Higher Cortical Dysfunction Research Articles

Allesthesia

Allesthesia is a peculiar symptom in which sensory stimulation to one side of the body is perceived on the opposite side. This was first described by Obersteiner1) in patients with spinal cord lesions in 1881. Thereafter, it has occasionally been reported for brain lesions and was classified into higher cortical dysfunction as a right parietal lobe symptom. No detailed studies on this symptom have long been reported in association with lesions of either the brain or spinal cord, partly because of difficulties in its pathological evaluation. Being scarcely mentioned in recent books on neurology, allesthesia has virtually become a forgotten neural symptom. The author identified allesthesia in some patients with hypertensive intracerebral hemorrhage and three patients with spinal cord lesions, and studied its clinical signs and mechanism of pathogenesis2). The following sections discuss allesthesia in light of its definition, cases and responsible lesions, clinical signs, and mechanism of pathogenesis.

NCOG-57. NEUROPSYCHOLOGICAL FUNCTION IN MENINGEAL MELANOCYTOMA: A CASE REPORT

Abstract Meningeal melanocytomas are rare tumors derived from leptomeningeal melanocytes. The aggressive subtype is considered a rare variant of malignant melanoma, carrying a poor prognosis. To date, the extant literature is void of any studies that have systematically assessed cognition or neuropsychological function in these patients. We present a case report of a 61-year-old male, right-hand dominant, Caucasian male with 16 years of formal education. He has an unremarkable medical history, and his presenting symptom were syncopal episodes. An extensive work-up revealed a complex pineal region mass. Serum markers (CEA, AFP, and beta HcG) were negative. The patient continued to deteriorate neurologically and underwent a gross total resection of the mass. At the time of resection, there was evidence of definitive leptomeningeal dissemination. Because of the leptomeningeal spread, he underwent whole brain radiation therapy with a boost to the cerebellum and surgical site. He subsequently required implantation of a ventriculoperitoneal shunt for treatment of communicating hydrocephalus. Prior to WBRT, the patient underwent comprehensive neuropsychological testing. According to the patient and his spouse, they estimated functioning at approximately 50% of normal cognition, with precipitous decline noted prior to resection of the lesion. At the time of the exam, he was dependent in essentially all instrumental activities of daily living. The examination identified a pattern consistent with diffuse subcortical involvement, characterized by impairments in cognitive processing speed, attention, working memory and executive function. Conversely, the patient demonstrated no amnesia or higher cortical dysfunction. Moreover, mood, anxiety, or other psychopathology failed to account for his cognitive impairment. In our review, this is the first case in the literature of a patient with meningeal melanocytoma (with leptomeningeal spread) who has been comprehensively assessed neuropsychologically. Conceptualizations, treatment, and prognosis will be discussed.

Higher Cortical Dysfunction Resembling Corticobasal Syndrome in 2 Patients with Multiple Sclerosis

The cognitive dysfunction in Multiple Sclerosis is classically defined as deficits in executive function, processing speed and memory. This pattern has been termed “subcorticaldy sfunction”. However, a growing body of literature suggests that there is cortical disruption in patients with Multiple Sclerosis and higher cortical dysfunction may manifest as symptoms. Here is presented two patients with Multiple Sclerosis who developed symptoms suggestive of Corticobasal syndrome, a disorder of higher cortical dysfunction due to a number of different pathologic substrates.

Correlation between Decreased Parasympathetic Activity and Reduced Cerebrovascular Reactivity in Patients with Lacunar Infarct.

