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https://www.readbyqxmd.com/read/27827379/kl%C3%A3-ver-bucy-syndrome-associated-with-a-recessive-variant-in-hgsnat-in-two-siblings-with-mucopolysaccharidosis-type-iiic-sanfilippo-c
#1
Hao Hu, Christoph Hübner, Zoltan Lukacs, Luciana Musante, Esther Gill, Thomas F Wienker, Hans-Hilger Ropers, Ellen Knierim, Markus Schuelke
Klüver-Bucy syndrome (KBS) comprises a set of neurobehavioral symptoms with psychic blindness, hypersexuality, disinhibition, hyperorality, and hypermetamorphosis that were originally observed after bilateral lobectomy in Rhesus monkeys. We investigated two siblings with KBS from a consanguineous family by whole-exome sequencing and autozygosity mapping. We detected a homozygous variant in the heparan-α-glucosaminidase-N-acetyltransferase gene (HGSNAT; c.518G>A, p.(G173D), NCBI ClinVar RCV000239404.1), which segregated with the phenotype...
November 9, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27530874/partial-kluver-bucy-syndrome-secondary-to-tubercular-meningitis
#2
Kunal Kishor Jha, Satyajeet Kumar Singh, Prem Kumar, Charu Dutt Arora
Tubercular meningitis (TBM) is a devastating extra pulmonary manifestation of tuberculosis and demonstrates a high neurological morbidity. A rare complication of this condition is Kluver-Bucy syndrome (KBS), which is a neurobehavioral disorder characterised by hyper-sexuality, visual agnosia, bulimia, placidity, hyperorality and memory deficits caused by lesions to the amygdala. The amygdala lesions can be due to many causes, including traumatic brain injury, systemic conditions and infections such as tuberculosis...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27380585/mesiotemporal-disconnection-and-hypoactivity-in-kl%C3%A3-ver-bucy-syndrome-case-series-and-literature-review
#3
REVIEW
Mario A Caro, Xavier F Jimenez
OBJECTIVE: Klüver-Bucy syndrome (KBS) is often perceived as rare and limited to cases with bilateral amygdala destruction. In fact, various alternate mechanisms may be involved, warranting exploration of the syndrome's presentation, pathophysiology, prognosis, and management. DATA SOURCES: Clinical management and the electronic medical records were examined for 2 patients diagnosed with partial KBS (ICD-10 F07.0) after experiencing ≥ 3 of the following: placidity, indiscriminate dietary behavior, hyperorality, hypersexuality, visual agnosia, and hypermetamorphosis...
August 2016: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/26955383/daphne-a-new-tool-for-the-assessment-of-the-behavioral-variant-of-frontotemporal-dementia
#4
Claire Boutoleau-Bretonnière, Christelle Evrard, Jean Benoît Hardouin, Laëtitia Rocher, Tiphaine Charriau, Frédérique Etcharry-Bouyx, Sophie Auriacombe, Aurélie Richard-Mornas, Florence Lebert, Florence Pasquier, Anne Sauvaget, Samuel Bulteau, Martine Vercelletto, Pascal Derkinderen, Cédric Bretonnière, Catherine Thomas-Antérion
BACKGROUND: The diagnosis of behavioral variant of frontotemporal dementia (bvFTD) relies primarily on clinical features and remains challenging. The specificity of the recently revised criteria can be disappointing, justifying development of new clinical tools. OBJECTIVE: We produced a behavioral inventory named DAPHNE. This scale (adapted from Rascovsky's criteria) explores six domains: disinhibition, apathy, perseverations, hyperorality, personal neglect and loss of empathy...
September 2015: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/26426392/behavioral-evolution-of-progressive-semantic-aphasia-in-comparison-with-nonfluent-aphasia
#5
COMPARATIVE STUDY
Estrella Gómez-Tortosa, Ricardo Rigual, Cristina Prieto-Jurczynska, Ignacio Mahillo-Fernández, Rosa Guerrero-López, Julián Pérez-Pérez, M José Sainz
BACKGROUND: Patients with primary progressive aphasia (PPA) usually develop significant behavioral disturbances with progression of the disease. We tested our clinical observation that development of disruptive agitation is more likely in semantic than in nonfluent PPA and examined which clinical variables could be associated with this behavior. METHODS: We retrospectively analyzed neuropsychiatric scores and the need for behavioral treatments in semantic PPA (n = 41) and nonfluent PPA (n = 39) cases and compared first (1-3 years since the onset of symptoms) and last (5-13 years since the onset) evaluations...
