frontotemporal dementia/crise epiléptica

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Complex partial seizures presenting as frontotemporal dementia: a case report.

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Both epilepsy and dementia are common after the age of 65. Epilepsy, originating in the temporal lobes, can present clinically in a variety of ways and can be difficult to diagnose. Loss of consciousness may not be evident. Reported here is a unique case of a 65 year old man who presented with

Hyperexcitability and epileptic seizures in a model of frontotemporal dementia.

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Epileptic seizures are more common in patients with Alzheimer disease than in the general elderly population. Abnormal forms of hyperphosphorylated tau accumulate in Alzheimer disease and other tauopathies. Aggregates of tau are also found in patients with epilepsy and in experimental models of

Relative Incidence of Seizures and Myoclonus in Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia.

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BACKGROUND Patients with Alzheimer's disease (AD) are more prone to seizures and myoclonus, but relative risk of these symptoms among other dementia types is unknown. OBJECTIVE To determine incidence of seizures and myoclonus in the three most common neurodegenerative dementias: AD, dementia with

Severe hyponatremia and seizures secondary to psychogenic polydipsia in a case of frontotemporal dementia.

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Non-Convulsive Status Epilepticus in Behavioral Variant Frontotemporal Dementia

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Epilepsy in frontotemporal dementia is considered to be less frequent than in Alzheimer's disease. We report two cases of patients with non-convulsive status epilepticus associated with behavioral variant frontotemporal dementia. In the first case, status epilepticus was the first symptom of the

[Pathological gambling and epilepsy in patients with frontotemporal dementia: Two case reports].

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Frontotemporal dementia is a neurodegenerative disorder of which the behavioral variant is most common. This condition is currently considered the most common cause of dementia in people younger than 60 years. Here, we present two unrelated cases in which the typical symptoms were cognitive and

Early-onset dementia with prolonged occipital seizures: an atypical case of Kufs disease.

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OBJECTIVE Kufs disease is the adult-onset form of neuronal ceroid lipofuscinosis (NCL). Its two clinical phenotypes are type A (progressive myoclonus epilepsy with dementia) and type B (behavioral abnormalities and dementia, associated with pyramidal and extrapyramidal signs). METHODS We describe

Alzheimer's disease neuropathologic changes in semantic dementia.

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Neuropathologic change underlying primary progressive aphasia (PPA) most commonly includes one of the frontotemporal lobar degenerations, such as FTLD-tau or FTLD-ubiquitin. The next most frequent etiology of PPA is Alzheimer's disease (AD). We describe 5 subjects with clinical diagnoses of semantic

Convulsive responses to seizure-inducible drugs are exacerbated in progranulin-deficient mice.

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Progranulin (PGRN) is a glycoprotein that is widely expressed among organs, including the central nervous system. PGRN insufficiency is involved in various neurodegenerative disorders such as frontotemporal dementia, Alzheimer's disease, and neuronal ceroid lipofuscinosis. One of the major causes of

FTDP-17: an early-onset phenotype with parkinsonism and epileptic seizures caused by a novel mutation.

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Recently, mutations in the tau gene on chromosome 17 were found causative for autosomal dominantly inherited frontotemporal dementia and parkinsonism (FTDP-17). We describe a family carrying a missense mutation at nucleotide 1137 C --> T, resulting in the amino acid substitution P301S. Methods of

Homozygous TREM2 mutation in a family with atypical frontotemporal dementia.

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TREM2 mutations were first identified in Nasu-Hakola disease, a rare autosomal recessive disease characterized by recurrent fractures because of bone cysts and presenile dementia. Recently, homozygous and compound heterozygous TREM2 mutations were identified in rare families with frontotemporal

New-Onset Delusions Heralding an Underlying Neurodegenerative Condition: A Case Report and Review of the Literature.

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To present a striking case of new-onset psychosis in a middle-aged woman subsequently diagnosed with behavioral variant frontotemporal dementia (bvFTD). To review the data regarding key red-flag features that may suggest a diagnosis of a neurodegenerative process, and specifically

DNMT1 mutation hot spot causes varied phenotypes of HSAN1 with dementia and hearing loss.

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BACKGROUND Mutations in DNA methyltransferase 1 (DNMT1) have been identified in 2 autosomal dominant syndromes: 1) hereditary sensory autonomic neuropathy with dementia and hearing loss (HSAN1E); and 2) cerebellar ataxia, deafness, and narcolepsy. Both syndromes have mutations in targeting sequence

Clinical characterization of a kindred with a novel 12-octapeptide repeat insertion in the prion protein gene.

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OBJECTIVE To report the clinical, electroencephalographic, and neuroradiologic findings in a kindred with a novel insertion in the prion protein gene, PRNP. METHODS Clinical description of a kindred. METHODS Mayo Clinic Alzheimer Disease Research Center (Rochester, Minnesota). METHODS Two

Identification of PSEN1 and PSEN2 gene mutations and variants in Turkish dementia patients.

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In order to assess the frequency of mutations in the known Alzheimer's disease causative genes in Turkish dementia patients we screened amyloid precursor protein (APP), PSEN1 and PSEN2 for mutations in a cohort of 98 Turkish dementia families. Six families were found to carry PSEN1 mutations

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