Cases reported "Dementia"

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1/56. Creutzfeldt-Jakob disease presenting with visual blurring, diplopia and visual loss: Heidenhain's variant.

    Focal electroencephalographic abnormalities as described in Heidenhain's variant of Creutzfeldt-Jakob disease are uncommon. We report a 73-year-old male presenting with visual symptoms, right hemianopia and rapidly progressive dementia. myoclonus was synchronous with generalised periodic epileptiform discharges on electroencephalography (EEG). In addition, there were periodic focal sharp waves at the left occipital region. diffusion-weighted magnetic resonance brain images showed slightly increased signal intensity in the occipital parasagittal area, left more than right. 14-3-3 protein was detected in the cerebrospinal fluid. The patient died within 5 months of presentation.
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2/56. self-injurious behavior in elderly patients with dementia: four case reports.

    self-injurious behavior (SIB) is a polymorphous and poorly understood phenomenon, probably representing the final common pathway arising from a variety of etiologies. SIB is a clinical problem that affects elderly patients, but has received little attention. Although the specific prevalence rates of SIB in elderly patients with dementia is unknown, the lack of data is striking, considering the frequency with which geriatric psychiatrists may be consulted for these and related behavioral problems. The authors present four cases of elderly patients with SIB and dementia who responded favorably to psychopharmacologic treatment.
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3/56. 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.
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4/56. 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.
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5/56. risperidone for control of agitation in dementia patients.

    Currently available research on the use of risperidone to manage agitation in patients with dementia is discussed. dementia affects up to 70% of nursing-home patients, and more than 90% of them exhibit aggressive or agitated behavior or severe depression. Agitation includes combativeness, hyperactivity, disinhibition, wandering, and restlessness. Environmental interventions are preferred for mild symptoms; medications are the treatment of choice for severe manifestations. Traditional neuroleptics have been the mainstay of treatment for agitated behavior in persons with dementia, but these agents have limited efficacy and are associated with high rates of adverse effects, including worsening of already poor cognitive functioning. Although the literature on the use of risperidone in elderly patients with dementia consists largely of uncontrolled trials, case reports, and chart reviews, it appears that this agent is effective for managing agitation in this population and does so with a low frequency of extrapyramidal symptoms (EPS). risperidone may also be useful for treating acute agitation in patients with a high risk of EPS and for long-term treatment of "sundowning" (agitation and confusion starting in the late afternoon and worsening at night). A low initial dosage that is gradually adjusted upward is recommended. risperidone appears effective in controlling agitation in patients with dementia and has a relatively benign adverse-effect profile, but more clinical trials are needed to elucidate its role for this indication.
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6/56. retinitis and dementia in a pregnant girl: an unusual case.

    A 14-year-old pregnant Caucasian girl presented with a 1-week history of dementia. She had presented 1 year prior with acute unilateral visual impairment and was noted to have macular degeneration of presumed infective aetiology. This evolved to a pigmentary macular lesion. During the course of the current presentation she developed myoclonic jerks. An electroencephalogram revealed periodic spike and slow wave complexes at 1-2 second intervals. blood and cerebrospinal fluid examination showed raised anti-measles IgG antibody titres. Intrathecal synthesis of anti-measles virus antibody was demonstrated unequivocally and a diagnosis of subacute sclerosing panencephalitis was made. A healthy male infant was delivered by elective caesarean section at 33 weeks' gestation. She continued to deteriorate clinically despite treatment with intraventricular alpha-interferon. She had not had primary immunization against measles.
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7/56. epilepsia partialis continua in Creutzfeldt-Jakob disease.