2016: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/26141491/the-behavioural-dysexecutive-variant-of-alzheimer-s-disease-clinical-neuroimaging-and-pathological-features
#6
Rik Ossenkoppele, Yolande A L Pijnenburg, David C Perry, Brendan I Cohn-Sheehy, Nienke M E Scheltens, Jacob W Vogel, Joel H Kramer, Annelies E van der Vlies, Renaud La Joie, Howard J Rosen, Wiesje M van der Flier, Lea T Grinberg, Annemieke J Rozemuller, Eric J Huang, Bart N M van Berckel, Bruce L Miller, Frederik Barkhof, William J Jagust, Philip Scheltens, William W Seeley, Gil D Rabinovici
A 'frontal variant of Alzheimer's disease' has been described in patients with predominant behavioural or dysexecutive deficits caused by Alzheimer's disease pathology. The description of this rare Alzheimer's disease phenotype has been limited to case reports and small series, and many clinical, neuroimaging and neuropathological characteristics are not well understood. In this retrospective study, we included 55 patients with Alzheimer's disease with a behavioural-predominant presentation (behavioural Alzheimer's disease) and a neuropathological diagnosis of high-likelihood Alzheimer's disease (n = 17) and/or biomarker evidence of Alzheimer's disease pathology (n = 44)...
September 2015: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/25413509/hypersexuality-in-neurocognitive-disorders-in-elderly-people-a-comprehensive-review-of-the-literature-and-case-study
#7
Chady Ibrahim, Christine Reynaert
BACKGROUND: Hypersexuality is defined as an increase in libido. It is often confounded with sexual disinhibition and inappropriate sexual behavior directed against oneself or against others. It is described in 2.9 to 8% of patients living at home and in 3.8 to 7% of patients in institutions. The pathophysiology of hypersexuality is complex. Several brain areas are involved. The psychological factors are also important. We found it useful to present a clinical case of a patient who presented with symptoms of hypersexuality and to discuss the diagnosis and the management...
November 2014: Psychiatria Danubina
https://www.readbyqxmd.com/read/25059437/what-is-frontotemporal-dementia
#8
REVIEW
Alexander Kurz, Carolin Kurz, Kathryn Ellis, Nicola T Lautenschlager
Frontotemporal dementia (FTD) is the clinical manifestation of progressive nerve cell loss in the frontal and anterior temporal lobes. It represents the second most frequent form of early-onset dementia. The two major types of FTD are determined by the localisation of the underlying pathology. The behaviour variant is characterised by disinhibition, socially inappropriate manners, loss of empathy, blunting of affect and hyperorality. Key features of the language variant are either non-fluent effortful speech and grammatical errors or impaired word finding and loss of meaning of words and objects...
October 2014: Maturitas
https://www.readbyqxmd.com/read/24760339/tdp-43-in-alzheimer-s-disease-is-not-associated-with-clinical-ftld-or-parkinsonism
#9
Youngsin Jung, Dennis W Dickson, Melissa E Murray, Jennifer L Whitwell, David S Knopman, Bradley F Boeve, Clifford R Jack, Joseph E Parisi, Ronald C Petersen, Keith A Josephs
Widespread deposition of TAR DNA-binding protein of 43 kDa (TDP-43), a major protein inclusion commonly found in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) can also be seen in a subset of cases with Alzheimer's disease (AD). Some of these AD cases have TDP-43 immunoreactivity in basal ganglia (BG) and substantia nigra (SN), regions that when affected can be associated with parkinsonian signs or symptoms, or even features suggestive of frontotemporal dementia. Here, we examined the presence of clinical features of FTLD, parkinsonian signs and symptoms, and BG atrophy on MRI, in 51 pathologically confirmed AD cases (Braak neurofibrillary tangle stage IV-VI) with widespread TDP-43 deposition, with and without BG and SN involvement...
July 2014: Journal of Neurology
https://www.readbyqxmd.com/read/24630285/kleine-levin-syndrome-a-case-report-and-review-of-literature
#10
REVIEW
Sourav Das, Ravi Gupta, Mohan Dhyani, Shailendra Raghuvanshi
BACKGROUND: Kleine-Levin syndrome presents with recurrent hypersomnia along with a number of other neuropsychiatric features, of which hyperorality has not been described frequently. METHOD: We report a male adolescent who presented with recurrent hypersomnia, hypersexuality, and hyperorality. Magnetic resonance imaging of the brain and overnight polysomnography followed by a multiple sleep latency tests were ordered. Excessive daytime sleepiness was assessed with the Epworth Sleepiness Scale...
