Frontal Lobe Involvement Research Articles

Meningioma da Fossa Craniana Anterior Associado a Depressão

Introdução: Meningiomas intracranianos correspondem a 20% dos tumores primários do sistema nervoso central. A localização frontobasal ocorre entre 8% a 15% dos casos. Pode apresentar como manifestação clínica inicial a síndrome de Foster Kennedy (anosmia, atrofia ótica homolateral e papiledema contralateral), transtorno de comportamento por acometimento do lobo frontal (apatia, abulia, depressão), incontinência urinária e crises convulsivas. Relato do caso: Paciente feminino de 49 anos, em tratamento ambulatorial de depressão há quatro anos. Resultado: Exame de ressonância magnética revelou lesão expansiva frontobasal bilateral. Submetida a exérese total da lesão que revelou meningioma meningotelial. Conclusão: As lesões do lobo frontal provocam alterações da personalidade e distúrbios emocionais, o que pode ocasionar um diagnóstico errôneo e tratamento inadequado antes de conhecer a sua verdadeira causa.

Epileptic ictal strabismus: a case report and review of the literature

Ictal strabismus, sometimes associated with epileptic nystagmus, is an extremely rare epileptic phenomenon, suggestive of cortical involvement in monocular eye movement control. We describe a patient with ictal disconjugate contraversive horizontal eye deviation of cortical origin as the main clinical feature of a focal seizure. A 17-year-old, previously healthy woman had a seizure characterized by initial rightward conjugate eye deviation, followed by convergent strabismus due to adduction of the right eye towards the nose without conjugate left eye abduction (esotropia), forced leftward head deviation with impaired awareness, and subsequent evolution into a bilateral tonic-clonic seizure. Postictal and interictal neurological status were unremarkable; more specifically, neuro-ophthalmological examination revealed no nystagmus or altered eye motility. Ictal EEG showed a rhythmic theta activity over the right posterior temporal region, involving fronto-central regions when strabismus appeared. MRI showed cortical dysplasia in the right temporal lobe. Due to the low spatial resolution of scalp EEG, we could not identify with precision the symptomatogenic zone underlying ictal strabismus. However, the concomitant appearance of rhythmic theta activity over the right fronto-central region and the leftward head version with MRI perfusion sequences, showing cerebral blood flow increase in the right frontal eye field area, suggest involvement of the right frontal lobe. [Published with video sequence on www.epilepticdisorders.com].

Fulminant Anti-GAD antibody encephalitis presenting with status epilepticus requiring aggressive immunosuppression

Antibodies against glutamic acid decarboxylase (GAD) are reported in association with numerous neurological conditions including temporal lobe epilepsy and limbic encephalitis. We report a case of Anti-GAD-Antibody associated encephalitis presenting with epilepsia partialis continua (EPC) progressing to a fulminant encephalopathy preferentially affecting the frontal lobes associated with coma and refractory status epilepticus. The abnormalities identified on MRI included marked bilateral frontal lobe involvement which has not been reported in other auto-immune encephalitides and may be specific for Anti-GAD-Antibody associated encephalitis. Similar to the majority of cases of Anti-GAD associated neurological disturbance no underlying malignancy was identified. Treatment with high dose corticosteriods, IVIG and plasmapheresis had minimal response, but escalation of treatment with rituximab and cyclophosphamide was associated with clinical improvement, reducing antibody titers and resolution of MRI changes.

Unusual Aspect of Posterior Reversible Encephalopathy Syndrome in an Elderly Adult

BackgroundPosterior reversible encephalopathy syndrome (PRES) classically occurs in patients presenting with a sudden increase of arterial pressure or eclampsia, but the range of etiologies is very wide. Although the mechanisms underlying PRES remain unclear, research suggests that when the extent of hypertension exceeds the threshold of cerebral blood flow autoregulation, this induces blood–brain barrier disruption that leads to brain edema. Unusual presentations on magnetic resonance imaging (MRI) are possible, including the involvement of frontal or temporal lobes, cerebellar hemispheres, basal ganglia, brainstem, or deep white matter. However, in these atypical locations, edema is mostly accompanied by the classical parieto-occipital region involvement. Case ReportWe report the case of an elderly adult presenting with an unusual presentation of PRES with exclusive involvement of the frontal lobes, associated with severe hypertension. In our case, computed tomography and MRI abnormalities were exclusively localized in the frontal regions without involvement of the classical parieto-occipital regions. A favorable clinical course after hypertension management and complete clearance on an MRI scan at 3 months' follow-up confirmed the diagnosis. Why Should An Emergency Physician Be Aware of This?PRES is a condition potentially encountered by many doctors, especially in the emergency department. However, atypical clinical or imaging presentations are possible and could delay the diagnosis. These must be known to begin adequate treatment as quickly as possible.

