Cases reported "Memory Disorders"

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1/20. Effects of a checklist on self-assessment of blood glucose level by a memory-impaired woman with diabetes mellitus.

    This study evaluated effects of a checklist on the accuracy of self-assessment of blood glucose level by a diabetic woman with memory impairments caused by viral encephalitis. The checklist consisted of 54 steps for operating an electronic glucometer, which the subject performed in sequence and checked off when completed. Following introduction of the checklist, the percentage of steps completed correctly increased in simulated and actual blood glucose tests and yielded clinically useful information.
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ranking = 1
keywords = encephalitis
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2/20. Profound amnesia after damage to the medial temporal lobe: A neuroanatomical and neuropsychological profile of patient E. P.

    E. P. became profoundly amnesic in 1992 after viral encephalitis, which damaged his medial temporal lobe bilaterally. Because of the rarity of such patients, we have performed a detailed neuroanatomical analysis of E. P.'s lesion using magnetic resonance imaging, and we have assessed his cognitive abilities with a wide range of neuropsychological tests. Finally, we have compared and contrasted the findings for E. P. with the noted amnesic patient H.M, whose surgical lesion is strikingly similar to E. P.'s lesion.
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ranking = 1
keywords = encephalitis
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3/20. memory lost, memory regained: neuropsychological findings and neuroimaging in two cases of paraneoplastic limbic encephalitis with radically different outcomes.

    OBJECTIVE: To report two cases of paraneoplastic limbic encephalitis (PNLE) with similar clinical presentation, but dramatically different outcome and to highlight the role of neuropsychological and radiological evaluation in PNLE. methods: Both patients underwent an extensive battery of neuropsychological tests designed to document general intellectual function, anterograde verbal and visual memory, naming, knowledge and executive ability. In addition, structural (CT and MRI) and functional (HMPAO-SPECT) brain scans were performed. RESULTS: Both patients presented with fairly sudden onset of profound and persistent memory loss in the absence of other neurological symptoms. Their subsequently diagnosed small cell lung cancer was treated with a combination of radiotherapy and chemotherapy, leading to remission of the tumour. The memory of patient 1 recovered fully and he died from an unrelated cause 1 year later; neuropsychological testing showed a severe, but isolated, anterograde amnesia, brain MRI was normal and HMPAO-SPECT showed left medial temporal hypoperfusion. Patient 2 remained densely amnesic despite regression of her lung tumour; neuropsychological testing disclosed both anterograde and extensive retrograde amnesia together with more generalised cognitive deficits including anomia and executive impairments, MRI showed gross atrophy of the hippocampus and amygdala bilaterally, and HMPAO-SPECT showed pronounced frontal and temporal hypoperfusion. CONCLUSION: Complete remission from PNLE may occur and seems to be associated with pure anterograde amnesia without evidence of structural hippocampal damage in MRI. By contrast, cognitive deficits beyond severe anterograde amnesia and evidence of destructive medial temporal lobe pathology on MRI seem to be poor prognostic features.
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ranking = 5
keywords = encephalitis
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4/20. Postencephalitic pure anomic aphasia: 2-year follow-up.

    We report a patient with pure anomic aphasia following encephalitis. brain magnetic resonance imaging (MRI) revealed bilateral temporal lesions, and subsequent focal atrophy in the left anterior inferior temporal lobe. Over the course of a 2-year follow-up, the patient's naming difficulty persisted without other dysfunction of language or memory. These observations indicate a contribution of the left anterior inferior temporal region to object naming.
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ranking = 1
keywords = encephalitis
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5/20. Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma: a case report.

    Paraneoplastic limbic encephalitis is a rare clinical entity, associated most often with the oat cell carcinoma of the lung. Clinically, it presents with affective changes in personality, memory loss, confusional state, hallucinations, and seizures; with dementia being the common feature as the disorder progresses. Response to treatment is disappointingly poor.
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ranking = 5
keywords = encephalitis
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6/20. Human herpesvirus 6 limbic encephalitis after stem cell transplantation.

