Background: Mid-dermal elastolysis is a rare disorder of elastic tissue which is characterized clinically by wrinkling of the skin and histologically by the selective absence of elastic fibers in the mid dermis. Two patients with severe ptosis and a loss of the lateral tarsal suspension are reported.
Patients And Methods: A 36-year-old male patient presented with bilateral ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. With normal clinical and routine laboratory investigations, punch biopsy revealed clear signs of mid-dermal elastolysis. Plastic surgery of both eyelids (levator advancement) was performed with good success after a 1-year interval without progression of ptosis. Another 37 year old patient suffered from mild ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. As the patient chose not to have surgical treatment, he was treated with oral corticosteroids. To differentiate between an acute and a chronic type of elastolysis a punch biopsy is required. In addition, we want to highlight the surgical option in chronic and non-progressive cases.
Conclusions: In contrast to formerly reported localized forms of acute acquired cutis laxa with complete loss of elastic fibres in the reticular and papillary dermis, we report a chronic type of elastolysis in these patients. In our experience in cases without any apparent progression of symptoms a surgical repair can provide long-term success.
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http://dx.doi.org/10.1007/s00347-003-0897-9 | DOI Listing |
Adv Drug Deliv Rev
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Self-renewal capacity and potential to differentiate into almost any cell type of the human body makes pluripotent stem cells a valuable starting material for manufacturing of clinical grade cell therapies. Neurodegenerative diseases are characterized by gradual loss of structure or function of neurons, often leading to neuronal death. This results in gradual decline of cognitive, motor, and physiological functions due to the degeneration of the central nervous systems.
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Salk Institute for Biological Studies, Molecular Neurobiology Laboratory, 10010 North Torrey Pines Rd, La Jolla, CA, 92037, USA. Electronic address:
Astrocytes perform multiple functions in the nervous system, many of which are altered in neurodegenerative disorders. In this review, we explore shared astrocytic alterations across neurodegenerative disorders, including Alzheimer's disease, Huntington's disease, Parkinson's disease, amyotrophic lateral sclerosis, and frontotemporal lobe degeneration. Assessing recent datasets of single-nucleus RNA-sequencing of human brains, a theme emerges of common alterations in astrocyte state across disorders including in neuroinflammation, synaptic organization, metabolic support, and the cellular stress response.
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Oral and Maxillofacial Surgery Department, Faculty of Dentistry, Tishreen University. Electronic address:
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Department of Cell Biology and Physiology, Washington University School of Medicine, Saint Louis, MO 63110, USA.
Sci Transl Med
January 2025
University of Strasbourg, INSERM, Strasbourg Translational Neuroscience & Psychiatry STEP-CRBS, UMR-S 1329, 67000 Strasbourg, France.
Sleep alterations have been described in several neurodegenerative diseases yet are currently poorly characterized in amyotrophic lateral sclerosis (ALS). This study investigates sleep macroarchitecture and related hypothalamic signaling disruptions in ALS. Using polysomnography, we found that both patients with ALS as well as asymptomatic and mutation carriers exhibited increased wakefulness and reduced non-rapid eye movement sleep.
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