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Frontal Lobe (frontal + lobe)
Kinds of Frontal Lobe Terms modified by Frontal Lobe Selected AbstractsAngiocentric glioma: A case report and review of the literatureDIAGNOSTIC CYTOPATHOLOGY, Issue 6 2010Ryan T. Mott M.D. Abstract Angiocentric glioma (AG) is a rare central nervous system (CNS) neoplasm that was only recently recognized by the World Health Organization (WHO). AG occurs in a broad age range, shows no gender predilection, and arises superficially in the cerebrum, usually resulting in medically intractable seizures. Most cases are cured by surgical excision alone, consistent with a WHO grade I neoplasm. We report a case of an AG in the right frontal lobe of a 57-year-old female, emphasizing the cytologic and immunohistochemical features, including confirmation and comparison with the surgical specimen. To our knowledge, this is the first report detailing the cytology of AG, including demonstration of important diagnostic findings that were only appreciated in the cytologic preparations and not in the smears or the surgical specimen. We also compare and contrast AG to other entities in the differential diagnosis and include a review of the literature. Diagn. Cytopathol. 2010. © 2009 Wiley-Liss, Inc. [source] Palilalia, echolalia, and echopraxia,palipraxia as ictal manifestations in a patient with left frontal lobe epilepsyEPILEPSIA, Issue 6 2009Yang-Je Cho Summary Palilalia is a relatively rare pathologic speech behavior and has been reported in various neurologic and psychiatric disorders. We encountered a case of palilalia, echolalia, and echopraxia,palipraxia as ictal phenomena of left frontal lobe epilepsy. A 55-year-old, right-handed man was admitted because of frequent episodes of rapid reiteration of syllables. Video-electroencephalography monitoring revealed stereotypical episodes of palilalia accompanied by rhythmic head nodding and right-arm posturing with ictal discharges over the left frontocentral area. He also displayed echolalia or echopraxia,palipraxia, partially responding to an examiner's stimulus. Magnetic resonance imaging revealed encephalomalacia on the left superior frontal gyrus and ictal single photon emission computed tomography showed hyperperfusion just above the lesion, corresponding to the left supplementary motor area (SMA), and subcortical nuclei. This result suggests that the neuroanatomic substrate involved in the generation of these behaviors as ictal phenomena might exist in the SMA of the left frontal lobe. [source] fMRI Lateralization of Expressive Language in Children with Cerebral LesionsEPILEPSIA, Issue 6 2006Dianne P. Anderson Summary:,Purpose: Lateralization of language function is crucial to the planning of surgery in children with frontal or temporal lobe lesions. We examined the utility of functional magnetic resonance imaging (fMRI) as a determinant of lateralization of expressive language in children with cerebral lesions. Methods: fMRI language lateralization was attempted in 35 children (29 with epilepsy) aged 8,18 years with frontal or temporal lobe lesions (28 left hemisphere, five right hemisphere, two bilateral). Axial and coronal fMRI scans through the frontal and temporal lobes were acquired at 1.5 Tesla by using a block-design, covert word-generation paradigm. Activation maps were lateralized by blinded visual inspection and quantitative asymmetry indices (hemispheric and inferior frontal regions of interest, at p < 0.001 uncorrected and p < 0.05 Bonferroni corrected). Results: Thirty children showed significant activation in the inferior frontal gyrus. Lateralization by visual inspection was left in 21, right in six, and bilateral in three, and concordant with hemispheric and inferior frontal quantitative lateralization in 93% of cases. Developmental tumors and dysplasias involving the inferior left frontal lobe had activation overlying or abutting the lesion in five of six cases. fMRI language lateralization was corroborated in six children by frontal cortex stimulation or intracarotid amytal testing and indirectly supported by aphasiology in a further six cases. In two children, fMRI language lateralization was bilateral, and corroborative methods of language lateralization were left. Neither lesion lateralization, patient handedness, nor developmental versus acquired nature of the lesion was associated with language lateralization. Involvement of the left inferior or middle frontal gyri increased the likelihood of atypical language lateralization. Conclusions: fMRI lateralizes language in children with cerebral lesions, although caution is needed in interpretation of individual results. [source] Seizure Semiology in the Elderly: A Video AnalysisEPILEPSIA, Issue 3 2004Christoph Kellinghaus Summary: Purpose: To describe the seizure semiology of patients older than 60 years and to compare it with that of a control group of younger adults matched according to the epilepsy diagnosis. Methods: Available videotapes of all patients aged 60 years and older who underwent long-term video-EEG evaluation at the Cleveland Clinic Foundation (CCF) between January 1994 and March 2002 were analyzed by two observers blinded to the clinical data. A younger adult control group was matched according to the epilepsy diagnosis, and their seizures also were analyzed. Results: Fifty-four (3.3%) of the 1,633 patients were 60 years or older at the time of admission. For 21 of them, at least one epileptic seizure was recorded. Nineteen patients had focal epilepsy (nine temporal lobe, two frontal lobe, two parietal lobe, eight nonlocalized), and two patients had generalized epilepsy. Seventy-three seizures of the elderly patients and 85 seizures of the 21 control patients were analyzed. In nine elderly patients and 14 control patients, at least one of their seizures started with an aura. Eleven elderly patients and 19 control patients lost responsiveness during their seizures. Approximately two thirds of the patients in both groups had automatisms during the seizures. Both focal and generalized motor seizures (e.g., clonic or tonic seizures) were seen less frequently in the elderly. Conclusions: Only a small percentage of the patients admitted