    central nervous system complications are common in stem cell transplant recipients, but selective involvement of the medial temporal area is unusual. The 5 patients reported here presented after stem cell transplantation with increased hippocampal T2 signal on magnetic resonance imaging and increased hippocampal glucose uptake on [F-18]fluorodeoxyglucose-positron emission tomography (FDG-PET) associated with short-term memory loss, insomnia, and temporal lobe electrographic seizure activity. The initial scalp electroencephalograms (EEGs) failed to detect seizure activity in these patients, although the memory dysfunction along with the magnetic resonance imaging and FDG-PET findings suggested subcortical seizure activity. However, extended EEG monitoring revealed repetitive temporal lobe electrographic seizure activity. Follow-up MRIs in 2 patients and postmortem findings on 1 patient suggested that hippocampal sclerosis had developed following the clinical syndrome. cerebrospinal fluid studies revealed the presence of human herpesvirus 6, variant B, dna in all of 3 patients who had lumbar punctures. Immunohistochemical staining for the P41 and P101 human herpesvirus 6 protein antigens showed numerous immunoreactive astrocytes and neurons in the hippocampus of 1 of the patients who died from other causes. Because of its subtle clinical presentation, this syndrome may be underrecognized, but can be diagnosed with appropriate magnetic resonance imaging techniques, EEG monitoring, and cerebrospinal fluid viral studies.
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ranking = 4
keywords = encephalitis
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7/20. limbic encephalitis presenting with topographical disorientation and amnesia.

    A case of paraneoplastic limbic encephalitis presenting with topographical disorientation is reported. A 70 year old woman became unable to identify familiar buildings and landscapes and could not recall the way to destinations she had known very well for years. She also showed attentional disturbance and severe anterograde amnesia. Her retrograde amnesia extended for one year at most. No other neuropsychological deficits were noted. Thus her topographical disorientation was of the primary form. Specific tests related to topographical disorientation showed that her two main symptoms seem to fall into the categories of landscape agnosia and heading disorientation. T2 weighted magnetic resonance imaging revealed high intensity signals in the anteromedial temporal lobes bilaterally, in the right posterior parahippocampal gyrus, in the right retrosplenial region, and in the right inferior precuneus. Anti-Hu antibody was found in the serum. This case shows that topographical disorientation can be a primary symptom of limbic encephalitis.
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ranking = 6
keywords = encephalitis
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8/20. Nonparaneoplastic limbic encephalitis with relapsing polychondritis.

    Relapsing polychondritis (RP), which shows pain, swelling and destruction of the affected parts, is a rare autoimmune disorder affecting cartilage. We report a patient with RP that affected skull cartilage, who subsequently developed multifocal meningoencephalitis. The patient presented with severe recent memory disturbance, anxiety and moderate depression. MRI study showed bilateral median temporal lobe lesions including hippocampi and amygdaloidal bodies, abnormal findings that disappeared after treatment with high-dose steroids. This is thought to be the first case of RP presenting amnesic syndrome and mental disorder associated with nonparaneoplastic limbic encephalitis involving bilateral hippocampi and amygdaloidal bodies detected by MRI.
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ranking = 6
keywords = encephalitis
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9/20. Reversible cortical lesions in primary sjogren's syndrome presenting with meningoencephalitis as an initial manifestation.

    We report a 50-year-old woman with primary sjogren's syndrome (SjS) who initially showed forgetfulness, and later developed disturbance of consciousness. In addition to aseptic meningoencephalitis revealed by cerebrospinal fluid examination and magnetic resonance imaging (MRI), the presence of serum anti-SS-A and anti-SS-B antibodies and inflammatory findings in lip biopsy indicated primary SjS. Fluid attenuated inversion recovery (FLAIR) of MRI revealed well defined small, high signal intensity areas in the cortex involving the subcortical white matter. Corticosteroid therapy resulted in rapid and nearly complete resolution of the cortical lesions with marked improvement of the clinical manifestations. memory disturbance is a rare initial manifestation in meningoencephalitis associated with SjS. Our patient with SjS showed inflammatory cortical lesions on MRI, which were reversed by corticosteroid therapy.
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ranking = 6
keywords = encephalitis
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10/20. pathology of pure hippocampal sclerosis in a patient with dementia and Hodgkin's disease: the Ophelia syndrome.

    An archive autopsy case of a 50-year-old man who died of Hodgkin's lymphoma had a 4-year, 4-month history of dementia. After radiochemotherapy, the lymphoma subsided except for involvement of the spleen, but the dementia remained. Neuropathological examination revealed that the pathology was confined to the hippocampus, both hippocampi showing sclerosis without inflammation. neurons of sector cornu ammonis (CA) 1 were completely lost whereas moderate neuron loss was also observed in sectors CA3 and 4, and the dentate gyrus. neurons of sector CA2 were relatively well preserved and the subiculum was intact. There was no evidence of global hypoxia, or of neurodegenerative disorders with pathological changes affecting the hippocampus. Although there was a long preneoplastic history, and no inflammatory changes were found at autopsy, the present case of hippocampal sclerosis could be included in the category of paraneoplastic limbic encephalitis associated with Hodgkin's lymphoma or the Ophelia syndrome.
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ranking = 1
keywords = encephalitis
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