1/141. Relearning and subsequent forgetting of semantic category exemplars in a case of semantic dementia.Over 15 months of longitudinal assessment, a patient with semantic dementia, D.M., improved on tests of naming and category fluency for a specific set of items (Experiment 1). The authors attribute this to his home drill with the names of these concepts plus pictures and descriptions of them. In Experiment 2, D.M. produced significantly more exemplars on category fluency for semantic categories that he had been practicing at home than for nonpracticed categories, an effect that cannot be attributed to an inherent difference between the 2 sets because the fluency performance of control participants revealed no significant difference between the 2 sets. In Experiment 3, D.M. rehearsed some of his previously nonpracticed categories daily for a period of 2 weeks: His fluency scores on the experimental categories improved substantially, but they declined once he ceased the daily drill. The results are discussed with respect to current views of long-term memory, particularly new word learning and forgetting, and to current techniques for facilitating word finding in aphasia.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
2/141. adult-type metachromatic leukodystrophy with a compound heterozygote mutation showing character change and dementia.A 26-year-old Japanese woman slowly developed a change of character such as hypospontaneity and blunted affect, followed by obvious mental deterioration. She was diagnosed as having a disorganized type of schizophrenia at the first examination. brain magnetic resonance imaging demonstrated diffuse high intensity in the cerebral white matter, particularly in the frontal lobes. The single photon emission computed tomography images using 123I-IMP disclosed diffuse cerebral hypofusion, especially in the frontal lobes. Electroencephalogram showed a moderate amount of 5-6Hz theta waves on the background of alpha activity. Nerve conduction velocities in the extremities were delayed. The level of leucocyte arylsulphatase was low. In the arylsulphatase A gene analysis, a compound heterozygote having the 99Gly-->Asp and 409Thr-->Ile mutations was confirmed. The patient was diagnosed as having metachromatic leukodystrophy. She gradually showed obvious dementing symptoms such as memory disturbance and disorientation. The characteristics of the psychiatric symptoms in the leukodystrophy are discussed.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
3/141. Early thalamic and cortical hypometabolism in adult-onset dementia due to metachromatic leukodystrophy.A case of early-onset adult dementia with family history of dementia is reported, characterised by neuropsychological deficits, suggesting frontal involvement, with mild non specific white matter abnormalities on CT scan. Familial Alzheimer's disease was suspected but the neuropathological diagnosis on brain biopsy was metachromatic leukodystrophy. 18FDG-PET revealed a very peculiar pattern of metabolic impairment in thalamic areas, in medial and frontopolar regions, and in occipital lobes. Neuropsychological follow-up showed relatively stable difficulties of long-term memory and signs of frontal lobe dysfunction, similar to those observed in subcortical dementias. MRI subsequently showed periventricular leukoencephalopathy. The brain metabolic pattern observed in that case of metachromatic leukodystrophy was quite different from that reported in other types of dementia.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
4/141. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies.Three cases are reported with dementia and ubiquitin-positive but tau-negative inclusion bodies. All patients had a semantic dementia and the clinical details of two of these have been published as the first description of a selective semantic memory impairment. The original diagnosis had been of Pick's disease based on frontotemporal atrophy, but re-examination has revealed ubiquitin-positive but tau-negative inclusions as well as neurites in the frontotemporal cortices and ubiquitin-positive, intracytoplasmic inclusions in the granule cells of the dentate fascia. These inclusions are identical to those reported in association with amyotrophic lateral sclerosis (motor neuron disease), but none were seen in brainstem or spinal cord motor neurons.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
5/141. The impact of semantic memory impairment on spelling: evidence from semantic dementia.We assessed spelling and reading abilities in 14 patients with semantic dementia (with varying degrees of semantic impairment) and 24 matched controls, using spelling-to-dictation and single-word reading tests which manipulated regularity of the correspondences between spelling and sound, and word frequency. All of the patients exhibited spelling and reading deficits, except at the very earliest stages of disease. Longitudinal study of seven of the patients revealed further deterioration in spelling, reading, and semantic memory. The performance of both subject groups on both spelling and reading was affected by regularity and word frequency, but these effects were substantially larger for the patients. Spelling of words with exceptional (or more precisely, unpredictable) sound-to-spelling correspondences was most impaired, and the majority of errors were phonologically plausible renderings of the target words. reading of low frequency words with exceptional spelling-to-sound correspondences was also significantly impaired. The spelling and reading deficits were correlated with, and in our interpretation are attributed to, the semantic impairment.- - - - - - - - - - ranking = 5keywords = memory (Clic here for more details about this article) |