    OBJECTIVES: We describe a patient with Creutzfeldt-Jakob disease (CJD) with epilepsia partialis continua (EPC) and complex partial seizures. MATERIAL, methods AND RESULTS: The patient presented with semi-rhythmic jerking movements of the right upper extremity. Serial EEG findings showed progressive changes with initial periodic lateralizing epileptiform discharges (PLEDs) on the left hemisphere which evolved into more generalized periodic sharp wave complexes (PSWCs) at later stage. Video-EEG monitoring recorded complex partial seizures originating from the left hemisphere in addition to EPC. CONCLUSIONS: A diagnosis of CJD should be considered when a rapidly progressive dementia is accompanied by abnormal movements. EPC, although rare, may present as an initial manifestation of CJD.
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8/56. Repeating without semantics: surface dysphasia?

    We describe our investigations of MNA, who had a progressive, severe and global loss of semantic knowledge (semantic dementia). Her verbal vocabulary was restricted to a few common words and she was also unable to recognize common objects from sight. By contrast, she had a well-preserved digit span (7-8 digits). In this series of experiments, we focused on her ability to repeat lists and sentences in which familiarity, meaningfulness, morphology and syntactic structure were manipulated. In list repetition tasks, we found that MNA showed a reliable effect of phonological similarity, word frequency and stimulus lexicality, but was unaffected by linguistic complexity, word length, semantic coherence or the status of individual stimuli as "known" or "unknown". In sentence repetition, her performance was not influenced by any semantic variables. However, there was a substantial effect of the frequency of the constituent vocabulary, even for words outside the range of her retained vocabulary. The influence of syntax was restricted to minor effects of morphology. The phonemes of syllables and the syllables of words are bound by their co-occurrence rather than their meaning. We conclude that the phonological representation of words is functionally independent of the semantic system.
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9/56. Deficits in irregular past-tense verb morphology associated with degraded semantic knowledge.

    Two distinct mechanisms are often considered necessary to account for generation of the past-tense of English verbs: a lexical associative process for irregular forms like speak-->spoke, and a rule-governed process ("add -ed") for regular and novel forms like talk-->talked and wug-->wugged. An alternative account based on a parallel-distributed processing approach proposes that one complex procedure processes all past-tense types. In this alternative view, neuropsychological dissociations are explained by reduced input from word meaning that plays a greater role in successful generation of the past-tense for lower frequency irregular verbs, and by phonological deficits that disproportionately affect regular and novel forms. Only limited evidence has been available concerning the relationship between knowledge of word meaning and verb-tense processing. The study reported here evaluated the past-tense verb abilities of 11 patients with semantic dementia, a neurodegenerative condition characterised by degraded semantic knowledge. We predicted and confirmed that the patients would have essentially normal ability to generate and recognise regular (and novel) past-tense forms, but a marked and frequency-modulated deficit on irregular verbs. Across the set of 11 patients, the degree of impairment for the irregular past-tense was significantly correlated with the degree of comprehension impairment as measured by verb synonym judgements. These results, plus other features of the data such as the nature of the errors to irregular verbs, are discussed in relation to currently developing theories of the language system.
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10/56. Late-onset myoclonic epilepsy in Down's syndrome (LOMEDS).

    The aim of this paper is to report a patient with late-onset myoclonic epilepsy in Down's syndrome (LOMEDS) as a differential diagnosis of adult-onset progressive myoclonic epilepsies. A 55-year-old male with Down's syndrome (DS) is described who developed progressively frequent myoclonus and generalized myoclonic-tonic seizures (GMTSs) at the age of 52. EEG recordings demonstrated background slowing and generalized polyspike-wave discharges occasionally associated with myoclonic jerks, leading to the classification of primary generalized epileptic myoclonus. Descriptions of late-onset epilepsy in DS patients are rare. However, a review of the pertinent literature revealed at least two other cases of elderly DS patients developing progressive myoclonic epilepsy after the onset of dementia. We suggest that late-onset myoclonic epilepsy in Down's syndrome as characterized here should be considered in the differential diagnosis of adult-onset myoclonic epilepsies. LOMEDS apparently shares features with myoclonic epilepsy in Alzheimer's disease (AD) and Unverricht-Lundborg disease (ULD) caused by a mutation on chromosome 21. Since life expectation of DS patients has markedly increased, LOMEDS may be more frequent than currently acknowledged.
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