results seem to indicate that nonlesional paroxysmal activity in the posterior temporal lobe can cause chronic dysfunctions of the visual system and that it may be reversible with effective seizure control.

Ultra-fast recovery from right neglect after 'awake surgery' for slow-growing tumor invading the left parietal area.

It is now possible to perform resections of slow-growing tumors in awake patients. Using direct electrical stimulation, real-time functional mapping of the brain can be used to prevent the resection of essential areas near the tumor. Simple clinical observations of patients with a resection of slow-growing tumors have demonstrated substantial recovery within a few days of such 'awake surgery'. The aim of this study was to investigate the kinetics of recovery following the resection of slow-growing tumors invading the left parietal area and to focus mainly on its rapidity. Two patients were assessed by standard line bisection tests and compared with eight healthy individuals. Independently of the pure nature of the symptoms, we report that the patients rapidly and substantially recovered from pronounced right neglect. They were tested 48 hours after the surgery and the recovery was significant for both patients after less than 4 hours. Strikingly, for one patient, recovery was ultra fast and substantial in the first practice session within less than 7 minutes: it occurred without verbal feedback and was substantially retained during the following testing session. Its rapidity suggests a process of unmasking redundant networks. With the slow growth of the lesion, the contralesional hemisphere is probably progressively prepared for rapid unmasking of homologue networks. These results have major clinical implications. For patients with an invading left-side tumor, it is now clear that line bisections are required before, during, and after awake surgery to: plan the surgery, control the quality of the resection, and also optimize the rehabilitation of the patient.

Electrostimulation mapping of spatial neglect.

BACKGROUND: Cortical and subcortical electrostimulation mapping during awake brain surgery for tumor removal is usually used to minimize deficits. OBJECTIVE: To use electrostimulation to study neuronal substrates involved in spatial awareness in humans. METHODS: Spatial neglect was studied using a line bisection task in combination with electrostimulation mapping of the right hemisphere in 50 cases. Stimulation sites were identified with Talairach coordinates. The behavioral effects induced by stimulation, especially eye movements and deviations from the median, were quantified and compared with preoperative data and a control group. RESULTS: Composite and highly individualized spatial neglect maps were generated. Both rightward and leftward deviations were induced, sometimes in the same patient but for different stimulation sites. Group analysis showed that specific and reproducible line deviations were induced by stimulation of discrete cortical areas located in the posterior part of the right superior and middle temporal gyri, inferior parietal lobe, and inferior postcentral and inferior frontal gyri (P < .05). Fiber tracking identified stimulated subcortical areas important to spare as sections of fronto-occipital and superior longitudinal II fascicles. According to preoperative and postoperative neglect battery tests, the specificity and sensitivity of intraoperative line bisection tests were 94% and 83%, respectively. CONCLUSION: In humans, discrete cortical areas that are variable in location between individuals but mainly located within the right posterior Sylvian fissure sustain visuospatial attention specifically toward the contralateral or ipsilateral space direction. Line bisection mapping was found to be a reliable method for minimizing spatial neglect caused by brain tumor surgery.

Lumbar microdiscectomy in pediatric patients: a large single-institution series.

OBJECT: Lumbar disc herniation is a rare but significant cause of pain and disability in the pediatric population. Lumbar microdiscectomy, although routinely performed in adults, has not been described in the pediatric population. The objective of this study was to determine the surgical results of lumbar microdiscectomy in the pediatric population by analyzing the experiences at Children's Hospital Boston over the past decade. METHODS: A series of 87 consecutive cases of lumbar microdiscectomy performed by the senior author (M.R.P.) from 1999 to 2008 were reviewed. Presenting symptoms, physical examination findings, and preoperative MR imaging findings were obtained from medical records. Immediate operative results were assessed including operative duration, blood loss, length of stay, and complications, along with long-term outcome and need for repeat surgery. RESULTS: This series represents the first surgical series of pediatric microdiscectomies. The mean patient age was 16.6 years (range 12-18 years) and 60% were female. The preoperative physical examination results were notable for motor deficits in 26% of patients, sensory changes in 41%, loss of deep tendon reflex in 22%, and a positive straight leg raise in 95%. Conservative management was the first line of treatment in all patients and the mean duration of symptoms until surgical treatment was 12.2 months. The mean operative time was 110 minutes and the mean postoperative length of stay was 1.3 days. Complications were rare: postoperative infection occurred in 1%, postoperative CSF leak in 1%, and new postoperative neurological deficits in 1%. Only 6% of patients needed repeat lumbar surgery and 1 patient ultimately required lumbar fusion. CONCLUSIONS: The treatment of pediatric lumbar disc herniation with microdiscectomy is a safe procedure with low operative complications. Nuances of the presentation, treatment options, and surgery in the pediatric population are discussed.

Vision in children with hydrocephalus.

Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7 y 4 mo-12 y 10 mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p < 0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p < 0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p < 0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability.

Now you see it, now you don't: variable hemineglect in a commissurotomized man.