to a tertiary epilepsy referral center for long-term video-EEG monitoring are older than 60 years. All seizure types observed in the elderly also were seen in the younger control group, and vice versa. Simple motor seizures were seen less frequently in the elderly. [source] Different Electroclinical Manifestations of the Epilepsy Associated with Hamartomas Connecting to the Middle or Posterior HypothalamusEPILEPSIA, Issue 9 2003Alberto J. R. Leal Summary:,Purpose: The epilepsy associated with hypothalamic hamartomas (HHs) has typical clinical, electrophysiologic, and behavioral manifestations refractory to drug therapy and with unfavorable evolution. It is well known that only sessile lesions produce epilepsy, but no correlation has been established between the different types of sessile hamartomas and the diverse manifestations of the epilepsy. We correlate anatomic details of the hamartoma and the clinical and neurophysiologic manifestations of the associated epilepsy. Methods: HHs of seven patients with epilepsy (ages 2, 25 years) were classified as to lateralization and connection to the anteroposterior axis of the hypothalamus by using high-resolution brain magnetic resonance imaging. We correlated the anatomic classification with the clinical and neurophysiologic manifestations of the epilepsy as evaluated in long-term (24 h) video-EEG recordings. Results: HHs ranged in size from 0.4 to 2.6 cc, with complete lateralization in six of seven patients. Ictal manifestations showed good correlation with the lobar involvement of ictal/interictal EEGs. These manifestations suggest the existence of two types of cortical involvement, one associated with the temporal lobe, produced by hamartomas connected to the posterior hypothalamus (mamillary bodies), and the other associated with the frontal lobe, seen in lesions connecting to the middle hypothalamus. Conclusions: A consistent clinical and neurophysiologic pattern of either temporal or frontal lobe cortical secondary involvement was found in the patients of our series. It depends on whether the hamartoma connects to the mamillary bodies (temporal lobe cases) or whether it connects to the medial hypothalamus (frontal lobe cases). [source] Operative Results without Invasive Monitoring in Patients with Frontal Lobe Epileptogenic LesionsEPILEPSIA, Issue 10 2001A. Mariottini Summary: ,Purpose: To further explore the still controversial issues regarding whether all or most candidates for epilepsy surgery should be investigated preoperatively with invasive long-term video-EEG monitoring techniques (ILTVE). Methods: We studied five patients with intractable seizures since early childhood using the same protocol: clinical evaluation, magnetic resonance imaging (MRI) with fluid-attenuated inversion recovery (FLAIR) sequences, long-term video-EEG (LTVE) monitoring with scalp electroencephalogram (EEG), interictal single photon emission computed tomography (SPECT), positron emission tomography (PET), and neuropsychological testing. The patients' seizures had clinical features suggesting a frontal lobe (FL) origin. MRI scans revealed focal cortical dysplasia (CD) in four patients and a probable gliotic lesion in the fifth. The findings in both PET and SPECT images were congruent with those of the MRI. Scalp LTVE failed to localize the ictal onset, although the data exhibited features suggestive of both CDs and FL seizures. On the basis of these results, surgery was performed with intraoperative corticography, and the cortical area exhibiting the greatest degree of spiking was ablated. Results: Histopathologic study of four of the resected specimens confirmed the presence of CD, whereas in the fifth, there were features consistent with a remote encephaloclastic lesion. There were no postoperative deficits. Seizures in three of the patients were completely controlled at 2,3.5 years of follow-up; a fourth patient is still having a few seizures, which have required reinstitution of pharmacotherapy, and the fifth has obtained 70% control. All patients have had significant improvement in psychosocial measures. For comparison, five patients with generally similar clinical and neuroradiologic features to the previous group underwent preoperative ILTVE monitoring. The surgical outcomes between the two groups have not differed significantly. Conclusions: We conclude that patients with FL epilepsies may be able to undergo successful surgery without preoperative ILTVE monitoring, provided there is high concordance between neuroimaging tests (MRI, SPECT, PET) and the seizure phenotypes, even when routine EEGs and scalp LVTE fail to localize ictal onset unambiguously. The surgical outcomes of these patients generally paralleled those of the other subjects who also had FL epilepsy but who were operated on only after standard ILTVE monitoring. [source] Cerebral blood flow in patients with diffuse axonal injury , examination of the easy Z -score imaging system utilityEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2007T. Okamoto To evaluate the utility of easy Z -score imaging system (eZIS) in 27 diffuse axonal injury (DAI) cases. Twenty-seven DAI patients were examined with an magnetic resonance imaging (MRI) T2* sequence and with eZIS (seven women, 20 men; age range, 19,35 years; median age: 26.6 years). In this investigation, we excluded patients who exhibited complications such as acute subdural hematoma, acute epidural hematoma, intracerebral hematoma, or brain contusion. We examined the neuropsychological tests and correlated with findings from MRI/eZIS. Furthermore, we evaluated the degree of ventricular enlargement in the bifrontal cerebroventricular index (CVI). Patients were divided into two groups: the enlargement group (bifrontal CVI > 35%, 12 patients) and the non-enlargement group (bifrontal CVI < 35%, 15 patients). All of the patients showed cognitive deficits as observed from the neuropsycological test results. Fifteen out of 27 patients by MRI T1/T2 weighted images and fluid attenuated inversion recovery (FLAIR), 22 out of 27 patients by MRI T2* weighted images and 24 out of 27 patients by eZIS showed abnormal findings. In MRI T2* weighted imaging, the white matter from the frontal lobe, corpus