11/71. Extensive metabolic and neuropsychological abnormalities associated with discrete infarction of the genu of the internal capsule.OBJECTIVE: The clinical presentation of capsular genu infarct varies. Prominent faciolingual weakness and subcortical dementia are the rule, but symptoms depend on the precise location and extension of the lesion beyond the genu. The aim was to characterise the radiographic, electroencephalographic, and neuropsychometric abnormalities in a woman who had a history of recurrent transient memory loss. METHOD: Case report. RESULTS: magnetic resonance imaging showed an infarct in the genu of the left internal capsule. Positron emission tomography scan demonstrated decreased metabolic activity in the ipsilateral temporal, occipitotemporal, and contralateral cerebellar hemispheres. electroencephalography showed intermittent rhythmic delta activity in the left frontotemporal region, and findings on neuropsychometric evaluation were consistent with cognitive impairment. Follow up evaluation 7 months after the stroke showed improvement in some areas of the cognitive domain, but residual neuropsychometric and neurophysiological abnormalities persisted. CONCLUSION: This case illustrates that cerebral and cerebellar diaschisis may contribute to the symptomatic presentation and recovery from capsular genu infarct, although its precise role remains elusive.- - - - - - - - - - ranking = 1keywords = dementia, frontotemporal (Clic here for more details about this article) |
12/71. 11: disorders of memory and intellect.The clinical approach to the patient with a suspected disorder of memory and intellect is to establish whether it is dementia, which parts of the brain are affected, what is the cause, what is the prognosis, and what can be done about it. The diagnosis of dementia usually requires the involvement of memory and at least one other cognitive system. delirium and depression are important differential diagnoses. patients with dementia should usually have some simple investigations after a careful history-taking and examination to identify "reversible" causes. The commonest cause of dementia is Alzheimer's disease, in which short-term memory disturbance is usually prominent. Other causes of dementia include cerebrovascular disease, Lewy-body disease and Pick's disease. There is now hope for patients with Alzheimer's disease (which can be treated with some success with cholinesterase inhibitors) and patients with vascular dementia, in whom aggressive control of causal risk factors may retard progression.- - - - - - - - - - ranking = 3.52469662259keywords = dementia (Clic here for more details about this article) |
13/71. Is surface dysgraphia tied to semantic impairment? Evidence from a case of semantic dementia.Recent models of lexical processing suggest that written spelling partly depends upon the semantic system. According to these models, a deterioration in word meanings would necessarily lead to a spelling impairment, especially for low-frequency and orthographically irregular words. We report a case study of a patient (MK) with semantic dementia who presents impairments in knowledge of word meanings and surface dysgraphia. By specifically identifying concepts that are still known or that are partially or completely deteriorated, we show that the patient's spelling deficit is highly correlated with word comprehension. These data confirm that surface dysgraphia is directly linked to the breakdown in semantic memory.- - - - - - - - - - ranking = 2.9372471854916keywords = dementia (Clic here for more details about this article) |
14/71. Creutzfeldt-Jakob disease associated with a deletion of two repeats in the prion protein gene.A two-octapeptide repeat deletion of the prion protein gene has been recently observed in a patient with a 2-year history of dementia and a clinical diagnosis of possible Creutzfeldt-Jakob disease (CJD). The authors report a similar deletion in a patient with a definitive diagnosis of CJD. Since the two-repeat deletion has not been observed in large, population-based studies, the two cases suggest that this deletion is a new pathogenic mutation associated with CJD.- - - - - - - - - - ranking = 0.58744943709833keywords = dementia (Clic here for more details about this article) |