April 2014: Pediatric Neurology
https://www.readbyqxmd.com/read/24197370/effects-of-microtubule-agents-on-the-spatial-and-electrical-properties-of-the-plasma-membrane-inchara-corallina
#11
J Fisahn, W J Lucas
The freshwater algaChara corallina Klein ex Willd., em. R.D.W. (=C. australis R.Br.) develops alternating outward (acid) and inward (alkaline) current areas on its surface when illuminated. Exposure of cells to vinblastine, colchicine, or oryzalin caused a reduction in and a shifting of this extracellular current pattern. Removal of these agents from the bathing media resulted in regeneration of the initial current profile. Because these agents all affect tubulin, microtubules may be responsible for orchestrating the transmembrane currents responsible for the acid and alkaline banding phenomenon...
November 1990: Planta
https://www.readbyqxmd.com/read/23635967/interrater-reliability-of-the-new-criteria-for-behavioral-variant-frontotemporal-dementia
#12
Amanda K Lamarre, Katya Rascovsky, Alan Bostrom, Parnian Toofanian, Sarah Wilkins, Sharon J Sha, David C Perry, Zachary A Miller, Georges Naasan, Robert Laforce, Jayne Hagen, Leonel T Takada, Maria Carmela Tartaglia, Gail Kang, Douglas Galasko, David P Salmon, Sarah Tomaszewski Farias, Berneet Kaur, John M Olichney, Lovingly Quitania Park, Mario F Mendez, Po-Heng Tsai, Edmond Teng, Bradford Clark Dickerson, Kimiko Domoto-Reilly, Scott McGinnis, Bruce L Miller, Joel H Kramer
OBJECTIVE: To evaluate the interrater reliability of the new International Behavioural Variant FTD Criteria Consortium (FTDC) criteria for behavioral variant frontotemporal dementia (bvFTD). METHODS: Twenty standardized clinical case modules were developed for patients with a range of neurodegenerative diagnoses, including bvFTD, primary progressive aphasia (nonfluent, semantic, and logopenic variant), Alzheimer disease, and Lewy body dementia. Eighteen blinded raters reviewed the modules and 1) rated the presence or absence of core diagnostic features for the FTDC criteria, and 2) provided an overall diagnostic rating...
May 21, 2013: Neurology
https://www.readbyqxmd.com/read/23611344/phenotypic-variety-in-the-presentation-of-frontotemporal-lobar-degeneration
#13
Shunichiro Shinagawa
Frontotemporal lobar degeneration (FTLD) is a heterogeneous syndrome characterized by a progressive decline in behaviour or language associated with degeneration of the frontal and anterior temporal lobes. FTLD can be classified into three clinical syndromes based on the early and predominant symptoms: behavioural variant frontotemporal dementia (bvFTD); semantic dementia (SD); and progressive non-fluent aphasia (PNFA). Patients with bvFTD present with marked changes in personality and behaviour such as disinhibition, apathy, loss of sympathy, compulsive behaviours, hyperorality...
April 2013: International Review of Psychiatry
https://www.readbyqxmd.com/read/23546304/spongiform-encephalopathy-in-siblings-with-no-evidence-of-protease-resistant-prion-protein-or-a-mutation-in-the-prion-protein-gene
#14
Daniela Varges, Walter J Schulz-Schaeffer, Wiebke M Wemheuer, Insa Damman, Matthias Schmitz, Maria Cramm, Kai Kallenberg, Katayoon Shirneshan, Manar Elkenani, Susanne Markwort, Michael Faist, Jürgen Kohlhase, Otto Windl, Inga Zerr
We discuss relevant aspects in two siblings with a neurodegenerative process of unclear aetiology who developed progressive dementia with global aphasia and hyperoral behaviour at the ages of 39 and 46 years and who died 6 and 5 years after disease onset. The cases were reported to the National Reference Center for TSE Surveillance in Göttingen, Germany. Detailed clinical examinations, CSF, blood samples, and copies of the important diagnostic tests (magnetic resonance imaging, electroencephalogram, laboratory tests) were obtained...