callosum, and brainstem showed abnormal findings. With eZIS, 22 patients (81.5%) showed blood flow degradation in the frontal lobe, and 12 patients (44.4%) in cingulate gyrus. In the enlargement group, Functional Independence Measure, Mini-Mental State Examination, Verbal IQ (VIQ)/Full Scale IQ (FIQ), Trail Making Test-B (TMT-B), and Non-paired of Miyake Paired Test were significantly lower. Amongst 12 patients without ventricular enlargement who had no abnormal findings in MRI T1/T2 weighted images and FLAIR, abnormal findings were detectable in seven patients with MRI T2* weighted imaging and to 10 patients with eZIS. Results of the MRI examination alone cannot fully explain DAI frontal lobe dysfunction. However, addition of the eZIS-assisted analysis derived from the single photon emission computed tomography (SPECT) data enabled us to understand regions where blood flow was decreased, i.e., where neuronal functions conceivably might be reduced. [source] Hereditary neuropathy with liability to pressure palsies associated with central nervous system myelin lesionsEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2001J. Dac Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder most commonly caused by a 1.5-Mb deletion in chromosome 17p11.2 which contains the peripheral myelin protein-22 (PMP22) gene. Mutations resulting in functional loss of one PMP22 gene copy are less frequent. We present a 51-year-old patient with a l.5-Mb deletion in chromosome 17p11.2 who exhibited signs of peripheral as well as central nervous system lesions. He gave a history of recurrent episodes of limb numbness and weakness with spontaneous but incomplete recovery since age 20. His father and two brothers had similar symptoms. Neurological examination revealed signs of multiple mononeuropathy associated with frontal lobe, corticospinal tract and cerebellar dysfunction, as well as signs of initial cognitive impairment. Electrophysiological investigations showed a demyelinating peripheral nerve disease with multiple conduction blocks and conduction disturbances in both optic nerves. Magnetic resonance imaging of the brain revealed multiple subcortical and periventricular foci of myelin lesions. The association of central and peripheral nervous system lesions in this patient indicates a possible role of PMP22 not only in peripheral but also in central nervous system myelin structure. [source] Cloning, distribution and functional analysis of the type III sodium channel from human brainEUROPEAN JOURNAL OF NEUROSCIENCE, Issue 12 2000Yu Hua Chen Abstract The type III voltage-gated sodium channel was cloned from human brain. The full-length cDNA has 89% identity with rat type III, and the predicted protein (1951 amino acids) has 55 differences. The expression pattern of human type III mRNA was determined in adult brain tissue and, in contrast to rat, was detected in many regions, including caudate nucleus, cerebellum, hippocampus and frontal lobe. The human type III channel was stably expressed in Chinese hamster ovary (CHO) cells and its biophysical properties compared to the human type II channel using identical conditions. The voltage dependence and kinetics of activation were found to be similar to that of type II. The kinetics of inactivation of the two human subtypes were also similar. However, type III channels inactivated at more hyperpolarized potentials and were slower to recover from inactivation than type II. When expressed in human embryonic kidney (HEK293T) cells, type III channels produced currents with a prominent persistent component, which were similar to those reported for rat type II [Ma et al. (1997) Neuron, 19, 443,452]. However, unlike type II, this was prominent even in the absence of coexpressed G-proteins, suggesting type III may adopt this gating mode more readily. The distinct properties of the channel, together with its wide distribution in adult brain, suggest that in humans, type III may have important physiological roles under normal, and perhaps also pathological conditions. [source] Coronary heart disease is associated with regional grey matter volume loss: implications for cognitive function and behaviourINTERNAL MEDICINE JOURNAL, Issue 7 2008O. P. Almeida Abstract Coronary heart disease (CHD) has been associated with impaired cognition, but the mechanisms underlying these changes remain unclear. We designed this study to determine whether adults with CHD show regional brain losses of grey matter volume relative to controls. We used statistical parametric mapping (SPM5) to determine regional changes in grey matter volume of T1 -weighted magnetic resonance images of 11 adults with prior history of myocardial infarction relative to seven healthy controls. All analyses were adjusted for total grey and white matter volume, age, sex and handedness. CHD participants showed a loss of grey matter volume in the left medial frontal lobe (including the cingulate), precentral and postcentral cortex, right temporal lobe and left middle temporal gyrus, and left precuneus and posterior cingulate. CHD is associated with loss of grey matter in various brain regions, including some that play a significant role in cognitive function and behaviour. The underlying causes of these regional brain changes remain to be determined. [source] Impact of cerebrovascular pathology on behavioural and neuropsychiatric symptoms in patients with Alzheimer's dementia: findings from a retrospective, naturalistic studyINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 7 2009A. Klugman Summary Aim:, Cerebrovascular disease (CVD) has been associated with depression and a host of neuropsychiatric conditions including dementia. This study assessed the relationship between cerebrovascular findings reported on MRI brain scans and neuropsychiatric symptoms (NPS) and behavioural problems in patients with Alzheimer's disease (AD). Methods:, Medical notes were retrospectively reviewed in patients undergoing brain MRI following referral for cognitive impairment to a memory clinic between January 2004 and June 2008. Patients with AD were graded into four categories of CVD severity based on neuroradiology reports and assessed for behavioural and NPS and activities of daily