Results 11 to 20 of about 1,156 (180)

Urbach-Wiethe disease [PDF]

open access: yesInternational Journal of Pediatric Otorhinolaryngology, 2006
Objective: Urbach-Wiethe disease (lipoid proteinosis) is a rare, recessively inherited disorder, characterized by infiltration of a hyalin-like material in skin, mucous membranes, brain and other internal organs. We present a case of a child with this rare disease.
Rallis, E.   +5 more
exaly   +11 more sources

Lipoid proteinosis (urbach-wiethe disease): A rare genodermatosis with characteristic dermatological and neuroimaging findings [PDF]

open access: yesAnnals of Indian Academy of Neurology, 2021
Aparajita Chatterjee   +3 more
doaj   +2 more sources

Atypical intracranial calcifications in a conventional Radiographic exam [PDF]

open access: yesClinical and Biomedical Research, 2015
An 18-year-old male patient of consanguineous parents, delivered at full-term by cesarean section and having no changes in neurodevelopment, presented with skin blisters that evolved to eruptions and scars immediately after birth.
Marília Sfredo Kruger   +7 more
doaj   +4 more sources

Recent advances in studying brain‐behavior interactions using functional imaging: The primary startle response pathway and its affective modulation in humans

open access: yesPsychophysiology, Volume 60, Issue 12, December 2023., 2023
Abstract The startle response is a cross‐species defensive reflex that is considered a key tool for cross‐species translational emotion research. While the neural pathway mediating (affective) startle modulation has been extensively studied in rodents, human work on brain‐behavior interactions has lagged in the past due to technical challenges, which ...
Julia Wendt   +3 more
wiley   +1 more source

Behavioral, neurological, and psychiatric frailty of autobiographical memory

open access: yesWIREs Cognitive Science, Volume 14, Issue 3, May/June 2023., 2023
The five long‐term memory systems and their assumed brain bases. Abstract Autobiographical‐episodic memory is considered to be the most complex of the five long‐term memory systems. It is autonoetic, which means, self‐reflective, relies on emotional colorization, and needs the features of place and time; it allows mental time traveling. Compared to the
Hans J. Markowitsch, Angelica Staniloiu
wiley   +1 more source

Lipoid proteinosis: Novel ECM1 pathogenic variants and intrafamilial variability in four unrelated Arab families

open access: yesPediatric Dermatology, Volume 40, Issue 1, Page 113-119, January/February 2023., 2023
Abstract Background/objectives Lipoid proteinosis (LP) is a rare autosomal recessive multisystem disorder that is caused by loss‐of‐function pathogenic variants in the extracellular matrix protein‐1 (ECM1) gene. The typical clinical manifestations of LP include hoarseness of voice, beaded papules on the eyelids, infiltration and scarring of the skin ...
Mingfeng Li   +7 more
wiley   +1 more source

Paradoxical facilitation of working memory after basolateral amygdala damage. [PDF]

open access: yesPLoS ONE, 2012
Working memory is a vital cognitive capacity without which meaningful thinking and logical reasoning would be impossible. Working memory is integrally dependent upon prefrontal cortex and it has been suggested that voluntary control of working memory ...
Barak Morgan   +4 more
doaj   +1 more source

Lipoid proteinosis coexisting with rare psychiatric manifestations: a case report with a review of literature

open access: yesPrzegląd Dermatologiczny, 2023
Lipoid proteinosis is a rare autosomal recessive genodermatosis characterized by the deposition of hyaline material in the skin and internal organs. Skin involvement is in the form of blisters in infancy healing with pock-like scars, yellow waxy papules,
Kritika Gupta   +5 more
doaj   +1 more source

Lipoid proteinosis [PDF]

open access: yes, 2008
OBJETIVO: Apresentar as características clínicas, tratamento cirúrgico e achado histológico de um caso de lipoidoproteinose. DESCRIÇÃO DO CASO: Criança do sexo masculino, cinco anos de idade, branco, que procurou atendimento odontológico na Universidade.
BRESAOLA, Marco Dapieve   +6 more
core   +5 more sources

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