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3761. Deafferentation-disconnection neglect induced by posterior cerebral artery infarction. (Abstract)

. Isolated occipital lesions were rarely associated with hemispatial neglect, and it was only the occipital plus splenial lesion that significantly influenced the frequency and severity of neglect.This study suggests that after excluding such confounding factors as aphasia or hemiplegia, neglect frequency does not differ between the right and left posterior cerebral artery (PCA) groups, but the severity of neglect is greater after right PCA infarctions; even in the acute stage of PCA infarction; visual

2006 Neurology

3762. Patterns of neuropsychological impairment in frontotemporal dementia. Full Text available with Trip Pro

) patients, but these patients made more errors on some tests. Patients with corticobasal degeneration (CBD) and progressive nonfluent aphasia (PNFA) were impaired on tests of working memory. Logistic regression analyses using factor scores successfully assigned FTD subgroups and AD patients into their respective diagnostic categories.Patients with differing frontotemporal dementia phenotypes can be distinguished from each other and from Alzheimer disease using neuropsychological tests.

2007 Neurology

3763. Clinical and psychometric distinction of frontotemporal and Alzheimer dementias. Full Text available with Trip Pro

indicators that distinguish AD from FTLD at initial presentation.Longitudinal study of memory and aging.Alzheimer's Disease Research Center, Washington University School of Medicine.Forty-eight clinically well-characterized cases of autopsy-confirmed FTLD (27 with psychometric testing results) were compared with 27 autopsy-confirmed AD cases.Behavioral abnormalities, particularly impulsivity (P<.001), disinhibition (P<.001), social withdrawal (P = .01), and progressive nonfluent aphasia, distinguished (...) individuals with FTLD from those with AD. The individuals with FTLD performed better than those with AD on a visual test of episodic memory (P = .01), but worse on word fluency (P = .02) (performance correlated with aphasic features). Other cognitive and clinical features, including executive dysfunction and memory impairment, were comparable between the FTLD and AD groups. Concomitant histopathological AD was present in 11 of the 48 individuals with FTLD.Clinical and cognitive features of FTLD may

2007 Archives of Neurology

3764. Asymmetry of long-latency auditory evoked potentials in LGI1-related autosomal dominant lateral temporal lobe epilepsy. (Abstract)

Asymmetry of long-latency auditory evoked potentials in LGI1-related autosomal dominant lateral temporal lobe epilepsy. To investigate auditory processing with cortical long-latency auditory evoked potentials (AEPs) in patients with autosomal dominant lateral temporal lobe epilepsy (ADTLE).Eight patients with LGI1-related ADTLE belonging to a family with predominantly aphasic seizures were studied. Sixty-five individuals without epilepsy served as controls. AEPs (N1-P2 amplitudes) to binaural (...) in N1-P2 AEP amplitudes over the left hemisphere was demonstrated in patients with ADTLE. This finding corresponds to the cardinal symptom of aphasia in this family, and also to the generally prevailing left-sided EEG abnormalities in this condition. The background for this electrophysiologic lateralization in LGI1-related epilepsy is unknown. It may be related to a specific function of LGI1 in the dominant hemisphere.

2005 Epilepsia

3765. Paul Broca's historic cases: high resolution MR imaging of the brains of Leborgne and Lelong. Full Text available with Trip Pro

Paul Broca's historic cases: high resolution MR imaging of the brains of Leborgne and Lelong. In 1861, the French surgeon, Pierre Paul Broca, described two patients who had lost the ability to speak after injury to the posterior inferior frontal gyrus of the brain. Since that time, an infinite number of clinical and functional imaging studies have relied on this brain-behaviour relationship as their anchor for the localization of speech functions. Clinical studies of Broca's aphasia often

2007 Brain

3766. Stimulating language: insights from TMS. Full Text available with Trip Pro

and language functions absent in functional neuroimaging, but also spatial and temporal precision not typically available in patient studies. For instance, TMS has been used to demonstrate functionally distinct sub-regions of the left inferior frontal gyrus; to clarify the relationship between pre-morbid language organization and susceptibility to unilateral lesions and to investigate the contribution of both left and right hemisphere language areas in recovery from aphasia. When TMS is used as a measure (...) of functional connectivity, it demonstrates a close link between action words and motor programmes; it suggests a potential evolutionary link between hand gestures and language and it suggests a role in speech perception for the motor system underlying speech production. In combination with functional neuroimaging, it can elucidate the circuits responsible for this involvement. Finally, TMS may even be useful for enhancing recovery in aphasic patients. In other words, TMS has already become an important

