Cases reported "dementia"

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11/1340. 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. ( info)

12/1340. Reversible dementia in idiopathic normal pressure hydrocephalus. A case report.

    OBJECTIVE: To present a woman with idiopathic normal pressure hydrocephalus (NPH) showing marked improvement in mental function after shunt operation. DESIGN: Case report. SETTING: primary health care. PATIENT: The clinical symptoms of gait disturbance, occasional urine incontinence and severely impaired mental function typical of NPH were all present. Although dementia predominated in the clinical picture, the diagnosis was confirmed by computer tomography (CT) and cerebrospinal fluid infusion test and cisternography. MAIN OUTCOME MEASURES: A marked improvement of mental functions was observed after shunt operation. RESULTS: Mini Mental State Examination score before shunt operation was 9 compared with 20 one year after operation. Also ventricle size was reduced as evaluated by CT scan. CONCLUSION: This reported case contrasts previous reports concluding that shunt responsive patients have only slight mental disturbances without significant improvement of mental status after shunt operation. ( info)

13/1340. Missense and silent tau gene mutations cause frontotemporal dementia with parkinsonism-chromosome 17 type, by affecting multiple alternative rna splicing regulatory elements.

    frontotemporal dementia with parkinsonism, chromosome 17 type (FTDP-17) is caused by mutations in the tau gene, and the signature lesions of FTDP-17 are filamentous tau inclusions. Tau mutations may be pathogenic either by altering protein function or gene regulation. Here we show that missense, silent, and intronic tau mutations can increase or decrease splicing of tau exon 10 (E10) by acting on 3 different cis-acting regulatory elements. These elements include an exon splicing enhancer that can either be strengthened (mutation N279(K)) or destroyed (mutation Delta280(K)), resulting in either constitutive E10 inclusion or the exclusion of E10 from tau transcripts. E10 contains a second regulatory element that is an exon splicing silencer, the function of which is abolished by a silent FTDP-17 mutation (L284(L)), resulting in excess E10 inclusion. A third element inhibiting E10 splicing is contained in the intronic sequences directly flanking the 5' splice site of E10 and intronic FTDP-17 mutations in this element enhance E10 inclusion. Thus, tau mutations cause FTDP-17 by multiple pathological mechanisms, which may explain the phenotypic heterogeneity observed in FTDP-17, as exemplified by an unusual family described here with tau pathology as well as amyloid and neuritic plaques. ( info)

14/1340. 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. ( info)

15/1340. Treatment of vocally disruptive behaviour of multifactorial aetiology.

    OBJECTIVE: To describe the treatment of vocally disruptive behaviour (VDB) of multifactorial aetiology. METHOD: Three case reports were used to illustrate the treatment of multifactorial VDB. RESULTS: A biopsychosocial assessment is required to identify the different aetiologies involved and the way they interact. Acute medical and psychiatric factors may demand that interventions are introduced simultaneously rather than in succession. CONCLUSION: Successful interventions require the combination of biopsychosocial strategies tailored to the individual case with realistic goals that include the acceptance of a residual level of VDB as a reasonable outcome. ( info)

16/1340. A case of coprophagia presenting with sialadenitis.

    PRESENTATION: a 94-year-old woman with dementia was admitted to an acute geriatric ward with increasing confusion and falls. On two occasions she developed submandibular masses. Faeces were aspirated from her mouth and a diagnosis made of recurrent submandibular sialadenitis secondary to coprophagia. OUTCOME: the submandibular mass settled with antibiotics and oral care. coprophagia was not observed on the ward, but faecal smearing was noted. With regular toileting, this behaviour ceased and sialadenitis did not recur. ( info)

17/1340. frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau.

    The tau gene has been found to be the locus of dementia with rigidity linked to chromosome 17. Exonic and intronic mutations have been described in a number of families. Here we describe a P301S mutation in exon 10 of the tau gene in a new family. Two members of this family were affected. One individual presented with frontotemporal dementia, whereas his son has corticobasal degeneration, demonstrating that the same primary gene defect in tau can lead to 2 distinct clinical phenotypes. Both individuals developed rapidly progressive disease in the third decade. Neuropathologically, the father presented with an extensive filamentous pathology made of hyperphosphorylated tau protein. Biochemically, recombinant tau protein with the P301S mutation showed a greatly reduced ability to promote microtubule assembly. ( info)

18/1340. Clinical genetics of familial progressive supranuclear palsy.

    Recent studies have shown that progressive supranuclear palsy (PSP) could be inherited, but the pattern of inheritance and the spectrum of the clinical findings in relatives are unknown. We here report 12 pedigrees, confirmed by pathology in four probands, with familial PSP. Pathological diagnosis was confirmed according to recently reported internationally agreed criteria. The spectrum of the clinical phenotypes in these families was variable including 34 typical cases of PSP (12 probands plus 22 secondary cases), three patients with postural tremor, three with dementia, one with parkinsonism, two with tremor, dystonia, gaze palsy and tics, and one with gait disturbance. The presence of affected members in at least two generations in eight of the families and the absence of consanguinity suggests autosomal dominant transmission with incomplete penetrance. We conclude that hereditary PSP is more frequent than previously thought and that the scarcity of familial cases may be related to a lack of recognition of the variable phenotypic expression of the disease. ( info)

19/1340. Olanzapine in dementia with lewy bodies: a clinical study.

    OBJECTIVES: Dementia with lewy bodies (DLB) is now a well-recognized form of dementia in which psychosis and behavioural disturbance are common. Treatment with conventional neuroleptics is often very poorly tolerated. Olanzapine, a newly introduced atypical neuroleptic which binds to multiple receptor types with relatively low affinity for D2 receptors, may be a useful treatment option in DLB. MAIN OUTCOME MEASURES: The Behavioural pathology in Alzheimer's disease Rating Scale, The Neuropsychiatric Inventory, Unified Parkinson's disease Rating Scale and The Webster Disability Scale. DESIGN: We present the results of eight DLB patients with associated psychotic and behavioural difficulties. All patients were given olanzapine 2.5-7.5 mg. Their psychotic phenomena and behavioural and extrapyramidal symptoms were monitored at 2-weekly intervals. RESULTS: Three out of the eight patients could not tolerate olanzapine even at the lowest available dose. Two patients had clear improvement in psychotic and behavioural symptoms. Three patients were able to tolerate olanzapine but gained only minimal benefit. CONCLUSIONS: Olanzapine at the doses used conferred little advantage over conventional neuroleptics and should only be given with great caution to patients with DLB. The utility of smaller doses deserves further evaluation. ( info)

20/1340. Dementia as a risk factor for homicide.

    We report a patient with dementia due to B-12 deficiency and syphilis who presented to a forensic hospital after killing his ex-wife with a gun. Despite current awareness on the occurrence of aggression and violence in patients with dementia, there has been no report discussing dementia secondary to an infectious or nutritional origin causing homicide or severe violent behavior. We discuss possible mechanisms and several predisposing factors for violent behavior in the elderly. We also discuss use and access of a gun in demented patients and its complications. We recommend availability of neuropsychiatric assessments in the elderly, limitation of gun access to demented patients and inquiry about weapon possession in the elderly. ( info)
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