Reduced cerebrovascular reactivity (CVR) was found in patients with recent lacunar infarct. However, its mechanisms were controversial. The breath holding maneuver as a vasodilatory stimulus is clinically useful for an estimation of cerebrovasomotor reactivity in well co-operative patients. Patients with lacunar infarct have no higher cortical dysfunction and remain well co-operation. The breath holding maneuver is feasible and safe to perform in patients with lacunar infarct. Autonomic nervous system regulates systemic vascular activity. Regulation of autonomic function to cerebrovascular reactivity has been reported in the literature. We examined the correlation between autonomic functions with frequency and nonlinear heart rate variability (HRV) and cerebrovascular reactivity in patients with lacunar infarct by application of breath holding maneuver. Fifteen patients with lacunar infarct (8 women, age 65.6 ± 13.61) and 16 healthy controls (11 women, age 27.33 ± 3.85) were continuously monitored at baseline before maneuver (basal phase), during CVR induction (experimental phase) with breath holding maneuver and after maneuver (recovery phase), for arterial blood pressure (ABP), electrocardiography (EKG), mean cerebral blood flow velocity (mCBFV) of middle cerebral arteries (MCA) by transcranial doppler (TCD). The short term-one minute HRV was analyzed from EKG signals for low frequency (LF)/ high frequency (HF) ratio, nonlinear of standard deviation 1 (SD1), standard deviation 2 (SD2), cardiac Sample Entropy (SampEn) and Shannon Entropy. Significant increasing in mCBFV, LF/HF ratio, SD2/SD1, Shannon Entropy and inversely decreasing SampEn during breath holding maneuver compared with baseline were found in both groups (p<0.05). The trend of cerebrovascular reactivity is similar in both groups. However, there were differences of mCBFV, systolic blood pressure (SysBP) in the whole phases (basal, experiment and recovery) between patients and controls (p<0.05). Less scattered signals of SD1 with low value in patient group were illustrated from Poincaré (p<0.05). This indicated less degree of parasympathetic drive in the patients compared to the controls. Moreover, significant positive correlation between systolic bloods pressure and mCBFV in patients suggests impact of autonomic control and cerebral blood flow on the patho-physiological mechanism of vasodilatation, triggered by hypercapnia from breath holding maneuver in patients with lacunar infarct. Reduction of cerebrovascular reactivity in patients with lacunar infarct may relate with decreased parasympathetic activity. Further study is required to demonstrate whether these findings mean mechanisms of lacunar infarct or the effect of hypertensive response.

Development and validity of an evaluation of higher cortical dysfunction in the daily life of patients with stroke and traumatic brain injury

Development and validity of an evaluation of higher cortical dysfunction in the daily life of patients with stroke and traumatic brain injury

Do interictal microembolic signals play a role in higher cortical dysfunction during migraine aura?

The aim of this study was to evaluate the prevalence and clinical impact of interictal microembolic signals (MES) in patients suffering from migraine with higher cortical dysfunction (HCD), such as language and memory impairment, during an aura. This study was carried out on 34 migraineurs with language and memory impairment during aura (HCD group), 31 migraineurs with only visual or visual and somatosensory symptoms during aura (Control group I), and 34 healthy controls (Control group II). We used a Doppler instrument to detect microemboli. Demographic data, disease features and the detection of MES between these groups, as well as the predictors of HCD during the aura, were analyzed. The duration of aura was longer and the frequency of aura was higher among patients with language and memory impairment during aura compared to Control group I. MES was detected in 29.4% patients from the HCD group, which was significantly higher compared to 3.2% in Control group I and 5.9% in Control group II. Regarding the absence or presence of MES, demographic and aura features were not different in the HCD subgroups. A longer duration of aura, the presence of somatosensory symptoms during the aura and the presence of interictal MES were independent predictors of HCD during the aura. The present findings indicate that HCD and MES are related in patients with migraine with aura. Further research is needed to better understand the exact pathophysiological mechanism.

Features of migraine aura in teenagers

BackgroundComplex migraine aura in teenagers can be complicated to diagnose. The aim of this study was to present detailed features of migraine aura in teenage migraineurs.MethodsThis cross-sectional study was conducted in the period from 2008 till 2013. A total number of 40 teenage migraineurs (20 females and 20 males) met criteria for this study. The patients were interviewed using a specially designed questionnaire for collecting data about migraine aura features. Main outcome measures were frequency of visual, somatosensory and higher cortical dysfunction (HCD) symptoms in teenage migraineurs population during the aura, and also within each individual.ResultsVisual aura was reported in every attack, followed by somatosensory (60%) and dysphasic (36.4%) aura. Scintillating scotoma and blurry vision were mostly reported and predominant visual symptoms. The most common somatosensory symptom was numbness in hand. HCD were reported by 22 (55%) patients. Slowed speech was mostly reported symptom of HCD, followed by dyslexia, déjà vu phenomenon, color dysgnosia, and dyspraxia. In patients with HCD, aura frequency per year (6.18 ± 3.17 vs. 3.33 ± 2.03, p = 0.003) and prevalence of somatosensory symptoms (77.3% vs. 38.9%, p = 0.014) were significantly higher than in patients without HCD.ConclusionsAura symptoms vary to a great extent in complexity in teenage migraineurs. Consequently, results obtained in this study provide useful information for clinicians when faced with unusual migraine aura.

