Background: Diencephalic Syndrome is an atypical early manifestation of low-grade gliomas; so, it is important to detect it in patients that experience a failure to thrive despite adequate length growth and food intake. The purpose of this article is to focus attention on this rare but potentially dangerous cause of poor weight gain or stunting in childhood.
Materials And Methods: We describe four patients with Diencephalic Syndrome and low-grade gliomas who were evaluated in our institution from January 2017 to December 2021.
Case Description And Results: two patients presented with suspected malabsorption, and two presented with a suspected eating disorder. In all cases, neurological symptoms appeared late, explaining the reason for the diagnostic delay, which impacts negatively on prognosis and on quality of life. Currently, patients 1 and 2 have stable disease in second-line therapy, patient 3 has stable disease post end of second-line therapy, and patient 4 has stable disease in first-line therapy. Everyone is in psychophysical rehabilitation.
Conclusions: A multidisciplinary evaluation is essential in order to make an early diagnosis and improve prognosis and quality of life.
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http://dx.doi.org/10.3390/curroncol30090610 | DOI Listing |
Front Immunol
December 2024
Department of Neurology, University of Virginia, Charlottesville, VA, United States.
Background: We evaluated comprehension and application of the 2015 neuromyelitis optica spectrum disorder (NMOSD) criteria core elements by neurologists in Latin America (LATAM) who routinely diagnose and care for NMOSD patients by (i) identifying typical/suggestive NMOSD syndromes, (ii) detecting typical MRI NMOSD lesions and meeting MRI dissemination in space (DIS) criteria, and (iii) evaluating historical symptoms suggestive of NMOSD.
Methods: We conducted an anonymous, voluntary, self-administered web- and case-based survey cross-sectional study from October 2023 to January 2024 of neurologists identified through the LACTRIMS database. Questions were presented first through iterative clinical cases or imaging, followed by questions directly evaluating comprehension of definitions.
Mult Scler Relat Disord
December 2024
CIEM MS Research Center, Federal University of Minas Gerais Medical School, Belo Horizonte, MG, Brazil.
Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease most frequently characterized by a neuromyelitis optica (NMO) phenotype, comprising both simultaneous or sequential optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM). Symptoms of brainstem, diencephalic and cerebral involvement may also occur. While most NMOSD patients test positive for serum aquaporin-4 (AQP4) antibodies, some seronegative patients test positive for oligodendrocyte glycoprotein-IgG (MOG-IgG).
View Article and Find Full Text PDFAJNR Am J Neuroradiol
October 2024
From the Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, UK (U.L.,F.D.), Laboratory of Developmental Biology, CNRS, Sorbonne-University, IPBS, Paris, France (M.C.), Department of Radiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, United States (M.H.L.), Department of Developmental Neuroscience, IRCCS Stella Maris Foundation, Pisa, Italy (R.P.), Department of Radiology, Tartu University Hospital, Tartu, Estonia (P.I., D.L., A.T.), Department of Radiology, The University of Tartu, Tartu, Estonia (P.I.), UOC Neuroradiologia, ASST Papa Giovanni XXIII, Bergamo, Italy (G.P.), Department of Radiology, Leeds Teaching Hospitals NHS Trust, Leeds, UK (I.C.), Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy (M.S., A.R.) and Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy (A.R.).
Pediatr Blood Cancer
December 2024
Department of Ophthalmology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Arch Argent Pediatr
October 2024
Clínica Universitaria Reina Fabiola, Córdoba, Argentina.
Russell's diencephalic syndrome is a set of signs and symptoms characterized by extreme weight loss, with no impairment of height or head circumference, without changes in intake or appetite. It is due to hypothalamic dysfunction associated with space-occupying lesions in this region. It is a rare cause of malnutrition in pediatrics, so its diagnosis is often delayed.
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