15/71. Neuropsychological testing following head injuries: prosopagnosia without visual field defect.Assessment of residual cerebral dysfunction in the post-traumatic patient poses considerable problems particularly when the neurological examination yields minimal or equivocal findings. The clinical picture is frequently complicated by emotional disturbance not easily differentiated from "post-traumatic neurosis." This report describes such a patient whose disorder was elicidated by neuropsychological testing. Numerous studies have established the validity of neuropsychological tests particularly when they are interpreted by psychologists specifically trained in their use. These procedures are also useful in differentiating patients with neurologic complaints of a nonorganic etiology from patients with similar complaints secondary to confirmed brain lesions. Although the findings reported here pertain to a patient exhibiting a rare neurologic consequence of closed head injury, the methods employed are applicable to subtle behavioral manifestations of diverse etiologies. Neurophyshological findings in a patient unable to recognize faces of familiar persons (prosopagnosia) disclosed a severe impairment of visual perception despite intact visual acuity and fields. The prosopagnosia was also associated with a pervasive memory deficit without dementia. Our results challenge current concepts of prosopagnosia and support the need for neuropsychological evaluation of post-traumatic patients.- - - - - - - - - - ranking = 0.58744943709833keywords = dementia (Clic here for more details about this article) |
16/71. Neuroradiologic and clinical abnormalities in dementia of diffuse neurofibrillary tangles with calcification (Kosaka-Shibayama disease).We describe a characteristic dementia patient diagnosed as diffuse neurofibrillary tangles with calcification (DNTC). Neuropsychologically, dementia, including a decline in memory retention and intelligence, and anomic aphasia were recognized. Imaging revealed circumscribed temporal dominant atrophy and calcification of the basal ganglia and cerebellum. SPECT and FDG-PET revealed a remarkable reduction of blood flow and metabolism in the temporal lobes; however, there is no reduction in the basal ganglia and cerebellum, and FDOPA-PET also disclosed no abnormalities. This suggests that calcification and neuronal degeneration occur independently in DNTC.- - - - - - - - - - ranking = 3.52469662259keywords = dementia (Clic here for more details about this article) |
17/71. Presenilin 1 mutation in an african american family presenting with atypical Alzheimer dementia.BACKGROUND: alzheimer disease (AD) is characterized by memory and visuospatial deficits with relative sparing of personality. Mutations in 3 genes (presenilin 1 and 2 and amyloid precursor protein) are associated with presenile AD. Presenilin 1 gene mutations have not been described in african americans. methods: We studied an African American family with autosomal dominant rapidly progressive dementia and psychosis occurring early in the fifth decade of life. We performed neurologic evaluations, psychometrics, and neuroimaging. We sequenced the amyloid precursor protein gene, presenilin 1 and 2, and tau in affected and unaffected family members. One patient underwent a brain biopsy and subsequent autopsy. RESULTS: personality change, auditory and visual hallucinations, delusions, memory impairment, word-finding difficulties, and subsequent rigidity, dystonia, myoclonus, and mutism developed in 2 brothers. Neuropsychometric testing in one was consistent with frontotemporal dementia or atypical AD. neuroimaging studies showed diffuse cortical involvement. A clinical diagnosis of familial non-Alzheimer dementia was made. However, results of temporal lobe biopsy in one revealed amyloid neuritic plaques, and autopsy results confirmed the diagnosis of AD. Gene sequencing revealed a presenilin 1 point mutation (M139V) cosegregating with the disease. A tau polymorphism in exon 7 (A178T) was found in an affected brother and unaffected relatives. CONCLUSIONS: We report the first documented presenilin mutation in African American patients presenting with early personality change, psychosis, and memory loss with preserved praxis. The M139V mutation can present differently between kindreds, with some features suggestive of a frontal lobe syndrome. The M139V mutation can lead to atypical AD, and genetic background may have a role in determining the phenotype of genetically defined AD.- - - - - - - - - - ranking = 7.6442342503363keywords = frontotemporal dementia, dementia, frontotemporal (Clic here for more details about this article) |