Validity of the progress notebook in supporting patients with higher cortical dysfunction

Sonoda S, Shirayama Y, Tanabe S, Shimomura K, Suzuki S. Validity of the progress notebook in supporting patients with higher cortical dysfunction. Jpn J Compr Rehabil Sci 2014; 5: 93-96. Purpose: To validate the usefulness of a progress notebook (p-notebook) in supporting patients with higher cortical dysfunction by analyzing the difference in interview time in a new hospital or facility with and without the p-notebook. The p-notebook was created to assist those with higher cortical dysfunction, which is not well understood by non-experts. Methods: Interview times with and without the p-notebook (notebook group: n=34, non-notebook group: n=32) were compared when patients with higher cortical dysfunction went to a new hospital or facility. Impressions of the families and support coordinators of patients in the notebook group were evaluated using visual analogue scales. Results: The average interview time was 32.4 ± 10.7 minutes in the notebook group and 57.2 ± 28.9 minutes in the non-notebook group, and this difference was significant (p<0.0001). Regarding the impressions of family and support coordinators, the length of explanation was shorter and understandability was improved when using the p-notebook. These tendencies were dominant in families compared with support coordinators. Conclusion: Our p-notebook is useful since it decreases the interview time and lowers the burden of giving explanations.

Trends in stroke care and higher cortical dysfunction

Trends in stroke care and higher cortical dysfunction

発作時にＧｅｒｓｔｍａｎｎ症候群を呈した症候性頭頂葉てんかんの１例

A 34-year-old man with astrocytoma in the left parietal lobe had symptomatic partial epilepsy, and he presented transient episodes of acalculia, agraphia and finger agnosia. Occasionally he had difficulty in finding appropriate letters when making an e-mail, and difficulty in writing and calculation. Neurological examinations revealed ictal symptoms of Gerstmann's syndrome without right to left disorientation. No other higher cortical dysfunction or neurological deficits were noted. Scalp EEGs showed frequent, regional ictal discharges in the left parietal area lasting for 60-240 seconds. These clinico-electrographical observations strongly support that epileptic seizures produced a loss of cortical higher function manifesting Gerstmann's syndrome.

Verbal perseveration as the initial symptom in a case of Creutzfeldt‐Jakob disease

Background: In the literature on cortical degenerative processes and primary progressive aphasia, language disorders have been discussed as the initial sign of higher cortical dysfunction. Verbal perseveration has also been cited to precede the occurrence of other cortical signs. In this paper we present language data from an autopsy‐confirmed case of Creutzfeldt‐Jakob disease (CJD) who exhibited profound verbal perseveration as the initial symptom. The authors would like to thank Karin Geisler, Maura Bayer, and Caroline Brew for their critical reading of this paper, and the colleagues who provided the two reviews of this manuscript for their constructive comments and suggestions. Aims: This case report aims to provide a description of the extent and pattern of verbal perseveration in a client with the rapidly progressing fatal CJD. Language test data and medical data, i.e., EEG and neuropathology—which confirm the diagnosis of CJD—are presented. Methods & Procedures: Language testing was performed with our patient to determine the nature of the language disorder at a time when the diagnosis of CJD had not yet been made. The tape‐recorded sessions were transcribed and the language data analysed. Outcomes & Results: The pervasive perseverative behaviour mirrors the underlying language‐processing difficulties and the magnitude of the progression of the disease process. Perseverative errors account for between 87% and 95% of the language‐processing errors on the various language tasks. In general, the first items of each task were responded to correctly, indicating that our client was trying to meet the various test demands and at the time of testing was still able to process language, albeit in a very restricted manner. Conclusions: The rapid deterioration of language functions in our case and in other reported cases necessitates acting quickly without delay. Immediate recognition and analysis of the language disturbance and verbal perseverative behaviour may provide relevant information for differentially diagnosing clients presenting with various cortical degenerative diseases at an early stage.

