frontotemporal dementia/kalis

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Potassium channel antibody associated encephalopathy presenting with a frontotemporal dementia like syndrome.

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OBJECTIVE To describe a patient who presented with features suggestive of frontotemporal dementia (FTD) but with some atypical findings and antibodies to neuronal voltage-gated potassium channels (VGKC-Abs). METHODS Case report. METHODS Mater Misericordiae University Hospital, Dublin,

A Case of Morvan Syndrome Mimicking Amyotrophic Lateral Sclerosis With Frontotemporal Dementia.

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BACKGROUND Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying

A frontotemporal dementia-like syndrome mimicking postpartum depression detected by 18F fluorodeoxyglucose positron emission tomography.

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FDG (fluorodeoxyglucose) PET (positron emission tomography) is a useful tool in the clinical workup of patients being evaluated for dementing illnesses. It is particularly effective at differentiating primary neurodegenerative diseases such as Alzheimer disease from alternative considerations

Control of clustered action potential firing in a mathematical model of entorhinal cortex stellate cells.

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The entorhinal cortex is a crucial component of our memory and spatial navigation systems and is one of the first areas to be affected in dementias featuring tau pathology, such as Alzheimer's disease and frontotemporal dementia. Electrophysiological recordings from principle cells of medial

Consequences of hyperphosphorylated tau on the morphology and excitability of hippocampal neurons in aged tau transgenic micew.

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The intracellular accumulation of hyperphosphorylated tau characterizes many neurodegenerative diseases such as Alzheimer's disease and frontotemporal dementia. A critical role for tau is supported by studies in transgenic mouse models expressing the P301L mutation with accumulation of

Late-onset temporal lobe epilepsy with unilateral mesial temporal sclerosis and cognitive decline: a diagnostic dilemma.

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We present a patient with new onset temporal lobe epilepsy and cognitive decline in his sixth decade with unilateral hippocampal atrophy on structural brain imaging, compatible with mesial temporal sclerosis. This unusual clinical scenario presented a challenging differential diagnosis since it may

Syndromes of Rapidly Progressive Cognitive Decline-Our Experience.

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BACKGROUND Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite

Loss of DPP6 in neurodegenerative dementia: a genetic player in the dysfunction of neuronal excitability.

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Emerging evidence suggested a converging mechanism in neurodegenerative brain diseases (NBD) involving early neuronal network dysfunctions and alterations in the homeostasis of neuronal firing as culprits of neurodegeneration. In this study, we used paired-end short-read and direct long-read whole

Topology of a G-quadruplex DNA formed by C9orf72 hexanucleotide repeats associated with ALS and FTD.

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Abnormal expansions of an intronic hexanucleotide GGGGCC (G4C2) repeat of the C9orf72 gene are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Previous studies suggested that the C9orf72 hexanucleotide repeat expansion (HRE), either as DNA or

Characterizations of distinct parallel and antiparallel G-quadruplexes formed by two-repeat ALS and FTD related GGGGCC sequence.

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The large expansion of GGGGCC (G4C2) repeats of the C9orf72 gene have been found to lead to the pathogenesis of devastating neurological diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The structural polymorphisms of C9orf72 HRE DNA and RNA may cause aberrant

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