T69. Continuous Electroencephalogram (CEEG) as a biomarker for progression of Herpes Simplex Virus Encephalitis (HSE)

Introduction HSE is the most common cause of sporadic fatal encephalitis worldwide and remains a devastating disease despite antiviral therapy. Clinically, it is often characterized by fever, headache, seizures and focal neurologic signs. EEG can be an important tool in the diagnosis of HSV. In the acute stage, EEG can show different EEG patterns, commonly including lateralized periodic discharges (LPDs), sharp waves, focal or generalized slowing. As such, EEG can be used to monitor the progression of the disease in patients with treatment refractory HSE. Methods Case report of a patient with HSE who was monitored on CEEG throughout course of her disease. Results A 62-year old female with no history of neurologic disease, who presented with generalized weakness and lethargy for 2 weeks and rapidly deteriorated with encephalopathy, fever, and seizures. Lumbar puncture demonstrated elevated protein and WBC with positive HSV DNA. CEEG initially showed right prefrontal 2-Hz LPD’s. Over the next 48 h, these evolved into focal right prefrontal status epilepticus. After a period of iatrogenic burst suppression, the CEEG showed right frontotemporal LPD’s which evolved into right frontotemporal electrographic seizures. As the CEEG progressed, the right sided seizures appeared to have a more posterior temporal focus. Later, there was additional independent semi-rhythmic 2–4 Hz delta over the left frontotemporal region which was on the ictal-interictal continuum but did not clearly evolve into seizures. As the recording continued, there were left frontal LPD’s more than right-sided ones. The final CEEG demonstrated bilateral independent sharp waves and generalized periodic discharges (GPD’s) with triphasic morphology with resolution of electrographic seizures. As the CEEG revealed evolution of the underlying disease, treatment was escalated with a 21 day course of acyclovir, multiple anti-epileptic agents, including suppressing medications (i.e., midazolam and ketamine), as well as use of plasma exchange and steroids given the refractory seizures. MRI was obtained at three points during hospital stay and confirmed what CEEG heralded. Initially, there was diffusion restriction of the bilateral (right > left) insular cortices and right hippocampus/amygdala. Repeat MRI showed diffusion normalization with T2-weighted and FLAIR hyperintensity in the right > left insula and new involvement of the right temporal lobe. The final MRI demonstrated involvement of the bilateral inferior frontal regions and left temporal lobe, consistent with evolution from the prior studies and with new findings seen on CEEG, suggesting spread of HSE to these regions. Conclusion We report the case of a patient with HSE who was monitored on CEEG and propose that CEEG can be used as a marker for progression of disease and thus may prompt escalation in therapy. While imaging studies may confirm spread of disease, using CEEG may prevent the delay of treatment and lead to more rapid therapy in cases of refractory HSE.

Spatial Synchronization of Brain Bioelectric Potentials Differs in Boys and Girls Aged 12–13 Years Reading Narrative Texts

The contribution of each cortical zone to the organization of spatial synchronization of brain bioelectric potential s (SSBP) was estimated using cross-correlation matrices of 20-channell EEG during reading in boys and girls aged 12–13 years. Differences in the topical distribution of the SSBP levels in young boys and girls were revealed both in the resting state and during reading. In boys, the SSBP level in the left temporal lobe was lower than that in girls, and in the right parietal lobe it was higher than that in girls in all the states studied. A decrease of SSBP in central cortex zones was revealed, indicating the activation of motor cortical areas in all subjects during reading. Enhancement of SSBP in the parietal regions of the left hemisphere and the occipital zones of both hemispheres is observed in boys during reading, while in girls SSBP is increased bilaterally in the frontotemporal areas with the involvement of the left frontal lobe. Moreover, during reading intrahemispheric correlations between EEGs of the central and temporoparietal regions with emphasis on the right hemisphere were more pronounced in boys, while both intra- and interhemispheric correlations of the left temporal regions were pronounced in girls.

