83 results about "Frontotemporal Lobe Dementia" patented technology

Disclosed herein are methods and compositions for identifying and / or treating subjects having or likely to have amyotrophic lateral sclerosis (ALS) or frontotemporal dementia (FTD). Antibodies specific for one or more di-amino acid repeat-containing proteins are also provided herein.

The invention belongs to the technical field of artificial intelligence, and discloses a signal data processing method of an Alzheimer's disease and frontotemporal dementia diagnosis method, which comprises the following steps: selecting an electroencephalogram channel by adopting PSO (Particle Swarm Optimization) and converting computer signal time sequence data into a time-frequency diagram by using wavelet transform, so as to realize accurate diagnosis of Alzheimer's disease patients and healthy subjects. And patients with frontotemporal dementia and healthy subjects as well as patients with Alzheimer's disease and patients with frontotemporal dementia can be accurately and efficiently classified. The PSO is used for selecting a channel combination with good electroencephalogram energy and reducing information interference caused by redundant electroencephalogram channels, a time-frequency graph obtained through wavelet transformation can provide time information and frequency information of electroencephalogram signals at the same time, the model can learn features more effectively, and the classification performance of the model is improved. According to the method disclosed by the invention, the accuracy of classification of the Alzheimer's disease patients and healthy subjects, the frontotemporal dementia patients and healthy subjects, and the Alzheimer's disease patients and the frontotemporal dementia patients is remarkably improved.

The invention discloses a dementia identification method based on brain computer network space cross attention fusion. The dementia identification method comprises the steps that resting-state electroencephalogram signals are acquired, preprocessing and brain network construction are carried out, the frequency band power ratio is calculated, and a training set and a test set are divided; the brain network fuses the spatial information, and spatial features are obtained through an isotropic graph neural network and a local feature enhancement module; the frequency band power ratio is coded by a multi-layer perceptron to obtain frequency spectrum statistical characteristics; a bidirectional cross attention module is input, and complementary fusion features are extracted; and performing mixed pooling and splicing on the complementary fusion features, then sending the fused features to a Chebyshev Kolmogorov-Arnold network classifier, and outputting an Alzheimer's disease / frontotemporal dementia / health control (AD / FTD / HC) classification result and a cognitive scale evaluation score (MMSE). According to the method, the multi-feature complementary information is effectively integrated through joint modeling of the spatial diagram features and the global spectrum features, and the accuracy, stability and generalization ability of AD and FTD classification in a complex brain network are improved.

Aspects of the disclosure relate to methods and compositions (e.g., kits) for detecting anti-repeat-associated non-ATG (RAN) protein antibodies in a subject (e.g., a subject that has been administered a therapeutic anti-RAN protein antibody or a vaccine against a disease or disorder associated with RAN protein expression, translation, and / or accumulation, for example amyotrophic lateral sclerosis (ALS) and / or frontotemporal dementia (FTD)). In some embodiments, methods described by the disclosure comprise detecting one or more anti-RAN protein antibodies in a biological sample obtained from a subject by an electrochemiluminescence-based immunoassay using one or more target di-amino acid repeat peptides. In some embodiments, the disclosure relates to kits comprising one or more di-amino acid repeat peptides and an electrochemiluminescence-based immunoassay plate and / or reagents.

The invention discloses an application of serratia marcescens in preparation of a medicine for preventing or treating gradual freezing (amyotrophic lateral sclerosis, ALS) or frontotemporal dementia (FTD). Specifically, the serratia marcescens of the present invention can improve the phenotype of an asymptomatic and frontotemporal dementia model S59L by reducing mitochondrial unfolded protein response (UPRmt); meanwhile, expression of excitatory amino acid glutamic acid can be reduced, excitatory neurotoxicity can be relieved, and ALS and FTD phenotypes can be relieved through cooperation of the two.

The present invention relates to a compound of formula (I) wherein R1 is a hydrogen atom, halogen or -(C1-C2)alkyl, R2 is -halo(C1-C2)alkoxy, and R3 and R4 form together with N to which they are attached an optionally substituted 5-7 membered monocyclic heterocycloalkyl ring or an optionally substituted 8-11 membered bicyclic heterocycloalkyl ring. The present invention also relates to a medicament and a pharmaceutical composition comprising said compound of formula (I), as well as their therapeutic uses, in particular as inhibitor of NOD-like receptor protein 3 inflammasome for preventing and / or treating Parkinson's disease, frontotemporal Dementia, Multiple System Atrophy, Alzheimer's disease, Multiple Sclerosis, Amyotrophic Lateral Sclerosis or brain injury.

