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113:. 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. Of note the syndrome is not associated with developmental delay. There may be associated
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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".
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despite normal or slightly decreased caloric intake. Classically there is also locomotor hyperactivity and euphoria. Less commonly diencephalic syndrome may involve skin pallor without
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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".
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Vlachopapadopoulou E, Tracey KJ, Capella M, Gilker C, Matthews DE (June 1993). "Increased energy expenditure in a patient with diencephalic syndrome".
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region. It is not yet understood how diencephalic syndrome causes the effects on appetite and metabolism which are seen, though inappropriately high
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secretion and overall increased metabolic demand. It is treated with nutritional optimisation while the underlying lesion is treated with
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Diencephalic syndrome was first described by Dr. A. Russell in 1951. It is usually caused by a brain tumor such as a low-grade
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182:"Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants"
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The diencephalon, which is affected by diencaphalic syndrome, consists of the
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This article about a medical condition affecting the nervous system is a
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Spinal muscular atrophy with lower extremity predominance (SMALED)
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275:"A diencephalic syndrome of emaciation in infancy and childhood"
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Kim A, Moon JS, Yang HR, Chang JY, Ko JS, Seo JK (Jan 2015).
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1421:Syndromes affecting the nervous system
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1192:Distal hereditary motor neuronopathies
865:Template:Demyelinating diseases of CNS
82:Weight loss from diencephalic syndrome
384:
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1043:Idiopathic intracranial hypertension
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31:Diencephalic syndrome of emaciation
13:
1401:Neurological disorders in children
1274:Infantile progressive bulbar palsy
320:10.1111/j.1365-2265.1980.tb01040.x
14:
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977:Template:Cerebrovascular diseases
780:Frontotemporal lobar degeneration
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997:For more detailed coverage, see
975:For more detailed coverage, see
946:For more detailed coverage, see
912:For more detailed coverage, see
863:For more detailed coverage, see
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1:
1290:Amyotrophic lateral sclerosis
1175:Hereditary spastic paraplegia
1033:Normal pressure hydrocephalus
363:10.1016/s0022-3476(09)90021-x
159:
1431:Nervous system disease stubs
1349:. You can help Knowledge by
1259:Progressive muscular atrophy
7:
769:Primary progressive aphasia
10:
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1091:Hashimoto's encephalopathy
797:Posterior cortical atrophy
618:Striatonigral degeneration
469:Cavernous sinus thrombosis
1197:Spinal muscular atrophies
1165:Primary lateral sclerosis
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1023:Intracranial hypertension
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803:Creutzfeldt–Jakob disease
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1264:Progressive bulbar palsy
1055:Intracranial hypotension
1038:Choroid plexus papilloma
452:Herpesviral encephalitis
198:10.3345/kjp.2015.58.1.28
776:Frontotemporal dementia
462:Encephalitis lethargica
1396:Neurological disorders
1081:Hepatic encephalopathy
292:10.1136/adc.26.127.270
244:10.1542/peds.2004-2237
83:
1411:Syndromes with tumors
1139:Ataxia–telangiectasia
1096:Static encephalopathy
819:Mitochondrial disease
657:Spasmodic torticollis
567:Basal ganglia disease
87:Diencephalic syndrome
81:
22:Diencephalic syndrome
1086:Toxic encephalopathy
792:Lewy bodies dementia
1134:Friedreich's ataxia
517:Meningoencephalitis
457:Limbic encephalitis
1170:Pseudobulbar palsy
908:Status epilepticus
859:Multiple sclerosis
696:Myoclonic epilepsy
559:movement disorders
505:Acute disseminated
447:Viral encephalitis
273:Russell A (1951).
91:Russell's syndrome
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948:Template:Headache
914:Template:Epilepsy
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809:Vascular dementia
652:Status dystonicus
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500:Encephalomyelitis
95:failure to thrive
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16:Medical condition
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1071:Brain herniation
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722:Intention tremor
717:Essential tremor
582:Postencephalitic
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411:Diseases of the
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134:optic chiasmatic
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1222:Congenital DSMA
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993:Sleep disorders
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684:Choreoathetosis
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128:located in the
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138:growth hormone
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1426:Diencephalon
1351:expanding it
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1183:
1156:
1117:Degenerative
854:Inflammatory
801:
734:Stiff-person
572:Parkinsonism
545:Degenerative
442:Encephalitis
425:Inflammation
415:, primarily
357:(6): 922–4.
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350:
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314:(2): 181–7.
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278:
268:
238:(6): 724–6.
235:
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192:(1): 28–32.
189:
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154:radiotherapy
146:chemotherapy
142:β-lipotropin
130:hypothalamic
119:
90:
86:
85:
56:hypothalamus
30:
1406:Neurotrauma
1269:Fazio–Londe
1109:Both/either
903:Generalised
762:Early-onset
757:Alzheimer's
492:spinal cord
126:astrocytoma
111:hypotension
97:and severe
60:epithalamus
52:subthalamus
27:Other names
1390:Categories
877:paroxysmal
849:Autoimmune
642:Dyskinesia
512:Meningitis
490:Brain and
232:Pediatrics
160:References
99:emaciation
58:, and the
874:Episodic/
752:Tauopathy
703:Akathisia
691:Myoclonus
674:Athetosis
606:Tauopathy
351:J Pediatr
72:Neurology
68:Specialty
1416:Oncology
1327:Medicine
1240:SMALED2B
1235:SMALED2A
932:Migraine
924:Headache
890:epilepsy
886:Seizures
744:Dementia
647:Dystonia
336:41448424
260:11980144
252:15930202
216:25729396
48:thalamus
1252:SMA-PME
1247:SMA-PCH
1230:SMALED1
942:Tension
937:Cluster
662:Meige's
479:Amoebic
371:8501572
328:7438472
285:: 274.
207:4342778
150:surgery
103:anaemia
1313:Portal
971:Stroke
785:Pick's
712:Tremor
679:Chorea
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258:
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214:
204:
122:glioma
109:, and
54:, the
50:, the
1285:both:
1217:DSMA1
1212:SMAX2
1207:SMAX1
1187:only:
1160:only:
1064:Other
986:Other
898:Focal
531:Brain
434:Brain
332:S2CID
256:S2CID
89:, or
1347:stub
888:and
599:PKAN
594:NBIA
367:PMID
324:PMID
248:PMID
212:PMID
1202:SMA
1185:LMN
1158:UMN
1149:MND
1015:CSF
966:TIA
611:PSP
587:NMS
556:and
417:CNS
359:doi
355:122
316:doi
287:doi
240:doi
236:115
202:PMC
194:doi
152:or
124:or
1392::
1126:SA
633:OA
628:HD
577:PD
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168:^
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132:-
62:.
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