Diencephalic syndrome, or Russell's syndrome, is a rare neurological disorder seen in infants and children and characterised by failure to thrive and severe emaciation despite normal or slightly decreased caloric intake. Classically there is also locomotor hyperactivity and euphoria. Less commonly diencephalic syndrome may involve skin pallor without anaemia, hypoglycaemia, and hypotension.[1] The syndrome is a rare but potentially fatal cause of failure to thrive in children. Failure to thrive presents on average at seven months of age.[1] Of note the syndrome is not associated with developmental delay.[2] There may be associated hydrocephalus.[citation needed]
Diencephalic syndrome was first described by Dr. A. Russell in 1951.[3] It is usually caused by a brain tumor such as a low-grade glioma or astrocytoma located in the hypothalamic-optic chiasmatic region.[1] It is not yet understood how diencephalic syndrome causes the effects on appetite and metabolism which are seen, though inappropriately high growth hormone release has been proposed,[4] as has excessive β-lipotropin secretion[1] and overall increased metabolic demand.[5] It is treated with nutritional optimisation while the underlying lesion is treated with chemotherapy, surgery or radiotherapy.[citation needed]
Referencesedit
^ abcdKim A, Moon JS, Yang HR, Chang JY, Ko JS, Seo JK (Jan 2015). "Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants". Korean J Pediatr. 58 (1): 28–32. doi:10.3345/kjp.2015.58.1.28. PMC4342778. PMID 25729396.
^Fleischman A, Brue C, Poussaint TY, Kieran M, Pomeroy SL, Goumnerova L, Scott RM, Cohen LE (June 2005). "Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance". Pediatrics. 115 (6): 724–6. doi:10.1542/peds.2004-2237. PMID 15930202. S2CID 11980144.
^Russell A (1951). "A diencephalic syndrome of emaciation in infancy and childhood". Arch Dis Child. 26: 274. doi:10.1136/adc.26.127.270.
^Drop SL, Guyda HJ, Colle E (August 1980). "Inappropriate growth hormone release in the diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up". Clin Endocrinol. 13 (2): 181–7. doi:10.1111/j.1365-2265.1980.tb01040.x. PMID 7438472. S2CID 41448424.
^Vlachopapadopoulou E, Tracey KJ, Capella M, Gilker C, Matthews DE (June 1993). "Increased energy expenditure in a patient with diencephalic syndrome". J Pediatr. 122 (6): 922–4. doi:10.1016/s0022-3476(09)90021-x. PMID 8501572.
diencephalic, syndrome, russell, syndrome, rare, neurological, disorder, seen, infants, children, characterised, failure, thrive, severe, emaciation, despite, normal, slightly, decreased, caloric, intake, classically, there, also, locomotor, hyperactivity, eup. Diencephalic syndrome or Russell s syndrome is a rare neurological disorder seen in infants and children and characterised by failure to thrive and severe emaciation despite normal or slightly decreased caloric intake Classically there is also locomotor hyperactivity and euphoria Less commonly diencephalic syndrome may involve skin pallor without anaemia hypoglycaemia and hypotension 1 The syndrome is a rare but potentially fatal cause of failure to thrive in children Failure to thrive presents on average at seven months of age 1 Of note the syndrome is not associated with developmental delay 2 There may be associated hydrocephalus citation needed Diencephalic syndromeOther namesDiencephalic syndrome of emaciationThe diencephalon which is affected by diencaphalic syndrome consists of the thalamus the subthalamus the hypothalamus and the epithalamus SpecialtyNeurologyWeight loss from diencephalic syndromeDiencephalic syndrome was first described by Dr A Russell in 1951 3 It is usually caused by a brain tumor such as a low grade glioma or astrocytoma located in the hypothalamic optic chiasmatic region 1 It is not yet understood how diencephalic syndrome causes the effects on appetite and metabolism which are seen though inappropriately high growth hormone release has been proposed 4 as has excessive b lipotropin secretion 1 and overall increased metabolic demand 5 It is treated with nutritional optimisation while the underlying lesion is treated with chemotherapy surgery or radiotherapy citation needed References edit a b c d Kim A Moon JS Yang HR Chang JY Ko JS Seo JK Jan 2015 Diencephalic syndrome a frequently neglected cause of failure to thrive in infants Korean J Pediatr 58 1 28 32 doi 10 3345 kjp 2015 58 1 28 PMC 4342778 PMID 25729396 Fleischman A Brue C Poussaint TY Kieran M Pomeroy SL Goumnerova L Scott RM Cohen LE June 2005 Diencephalic syndrome a cause of failure to thrive and a model of partial growth hormone resistance Pediatrics 115 6 724 6 doi 10 1542 peds 2004 2237 PMID 15930202 S2CID 11980144 Russell A 1951 A diencephalic syndrome of emaciation in infancy and childhood Arch Dis Child 26 274 doi 10 1136 adc 26 127 270 Drop SL Guyda HJ Colle E August 1980 Inappropriate growth hormone release in the diencephalic syndrome of childhood case report and 4 year endocrinological follow up Clin Endocrinol 13 2 181 7 doi 10 1111 j 1365 2265 1980 tb01040 x PMID 7438472 S2CID 41448424 Vlachopapadopoulou E Tracey KJ Capella M Gilker C Matthews DE June 1993 Increased energy expenditure in a patient with diencephalic syndrome J Pediatr 122 6 922 4 doi 10 1016 s0022 3476 09 90021 x PMID 8501572 Portal nbsp Medicine nbsp This article about a medical condition affecting the nervous system is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title Diencephalic syndrome amp oldid 1204926681, wikipedia, wiki, book, books, library,