Cognitive and motor outcomes in children with unilateral Sturge–Weber syndrome: Effect of age at seizure onset and side of brain involvement

PurposeMost children with Sturge–Weber syndrome (SWS) develop seizures that may contribute to neurocognitive status. In this study, we tested the hypothesis that very early seizure onset has a particularly detrimental effect on the cognitive and/or motor outcomes of children with unilateral SWS. We also tested whether side of SWS brain involvement modulates the effect of seizure variables on the pattern of cognitive abnormalities. MethodsThirty-four children (22 girls; mean age 6.1years) with unilateral SWS and history of epilepsy in a longitudinal cohort underwent neurological and cognitive evaluations. Global intelligent quotient (GIQ), verbal intelligent quotient (VIQ), nonverbal intelligent quotient (IQ), and motor function were correlated with epilepsy variables, side and extent of brain involvement on magnetic resonance imaging (MRI). ResultsMean age at seizure onset was 1.3years (0.1–6years) and mean IQ at follow-up was 86 (45–118). Age at seizure onset showed a logarithmic association with IQ, with maximum impact of seizures starting before age 1year, both in uni- and multivariate regression analyses. In the left SWS group (N=20), age at seizure onset was a strong predictor of nonverbal IQ (p=0.001); while early seizure onset in the right-hemispheric group had a more global effect on cognitive functions (p=0.02). High seizure frequency and long epilepsy duration also contributed to poor outcome IQ independently in multivariate correlations. Children with motor involvement started to have seizures at/before 7months of age, while frontal lobe involvement was the strongest predictor of motor deficit in a multivariate analysis (p=0.017). ConclusionThese findings suggest that seizure onset prior to age 1year has a profound effect on severity of cognitive and motor dysfunction in children with SWS; however, the effect of seizures on the type of cognitive deficit is influenced by laterality of brain involvement.

A rare treatable cause for atypical frontotemporal dementia with multiple fractures in a young female

Frontal and temporal lobe involvement in young people is seen in infections like neurosyphilis, Vitamin B12 deficiency, NPH, tumors and neurometabolic disorders apart from neurodegenerative dementias. Involvement of other parts of neuraxis in addition to Fronto temporal features is a clue for the specific diagnosis. Cerebro tendinous xantamatosis (CTX) is a rare treatable inborn error of bile acid metabolism. Reported average delay from onset of symptoms to diagnosis is about 16 years as per reports. A patient responds very well to treatment, if diagnosed before significant neurological damage had occurred. 40 year old female had been symptomatic for 11 years with tendon xanthomas, severe osteopenia and multiple fractures. She presented to us with features of Frontal, temporal and cerebellar involvement. Frontal and temporal lobe dysfunction in neuropsychological tests with MRI showing frontal, temporal and cerebellar atrophy. Genetic testing with whole exome sequencing showed TREM2 (-) (ENS0373113) Exon 2 c.377T>G (p.Val126Gly) Homozygous Autosomal recessive. Short stature, tendon xanthomas, cognitive behavioral impairment with severe osteopenia is consistent with the diagnosis of CTX. CTX is a relatively rare and treatable cause for atypical Fronto temporal dementia.

Factors influencing neurocognitive function in patients with neuroepithelial tumors

Though cognitive function is proven to be an independent predictor of survival in patients with intrinsic brain tumors, cognitive functions are still rarely considered. Aim of this study was to assess neurocognitive function and to identify risk factors for neurocognitive deficits. 103 patients with primary neuroepithelial tumors who received tumor resections or biopsies were included in this prospective study. The following data was acquired: mini-mental state examination, preoperative tumor volume, WHO grade, tumor entity and location, and the Karnofsky performance status scale. Furthermore, patients participated in extensive neuropsychological testing of attentional, memory and executive functions. General factors like age, clinical status, WHO grade, tumor volume and tumor location correlated with patients’ neurocognitive functions. Affection of the parietal lobe resulted in significant impairment of attention and memory functions. Frontal lobe involvement significantly affected patients’ abilities in planning complex actions and novel problem solving. Patients with temporal lesions were more likely to have impaired memory and executive functions. Comparing results among neuroepithelial tumor patients enables the identification of risk factors for cognitive impairment. General parameters such as age, KPS score, tumor size, and WHO grade are apart from the respective tumor location of high importance for neurocognitive function.

