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M Pistacchi, M Gioulis, F Sanson, S M Marsala
INTRODUCTION: Fahr's disease is characterized by bilateral calcium deposition within the basal ganglia, cerebellar dentate nucleus and subcortical brain white matter. The main clinical manifestations are rigid or hyperkinetic syndrome, mood disorders and cognitive impairment. The correlation between neurological impairment and symmetrical basal ganglia calcification is not so frequent. Aim of the study was to report the results of neurological assessment of three sporadic cases of Fahr's disease highlighting a correlation between the clinical syndrome and neuroimaging...
2016: Folia Neuropathologica
Rangaswamy, V Ranjith, L Vikas, R Santosh
Fahr's disease or Fahr's syndrome is a rare neurodegenerative disorder characterized by abnormal symmetrical calcifications of the basal ganglia, thalami, sub-cortical hemispheric white matter and deep cerebellar nuclei. It can be idiopathic or associated with an endocrinopathy, frequently with parathyroid disorder. Clinical spectrum of the disease is wide ranging from neurological features like seizure, syncope, stroke like events, extra-pyramidal symptoms often combined with frontal sub-cortical pattern of behavioural dysfunction and psychiatric symptoms such as psychosis, mood disorder and dementia...
August 2016: Journal of the Association of Physicians of India
Stephan Holzschuh, Kathrin Kaeß, Guilherme Volpe Bossa, Christiane Decker, Alfred Fahr, Sylvio May
Liposomal delivery constitutes a promising approach for i.v. administration of mTHPC because lipid membranes can host these drug molecules. This study investigates the transfer and release of temoporfin (mTHPC) to plasma proteins and stability of various liposomal formulations. To this end, we employed traces of radioactive markers and studied the effects of fatty acid chain length and the degree of saturation in the lipophilic tail, addition of cholesterol, and PEGylation of the membrane surface and different drug-to-lipid ratios...
October 18, 2016: Journal of Liposome Research
Markus Sack, Jenny N Lenz, Mira Jakovcevski, Sarah V Biedermann, Claudia Falfán-Melgoza, Jan Deussing, Maximilian Bielohuby, Martin Bidlingmaier, Frederik Pfister, Günter K Stalla, Alexander Sartorius, Peter Gass, Wolfgang Weber-Fahr, Johannes Fuss, Matthias K Auer
Excessive intake of high-caloric diets as well as subsequent development of obesity and diabetes mellitus may exert a wide range of unfavorable effects on the central nervous system (CNS) in the long-term. The potentially harmful effects of such diets were suggested to be mitigated by physical exercise. Here, we conducted a study investigating early effects of a cafeteria-diet on gray and white brain matter volume by means of voxel-based morphometry (VBM) and region-of-interest (ROI) analysis. Half of the mice performed voluntary wheel running to study if regular physical exercise prevents unfavorable effects of a cafeteria-diet...
October 12, 2016: Brain Imaging and Behavior
Rakesh Mp, Kshitija Jain, Priyank S Dumade, Dumade Gala, Salman Ali Sayyed
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Malathi Latha Perugula, Steven Lippmann
No abstract text is available yet for this article.
July 2016: Innovations in Clinical Neuroscience
Sarah V Biedermann, Matthias K Auer, Laura Bindila, Gabriele Ende, Beat Lutz, Wolfgang Weber-Fahr, Peter Gass, Johannes Fuss
Beneficial effects of voluntary wheel running on hippocampal neurogenesis, morphology and hippocampal-dependent behavior have widely been studied in rodents, but also serious side effects and similarities to stereotypy have been reported. Some mouse strains run excessively when equipped with running wheels, complicating the comparability to human exercise regimes. Here, we investigated how exercise restriction to 6h/day affects hippocampal morphology and metabolism, stereotypic and basal behaviors, as well as the endocannabinoid system in wheel running C57BL/6 mice; the strain most commonly used for behavioral analyses and psychiatric disease models...
