1/120. myotonic dystrophy and progressive cognitive decline: a common condition or two separate problems?We report the case of NG, a 43-year old woman with myotonic dystrophy (MYD) who has shown a slow decline in both motor and cognitive abilities since her referral to us at age 32. MYD is an autosomal dominant disorder characterised by weakening and wasting of the muscles together with impaired muscle relaxation. Cognitive abilities are usually little affected in the adult onset form, although there is a high risk of cognitive impairment in those with childhood onset. Cognitive decline is also typically associated with maternal inheritance. NG, who was diagnosed with MYD at the age of 18, inherited it from her father. We report the decline in NG's cognitive abilities over 11 years of longitudinal assessment, and consider whether she is an atypical MYD patient or whether the MYD and cognitive decline are attributable to two separate pathological processes.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
2/120. 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.- - - - - - - - - - ranking = 13440.055701075keywords = frontal (Clic here for more details about this article) |
3/120. Reduplicative paramnesia: longitudinal neurobehavioral and neuroimaging analysis.Reduplicative paramnesia (RP) is a delusion in which the patient perceives familiar places, objects, or events to have been duplicated. The current case describes the development of RP in an 81-year-old male following a large right frontal lobe infarction. As the patient had been hospitalized previously with hemorrhagic contusions, neurologic, neuropsychological, and neuroimaging data were obtained both prior to and following RP onset. Psychophysiologic data were obtained following the development of the delusion. Both premorbidly and at follow-up, neuropsychological functioning was characterized by significant impairments of learning and memory and frontal-executive functions. language and visuospatial skills and motor speed were intact both before and after RP onset. The case is described within the context of preexisting theories of RP, and it is surmised that the delusion is secondary to temporal-limbic-frontal dysfunction giving rise to a distorted sense of familiarity and impaired ability to resolve the delusion via reasoning.- - - - - - - - - - ranking = 11164.644448891keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
4/120. life review of an older adult with memory difficulties.The natural life review process involves emotional processing of events from the individual's past. life review therapy (LRT) addresses issues regarding unresolved conflicts, guilt and resentment which the client has particular difficulty in reviewing independently. People with memory problems may have difficulty mentally manipulating a number of pieces of information at the same time. Since LRT involves recollection of past experiences (events, emotions and relationships) which need to be processed and integrated with the person's current understanding, individuals with memory problems may find this process difficult, though not impossible. This article is written in order to demonstrate that LRT can be useful for people with memory difficulties, provided that individual sessions are tailored to their particular cognitive abilities. A case is presented of an older adult, with above-average intellectual abilities, who became distressed when recollecting her childhood. LRT was conducted in a manner that took account of the memory difficulties and utilized a variety of therapeutic techniques. LRT was beneficial for this client despite her memory difficulties, since, following therapy, she was no longer distressed when thinking about her childhood.- - - - - - - - - - ranking = 2keywords = childhood (Clic here for more details about this article) |
5/120. Unknown people believed to be known: the 'assoziierende Erinnerungs- falschungen' by Kraepelin.A male patient with epilepsy, who developed a unique form of paramnesia after an episode of zonisamide-induced psychosis is reported. The patient consistently mistook people who were quite new to him, such as staff of the hospital, for persons whom he had met long ago. However, he did not misidentify their names or other attributes, such as their occupations. This extraordinary form of misidentification does not fall into any known subcategory of misidentification syndromes. Rather, this paramnesia falls into the classical description of 'assoziierende Erinnerungsfalschungen' by Kraepelin. The neuropsychological interpretation of the reported patient is difficult. However, loss of familiarity with environmental objects due to his long sustained epileptic history is supposed to be a possible mechanism of paramnesia.- - - - - - - - - - ranking = 923.93780793776keywords = epilepsy (Clic here for more details about this article) |
6/120. Effects of guided imagery on memory distortion in women reporting recovered memories of childhood sexual abuse.We tested whether having participants imagine unusual childhood events inflates their confidence that these events happened to them, and tested whether this effect is greater in women who report recovered memories of childhood sexual abuse than in women who do not. Participants were pretested on how confident they were that certain childhood events had happened to them before being asked to imagine some of these events in the laboratory. New confidence measures were readministered. Although guided imagery did not significantly inflate confidence that early childhood events had occurred in either group, the effect size of inflated confidence was more than twice as large in the control group as in the group with recovered memory. These data suggest that individuals can counteract memory distortions potentially associated with guided imagery, at least under some conditions.- - - - - - - - - - ranking = 8keywords = childhood (Clic here for more details about this article) |
7/120. "Developmental dysmnesia": a case report.We report a 9-year-old girl who presented from an early age a severe and specific verbal memory disorder, in the absence of a definite etiology. The results of an extensive neuropsychological assessment showed a dissociation between normal visuo-spatial memory abilities and a marked short-term and long-term verbal memory impairment. We argue that our case is affected by "developmental dysmnesia", a condition so far described in two cases only (De Renzi and Lucchelli, 1990, and Temple, 1997). The disorder affected episodic as well as semantic verbal memory. The long term effects on learning and cognition of a memory deficit occurring early in childhood are discussed.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
8/120. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.- - - - - - - - - - ranking = 2keywords = childhood (Clic here for more details about this article) |
9/120. Symptomatic arachnoid cyst of the left frontal convexity presenting with memory disturbance--case report.A 48-year-old female presented with vertiginous feeling and behavior disturbance. Computed tomography showed an arachnoid cyst on the left cerebral convexity. Single photon emission computed tomography revealed decreased cerebral blood flow (CBF) in the left frontal lobe. The Wechsler memory Scale-Revised test demonstrated memory dysfunction. The arachnoid cyst was partially removed. Disturbances in CBF and behavior disappeared postoperatively. Local ischemia induced by compression due to arachnoid cyst may cause memory dysfunction and behavior disturbance. Neuropsychometric examination is useful for the evaluation of such symptoms.- - - - - - - - - - ranking = 15004.660363484keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
10/120. Abnormal Wada and neuropsychological testing results due to topiramate therapy.A 46-year-old man experienced intractable seizures since childhood. Due to lack of response to antiepilepsy drugs (AEDs), he underwent a surgical evaluation that was consistent with seizure onset in the left medial temporal lobe. While on topiramate and carbamazepine, his preoperative neuropsychological scores and sodium amytal (Wada) scores were low and may have excluded him from surgery. Repeat testing on lamotrigine and carbamazepine showed improvement in his scores, allowing him to undergo surgery. physicians must therefore be cautious in evaluating such test scores while a patient is on topiramate.- - - - - - - - - - ranking = 1121.485133423keywords = epilepsy, lobe, childhood (Clic here for more details about this article) |
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