Poster 8: Percutaneous Endoscopic Button Gastrostomy Has Merits for Neurologic Rehabilitation of Patients With Oropharyngeal Dysphagia

Objective: To evaluate the merits of percutaneous endoscopic gastrostomy (PEG button) feeding during neurologic rehabilitation of stroke or traumatic brain injury (TBI). Design: Not provided. Setting: Inpatient rehabilitation hospital. Participants: 21 stroke and 4 TBI survivors, with severe oropharyngeal dysphagia and higher cortical dysfunction, who were initially fed via a nasogastric tube (NGT) for >3 months. Intervention: One-step PEG button placement. Main Outcome Measures: Peroral feeding condition and Barthel Index score. Results: Routine program of swallowing exercise was more effectively performed under the condition of PEG feeding. 12 patients attained complete and 5 patients did partial peroral diets, although 8 patients were totally dependent on long-term PEG feeding. An increase in the Barthel Index score during the PEG feeding (19±14) was significantly (P<.001) larger than that during the NGT feeding (4±4). 2 patients with tracheotomy were finally decanulated after placing the PEG. Patients with the PEG button could avoid psychologic stress associated with the NGT. Conclusions: The 1-step PEG button is valuable to oropharyngeal dysphagic patients not only for long-term alimentation, but also for transient feeding by temporary PEG during neurologic rehabilitation in early stage of stroke or TBI to improve functional outcome.

Children With Specific Language Impairment: Quantitative and Qualitative Analysis of Dichotic Listening Performance

Specific language impairment (SLI) is one of the most common developmental disorders of higher cerebral function in children. This study was undertaken to examine the dichotic listening performance of children with SLI. Participants included 37 school-age children who underwent a comprehensive neuropsychological evaluation that included measures of cognitive ability, receptive and expressive language, visual-spatial perception-construction, immediate-working memory, and achievement and ratings of behavior-emotionality. In addition, each child was administered a free-recall consonant-vowel syllable dichotic listening task (30 pairs). Results indicated that, although the children with SLI exhibited a weak right-ear preference as a group, nearly 52% demonstrated a significant right-ear deficiency, whereas 29% exhibited left-ear deficiency and approximately 10% demonstrated bilateral-ear deficiency. The implication of these results is discussed with regard to their corresponding patterns of higher cortical dysfunction and their support for a qualitative differences model of SLI.

Patient Participation in the Management of Stroke Rehabilitation

(1999). Patient Participation in the Management of Stroke Rehabilitation. Topics in Stroke Rehabilitation: Vol. 6, Higher Cortical Dysfunction in the Stroke Patient, pp. 43-59.

Tc-99m HMPAO brain perfusion imaging in young Down's syndrome patients.

Down's syndrome (DS) is characterized by moderate mental retardation and a variety of abnormalities involving multiple organ systems. There is a high incidence of Alzheimer's disease (AD) type dementia beyond the age of 35. In this study, single photon emission computed tomography (SPECT) brain perfusion imaging of young Down's syndrome patients was performed to define the perfusion pattern. Tc-99m HMPAO brain perfusion SPECT was performed on 17 young DS patients, aged 3-24 years (mean: 10.9+/-5.9 years). None of the patients had dementia symptoms. Brain perfusion scans were acquired 15 min after i.v. injection of 12 MBq/kg of Tc-99m HMPAO using a single head rotating gamma camera. Images were analyzed visually and semiquantitatively by defining side-to-side asymmetry index. Nine DS cases showed normal brain perfusion. Eight of the 17 cases revealed mostly unilateral parieto-temporal, parieto-occipital and frontal hypoperfusions. The side-to-side asymmetry indices for these visually interpreted regional brain perfusion abnormalities ranged from 6 to 15%. These findings revealing mostly unilateral parieto-temporal and frontal hypoperfusions may not be considered as predictive patterns of dementia related Alzheimer type perfusion deficits in DS. However, such findings may connect to other functional imaging studies related to the higher cortical dysfunction in mental retardation.

