Cases reported "Memory Disorders"

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1/26. early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?

    OBJECTIVE: To examine the role of structural (magnetic resonance imaging [MRI]) and functional (single photon emission computed tomography [SPECT]) imaging and neuropsychologic evaluation in the early diagnosis of frontal variant frontotemporal dementia (fvFTD). BACKGROUND: Current criteria for FTD stress the need for neuropsychologic and functional neuroimaging abnormalities, yet caregivers report lengthy histories of behavioral change. It is not known when, in the course of the disease, these investigations become abnormal, because few longitudinal studies have been reported. METHOD: Longitudinal study of two patients with serial neuropsychologic evaluation and MRI and HMPAO-SPECT scanning. RESULTS: Both patients, men aged 49 and 50, had major changes in personality, behavior, and social conduct that progressed over 5 to 6 years in a way that conformed to the clinical picture of fvFTD. There was remarkably little abnormality on neuropsychologic testing, and MRI and HMPAO-SPECT findings initially were normal. Over time, however, abnormalities on SPECT, frontal atrophy on MRI, or a neuropsychologic profile more typical of fvFTD developed in both patients. CONCLUSIONS: Standard neuropsychologic tests and conventional brain imaging techniques (MRI and SPECT) may not be sensitive to the early changes in fvFTD that occur in the ventromedial frontal cortex, and better methods of accurate early detection are required. These findings are relevant to the diagnostic criteria for FTD.
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2/26. A category-specific deficit of spatial representation: the case of autotopagnosia.

    Following a vascular lesion in the parietal cortex of the language dominant hemisphere (right in one case), two patients showed a striking dissociation between spared naming, recognition and use of their body parts and an inability in localising on verbal command the same body parts on themselves and on a mannequin (Autotopagnosia, AT). The patients were submitted to a modified version of Reed and Farah Test (1995), a test that taps the ability to encode changes of body position as opposed to changes of position of objects. Their performance differed from normal controls, showing a specific deficit in encoding body position.It is suggested that AT could be the consequence of a lesion in a specific neural circuit, located in the language dominant hemisphere, whose function is to encode the body position for both oneself and others.
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3/26. Semantic knowledge and episodic memory for faces in semantic dementia.

    Previous studies have documented poor recognition memory for faces in patients with semantic dementia. Preserved face recognition memory was found in this study, however, so long as atrophy was confined predominantly to the left temporal lobe. patients with structural damage to the right temporal lobe were typically impaired, with the status of the hippocampus and parahippocampal gyrus (including the perirhinal cortex) on the right being critical. Two single-case studies of patients with predominantly left temporal lobe pathology confirmed good recognition memory for famous faces, even if semantic knowledge about the celebrities depicted was severely degraded. An effect of semantic knowledge on recognition memory became apparent only when perceptually different photographs of the famous people were used at study and test. These results support the view that new episodic learning typically draws on information from both perceptual and semantic systems.
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4/26. Cortical deafness to dissonance.

    Ordinary listeners, including infants, easily distinguish consonant from dissonant pitch combinations and consider the former more pleasant than the latter. The preference for consonance over dissonance was tested in a patient, I.R., who suffers from music perception and memory disorders as a result of bilateral lesions to the auditory cortex. In Experiment 1, I.R. was found to be unable to distinguish consonant from dissonant versions of musical excerpts taken from the classical repertoire by rating their pleasantness. I.R.'s indifference to dissonance was not due to a loss of all affective responses to music, however, since she rated the same excerpts as happy or sad, as normal controls do. In Experiment 2, I.R.'s lack of responsiveness to varying degrees of dissonance was replicated with chord sequences which had been used in a previous study using PET, in examining emotional responses to dissonance. A CT scan of I.R.'s brain was co-registered with the PET activation data from normal volunteers. Comparison of I.R.'s scan with the PET data revealed that the damaged areas overlapped with the regions identified to be involved in the perceptual analysis of the musical input, but not with the paralimbic regions involved in affective responses. Taken together, the findings suggest that dissonance may be computed bilaterally in the superior temporal gyri by specialized mechanisms prior to its emotional interpretation.
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5/26. Postoperative cortical venous infarction in tumours firmly adherent to the cortex.

    It is sometimes difficult to separate extra-axial tumours from the cortical veins in case of tumours attaching tightly to the cortex and the cortical veins. We present two patients having a postoperative cortical venous infarction. A 59 year old female had convexity meningioma above the motor cortex where abnormal cortical anastomotic veins developed. Transient hemiparesis occurred after total removal of the tumour because of venous infarction and cyst formation resulting from sacrifice of these veins which were tightly adherent to the tumour surface. A 15 year old boy with immature teratoma of the pineal region, showing several draining veins around the vascular-rich tumour, presented transient drowsiness, diplopia and partial impairment of bilateral visual acuity postoperatively because of localised cortical brain and venous damage. It is important to make an effort to preserve main cortical veins during operation as much as possible, even if the tumour adheres to the cortical surface.
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6/26. Neuropsychological profile of a 9-year-old child with subcortical band heterotopia or 'double cortex'.

    Subcortical band heterotopia (SBH) or 'double cortex', is a congenital brain abnormality that results from aberrant migration of neurons during development of the cortex. MRI shows a continuous band of heterotopic grey matter located between the cortex and ventricular walls, separated from them by a thin layer of white matter. The condition is quite rare, found predominantly in females, and is occasionally familial with an X-linked dominant inheritance. Current research has focused on genetic and neurological correlates, with cognitive assessment restricted to a global measure of general intellectual functioning. This paper describes in detail the results of a neuropsychological assessment of a 9-year-old female recently diagnosed with SBH. Predominant features were a significantly reduced speed of processing for visuomotor and oral output and reduced immediate registration of information. This difficulty has functional implications affecting skill acquisition, learning in the classroom, and social interaction.
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7/26. Remembering what but not where: independence of spatial and visual working memory in the human brain.

    We report the neuropsychological and MRI investigation of a patient (MV) who developed a selective impairment of visual-spatial working memory (WM) with preservation not only of verbal, but also of visual shape WM, following an ischemic lesion in the cerebral territory supplied by one of the terminal branches of the right anterior cerebral artery. MV was defective in visual-spatial WM whether the experimental procedure involved arm movement for target pointing or not. Also, in agreement with the role generally assigned to visual-spatial WM in visual imagery, MV was extremely slow in the mental rotation of visually and verbally presented objects. In striking contrast with the WM deficit, MV's visual-spatial long-term memory was intact. The behavioral and neuroanatomical investigation of MV provides support for the hypothesis that the superior frontal gyrus (BA 6) and the dorsomedial cortex of the parietal lobe (BA 7) are part of the neural circuitry underlying visual-spatial WM in humans.
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8/26. Differential activation on fMRI of monozygotic twins discordant for AD.

    This is the first report of fMRI in monozygotic twins discordant for AD. FMRI brain activation patterns were examined during visuospatial and verbal working memory tasks. The affected twin had greater parietal involvement bilaterally during both working memory tasks and reduced left dorsolateral prefrontal cortex activity on the visuospatial memory task. Thus, fMRI may identify additional brain regions recruited in patients with AD to perform a given cognitive task.
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9/26. 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."
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keywords = cortex
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10/26. Alzheimer's disease.

    In this case study, we describe the symptoms, neuropsychological testing, and brain pathology of a man with Alzheimer's disease (AD). AD commonly presents with impairment of memory and language function. In this case, language difficulties were noted more prominently than was memory impairment. Throughout the limbic system and neocortex of the patient were large numbers of senile plaques and neurofibrillary tangles, the pathological hallmarks of AD.
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