6/141. Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings.Familial British dementia with amyloid angiopathy (FBD) is an autosomal dominant condition characterized by a dementia, progressive spastic tetraparesis and cerebellar ataxia with onset in the sixth decade. A point mutation in the BRI gene has been shown to be the genetic abnormality. Genealogical work with the large family originally reported by Worster-Drought and updated by Plant has identified nine generations dating back to the late eighteenth century. The pedigree now includes six living affected patients, 35 historical cases, and 52 descendants at risk of having inherited the disease. A common ancestor has been identified between the large pedigree and a case report of 'familial cerebellar ataxia with amyloid angiopathy'. An autopsy case from a separate family with an identical condition is described but no common ancestor with the large pedigree has been found. Case histories have been researched and updated in each pedigree. Eleven individuals at risk of FBD, aged between 44 and 56 years, agreed to undergo a clinical and neuropsychological assessment along with MRI brain imaging in order to clarify early diagnostic features. Five of the eleven were thought to show early clinical signs of the disease. Neurological examination was abnormal in three, with limb and gait ataxia and mild spastic paraparesis. Three had impaired recognition and recall memory and another had mild impairment of delayed visual recall. All affected individuals had an abnormal MRI of the brain, consisting of deep white-matter hyperintensity (T(2)-weighted scans) and lacunar infarcts, but no intracerebral haemorrhage. The corpus callosum was affected particularly, and in one patient it was severely atrophic.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
7/141. Two brothers with frontotemporal dementia and parkinsonism with an N279K mutation of the tau gene.OBJECTIVE: To characterize the clinical diagnostic features, neuropathologic phenotype of tau deposition, and subunit structure of tau filaments in patients who had an asparagine-to-lysine substitution at codon 279 (the N279K missense mutation) of the gene for microtubule-associated tau protein. BACKGROUND: The N279K mutation is a causative genetic defect for pallidopontonigral degeneration in an American kindred that presents with frontotemporal dementia (FTD) and parkinsonism. methods: The authors analyzed retrospectively the clinical symptoms of two Japanese brothers who carry this mutation. Postmortem neuropathologic and electron microscopic studies, and Western blot analysis of insoluble tau were performed to correlate tau-mediated lesions with neurologic deficits. RESULTS: Both patients exhibited impairment in recent memory, parkinsonism, and corticospinal disturbances in addition to FTD. Parkinsonism in one patient was responsive temporarily to l-dopa. There was intense tau deposition in the medial temporal cortices and upper and lower motor neurons with accompanying corticospinal tract degeneration. Two distinct tau isoforms with four microtubule-binding repeats, in hyperphosphorylated forms, were the primary constituents of insoluble tau, which aggregated to the filamentous component, termed "paired tubules," in neurons, oligodendrocytes, and astrocytes. The elemental filaments were hollow tubules measuring 11 to 12 nm in diameter, two of which adhered to each other along their longitudinal axes to form "paired tubules." CONCLUSIONS: Early memory loss and pyramidal signs, which are atypical of FTD, can be presenting symptoms in this disorder. The authors demonstrated that the subunit structure of tau filaments is a pair of hollow tubules despite the prevailing twisted ribbon model.- - - - - - - - - - ranking = 2.5940005800835keywords = memory, memory loss (Clic here for more details about this article) |
8/141. Neuropsychological sequelae of heat stroke: report of three cases and discussion.heat stroke includes neurologic impairment as a person's body temperature reaches 40.5 degrees C (105 degrees F) as a result of a failure of thermoregulation. The physiologic complications of heat stroke are well described in the literature. The domains of cognitive functioning affected and the severity of impairment resulting from heat stroke can vary from mild deficits in attention and memory to severe global dementia. There can also be changes in affect and personality that are equally debilitating. This article presents the neuropsychological test results of three active duty soldiers who suffered heat stroke and were tested within 2.5 months of injury.- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
9/141. Confabulation in a patient with fronto-temporal dementia and a patient with Alzheimer's disease.This paper describes two patients, O.I. and B.Y., with a confabulatory syndrome. O.I. was diagnosed with probable fronto-temporal dementia, whereas B.Y. met the criteria for probable Alzheimer's disease. O.I., but not B.Y., was impaired on tests of frontal/executive functions, and performed better than B.Y. on clinical tests of memory. Both patients confabulated in episodic/autobiographical memory tasks and in personal future planning tasks. B.Y. confabulated also in a semantic memory task. It is argued that the pattern of confabulation and the cognitive profile shown by the two patients is explained better by the hypothesis proposed by Dalla Barba and co-workers (Dalla Barba et al., 1997b) than by current theories of confabulation.- - - - - - - - - - ranking = 3keywords = memory (Clic here for more details about this article) |
10/141. Dementia with lewy bodies: a pure case.A pure case of autopsy-confirmed dementia with lewy bodies (DLB) is described. The patient presented with distinctive verbal fluency deficits in the context of mild language impairment, intact recognition memory, and impaired paragraph recall. neuroimaging (CT and SPECT) showed progressive medial temporal lobe atrophy. Neuropathology revealed lewy bodies, degeneration in the substantia nigra, nucleus basalis of Meynert (Nakano & Hirano, 1984), and locus ceruleus, but no pathology characteristic of Alzheimer's disease. It is in this sense that the case is "pure" DLB. Early neuropsychological diagnosis of DLB is essential (salmon et al., 1996) given the potentially fatal hazard of neuroleptics (McKeith et al., 1992) and the difficulties associated with clinical neurological diagnoses (Litvan et al., 1998).- - - - - - - - - - ranking = 1keywords = memory (Clic here for more details about this article) |
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