living using Neuropsychiatric Inventory (NPI), Geriatric Depression Scale (GDS) and Bristol Activities of Daily Living (BADL). Frontal lobe symptoms and parkinsonian features were also evaluated. Results:, Of the initial 232 patients who underwent MRI 72% were diagnosed with AD. 89% of AD patients had CVD findings reported on MRI. Moderate-to-severe CVD was present in 47% of patients. None of the AD patients satisfied a diagnosis of vascular dementia. There was no significant relationship observed between level of MRI CVD findings and scores on NPI (p = 0.57), GDS (p = 0.26) and BADL (p = 0.46). The level of CVD severity did not appear to influence frontal lobe and parkinsonian assessments (p = 0.60). Conclusion:, The contribution of CVD to the pathogenesis of various NPS is still debated. Our study, based on patients diagnosed with AD in a memory clinic setting, suggests that there is no relationship between the extent of CVD pathology and neuropsychiatric and behavioural measures in AD patients. Further prospective quantitative studies are needed to assess the role of CVD, if any, in neuropsychiatric and behavioural symptoms in AD. It is possible that the relatively small pathological contribution of CVD to the development of these symptoms is obscured by the effect of the wider neurodegeneration encountered in AD. [source] Primary CNS angiitis presenting as short-term memory loss: a case report and literature reviewINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 3 2008Bradley DUHON Abstract Primary angiitis of the central nervous system (PACNS) is an idiopathic vasculitis of the small to medium vessels that often eludes diagnosis because of its variable signs and symptoms. A 36-year-old woman presented with a 4-week history of progressive memory loss. Neurological examination revealed only severe cognitive deficits including anterograde amnesia. Magnetic resonance imaging demonstrated multiple ring-enhancing lesions involving the left frontal lobe and uncus and bilateral thalami. Stereotactic biopsy showed findings consistent with CNS angiitis. Further workup revealed no evidence of systemic disease. We report the first case of biopsy-proven PACNS presenting as profound isolated anterograde amnesia. [source] Unexpected cardiovascular collapse from massive air embolism during endoscopic retrograde cholangiopancreatographyACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 3 2010K. M. GOINS A 72 year-old woman with cholangiocarcinoma presented for endoscopic retrograde cholangio pancreatography (ERCP) for diagnostic intraductal endoscopy under GETA. During the technically difficult procedure the patient became suddenly hypoxic, hypotensive, bradycardic, and progressed to PEA code (ETCO2 5 mmHg). ACLS was initiated. Transesophageal echo demonstrated massive right heart air accumulation; abdominal X-Ray showed air filled bile ducts. Central access was obtained, a pulmonary artery catheter floated, and 30 ml of air aspirated from the RV. Within 5 minutes pulses returned; the patient was transferred to the ICU. MRI revealed two watershed infarcts in the right frontal lobe. The patient fully recovered and returned a month later for an uneventful ERCP. [source] Executive cognitive functioning and the recognition of facial expressions of emotion in incarcerated violent offenders, non-violent offenders, and controlsAGGRESSIVE BEHAVIOR, Issue 5 2007Peter N.S. Hoaken Violence is a social problem that carries enormous costs; however, our understanding of its etiology is quite limited. A large body of research exists, which suggests a relationship between abnormalities of the frontal lobe and aggression; as a result, many researchers have implicated deficits in so-called "executive function" as an antecedent to aggressive behaviour. Another possibility is that violence may be related to problems interpreting facial expressions of emotion, a deficit associated with many forms of psychopathology, and an ability linked to the prefrontal cortex. The current study investigated performance on measures of executive function and on a facial-affect recognition task in 20 violent offenders, 20 non-violent offenders, and 20 controls. In support of our hypotheses, both offender groups performed significantly more poorly on measures of executive function relative to controls. In addition, violent offenders were significantly poorer on the facial-affect recognition task than either of the other two groups. Interestingly, scores on these measures were significantly correlated, with executive deficits associated with difficulties accurately interpreting facial affect. The implications of these results are discussed in terms of a broader understanding of violent behaviour. Aggr. Behav. 33:412,421, 2007. © 2007 Wiley-Liss, Inc. [source] A neuropsychological assessment of frontal cognitive functions in Prader,Willi syndromeJOURNAL OF INTELLECTUAL DISABILITY RESEARCH, Issue 5 2007J. Jauregi Abstract Background Prader,Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects' IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders. [source] Radiation-induced brain disorders in patients with pituitary tumoursJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 3 2004A Bhansali Summary Radiation-induced brain disorders (RIBD) are uncommon and they are grave sequelae of conventional radiotherapy. In the present report, we describe the clinical spectrum of RIBD in 11 patients who received post-surgery conventional megavoltage irradiation for residual pituitary tumours. Of these 11 patients (nine men, two women), seven had been treated for non-functioning pituitary tumours and four for somatotropinomas. At the time of irradiation the age of these patients ranged from 30 to 59 years (mean, 39.4 ± 8.3; median, 36) with a follow-up period of 6,96 months (mean, 18.3 ± 26.4; median, 11). The dose of radiation ranged from 45 to 90 Gy (mean, 51.3 ± 13.4; median, 45), which was given in 15,30 fractions (mean, 18.6 ± 5.0; median, 15) with 2.8 ± 0.3 Gy (median, 3) per fraction. The biological effective dose calculated for late complications in these patients ranged from 78.7 to 180 Gy (mean, 99.1 ± 27.5; median, 90). The lag time between tumour irradiation and the