July 2013: Journal of Neurology
https://www.readbyqxmd.com/read/22847063/diagnosis-and-management-of-behavioral-issues-in-frontotemporal-dementia
#15
REVIEW
Masood Manoochehri, Edward D Huey
Frontotemporal lobar degeneration is an umbrella term for several different disorders. In behavioral variant frontotemporal dementia (bvFTD), patients show deterioration in cognition and social behavior. New diagnostic criteria proposed by the International Behavioral Variant FTD Consortium provide greater sensitivity in diagnosing bvFTD. Current pharmacological management of symptoms relies on medications borrowed from treating Alzheimer's disease (AD) and psychiatric disorders. The evidence for using AD medications such as acetylcholinesterase inhibitors is questionable...
October 2012: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/22366755/-frontotemporal-dementias
#16
REVIEW
K Witt, G Deuschl, T Bartsch
Frontotemporal dementias (FTD) account for only 5-7% of all dementia aetiologies. However, FTD is one common form of dementia in the presenile period with a symptom onset between an age of 45 and 65 years. FTD are clinically classified into a group of rare genetic variants, the behavioural variant, primary progressive aphasias and a variant including motor neuron symptoms (FTD-MNS). In recent years the pathobiological characteristics of some FTD variants was clarified, demonstrating a pathological accumulation of TAR-DNA binding protein 43 (TDP-43) as a common pathological substrate...
January 2013: Der Nervenarzt
https://www.readbyqxmd.com/read/22292140/peripheral-painful-traumatic-trigeminal-neuropathy-clinical-features-in-91-cases-and-proposal-of-novel-diagnostic-criteria
#17
COMPARATIVE STUDY
Rafael Benoliel, Yehuda Zadik, Eli Eliav, Yair Sharav
AIMS: To field-test carefully designed criteria for pain following trigeminal nerve trauma. METHODS: In order to characterize the clinical phenotype, posttraumatic pain patients were studied and compared with classical trigeminal neuralgia patients (CTN, defined according to the International Headache Society's criteria). Based on etiology and features, trigeminal pain following trauma was defined as "peripheral painful traumatic trigeminal neuropathy" (PPTTN). Data were analyzed with t tests, ANOVA, chi-square, and regression analyses...
2012: Journal of Orofacial Pain
https://www.readbyqxmd.com/read/22054627/a-case-of-utilization-behavior-and-hyperorality-following-bilateral-anterior-cerebral-artery-infarct-partially-responsive-to-carbamazepine-can-both-behaviors-be-attributed-to-lesions-in-different-frontal-lobe-circuits
#18
https://www.readbyqxmd.com/read/21810890/sensitivity-of-revised-diagnostic-criteria-for-the-behavioural-variant-of-frontotemporal-dementia
#19
Katya Rascovsky, John R Hodges, David Knopman, Mario F Mendez, Joel H Kramer, John Neuhaus, John C van Swieten, Harro Seelaar, Elise G P Dopper, Chiadi U Onyike, Argye E Hillis, Keith A Josephs, Bradley F Boeve, Andrew Kertesz, William W Seeley, Katherine P Rankin, Julene K Johnson, Maria-Luisa Gorno-Tempini, Howard Rosen, Caroline E Prioleau-Latham, Albert Lee, Christopher M Kipps, Patricia Lillo, Olivier Piguet, Jonathan D Rohrer, Martin N Rossor, Jason D Warren, Nick C Fox, Douglas Galasko, David P Salmon, Sandra E Black, Marsel Mesulam, Sandra Weintraub, Brad C Dickerson, Janine Diehl-Schmid, Florence Pasquier, Vincent Deramecourt, Florence Lebert, Yolande Pijnenburg, Tiffany W Chow, Facundo Manes, Jordan Grafman, Stefano F Cappa, Morris Freedman, Murray Grossman, Bruce L Miller
Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, 'possible' behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile)...
September 2011: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/21559874/behavioural-variant-frontotemporal-dementia-defining-genetic-and-pathological-subtypes
#20
REVIEW
Jonathan D Rohrer
Behavioural variant frontotemporal dementia (bvFTD) is a clinically, genetically and pathologically heterogeneous neurodegenerative disorder caused by FTLD-tau, FTLD-TDP and FTLD-FUS pathologies. Clinically, patients present with behavioural symptoms that may include one or more of disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative, stereotyped and compulsive/ritualistic behaviour or hyperorality/dietary changes. Cognitive deficits, particularly executive dysfunction, are also seen. Neuroanatomically, patients have frontal and/or temporal lobe atrophy on neuroimaging...
November 2011: Journal of Molecular Neuroscience: MN
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