The invention provides a compound used as a CaV1.22 activating agent. In particular, a compound according to formula (I) or a pharmaceutically acceptable salt thereof is provided as a CaV1.22 activator for the treatment of schizophrenia, bipolar disorder, severe depressive disorder, substance use disorder, ADHD, Ferren-Mecdemedes syndrome, autism spectrum disorder, multiple sclerosis, frontotemporal dementia, Alzheimer's disease, Bruggea syndrome, short QT syndrome, and the like. Or early repolarization syndrome. # imgabs0 #

The invention discloses a dementia disease identification method and system based on space-time-frequency multi-dimensional feature fusion, and belongs to the technical field of smart medical treatment. The method comprises the following steps: preprocessing an electroencephalogram signal of a target user; extracting space-time-frequency multi-dimensional features of the preprocessed electroencephalogram signals; and performing classification based on the space-time-frequency multi-dimensional features to obtain a dementia disease recognition result. According to the invention, high-precision and automatic identification can be carried out on dementia diseases such as Alzheimer disease and frontotemporal dementia.

To provide stabilization of a retromer for treating Alzheimer disease and other neurodegenerative disorders.SOLUTION: Provided is a method and a composition for increasing and stabilizing a retromer in order to treat and / or prevent Alzheimer disease and other neurodegenerative disorders. Furthermore, provided is treatment of an adenovirus base for treating Alzheimer disease (AD), and other neurodegenerative states, which are Parkinson disease (PD), neuronal ceroid lipofuscinosis (NCL), and transmissible spongiform encephalopathy (TSE or prion disease), multiple system atrophy (MSA), Down syndrome, and hereditary spastic paraplegia for example, and which are progressive supranuclear palsy (PSP), frontotemporal dementia linked to chromosomes 17q21 to 22 and subtype (FTLD-17 / FTLD-Tau) thereof, Lewy body disease (LBD), amyotrophic lateral sclerosis (AES), frontotemporal degeneration (FTD), ALS-FTD and chronic traumatic encephalopathy (CTE) for example.SELECTED DRAWING: None

The present disclosure describes improved vectors, such as adeno-associated virus (AAV) vectors, for expressing progranulin and its variants in transduced cells, and the use of such vectors to increase the amount of progranulin in subjects with progranulin deficiency, such as certain subjects with frontotemporal dementia (FTD) or frontotemporal lobar degeneration (FTLD).

To provide a C9orf72 oligonucleotide, a composition, and a method for treatment of C9orf72-associated conditions, disorders, or diseases including amyotrophic lateral sclerosis and frontotemporal dementia.SOLUTION: Provided is an oligonucleotide comprising at least one modification of a sugar, a base, or an internucleotidic linkage, wherein the base sequence of the oligonucleotide is or comprises at least 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, or 25 contiguous bases of a base sequence that is at least 80% identical with or complementary to a base sequence of a C9orf72 gene or a transcript thereof, and wherein a nucleobase at a 3' end of the oligonucleotide is optionally replaced by a replacement nucleobase selected from I, A, T, U, G, and C.SELECTED DRAWING: None

The present disclosure provides compositions and methods for modulating C9orf72 gene transcription in patients in need thereof, including patients suffering from C9orf72 related diseases, such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).

The invention discloses a 2-(piperidinyl) pyridazinone compound (I), pharmaceutically acceptable salts thereof, a preparation method of the 2-(piperidinyl) pyridazinone compound, a pharmaceutical composition of the 2-(piperidinyl) pyridazinone compound and application of the 2-(piperidinyl) pyridazinone compound in preparation of drugs for treating and / or preventing nervous system related diseases. Comprising but not limited to vascular dementia, Alzheimer's disease, frontotemporal dementia, Prion disease, dementia with Lewy bodies, Parkinson's disease, Huntington's disease, HIV-related dementia, multiple sclerosis, amyotrophic lateral sclerosis, neuropathic pain, cerebral arterial thrombosis, hemorrhagic stroke, nerve injury caused by cerebral trauma and other diseases;

The present invention relates to compounds which are suitable for imaging TDP-43 (Transactive response (TAR) DNA binding protein 43 kDa) aggregates. The compounds can be used, for example, for diagnosing a disease, disorder or abnormality associated with TDP-43 aggregates or a TDP-43 proteinopathy, such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Frontotemporal dementia (FTD) and limbic-predominant age-related TDP-43 encephalopathy (LATE).