onset of symptoms ranged from 6 to 168 months (mean, 46.3 ± 57.0; median, 57). The clinical spectrum of RIBD included new-onset visual abnormalities in five, cerebral radionecrosis in the form of altered sensorium in four, generalized seizures in four, cognitive dysfunction in five, dementia in three and motor deficits in two patients. Magnetic resonance imaging (MRI)/CT of the brain was suggestive of radionecrosis in eight, cerebral oedema in three, cerebral atrophy in two and second neoplasia in one patient. Associated hormone deficiencies at presentation were hypogonadism in eight, hypoadrenalism in six, hypothyroidism in four and diabetes insipidus in one patient. Autopsy in two patients showed primitive neuroectodermal tumour (PNET) and brainstem radionecrosis in one, and a cystic lesion in the left frontal lobe following radionecrosis in the other. We conclude that RIBD have distinctive but varying clinical and radiological presentations. Diabetes insipidus and PNET as a second neoplastic disorder in adults following pituitary irradiation have not been reported previously. [source] MR Spectroscopy Findings in Lafora DiseaseJOURNAL OF NEUROIMAGING, Issue 4 2009Ebru Altindag MD ABSTRACT PURPOSE Our aim was to investigate the [1H] MR spectroscopy (MRS) findings of Lafora Disease (LD), which is a disabling form of progressive myoclonic epilepsy. METHODS Twelve patients diagnosed with LD and 12 control subjects underwent MRS studies with single-voxels of 8 cc obtained in the frontal lobe, pons, and cerebellum. The metabolites and NAA/Cr, NAA/Cho, Cho/Cr, mI/Cr ratios were calculated. Subgroup analysis was also done between 5 patients with EPM2B and 6 patients with EPM2A mutations. Two investigators scored neurological symptom severity. RESULTS We found a statistically significant difference of NAA/Cho ratio in LD patients compared with normal controls in cerebellum (P= 0.04). In addition, both myoclonus and ataxia scores showed significant correlation with NAA/Cho ratios in the pons (P= 0.03, P= 0.04) and in the cerebellum (P= 0.04, P= 0.01), respectively. CONCLUSION We conclude that the cerebellum is the mostly affected structure in LD and there are significant correlations of MRS findings with some clinical parameters. The differences in the group may be related to different genetic mutations besides disease duration and other clinical variables. MRS studies could provide insights about the severity of the involvement of LD. [source] Greater Activation in Left Hemisphere Language-Related Regions During Simple Judgment Tasks Among Substance-Dependent Patients in Treatment for AlcoholismALCOHOLISM, Issue 2 2010Jodi M. Gilman Background:, Alcoholism is often associated with impaired emotional control. Alcoholics have also been found to have deficits in frontal lobe executive functions. Recent functional imaging studies have suggested that alcoholics show greater activation than nonalcoholics in circuits involving frontal lobes, as well as more posterior brain regions, when engaged in executive-type tasks. In this study, we compared brain activations of alcohol-dependent patients and healthy nonalcoholics while they performed 2 simple judgment tasks designed to activate frontal circuits involved in a basic form of decision making. Participants completed 1 judgment task that required an emotional judgment and 1 task that did not, which enabled us to study whether alcoholics had greater brain activation while performing executive tasks, and to determine if emotional tasks elicited even greater activation than nonemotional tasks. Methods:, We performed functional magnetic resonance imaging scans while alcoholic patients and nonalcoholic controls viewed pictures from the International Affective Picture System. In 3 separate runs, participants viewed the images without making a judgment, determined whether the images were indoor or outdoor scenes, or decided if they liked or disliked the images. Results:, There was little difference in brain activation between alcoholics and controls when no judgment was required. When participants made judgments about either the location or whether they liked or disliked an image, however, we observed significantly increased activation in frontal, limbic, and temporal regions in the patients relative to the controls. Increases were particularly robust in the frontal lobe and in areas of the brain associated with language. When we compared the emotional to the nonemotional judgment, the alcoholics, but not the controls, showed greater activation in the ventral mesial frontal cortex. Conclusions:, Alcoholic patients appear to use brain language areas more than nonalcoholics while making judgments about the setting or liking of emotionally arousing visual images. This increased activation may reflect a compensatory recruitment of brain regions to perform simple decision-making tasks. [source] Magnetic Resonance Imaging Outcomes From a Comprehensive Magnetic Resonance Study of Children With Fetal Alcohol Spectrum DisordersALCOHOLISM, Issue 10 2009Susan J. Astley Background:, Magnetic resonance (MR) technology offers noninvasive methods for in vivo assessment of neuroabnormalities. Methods:, A comprehensive neuropsychological/psychiatric battery, coupled with MR imaging, (MRI), MR spectroscopy (MRS), and functional MRI (fMRI) assessments, were administered to children with fetal alcohol spectrum disorders (FASD) to determine if global and/or focal abnormalities could be identified, and distinguish diagnostic subclassifications across the spectrum. The 4 study groups included: (i) fetal alcohol syndrome (FAS)/partial FAS (PFAS); (ii) static encephalopathy/alcohol exposed (SE/AE); (iii) neurobehavioral disorder/alcohol exposed (ND/AE) as diagnosed with the FASD 4-Digit Code; and (iv) healthy peers with no prenatal alcohol exposure. Presented here are the MRI assessments that were used to compare the sizes of brain regions between the 4 groups. The neuropsychological/behavioral, MRS, and fMRI outcomes are reported separately. Results:, Progressing across the 4 study groups from Controls to ND/AE to SE/AE to FAS/PFAS, the mean absolute size of the total brain, frontal lobe, caudate, putamen, hippocampus, cerebellar