18/71. The neuropsychological profile associated with variant Creutzfeldt-Jakob disease.We report the neuropsychological profile associated with variant Creutzfeldt-Jakob disease (vCJD). A retrospective study was carried out of data from neuropsychological reports prepared on 24 patients with vCJD. While there was some variability in neuropsychological profiles, the overall pattern was one of a combined cortical and subcortical dementia, with impaired performance being particularly prominent on tests of memory, executive function, speed of attention, and visuoperceptual reasoning. Across 16 cases where Wechsler adult intelligence Scale-Revised intelligence quotient (IQ) scores were available, this profile was in part reflected by an invariably low performance IQ (<90 in all patients). All patients who received tests of verbal fluency, digit-symbol substitution and faces recognition memory showed deficits on these tests. Basic vocabulary, digit span and verbal reasoning skills were relatively preserved in most patients. In four cases who underwent more detailed cognitive testing, additional observations were made of relatively intact long-term autobiographical memory and faces perception. Cognitive impairment may represent one of the earliest features of vCJD and it is possible that, at least in some cases, neuropsychological deficits precede the onset of psychiatric or neurological symptoms. Our findings may help in the early detection and management of patients with vCJD.- - - - - - - - - - ranking = 0.58744943709833keywords = dementia (Clic here for more details about this article) |
19/71. confusion and memory loss from capsular genu infarction: a thalamocortical disconnection syndrome?We examined six patients with an abrupt change in behavior after infarction involving the inferior genu of the internal capsule. The acute syndrome featured fluctuating alertness, inattention, memory loss, apathy, abulia, and psychomotor retardation, suggesting frontal lobe dysfunction. Contralateral hemiparesis and dysarthria were generally mild, except when the infarct extended into the posterior limb. Neuropsychological testing in five patients with left-sided infarcts revealed severe verbal memory loss. Additional cognitive deficits consistent with dementia occurred in four patients. A right-sided infarct caused transient impairment in visuospatial memory. Functional brain imaging in three patients showed a focal reduction in hemispheric perfusion most prominent in the ipsilateral inferior and medial frontal cortex. We infer that the capsular genu infarct interrupted the inferior and anterior thalamic peduncles, resulting in functional deactivation of the ipsilateral frontal cortex. These observations suggest that one mechanism for cognitive deterioration from a lacunar infarct is thalamocortical disconnection of white-matter tracts, in some instances leading to "strategic-infarct dementia."- - - - - - - - - - ranking = 1.1748988741967keywords = dementia (Clic here for more details about this article) |
20/71. Two further investigations of autobiographical memory in semantic dementia.A number of investigations in semantic dementia have documented better retrieval of recent personal events compared to those in the more distant past (Graham and Hodges, 1997). Westmacott et al. (2001) challenged this result, however, finding relative preservation of remote memories in a single case of semantic dementia when he was tested using family photographs. In Experiment 1, we tested two possible explanations for the discrepancy between the published papers: (a) that there is a significant effect of modality in autobiographical retrieval in semantic dementia (e.g., patients will show better, or even preserved, remote memory when tested on nonverbal, compared to verbal, tasks); and (b) that the distinct pattern seen between patients is attributable to the different methods adopted for scoring the episodic quality of the memories. A patient with semantic dementia, AM, produced autobiographical memories to both words and family photographs. These personal events were scored by two raters using the scoring method described by Westmacott et al. (2001) and that reported by Graham and Hodges (1997). It was found that AM showed similar levels of remote memory impairment regardless of whether the cue was verbal or nonverbal. In addition, significant effects of time were revealed in AM's performance on the verbal memory test, regardless of which method was used to score the memories. In Experiment 2, we investigated a related question: whether the relatively better retrieval of memories in semantic dementia could be due to over rehearsal of highly salient recent experiences? Four patients were tested on their ability to remember a recent event (either a trip to london or events from a Formula One Grand Prix race) using specially designed autobiographical questionnaires. It was found that all four were able to perform this task, although one patient showed evidence of some forgetting over three months, and another exhibited difficulty discriminating between two similar events. Together, the two experiments further confirm that patients with semantic dementia show a modality independent autobiographical memory deficit, with better retrieval of recent events.- - - - - - - - - - ranking = 5.8744943709833keywords = dementia (Clic here for more details about this article) |
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