Systematic Review of Cognitive Function in Euthymic Bipolar Disorder and Pre-Surgical Temporal Lobe Epilepsy.

Bipolar disorder (BD) and temporal lobe epilepsy (TLE) overlap in domains including epidemiology, treatment response, shared neurotransmitter involvement and temporal lobe pathology. Comparison of cognitive function in both disorders may indicate temporal lobe mediated processes relevant to BD. This systematic review examines neuropsychological test profiles in euthymic bipolar disorder type I (BD-I) and pre-surgical TLE and compares experimental designs used. A search of PubMed, PsychINFO, and Scopus using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Inclusion criteria were comparison group or pre- to post-surgical patients; reported neuropsychological tests; participants aged 18-60 years. Fifty six studies met criteria: 27 BD-I; 29 TLE. Deficits in BD-I compared to healthy controls (HC) were in executive function, attention span and verbal memory. Deficits in TLE compared to HC were in executive function and memory. In the pre- to post-surgical comparisons, verbal memory in left temporal lobe (LTL) and, less consistently, visuospatial memory in right temporal lobe (RTL) epilepsy declined following surgery. BD-I studies used comprehensive test batteries in well-defined euthymic patients compared to matched HC groups. TLE studies used convenience samples pre- to post-surgery, comparing LTL and RTL subgroups, few included comparisons to HC (5 studies). TLE studies typically examined a narrow range of known temporal lobe-mediated neuropsychological functions, particularly verbal and visuospatial memory. Both disorders exhibit deficits in executive function and verbal memory suggestive of both frontal and temporal lobe involvement. However, deficits in TLE are measured pre- to post-surgery and not controlled at baseline pre-surgery. Further research involving a head-to-head comparison of the two disorders on a broad range of neuropsychological tests is needed to clarify the nature and extent of cognitive deficits and potential overlaps.

Frontal Assessment Battery in Parkinson's Disease: Validity and Morphological Correlates.

Executive dysfunction is a common feature in Parkinson's disease (PD). However, there is a lack of brief validated instruments for executive dysfunction in PD. The aim of the present study was to assess the relation of Frontal Assessment Battery (FAB) scores to age and education, to verify the utility of FAB in the evaluation of executive dysfunction in PD and to differentiate between controls (n=41), PD patients with normal cognition (PD-NC; n=41; Hoehn and Yahr stages 2-3) and PD with mild cognitive impairment (PD-MCI; n=32; Hoehn and Yahr stages 2-3). In addition, we studied the relation between voxel-based morphometric (VBM) data and FAB results in PD. We found that FAB scores are significantly related to age and education. The FAB has shown discriminative validity for the differentiation of PD-MCI from PD-NC and controls (area under the curve >.80). Also, the VBM analysis revealed lower FAB scores are specifically related to lower gray matter density in the right ventromedial prefrontal areas and precuneus. The FAB can be recommended as a valid instrument for PD-MCI Level I screening. FAB is sensitive to frontal lobe involvement in PD as reflected by lower gray matter density in prefrontal areas. (JINS, 2017, 23, 675-684).

Differential white matter involvement associated with distinct visuospatial deficits after right hemisphere stroke

Visuospatial attention depends on the integration of multiple processes, and people with right hemisphere lesions after a stroke may exhibit severe or no visuospatial deficits. The anatomy of core components of visuospatial attention is an area of intense interest. Here we examine the relationship between the disruption of core components of attention and lesion distribution in a heterogeneous group (N = 70) of patients with right hemisphere strokes regardless of the presence of clinical neglect. Deficits of lateralized spatial orienting, measured as the difference in reaction times for responding to visual targets in the contralesional or ipsilesional visual field, and deficits in re-orienting attention, as measured by the difference in reaction times for invalidly versus validly cued targets, were measured using a computerized spatial orienting task. Both measures were related through logistic regression and a novel ridge regression method to anatomical damage measured with magnetic resonance imaging. While many regions were common to both deficit maps, a deficit in lateralized spatial orienting was more associated with lesions in the white matter underlying the posterior parietal cortex, and middle and inferior frontal gyri. A deficit in re-orienting of attention toward unattended locations was associated with lesions in the white matter of the posterior parietal cortex, insular cortex and less so with white matter involvement of the anterior frontal lobe. An hodological analysis also supports this partial dissociation between the white matter tracts that are damaged in lateralized spatial biases versus impaired re-orienting. Our results underscore that the integrity of fronto-parietal white matter tracts is crucial for visuospatial attention and that different attention components are mediated by partially distinct neuronal substrates.