vermis, and corpus callosum length decreased incrementally and significantly. The FAS/PFAS group (the only group with the 4-Digit FAS facial phenotype) had disproportionately smaller frontal lobes relative to all other groups. The FAS/PFAS and SE/AE groups [the 2 groups with the most severe central nervous system (CNS) dysfunction] had disproportionately smaller caudate regions relative to the ND/AE and Control groups. The prevalence of subjects in the FAS/PFAS, SE/AE, and ND/AE groups that had 1 or more brain regions, 2 or more SDs below the mean size observed in the Control group was 78, 58, and 43%, respectively. Significant correlations were observed between size of brain regions and level of prenatal alcohol exposure, magnitude of FAS facial phenotype, and level of CNS dysfunction. Conclusions:, Magnetic resonance imaging provided further validation that ND/AE, SE/AE, and FAS/PFAS as defined by the FASD 4-Digit Code are 3 clinically distinct and increasingly more affected diagnostic subclassifications under the umbrella of FASD. Neurostructural abnormalities are present across the spectrum. MRI could importantly augment diagnosis of conditions under the umbrella of FASD, once population-based norms for structural development of the human brain are established. [source] The clinical spectrum of freezing of gait in Parkinson's disease,MOVEMENT DISORDERS, Issue S2 2008Yasuyuki Okuma MD Abstract Freezing of gait (FOG) is a common and very disabling symptom in Parkinson's disease (PD). It is usually observed in the advanced stage of the disease, although a mild form can be seen in the early stage. Although some studies have suggested that longer duration of dopaminergic treatment is associated with FOG, the disease progression alone may be responsible for the development of FOG. FOG can be experienced on turning, in narrow spaces, while reaching a destination, and in stressful situations. In PD, FOG is strongly associated with motor fluctuation. FOG is commonly observed in the "off" state and is observed less frequently in the "on" state. Dual tasking (cognitive load) aggravates FOG. Visual or auditory cues often resolve FOG. Analysis of gait revealed that the stepping rhythm suddenly jumps into high frequency (4,5 Hz) in FOG (hastening), and that floor reaction forces are disregulated. Since the hastening phenomenon was also reported in patients with lesions in the striatum and/or the frontal lobe, fronto-basal ganglia projections are considered essential for FOG. Careful observation and gait pattern analysis may lead to a successful management of individual PD patients with FOG. © 2008 Movement Disorder Society [source] Freezing of gait and executive functions in patients with Parkinson's diseaseMOVEMENT DISORDERS, Issue 3 2008Marianna Amboni MD Abstract Freezing of gait (FOG) is a frequent, disabling symptom of Parkinson's disease (PD). FOG usually lasts a few seconds. It refers to brief paroxysmal events during which a subject is unable to start or continue locomotion. Despite its frequency, FOG pathophysiology is unclear. Because a frontal lobe dysfunction or a disconnection between the frontal lobe and basal ganglia has been implicated in FOG, we explored frontal functions in PD patients using neuropsychological tests. Thirteen early-stage PD patients [Hoehn & Yahr score (H&Y) , 2.5] with freezing during "on " state (FOG+), and 15 age-, H&Y score-, and disease-duration-matched PD patients without freezing (FOG,) were investigated. No patient was demented or depressed. Assessment included the Unified Parkinson's Disease Rating Scale (UPDRS), FOG questionnaire, Mini Mental State Examination (MMSE), frontal assessment battery (FAB), phonemic verbal fluency, Stroop test (parts II and III), and ten-point clock test (TPCT). UPDRS and MMSE scores did not differ between the two groups. FAB, verbal fluency, and TPCT scores were significantly lower in FOG+ patients than in FOG, patients (FAB: P = 0.008; phonemic verbal fluency: P = 0.011; TPCT: P = 0.024). FOG correlated with lower scores at frontal tests in patients with early-stage PD. © 2007 Movement Disorder Society [source] Corticobasal degeneration as cause of progressive non-fluent aphasia: Clinical, radiological and pathological study of an autopsy caseNEUROPATHOLOGY, Issue 6 2006Masaki Takao A Japanese male developed gradual loss of spontaneous speech at age 60. Three years later meaningful speech had deteriorated to the point that it had become restricted to monotonous utterances. Neuropsychological examination at age 62 showed that he had severe non-fluent aphasia. A brain MRI demonstrated mild cortical atrophy with ischemic lesions in the cerebral white matter. He was diagnosed as having primary progressive aphasia. At age 63, he was admitted to the hospital to reevaluate the neurological condition. Neurologic examination showed severe non-fluent aphasia, hyperreflexia, snout and sucking reflexes. No alien hand was observed. He was able to walk, dress, wash himself and use chopsticks as well as name real objects. At age 65, 99mTc-hexamethylpropyleneamine oxime single photon emission computed tomography (HMPAO-SPECT) revealed diffuse cerebral hypoperfusion that was particularly prominent in the left frontal lobe. An MRI showed progressive cortical atrophy with the definite atrophy of the left paracentral gyrus. The hippocampal formation and putamen were also atrophic. He died of pneumonia at age 67. The brain weighed 810 g with atrophy of the frontal lobe, globus pallidus, enlargement of the lateral ventricles and depigmentation of the substantia nigra. Microscopic examination showed severe neuronal loss and gliosis in the cerebral cortex, globus pallidus interna and substantia nigra. Ballooned neurons were observed in the cerebral cortex. Gallyas-Braak method revealed numerous astrocytic plaques and argentophilic threads in the cerebrum. Clinical diagnosis of corticobasal degeneration sometimes is difficult in individuals with atypical clinical presentations. More exact clinical and radiological criteria may warrant a diagnosis of corticobasal degeneration. [source] Leptomeningeal carcinomatosis from urinary bladder adenocarcinoma: A clinicopathological case studyNEUROPATHOLOGY, Issue 