2007 Brain

3767. Language and memory disorder in the case of Jonathan Swift: considerations on retrospective diagnosis. Full Text available with Trip Pro

changes, memory impairment, personality alterations, language disorder and facial paralysis have all been apportioned differing levels of significance in various attempts at retrospective diagnosis. The various medical arguments put forward from the 18th through 20th centuries will be critically examined. The diagnoses considered refer to evolving theories of insanity, phrenology, localization of cortical function, hydrocephalus, psychoanalysis, aphasia, dementia and depression in ageing. Re

2006 Brain

3768. The role of dominant striatum in language: a study using intraoperative electrical stimulations. Full Text available with Trip Pro

The role of dominant striatum in language: a study using intraoperative electrical stimulations. The role of the striatum in language remains poorly understood. Intraoperative electrical stimulation during surgery for tumours involving the caudate nucleus or putamen in the dominant hemisphere might be illuminating.To study the role of these structures in language, with the aim of avoiding postoperative definitive aphasia.11 patients with cortico-subcortical low grade gliomas were operated (...) on while awake, and striatal functional mapping was done. Intraoperative direct electrical stimulation was used while the patients carried out motor and naming tasks during the resection.In five cases of glioma involving the dominant putamen, stimulations induced anarthria, while in six cases of glioma involving the dominant caudate, stimulations elicited perseveration. There was no motor effect. The striatum was systematically preserved. Postoperatively, all patients except one had transient dysphasia

2005 Neurosurgery and Psychiatry

3769. Clinical study of 39 patients with atypical lacunar syndrome. Full Text available with Trip Pro

for 1.8% of all acute stroke patients, 2.1% of acute ischaemic stroke, and 6.8% of lacunar syndromes. ALS included dysarthria facial paresis (n = 12) or isolate dysarthria (n = 9), isolated hemiataxia (n = 4), pure motor hemiparesis with transient internuclear ophthalmoplegia (n = 4), pure motor hemiparesis with transient subcortical aphasia (n = 3), unilateral (n = 2) or bilateral (n = 3) paramedian thalamic infarct syndrome, and hemichorea hemiballismus (n = 2). Atypical lacunar syndromes were due

2006 Neurosurgery and Psychiatry

3770. Is it worth pursuing surgery for epilepsy in patients with normal neuroimaging? Full Text available with Trip Pro

(one hemiplegia caused by haematoma and one mild dysphasia resulting from haemorrhage).It is worth pursuing surgery in patients with normal neuroimaging because it results in good seizure control and the incidence of permanent deficits associated with intracranial studies is low.

2006 Neurosurgery and Psychiatry

3771. Hippocampal atrophy on MRI in frontotemporal lobar degeneration and Alzheimer's disease. Full Text available with Trip Pro

frontotemporal dementia, 13 semantic dementia, and 12 progressive non-fluent aphasia), 103 patients with Alzheimer's disease, and 73 controls were included. Hippocampal volumetry and the easily applicable medial temporal lobe atrophy (MTA) rating scale were applied to assess hippocampal atrophy.Multivariate analysis of variance for repeated measures showed an effect of diagnostic group on hippocampal volume. There was a significant diagnosis by side (left v right) interaction. Both FTLD and Alzheimer's

2006 Neurosurgery and Psychiatry

3772. Metacognitive deficits in frontotemporal dementia. Full Text available with Trip Pro

Metacognitive deficits in frontotemporal dementia. To investigate whether metacognitive impairments in self-awareness and self-monitoring occur in patients with frontotemporal dementia (FTD), particularly among those with prominent social and dysexecutive impairments.Patients diagnosed with FTD were divided by clinical subtype (social-dysexecutive (n = 12) aphasic (n = 15), and constituent subgroups of progressive non-fluent aphasia and semantic dementia) and compared with subjects (...) cognitive test performance adequately, with partial difficulties observed in the socially impaired and progressive non-fluent aphasia subgroups.FTD patients, particularly those with prominent social and dysexecutive impairments, exhibit profound metacognitive anosognosia that may represent a loss of self-awareness, self-monitoring, and self-knowledge, likely related to significant prefrontal pathophysiology. Other FTD clinical groups and AD patients showed less pervasive and more select metacognitive

2005 Neurosurgery and Psychiatry

3773. Oops! Resolving social dilemmas in frontotemporal dementia. Full Text available with Trip Pro

Oops! Resolving social dilemmas in frontotemporal dementia. Our social cognition model posits that social knowledge and executive resources guide interpersonal decision making. We investigated this model by examining the resolution of standardised social dilemmas in patients with a social and executive disorder (SOC/EXEC) caused by frontotemporal dementia (FTD).Patients with SOC/EXEC (n = 12) and those with progressive aphasia (APH, n = 14) completed measures requiring resolution of social