September 20, 2016: Hormones and Behavior
Felista L Tansi, Ronny Rüger, Claudia Böhm, Roland E Kontermann, Ulf K Teichgraeber, Alfred Fahr, Ingrid Hilger
The underlying data demonstrates that fibroblast activation protein (FAP) paves the way for fibrosarcoma cells, which require the proteolysis of the extracellular matrix (ECM) and basement membranes to intravasate from implanted subcutaneous primary tumors into blood vessels, be transported to distant organs where they extravasate from the blood vessels, reattach and proliferate to metastases. The data additionally shows that FAP, when overexpressed on fibrosarcoma cells induces their invasion and formation of spontaneous metastases in multiple organs, particularly after subcutaneous co-implantation of the FAP-expressing and wildtype fibrosarcoma...
December 2016: Data in Brief
Mukul Ashtikar, Kalpa Nagarsekar, Alfred Fahr
Strong barrier properties of stratum corneum often limits the efficiency of drug delivery through skin. Several strategies were tried to improve permeation of drug through skin for local as well as systemic drug delivery. Incorporation of the drug within flexible liposomal vesicles has been one of the popular and well-studied approaches for delivering drug to deeper layers of the skin or even systemic circulation. Flexible/deformable/elastic liposomal systems such as invasomes, Transfersomes®, ethosomes, niosomes, etc...
September 10, 2016: Journal of Controlled Release: Official Journal of the Controlled Release Society
Elisabetta Savino, Cecilia Soavi, Eleonora Capatti, Massimo Borrelli, Giovanni B Vigna, Angelina Passaro, Giovanni Zuliani
BACKGROUND: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome...
September 8, 2016: BMC Neurology
Duygu Kayar Calili, Nevzat Mehmet Mutlu, Ayse Pinar Mutlu Titiz, Zeynep Nur Akcaboy, Eda Macit Aydin, Isil Ozkocak Turan
Fahr Syndrome is a rare disease where calcium and other minerals are stored bilaterally and symmetrically in the basal ganglia, cerebellar dentate nucleus and white matter. Fahr Syndrome is associated with various metabolic disorders, mainly parathyroid disorders. The presented case discusses a 64-year old male patient admitted to the intensive care unit of our hospital diagnosed with aspiration pneumonia and urosepsis. The cranial tomography examination to explain his nonspecific neurological symptoms showed bilateral calcifications in the temporal, parietal, frontal, occipital lobes, basal ganglia, cerebellar hemisphere and medulla oblongata posteriorly...
August 2016: JPMA. the Journal of the Pakistan Medical Association
A Schattner, I Dubin, Y Drahy, M Gelber
No abstract text is available yet for this article.
August 12, 2016: QJM: Monthly Journal of the Association of Physicians
Umit Eroglu, Gökmen Kahilogullari, Altan Demirel, Anıl Arat, Agahan Unlu
Fahr's Syndrome is characterized by the presence of intracerebral, bilateral and symmetrical calcifications located in bilateral basal ganglia, thalamus, and cerebellum. The etiology is not exactly known. The authors reported a very rare case who had Fahr's Syndrome and intracerebral aneurysms simultaneously. The patient was female and presented with headache. Her examinations revealed aneurysms on the middle cerebral artery, internal carotid artery and ophthalmic artery. That is the first case reported in the literature having multiple intracranial aneurysms and Fahr's Syndrome together...
2016: Turkish Neurosurgery
Seby Elsy Varghese, Mayur K Fariya, Gopal Singh Rajawat, Frank Steiniger, Alfred Fahr, Mangal S Nagarsenker
The present study investigates the drug delivery potential of polymer lipid hybrid nanocomposites (Lecithmer®) composed of poly(D,L-lactide-co-glycolide (PLGA) and soya lecithin. Core-shell structure of Lecithmer was evident from cryo-TEM images. Daunorubicin (DNR) and lornoxicam (LNX)-incorporated Lecithmer nanocomposites were evaluated for anticancer and anti-inflammatory activity. DNR- and LNX-loaded Lecithmer had mean particle size of ∼335 and ∼282.7 nm, respectively. Lecithmer formulated with different cationic lipids resulted in lower particle size (∼120 nm) and positive zeta potential...