1 2005Kaoru Sugimori We report a 73-year-old male patient with leptomeningeal metastasis from urinary bladder adenocarcinoma. He was presented, with, prominent, hyperactive, delirium, during the course of the disease. Meningeal carcinomatosis was detected 5 days before his death, but the primary site of the malignant tumor could not be determined. Necropsy revealed leptomeningeal infiltration of many adenocarcinoma cells that covered the cerebrum. The leptomeninges of the right middle frontal gyrus, superior temporal gyrus, precentral gyrus and inferior parietal lobe were most severely affected by tumor cell infiltration. Cerebral edema was found to extensively cover the basal part of the temporal lobe. In the cerebrum, tumor cells were clustered in the perivascular spaces and had invaded localized areas of the frontal lobe. Vascular cell adhesion molecule (VCAM)-1 expression was detected in the small vessels of the cerebral upper cortical layers and of temporal subcortical u-fibers. Numerous astrocytes positive for cytokeratin AE1/AE3 were found in the frontal and temporal lobes. Meningeal carcinomatosis from urinary bladder adenocarcinoma is extremely rare and up-regulation of the adhesion molecules in the meningeal adenocarcinoma was confirmed. [source] Conservative treatment of L -asparaginase-associated lipid abnormalities in children with acute lymphoblastic leukemia,PEDIATRIC BLOOD & CANCER, Issue 5 2010Hofit Cohen MD Abstract Objective To determine the incidence and clinical consequences of asparaginase-associated lipid abnormalities in children with acute lymphoblastic leukemia (ALL). Methods Sixty-five newly diagnosed children and adolescents aged 0.4,21 years with ALL or lymphoblastic lymphoma were retrospectively evaluated for lipid abnormalities. They were treated according to the ALLIC-BFM 2002 protocol between 2002 and 2005. Fasting cholesterol levels were measured in all patients and triglycerides (TG) in 42/65 patients. Results Prior to treatment, mean cholesterol level was 149,±,50,mg/dl, and increased to maximal level 274,±,124,mg/dl during treatment. Mean TG level during treatment was 459,±,526,mg/dl (range 54,3,009). Twelve patients (28%) had TG levels <200,mg/dl, 18 (43%) had 200,400,mg/dl, 3 (7%) had 400,600,mg/dl, 4 (10%) between 600 and 1,000,mg/dl, and 5 (12%) patients had >1,000,mg/dl. No association was found between TG levels and age or gender. One of the 12 patients with TG >400,mg/dl developed left saggital sinus thrombosis and left frontal lobe infarct. TG level at the time of the event was 2,640,mg/dl. None of the five patients with TG levels >1,000,mg/dl developed pancreatitis. Children with TG levels between 400 and 600,mg/dl were treated by fasting. Fibrates and heparin were added to those with levels >600,mg/dl. Lipid abnormalities normalized in all children upon completion of asparaginase treatment. Conclusions Abnormalities of lipid profile in children with ALL during asparaginase therapy are relatively common. We recommend measuring TG before and during asparaginase treatment. Initiation of conservative treatment could prevent further increase of TG and decrease the risk of potential complications. Pediatr Blood Cancer 2010;54:703,706. © 2010 Wiley-Liss, Inc. [source] Study of image findings in rapid eye movement sleep behavioural disorderPSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 3 2002SHIN-ICHIRO SHIRAKAWA md Abstract To elucidate the cause of idiopathic rapid eye movement (REM) sleep behavior disorder (RBD), magnetic resonance imaging and single-photon emission computed tomography of the brain were conducted on 20 patients with RBD. Blood flow in the upper portion of both sides of the frontal lobe and pons was significantly lower in patients with RBD than in the normal elderly group. Among the patients with RBD, decreased blood flow in the frontal lobe showed no correlation with the extent of frontal lobe atrophy. Decreased blood flow in the upper portion of the frontal lobe and pons might be associated with the pathogenesis of idiopathic RBD. [source] Elbow flexion response as another primitive reflexPSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 2 2002Kazumasa Sudo MD Abstract In daily clinical practice we noticed that patients with intellectual impairment spontaneously flex the elbow within a few seconds of the forearm being manipulated during routine examination of spasticity of the muscles in the upper extremities. We termed this phenomenon elbow flexion response (EFR), and prospectively studied it in 229 patients who underwent in-hospital rehabilitation following brain damage. Evaluation of each patient included EFR, patient profile, ability to communicate, scores on three parameters from various intelligence tests, scores on seven parameters testing primitive reflexes, and scores on three parameters describing personality. We investigated for relationships among these parameters. Consequently, although EFR rarely have a statistical association with the varied profiles of patients, patients with bilateral lesion or bilateral paresis demonstrated significantly more marked EFR than those with unilateral lesion or unilateral paresis. Patients with involvement of the frontal lobe showed significantly more marked EFR than those without damage in this area. Elbow flexion responses occurred significantly more frequently in relation with lower scores on intelligence and occurred with significantly higher frequency in conjunction with the more marked appearance of conventional primitive reflexes. Therefore, we conclude that EFR have a strong association with intelligence and with the existence of frontal lobe lesion, and their mode of clinical presentation parallels that of primitive reflexes particularly that of the grasp reflex. We propose that EFR could be referred as a variation of the grasp reflex occurring in the more proximal or axial part of the body. [source] A study of the relationship between the seizure focus and 1H-MRS in temporal lobe epilepsy and frontal lobe epilepsyPSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 4 2000Senichiro Kikuchi MD Abstract Several studies of temporal lobe epilepsy (TLE) patients have investigated the relationship between