2007 Neurosurgery and Psychiatry

3774. A novel PRNP-P105S mutation associated with atypical prion disease and a rare PrPSc conformation. Full Text available with Trip Pro

conformation, or "PrP(Sc)-type," was determined.We detected a novel mutation at codon 105 of PRNP that results in a serine (S) substitution of proline (P) (P105S), in a young woman who developed progressive aphasia, behavioral changes, dementia, and parkinsonism, lasting 10 years to her death. Histopathologic findings included an intense focus of multicentric PrP-plaques within the hippocampus, punctate plaques scattered throughout the cerebellum, and intense spongiform degeneration focally within

2008 Neurology

3775. Association of GSK3B with Alzheimer disease and frontotemporal dementia. Full Text available with Trip Pro

the surrounding intronic sequence, in patients with FTD, patients with AD, and aged healthy subjects to identify single-nucleotide polymorphisms associated with disease.Single-nucleotide polymorphism frequency was examined in a case-control cohort of 48 patients with probable AD, 102 patients with FTD, 38 patients with primary progressive aphasia, and 85 aged healthy subjects. Results were followed up in 2 independent AD family samples consisting of 437 multiplex families with AD (National Institute of Mental

2008 Archives of Neurology

3776. Frequency and Prognostic Value of Cognitive Disorders in Stroke Patients. (Abstract)

-up.In the post-acute stage, 78% patients were impaired in one or more cognitive domains. The most frequently affected cognitive abilities were attention (48.5%), language (27%), short-term memory (24.5%) and executive functions (18.5%). At the 1-year follow-up, attention deficits were still the most frequent symptom. In contrast, executive dysfunction, aphasia, and long-term memory disorder were significantly less frequent than in the post-acute period. Logistic regression analysis showed that older

2008 Dementia and Geriatric Cognitive Disorders

3777. Spectrum of Pediatric Neuromyelitis Optica. (Abstract)

G antibody titer. Cerebral involvement on MRI was found in all subjects, 5 of whom were symptomatic with encephalopathy, seizures, hemiparesis, aphasia, vomiting, or hiccups. Immunosuppressive therapy reduced attack frequency and progression of disability.Pediatric neuromyelitis optica has a diverse clinical presentation and may be difficult to distinguish from multiple sclerosis in the early stages of the disease. The recognition of the broad spectrum of this disease to include signs

2008 Pediatrics

3778. Stent-Assisted Reconstructive Endovascular Repair of Cranial Fusiform Atherosclerotic and Dissecting Aneurysms. Long-Term Clinical and Angiographic Follow-Up. Full Text available with Trip Pro

showed complete occlusion in 12 and subtotal occlusion in 11 aneurysms, whereas no occlusion was seen in one aneurysm and in all 6 aneurysms treated with stents alone. A clinical improvement or stable outcome was achieved in 25 patients (89%). The 2 cases of permanent morbidity included a patient with a finger dysesthesia associated with a perforator stroke and another patient with hemiparesis and aphasia due to a delayed in-stent thrombosis. One patient died after treatment of a giant

2008 Stroke

3779. Mutations in progranulin (GRN) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia. (Abstract)

for clinical practice. Different clinical variants (ie, behavioural, aphasic, and motor neuron disease variants) are now recognised as part of the clinical spectrum of FTD. Neuropathologically, the disease can be divided into two main pathological subtypes: frontotemporal lobar degeneration (FTLD) with neuronal and glial tau inclusions (FTLD-tau); and FTLD with neuronal inclusions that are positive for ubiquitin (FTLD-U). 20-30% of cases of FTD follow an autosomal dominant pattern of inheritance, and half

2008 Lancet Neurology

3780. Development of methodology for conducting clinical trials in frontotemporal lobar degeneration. Full Text available with Trip Pro

nonfluent aphasia (PNFA), progressive logopenic aphasia (PLA) and semantic dementia (SMD). Patients with one of the four FTLD syndromes were recruited from five academic medical centres over a 2 year period. Standard operationalized diagnostic criteria were used. In addition to clinical inclusion and exclusion criteria, patients were required to exhibit focal frontal, temporal or insular brain atrophy or dysfunction by neuroimaging. Patients underwent neuropsychological, functional, behavioural

2008 Brain

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