We describe the case of a callosotomized man, D.D.V., who shows unusual neglect of stimuli in the left visual field (LVF). This is manifest in simple reaction time (RT) to stimuli flashed in the LVF and in judging whether pairs of filled circles in the LVF are of the same or different color. It may reflect strong left-hemispheric control and consequent attention restricted to the right side of space. It is not evident in simple RT when there are continuous markers in the visual fields to indicate the locations of the stimuli. In this condition, his RTs are actually faster to LVF than to right visual field (RVF) stimuli, suggesting a switch to right-hemispheric control that eliminates the hemineglect. Neglect is also not evident when D.D.V. responds by pointing to or touching the locations of the stimuli, perhaps because these responses are controlled by the dorsal rather than the ventral visual system. Despite his atypical manifestations of hemineglect, D.D.V. showed evidence of functional disconnection typical of split-brained subjects, including prolonged crossed-uncrossed different in simple reaction time, inability to match colors between visual fields, and enhanced redundancy gain in simple RT to bilateral stimuli even when the stimulus in the LVF was neglected.

Assessment of recovery in the hemiparkinson rat: drug-induced rotation is inadequate.

Recovery from apomorphine-induced rotational behavior was compared to sensorimotor and motor function in hemiparkinsonian rats receiving intrastriatal grafts of astrocytes expressing recombinant tyrosine hydroxylase (TH) or control beta-galactosidase (beta-gal). Rats received unilateral intranigral infusions of 6-hydroxydopamine (6-OHDA). Animals with large lesions, as determined by apomorphine-induced rotation, received grafts of astrocytes into the denervated striatum. Behavioral recovery was assessed on days 14-16 post-transplantation using apomorphine-induced rotation, somatosensory neglect, and reaching for pellets using the Montoya staircase method. Rats that received transplants of TH-transfected astrocytes showed a 34% decrease in rotational behavior, but no consistent recovery of somatosensory neglect or skilled reaching. Post-mortem histological analyses revealed survival of grafted astrocytes in host striatum and expression of TH at 17 days post-transplantation. We suggest that TH-expressing astrocytes may reverse post-synaptic dopamine (DA) receptor supersensitivity; however, sensorimotor and motor abilities are not restored due to a failure by TH-expressing astrocytes to reestablish dopaminergic circuitry. The present results demonstrate the need to utilize a variety of sensory and motor behavioral tests that cohesively provide greater interpretability than a single behavioral measure used in isolation, such as drug-induced rotational behavior, to assess the efficacy of experimental gene therapies.

Correction of the Pulfrich phenomenon by surgery and laser.

We describe 2 patients who presented with asymmetrical media changes and a spontaneous Pulfrich phenomenon. In the first case, the phenomenon was due to cataract formation and an interocular latency difference of 1.49 ms +/-0.07 (SD) was found. After cataract surgery, the symptoms resolved and no interocular latency difference could be measured. In the second case, the phenomenon was caused by posterior capsule opacification and an interocular latency difference of 1.16 +/- 0.08 ms was found. The symptoms were alleviated and no interocular latency difference could be measured after a neodymium:YAG laser capsulotomy was performed. The symptomatology of the Pulfrich phenomenon is often difficult for patients to explain. Clinicians should be aware of the phenomenon with cataract patients.

Surgical outcome after prism adaptation for esotropia with a distance-near disparity.

INTRODUCTION: Prism adaptation for patients with esotropia and a distance-near disparity is controversial. The purpose of this study was to evaluate the surgical outcome for patients who underwent prism adaptation for esotropia with a distance-near disparity and determine whether both preoperative sensory and motor fusion are necessary to determine surgical success. METHODS: The medical records of 65 prism-adapted patients with a distance-near disparity of 9 PD or more were reviewed. Prism responders had a fusion response to near Worth 4-dot test and a deviation with prisms of 8 PD or less of esotropia at near, and 5 PD or less of exotropia at distance, or both. The same criteria were used postoperatively to assess a successful surgical outcome. Patients were operated for the near angle or greater than the near angle. RESULTS: Fifty-eight of 65 patients (89%) demonstrated fusion with prisms. Twenty of 65 patients (31%) had increased deviations that were greater than their original near angle (prism builders). Eleven percent (7 of 65) had no fusion. Postoperatively, 88% (51 of 58) of all fusers, 95% (18 of 19) of prism builders, and 71% (5 of 7) of nonfusers had a good surgical outcome of 8 PD or less of esotropia at near, 5 PD or less of exotropia at distance, or both. CONCLUSIONS: Prism response for distance-near disparity esotropia is a good indicator of postoperative outcome. Responders to prism adaptation had a better surgical outcome compared with nonresponders. In particular, prism adaptation aids in detecting those patients who will benefit from larger amounts of surgical correction.

Successful treatment of phantosmia with preservation of olfaction.

A 26-year-old woman had an 8-year history of phantosmia in her left nostril. The phantosmia could be eliminated by nostril occlusion or cocainization of the olfactory epithelium on the involved side. Because her symptoms and testing suggested a peripheral problem, a full-thickness "plug" of olfactory epithelium from under the cribriform plate (including all the fila olfactoria) was excised. At 5 weeks postoperatively, the phantosmia was completely gone, and her olfactory ability had returned to preoperative levels. Either the removal of abnormal peripheral olfactory neurons from the nose or the interruption of incoming signals to the olfactory bulb eliminated the phantosmia. This form of therapy for phantosmia offers an alternative to more radical procedures such as olfactory bulbectomy and may offer a significant sparing of olfactory ability.