The invention discloses ursodesoxycholic acid and amino acid conjugates (I), pharmaceutically acceptable salts thereof, a preparation method of the ursodesoxycholic acid and amino acid conjugates, a pharmaceutical composition of the ursodesoxycholic acid and amino acid conjugates and application of the ursodesoxycholic acid and amino acid conjugates in preparation of drugs for treating and / or preventing comprising but not limited to vascular dementia, Alzheimer's disease, frontotemporal dementia, Prion disease, dementia with Lewy bodies, Parkinson's disease, Huntington's disease, HIV-related dementia, multiple sclerosis, amyotrophic lateral sclerosis, neuropathic pain, cerebral arterial thrombosis, hemorrhagic stroke, nerve injury caused by cerebral trauma and other diseases; # imgabs0 #

Aspects of this invention are related to the use of N-desmethyl ruboxistaurin and pharmaceutically acceptable formulations thereof to modulate GSK3|3 signaling. Some aspects of the invention relate to the use of N-desmethyl ruboxistaurin to inhibit protein kinase C. Some aspects of the invention provide methods of using N-desmethyl ruboxistaurin in the treatment of subjects having a neurological disease and / or psychiatric disorder, including Alzheimer's disease, frontotemporal dementia, behavioral complications of dementia, bipolar disorder, depression, schizophrenia, Parkinson's disease, or neuroinflammation. Some aspects of this invention provide methods of using N-desmethyl ruboxistaurin in treating conditions associated with diabetes mellitus or its complications, or ischemia, inflammation, pulmonary hypertension, congestive heart failure, cardiovascular disease, dermatological disease, or cancer. Some aspects of the invention relate to the use of N-desmethyl ruboxistaurin as an alternative to ruboxistaurin, with a better pharmacokinetic profile, lower potential for prolongation of the electrocardiogram QT interval, and / or a lower potential for adverse drug interactions. In some embodiments, N-desmethyl ruboxistaurin is administered in combination with lithium or other treatments for bipolar disorder.

The invention discloses an aminoamide alkyl thioester compound (I), pharmaceutically acceptable salts thereof, a preparation method of the aminoamide alkyl thioester compound, a pharmaceutical composition of the aminoamide alkyl thioester compound and application of the aminoamide alkyl thioester compound in preparation of drugs for treating and / or preventing nervous system related diseases. Comprising but not limited to vascular dementia, Alzheimer's disease, frontotemporal dementia, Prion disease, dementia with Lewy bodies, Parkinson's disease, Huntington's disease, HIV-related dementia, multiple sclerosis, amyotrophic lateral sclerosis, neuropathic pain, cerebral arterial thrombosis, hemorrhagic stroke, nerve injury caused by cerebral trauma and other diseases; # imgabs0 #

The invention discloses an amide alkyl thiol ester compound (I) as well as a preparation method and a pharmaceutical composition thereof, and application of the amide alkyl thiol ester compound in preparation of drugs for treating and / or preventing diseases by resisting oxidative stress, inhibiting amyloid protein aggregation and resisting neuroinflammation. Comprising but not limited to vascular dementia, Alzheimer's disease, frontotemporal dementia, Prion disease, dementia with Lewy bodies, Parkinson's disease, Huntington's disease, HIV-related dementia, multiple sclerosis, amyotrophic lateral sclerosis, neuropathic pain, cerebral arterial thrombosis, hemorrhagic stroke, nerve injury caused by cerebral trauma and other diseases; # imgabs0 #