Mutated CTSF in adult-onset neuronal ceroid lipofuscinosis and FTD.

Objective:To investigate the molecular basis of a Belgian family with autosomal recessive adult-onset neuronal ceroid lipofuscinosis (ANCL or Kufs disease [KD]) with pronounced frontal lobe involvement and to expand the findings to a cohort of unrelated Belgian patients with frontotemporal dementia (FTD).Methods:Genetic screening in the ANCL family and FTD cohort (n = 461) was performed using exome sequencing and targeted massive parallel resequencing.Results:We identified a homozygous mutation (p.Ile404Thr) in the Cathepsin F (CTSF) gene cosegregating in the ANCL family. No other mutations were found that could explain the disease in this family. All 4 affected sibs developed motor symptoms and early-onset dementia with prominent frontal features. Two of them evolved to akinetic mutism. Disease presentation showed marked phenotypic variation with the onset ranging from 26 to 50 years. Myoclonic epilepsy in one of the sibs was suggestive for KD type A, while epilepsy was not present in the other sibs who presented with clinical features of KD type B. In a Belgian cohort of unrelated patients with FTD, the same heterozygous p.Arg245His mutation was identified in 2 patients who shared a common haplotype.Conclusions:A homozygous CTSF mutation was identified in a recessive ANCL pedigree. In contrast to the previous associations of CTSF with KD type B, our findings suggest that CTSF genetic testing should also be considered in patients with KD type A as well as in early-onset dementia with prominent frontal lobe and motor symptoms.

AV-1451 TAU-PET BINDING IN TYPICAL AND ATYPICAL SYNDROMIC VARIANTS OF ALZHEIMER’S DISEASE

Patients with Alzheimer’s disease typically present with episodic memory loss (dementia of the Alzheimer’s type (DAT)). However, 25% can have an atypical presentation dominated by language difficulties (logopenic aphasia (lvPPA)), or visuo-spatial/perceptual deficits (posterior cortical atrophy (PCA)). Tau-PET imaging using AV-1451 demonstrates striking uptake in DAT. It remains unclear whether elevated uptake occurs in atypical AD and whether regional patterns of uptake differ from DAT. We aimed to investigate tau-PET imaging in atypical AD. Tau-PET imaging using AV-1451 was performed in three patients with PCA and three with lvPPA; all six had a positive beta-amyloid PiB-PET scan. These six patients were compared to an age-matched group of 101 controls and 19 patients with DAT. Regional tau-PET uptake was assessed within medial and lateral temporal, parietal, frontal and occipital grey matter regions using the automated anatomical labelling atlas. Compared to controls, elevated tau uptake was observed in all PCA and lvPPA subjects. The PCA subjects all showed greatest uptake in occipital lobe and asymmetric patterns of uptake, with greatest involvement of the right hemisphere. Two PCA subjects showed additional widespread involvement of frontal, temporal and parietal lobes, with more focal and mild patterns observed in the third. The lvPPA subjects all showed left-sided patterns of uptake with the lateral temporal lobes showing striking uptake in two subjects. The third lvPPA subject showed widespread uptake throughout the cortex. Regional patterns of uptake in PCA and lvPPA overlapped with those observed in DAT, although there was a tendency for the left hippocampus to be involved to a greater degree in DAT.

Predictors of Cognitive Functions in Children With Sturge–Weber Syndrome: A Longitudinal Study