August 2016: Drug Delivery and Translational Research
Verónica Montilla-Uzcátegui, Hilarión Araujo-Unda, Anilú Daza-Restrepo, Michel Sáenz-Farret, Federico Micheli
OBJECTIVE: This study aimed to report the case of a patient with paroxysmal nonkinesigenic dyskinesias and Fahr syndrome who had a marked response to carbamazepine. METHODS: We present the case of a 57-year-old female patient with episodes of paroxysmal choreoathetoid dyskinesias in the oromandibular region and distal region of upper and lower extremities, with fluctuating dystonic postures in the same distribution; duration was variable ranging from 30 minutes to 3 hours...
September 2016: Clinical Neuropharmacology
Asma Ben Houidi, Faten Ellouze, Mona Ben Djebara, Youssef Sidhom, Mohamed Fadhel Mrad, Riadh Gouider
No abstract text is available yet for this article.
December 2015: La Tunisie Médicale
Pankaj Pathak, Vivek Dhawan, Aniket Magarkar, Reinis Danne, Srinath Govindarajan, Sandipto Ghosh, Frank Steiniger, Pradip Chaudhari, Vijaya Gopal, Alex Bunker, Tomasz Róg, Alfred Fahr, Mangal Nagarsenker
We have developed active targeting liposomes to deliver anticancer agents to ASGPR which will contribute to effective treatment of hepatocellular carcinoma. Active targeting is achieved through polymeric ligands on the liposome surface. The liposomes were prepared using reverse phase evaporation method and doxorubicin hydrocholoride, a model drug, was loaded using the ammonium sulphate gradient method. Liposomes loaded with DOX were found to have a particle size of 200nm with more than 90% entrapment efficiency...
July 25, 2016: International Journal of Pharmaceutics
Roberta Biancheri, Mariasavina Severino, Angela Robbiano, Michele Iacomino, Massimo Del Sette, Carlo Minetti, Mariarosaria Cervasio, Marialaura Del Basso De Caro, Pasquale Striano, Federico Zara
Primary familial brain calcification (PFBC) (formerly idiopathic basal ganglia calcification; Fahr disease) is an autosomal dominant cerebral microvascular calcifying disorder with variable clinical and imaging features.(1) Four causative genes have been identified: SLC20A2,(2) PDGFRB,(3) PDGFB,(4) and XPR1.(5).
June 2016: Neurology. Genetics
Beatrice Roiter, Giorgio Pigato, Giulio Perugi
Age of onset can have a significant impact on clinical course and pathophysiological mechanism of bipolar disorder. Late-onset bipolar episodes are more likely linked to medical illnesses and so are frequently classified as "secondary" forms of mood disorder. We discuss the case of a patient who at the age of 58 presented his first delusional-manic episode. He also had mild frontal and occipital cortical atrophy, white matter posterior ischemic lesions, and small basal ganglia calcifications. Seven years later, he presented a second manic episode with new emergent hyperkinetic choreiform symptoms...
2016: Case Reports in Psychiatry
M P Keasey, R R Lemos, T Hagg, J R M Oliveira
Vitamin D deficiency (hypovitaminosis D) causes osteomalacia and poor long bone mineralization. In apparent contrast, hypovitaminosis D has been reported in patients with primary brain calcifications ("Fahr's disease"). We evaluated the expression of two phosphate transporters which we have found to be associated with primary brain calcification (SLC20A2, whose promoter has a predicted vitamin D receptor binding site, and XPR1), and one unassociated (SLC20A1), in an in vitro model of calcification. Expression of all three genes was significantly decreased in calcifying human bone osteosarcoma (SaOs-2) cells...
2016: Scientific Reports
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