The invention relates to a method for diagnosis, disease monitoring and / or therapy guidance in a patient suspected of having a neurodegenerative disease of the Frontotemporal Dementia-Amyotrophic Lateral Sclerosis-spectrum (FTD-ALS-spectrum), comprising (a.) providing a sample obtained from said patient, wherein said sample comprises extracellular vesicles, (b.) determining a level of one or more FTD-ALS-spectrum biomarkers in the extracellular vesicles of said sample, (c.) wherein the level of the one or more biomarkers is indicative of whether the patient has (and / or allows to distinguish between) a neurodegenerative disease of the FTD-ALS-spectrum comprising a Tau-proteinopathy or a neurodegenerative disease of the FTD-ALS-spectrum comprising a TAR DNA-binding protein 43 (TPD-43)-proteinopathy. The invention further relates to a kit for carrying out the method of the present invention, methods of treating patients identified using the method of the invention, methods of determining said FTD-ALS-spectrum biomarkers in the extracellular vesicles of said sample, and samples comprising extracellular vesicles and said FTD-ALS-spectrum biomarkers.

The present invention pertains to the anti-inflammatory therapeutic effect of xantohumol in spinal cord injury (SCI). The continuous administration of xanthohumol for 1-8 weeks to SCI rats resulted in improved results in 4 clinical tests used and in lowering and faster elimination of macrophages from the SCI lesion. Since the infiltration of the SCI lesion by numerous phagocytic macrophages indicates a severe destructive inflammation of extraordinary longevity, administration of xanthohumol is neuroprotective and resulted in a better and faster recovery of the locomotor function and the strength and sensory function in the hind limbs, in a shorter period of paralysis of the urinary bladder and in the recovery of the body weight lost due to the SCI surgery. Since the traumatic brain injury (TBI) involving the white matter, and stroke involving the white matter initiate the severe destructive inflammation as in the SCI, the administration of xanthohumol is expected to be anti-inflammatory and neuroprotective in both brain diseases. Since neurodegenerative diseases including Alzheimer's disease, frontotemporal dementia and Parkinson's disease, immune mediated neuroinflammation including multiple sclerosis and neuromyelitis optica and cerebrospinal infections have inflammatory pathogenesis involving microgliosis and infiltration by macrophages, the administration of xanthohumol is expected to result in therapeutic inhibition of progression of all above diseases as well as of related neurodegenerative, immune mediated and infectious diseases of the brain and of the spinal cord.

The invention is directed to immunological compositions of one or more peptides containing epitopes of PGN, LTA and LPS molecules that induce an immunological response in a mammal, and to multiple antibodies that bind to these epitopes. Immunological compositions and antibodies disclosed herein can be used in the treatment and / or prevention of human health disorders such as bacterial sepsis, inflammation, cancers, tumors, inflammatory diseases and disorders, and neurodegenerative disorders such as, but not limited to Alzheimer's disease, frontotemporal dementia, chronic traumatic encephalopathy (CTE), Lewy body dementia and / or limbic predominant age-related TDP-43 encephalopathy (LATE).

The present invention describes a method for the therapeutic treatment and / or for the diagnosis of a brain disorder including but not limited to cognitive disorders such as mild cognitive impairment, Alzheimer disease, frontotemporal dementia, dementia with Lowy body in a subject at risk of having or developing a brain disorder such as cognitive disorder, using long non-coding RNA (lncRNA).

A recombinant AAV (rAAV) suitable for use in treating adult onset neurodegeneration caused by granulin (GRN) haploinsufficiency, such as progranulin (PGRN)-related frontotemporal dementia (FTD), is provided. The rAAV comprises (a) an adeno-associated virus 1 capsid, and (b) a vector genome packaged in the AAV capsid, said vector genome comprising AAV inverted terminal repeats, a coding sequence for human progranulin, and regulatory sequences which direct expression of the progranulin. Also provided are a method for treating a human patient with PGRN-FTD and other adult onset neurodegeneration caused by granulin (GRN) haploinsufficiencies, comprising delivering to the central nervous system (CNS) a recombinant adeno-associated virus (rAAV) having an adeno-associated virus 1 (AAV1) capsid, said rAAV further comprising a vector genome packaged in the AAV capsid, said vector genome comprising AAV inverted terminal repeats, a coding sequence for human progranulin, and regulatory sequences which direct expression of the progranulin.

The present invention relates to compounds suitable for imaging TDP-43 (transactive response (TAR) DNA-binding protein 43 kDa) aggregates. The compounds can be used, for example, to diagnose diseases, disorders, or abnormalities associated with TDP-43 aggregates or TDP-43 proteinopathies, such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), frontotemporal dementia (FTD), and limbic-predominant late-stage TDP-43 encephalopathy (LATE).