BackgroundSturge–Weber syndrome is often accompanied by seizures and neurocognitive deterioration, although previous studies have suggested that early functional brain reorganization may diminish the cognitive sequelae in some children with unilateral Sturge–Weber syndrome. The “rules” governing these plasticity mechanisms are poorly understood. In this study, we evaluated longitudinal changes of cognitive functioning (intelligence quotient [IQ]) and assessed the performance of clinical, electroencephalography (EEG), and magnetic resonance imaging (MRI) variables for predicting IQ in children with Sturge–Weber syndrome. MethodsThirty-three young children (mean age: 3.3 years at baseline) with unilateral Sturge–Weber syndrome underwent MRI, scalp EEG, and neuropsychology evaluation twice, with a median follow-up of 2 years. None of the children had epilepsy surgery. Longitudinal IQ changes were calculated. Seizure variables, interictal EEG abnormalities, and extent and location of MRI brain involvement were correlated with IQ assessed at follow-up. ResultsGlobal IQ showed a highly variable course with both increases and decreases over time. Lower IQ at baseline was associated with interval IQ increase. In univariate analyses, lower outcome IQ was associated with baseline EEG abnormalities (P < 0.001), young age at seizure onset (P = 0.001), high seizure frequency (P = 0.02), and early frontal-lobe involvement on MRI (P = 0.01). In multivariate analysis, EEG abnormalities at baseline remained a robust, independent predictor of outcome IQ. ConclusionsThe early trajectory of cognitive changes in children with unilateral Sturge–Weber syndrome is highly variable; children with improving IQ likely undergo effective unimpeded functional reorganization. Early onset, frequent seizures, and interictal epileptiform abnormalities on EEG likely interfere with this process resulting in poor cognitive functions. Future studies assessing interventions should target this high-risk subgroup to optimize cognitive outcome in Sturge–Weber syndrome.

Progressive dysphasic dementia with bucco-facial apraxia: a case report.

A patient with progressive dementia, prominent non-fluent aphasia and signs of frontal lobe involvement, was evaluated by neuropsychological testing, magnetic resonance imaging (MRI) and high resolution single photon emission tomography (SPET). The presence of severe bucco-facial apraxia, associated with spared imitation of limb movements, correlated well with a marked reduction of cerebral perfusion in the left fronto-temporal cortex. This case emphasizes the usefulness of SPET as a valuable alternative to PET for the diagnosis of conditions, such as progressive neuropsychological syndromes, where a coupled reduction of metabolism and blood flow can be expected.

Acute seizures in cerebral venous sinus thrombosis: What predicts it?

BackgroundSeizures are the presenting feature of cerebral venous sinus thrombosis (CVST) in 12–31.9% of patients. 44.3% of patients have seizures in the early stage of the disease. Acute seizures (AS), refers to seizures which take place before the diagnosis or during the first 2 weeks afterward. ObjectiveTo report the predictors of acute seizures in cerebral venous sinus thrombosis (CVST). Methods100 patients with CVST were included in the study. The occurrence of acute seizures was noted. The predictors of acute seizure were evaluated by univariate analysis including the demographic (gender, age), clinical (headache, focal neurological deficit, papilloedema, GCS score), type and number of risk factors, MRI findings (Type of lesion: hemorrhagic infarction or hematoma, location of lesion) and MRV findings (superficial or deep sinus, cortical veins). ResultsA total of 46 patients had acute seizures. On univariate analysis, altered mental status (p<0.001), paresis (p=0.03), GCS score <8 (p=0.009), hemorrhagic infarct on imaging (p=0.04), involvement of frontal lobe (p=0.02), superior sagittal sinus (p=0.008), and high D-dimer levels (p=0.03) were significantly associated with acute seizure. On multivariate analysis, the hemorrhagic infarct on MRI and high D-dimer was independently predictive for early seizure. ConclusionThe predictive factors for the acute seizures are altered mental status (GCS<8), focal deficits, hemorrhagic infarct, involvement of frontal lobe and superior sagittal sinus with high D-dimer levels.

Neuropsychological profile in early Parkinson's disease: Comparison between patients with right side onset versus left side onset of motor symptoms.

Aims:Though impaired cognition in Parkinson's disease (PD) is well known, data in early PD is sparse. This study was designed to assess the cognitive profile in patients with early PD (motor symptoms <5 years and Hoehn and Yahr stage <2), and to compare the cognitive profile between these patients with right versus left side onset of motor symptoms.Materials and Methods:National Institute of National Health and Neurosciences (NIMHANS) neuropsychological battery was used to assess the cognitive profile in 50 patients with early PD and compared with 50 age-, education-, and gender-matched healthy controls. Within the PD group, the cognitive profile was also compared between patients with right side onset motor symptoms (RPD) versus those with left side onset (LPD). The neuropsychological tests assessed the executive functions, memory, attention, visuospatial functions, and psychomotor speed.Results:Among the 50 patients, 25 each were RPD and LPD. The two subgroups were matched for age, gender, education, age at disease onset, disease duration, and degree of motor disability. There was no significant difference between the groups on Hoehn and Yahr staging or Unified Parkinson Disease Rating Scale (UPDRS) motor score. Patients with early PD performed significantly worse in the tasks involving memory, executive functions, and attention compared to controls. However, there was no difference in the cognitive profile between RPD and LPD subgroups.Conclusions:Patients with early PD have cognitive dysfunction with predominant involvement of frontal and temporal lobes. Side of onset of motor symptoms probably does not have significant role in future development or profile of cognitive dysfunction in PD.