the seizure focus and 1H magnetic resonance spectroscopy (1H-MRS). There have also been a few reports in other types of partial epilepsy. We examined the relationship between the seizure focus and the reduction in N -acetylaspartate : creatine (NAA : Cr) ratio using 1H-MRS in both TLE and frontal lobe epilepsy (FLE) patients. We studied 21 patients with unilateral TLE and seven patients with unilateral FLE. We used a 1.5 Tesla magnetic resonance unit (Signa Horizon; General Electric). Approximately 15 × 15 × 20 mm3 voxel of interest (VOI) was placed over the anterior portion of the bilateral hippocampus in the TLE patients, and the anterodorsal position of bilateral frontal lobe in the FLE patients. The seizure focus was identified by interictal scalp electro-encephalogram (EEG). In the TLE patients the NAA : Cr ratios were reduced in the seizure focus, while in the FLE patients they were not always reduced in the seizure focus. In the TLE patients the coincidence rate between the seizure focus and the reduction in the NAA : Cr ratio was 90% (19 of 21 patients), while in the FLE patients the coincidence rate was only 57% (four of seven patients). [source] Chronological progression of a language deficit appearing to be postictally reversible in a patient with symptomatic localization-related epilepsyPSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 2 2000Tatsuya Kudo Abstract A language deficit occurring interictally, with chronological progression, and postictally in a patient with symptomatic localization-related epilepsy, which began at 1.6 years of age, is reported. The patient was a 30-year-old right-handed man whose seizures seemed to originate from the left frontal lobe and to involve the left temporal lobe. The deficit in oral language consisted mainly of features of motor aphasia, including delayed initiation of speech with great effort, echolalic and palilalic tendencies, and word-finding difficulty, but he also showed features of sensory aphasia. Written language had agraphia observed in sensory aphasia, including well-formed letters, paraphasias, neologisms, and paragrammatism. Postictally, the language deficit appeared to be superficially reversible, and evolved from mutism through non-fluent jargon to the interictal level of language. Analysis of the patient's diaries from 10 to 26 years of age disclosed chronologically progressive deterioration of language with paragrammatism, showing an increase of grammatical errors, neologismus, literal and verbal paraphasias and misconstruction of sentences. The results suggest that localization-related epilepsy of presumably left frontal lobe origin causes not only a postictal language deficit but also a slowly progressive deficit of language function. [source] A New Entodiniomorphid Ciliate, Troglocorys cava n. g., n. sp., from the Wild Eastern Chimpanzee (Pan troglodytes schweinfurthii) from UgandaTHE JOURNAL OF EUKARYOTIC MICROBIOLOGY, Issue 2 2010TOSHIHIRO TOKIWA ABSTRACT. Troglocorys cava n. g., n. sp. is described from the feces of wild eastern chimpanzee, Pan troglodytes schweinfurthii, in Uganda. This new species has a spherical body with a frontal lobe, a long vestibulum, a cytoproct located at the posterior dorsal side of the body, an ovoid macronucleus, a contractile vacuole near the cytoproct, and a large concavity on the left surface of the body. Buccal ciliature is non-retractable and consists of three ciliary zones: an adoral zone surrounding the vestibular opening, a dorso-adoral zone extending transversely at the basis of the frontal lobe, and a vestibular zone longitudinally extending in a gently spiral curve to line the surface of the vestibulum. Two non-retractable somatic ciliary zones comprise arches over the body surface: a short dorsal ciliary arch extending transversely at the basis of the frontal lobe and a wide C-shaped left ciliary arch in the left concavity. Because of the presence of three ciliary zones in the non-retractable buccal ciliature, the present genus might be a member of the family Blepharocorythidae, but the large left concavity and the C-shaped left ciliary arch are unique, such structures have never been described from other blepharocorythids. [source] Microvascular lesions in the brain and retina: The age, gene/environment susceptibility,Reykjavik study,ANNALS OF NEUROLOGY, Issue 5 2009Chengxuan Qiu MD Objective To investigate whether the severity and location of cerebral white matter hyperintensities (WMHs) and brain infarcts are correlated with the signs of retinal microvascular abnormalities in the elderly. Methods The study included 4,176 men and women (mean age, 76 years) who participated in the Age, Gene/Environment Susceptibility (AGES),Reykjavik Study. Digital retinal images of both dilated eyes were taken and evaluated for the presence of retinal focal arteriolar signs (focal arteriolar narrowing and arteriovenous nicking) and retinopathy lesions (retinal blot hemorrhages and microaneurysms). Brain magnetic resonance imaging scans were acquired and evaluated for the presence and distribution of cerebral infarcts and WMHs. Logistic and multinomial logistic models were constructed to estimate the association of retinal microvascular signs to brain lesions. Results Controlling for demographic and major cardiovascular risk factors, we found that retinal focal arteriolar signs, but not retinopathy lesions, were significantly associated with an increasing load of subcortical and periventricular WMHs. The strongest association was found between retinal arteriolar signs and a heavier WMH load, specifically in the subcortical frontal lobe, and periventricular frontal and parietal caps. There was a tendency toward bilateral retinal focal arteriolar narrowing being more strongly associated with the heavier load of subcortical WMHs. Arteriovenous nicking was significantly associated with subcortical infarcts. Interpretation In older adults, retinal focal arteriolar signs, but not retinopathy lesions, are correlated with the load of diffuse WMHs, particularly those located in the subcortical frontal lobe, and the periventricular frontal and parietal caps of the brain. Ann Neurol 2009;65:569,576 [source] | ||||||||||||||||||||||||||||||||||