The present invention provides a method of treating frontotemporal dementia, or a childhood genetic neurodegenerative disease such as Ataxia Telangiectasia (A-T), or neurodegenerative diseases such as Parkinson's disease or neuropsychiatric diseases comprising administering to a subject in need thereof an effective amount of chlorite composition, such as sodium chlorite. The present invention thereby provides a method of modulating the immune system in a subject in need thereof. Described herein are methods of administration and treatment.

The present invention is in the field of transactive response DNA binding protein with a molecular weight of 43 kDa (TARDB or also TDP-43). The invention relates to TDP-43 specific binding molecules, in particular to anti-TDP-43 antibodies or antigen-binding fragments or a derivative thereof, vectors delivering nucleic acid encoding antibodies of the invention, and uses thereof. The present invention provides means and methods to diagnose, prevent, alleviate and / or treat a disease, disorder and / or abnormality associated with TDP-43 aggregates including but not limited to Frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Parkinson's disease (PD), Chronic Traumatic Encephalopathy (CTE), and limbic-predominant age-related TDP-43 encephalopathy (LATE).

The present invention relates to a compound of formula (I) wherein n is 1 or 2, R1 is halogen, (C1-C4) alkyl, halo (C1-C4) alkyl, (C1-C4) alkoxy, halo (C1-C4) alkoxy, ethynyl, propargyl, or (C3-C6) cycloalkyl, or two R1 form a cyclopentane ring fused to phenol; r2 and R3 represent H, cyano, ethynyl, propargyl, (C1-C4) alkyl, hydroxy (C1-C4) alkyl, or halogenated (C1-C4) alkyl, or R2 and R3 together with the atoms linking them form a (C5-C6) carbocyclic ring fused to the pyridazine ring; r4 and R5 together with the N to which they are attached form an optionally substituted 3-7 membered monocyclic heterocycloalkyl ring, 8-11 membered bicyclic heterocycloalkyl ring or 7-12 membered bicyclic heterocyclic spiro ring. The invention also relates to a medicament and a pharmaceutical composition comprising said compounds of formula (I) and to their therapeutic use, in particular as inhibitors of NOD-like receptor protein 3 inflammasome for the treatment of e.g. Parkinson's disease or frontotemporal dementia.

To provide an antisense oligonucleotide that controls ATXN2 both in vivo and in vitro.SOLUTION: Provided is an antisense oligonucleotide capable of adjusting expression of ATXN2 in a target cell. An oligonucleotide hybridizes to ATXN2 mRNA. Also provided is a pharmaceutical composition and a method for treating neurodegenerative diseases such as Spinocerebellar ataxia type 2 (SCA2), Amyotrophic lateral sclerosis (ALS), frontotemporal dementia with Alzheimer's pathology (FTD), parkinsonism, and a condition associated with TDP-43 proteinopathy by using an oligonucleotide conjugate and an oligonucleotide.SELECTED DRAWING: None

An EEG-based dementia diagnosis assistance device according to the present embodiment comprises: an EEG data preprocessing unit for preprocessing a collected EEG data set of a test subject, the collected EEG data set being divided into time intervals by setting a window length and a shift length; an Hjorth parameter extraction unit for extracting, for each time interval, Hjorth parameters including an activity index, a mobility index, and a complexity index; and a dementia classification unit for classifying the test subject into one from among a Alzheimer's disease (AD) group, a frontotemporal dementia (FTD) group, and a cognitive normal (CN) group on the basis of the extracted Hjorth parameters. Therefore, AD, FTD, and CN subjects can be classified using Hjorth parameters composed of three major indicators of activity, mobility, and complexity through time domain analysis on EEG datasets.

The current invention relates to PLA2G15 inhibitors represented by formula (I), and corresponding compositions and uses. Preferably, the inhibitors and compositions are for use in the treatment of lysosomal storage diseases, Alzheimer's disease and Parkinson's disease; in particular for use in the treatment of Niemann Pick type C or a neuronal ceroid lipofuscinosis such as CLN3 disease or Batten disease, CLN5 disease, or GRN frontotemporal dementia.