Computed topographic electroencephalographic study in left hemiplegic patients with higher cortical dysfunction

Computed topographic electroencephalography (EEG) was performed on patients with right hemispheric injury due to cerebral infarction to investigate the relationship of wave form pattern with neurologic and neuropsychologic data and activities of daily living (ADL). Neurologic signs, unilateral spatial neglect, motor impersistence, and constructional apraxia were found to have no relation to the wave form pattern of computed topographic EEG. In patients with abnormalities detected by the “Mini-Mental State” examination (MMSE) or the “Kanahiroi” test, and in patients with abnormalities in word fluency, extensive slowing of EEG frequency was commonly observed ipsilateral to the side of the lesion or bilaterally, and the ADL were frequently poor. In contrast, among the patients with slow waves and retained alpha waves, the MMSE, “Kana-hiroi” test, word fluency, and ADL were less abnormal. Computed topographic EEG may thus be a useful tool to evaluate neuropsychological status in rehabilitation patients with cerebral infarction.

Hypoxic Brain Damage and Higher Cortical Dysfunction in Children

Hypoxic Brain Damage and Higher Cortical Dysfunction in Children

Patterns of vascular pathology in acute, first-ever cerebral infarction.

This was a preliminary study to see whether patterns of vascular pathology correlated with a simple clinical classification of acute cerebral infarction. Thirty-two patients with acute, first-ever hemispheric cerebral infarction were prospectively studied clinically together with an extra- and transcranial Doppler ultrasound. All 14 patients with the triad of ipsilateral motor/sensory deficit, homonymous hemianopia and higher cortical dysfunction (Total Anterior Circulation Infarction, [TACI]) had occlusion of the symptomatic middle cerebral artery and/or internal carotid artery, or a severe middle cerebral artery stenosis. Three patients with lacunar infarction had no evidence of major vessel occlusion. Eleven of thirteen patients with Partial Anterior Circulation, Infarction (PACI) I (i.e. only one or two clinical features of the TACI triad) had patent symptomatic major vessels, with indirect evidence to suggest distal branch occlusion(s) of the middle cerebral artery in six. The remaining two PACI patients had major vessel occlusions. Two patients were not clinically classifiable, but both had significant vascular pathology on ultrasound. The findings of this preliminary study therefore suggest that a simple clinical classification was generally capable of predicting patterns of vascular pathology in patients with acute cerebral infarction, which could have implications for the selection of patients for clinical trials of thrombolytic therapy.

Higher cortical dysfunction, antiphospholipid antibodies and neuroradiological examinations in systemic lupus erythematosus.

We performed neuropsychological tests to investigate higher cortical dysfunction in 21 patients with systemic lupus erythematosus (SLE). We also measured antiphospholipid antibodies (APA), performed brain computed tomography (CT), and obtained a single photon emission CT (SPECT) to measure regional cerebral blood flow (rCBF) in order to elucidate a possible relationship between APA and higher cortical dysfunction. Higher cortical dysfunction was noted in as many as 16 (76%) out of 21 cases. APA were positive in 8 (38%) out of 21 cases. Although the relationship between APA and higher cortical dysfunction was not significant, patients positive for lupus anticoagulant (LA) were found to have higher cortical dysfunction. Brain CT revealed at least one abnormality in 6 cases (29%) but none had a localized lesion, SPECT disclosed a reduced rCBF in 9 cases (43%). The findings on brain CT and SPECT were unrelated to higher cortical dysfunction.

Transient impairment of visual perception induced by single interictal occipital spikes.

We have previously reported that focal occipital interictal epileptiform discharges (spikes) cause transiently prolonged reaction time (RT) and increased nonperception of stimuli, especially in the visual field contralateral to the spike. One subject with very frequent spikes was capable of carrying out a visual recognition task along with the RT task. During central fixation, computer-generated random digits were flashed for 150 ms at random locations on a screen. Some stimuli were delivered during spikes, by means of an amplitude-threshold trigger, whereas control stimuli were delivered at random times between spikes. Following each stimulus, the subject had to press a button for RT and then report the digit perceived. There was a statistically significant increase in nonresponse rate (nonperception) during spikes compared to controls, and this effect was maximal contralateral to the spike. Moreover, among the responses, perceptual accuracy (correct vs incorrect) was significantly impaired during spikes, again predominantly in the visual field contralateral to the spike. Thus, not all focal interictal spikes are necessarily "subclinical;" at least some induce a transient cortical dysfunction of the same kind as produced more enduringly from a structural lesion in the same location. These findings may have clinical relevance in patients, especially children, with very frequent epileptiform discharges and higher cortical dysfunction.