右前頭葉内側面の発話・書字への関与

前頭葉内側面損傷はさまざまな言語障害を生じうる。超皮質性運動失語を代表とする失語のほか, 吃音や発話開始困難などのより基礎的な音声表出そのものの障害や, まれに失書の報告もみられる。今回の発表では, 前補足運動野を含む右前頭葉内側面損傷により一過性の発話開始困難と漢字の失書を呈した自験例を通して, 発話と書字に対する前頭葉内側面の関与を検討した。本例の発話の開始困難は一過性であったため, その病巣が発話開始に関わる中枢部位を含んでいたと主張することはできない。過去の報告において, 左前頭葉内側面損傷では発話障害の症状が持続するが, 右半球損傷では症状が持続しないことから, 発話に関与する前頭葉内側面の機能が側性化している可能性が示唆される。一方, 本例の漢字の失書は持続した。右前頭葉内側面損傷による失書は過去に報告されていない。本例の書字の成否は, 漢字の画数や使用頻度に依存せず, 構成要素 (偏や旁など漢字を構成する要素的単位) の数が増すほど書字が困難であり, その誤りの特徴は構成要素の置換であった。これらより, 右前頭葉内側面は漢字書字を実現する過程の終盤の段階で, 構成要素の選択・配列に関与している可能性が示唆される。

A clinicopathological study of primary central nervous system lymphomas & their association with Epstein-Barr virus.

Background & objectives:Primary central nervous system lymphomas (PCNSLs) are relatively uncommon, accounting for 2-3 per cent of primary brain tumours. Majority of these are diffuse large B cell lymphomas (DLBCL) occurring both in immunocompromised and immunocompetent patients. We undertook this study to classify PCNSL into germinal centre (GC) and non-germinal centre (NGC) type based on Hans classification and to find the role of Epstein-Barr virus (EBV) in pathogenesis both by conventional immunohistochemistry (IHC) and chromogenic in situ hybridization (CISH).Methods:The consecutive cases of PCNSL during a 10 years period were analysed by IHC for CD45, CD20, CD3, B-cell lymphoma 2 and 6 (Bcl-2 and Bcl-6), B-cell specific octamer binding protein-1 (BOB-1), multiple myeloma oncogene-1 (MUM-1), EBV latent-membrane protein 1 (LMP-1), cyclin-D1, CD10, CD5 and CD23, as well as by CISH for EBV.Results:During a period of 10 years, 65 PCNSL were diagnosed which comprised 0.69 per cent (65/9476) of all intracranial tumours. The mean age of presentation was 49 yr with sex ratio (M:F) of 1.4:1. Most common location was supratentorial region with predominant involvement of frontal lobe. Single lesions were seen in 38 (58.4%) and multifocal lesions in 27 (41.5%) patients. None of the patients were immunocompromised. All cases were B cell immunophenotype and were DLBCL except one case of follicular lymphoma. According to Hans classification, majority of them were NGC (n=51, 79.6%) and 13 (20.3%) were GC type. Bcl-2 expression was noted in 34 (52.3%) tumours. EBV was positive in three (4.6%) cases; two were detected both by IHC and CISH and one case by CISH only.Interpretation & conclusions:In Indian population, PCNSL occurs mainly in immunocompetent patients, and a decade earlier than in western population. Immunophenotyping revealed that all cases were DLBCL with predominance of NGC type. No prognostic difference was seen between GC and NGC DLBCL. Association of EBV was rare and this virus was possibly not involved in the pathogenesis of PCNSL in immunocompetent individuals. CISH was an easy, economical and less cumbersome method for detection of EBV in PCNSL.