Disease Information for Diencephalic syndrome

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Clinical Manifestations
Signs & Symptoms
Dizzy on Standing/Giddy Response
High blood pressure child
High blood pressure/sign
Low Blood Pressure
Orthostatic drop in blood pressure
Loss of body fat/trunk/limbs
Acromegalic habitus
Anorexia in Infant
Failure to Thrive
Failure to Thrive Child
Failure to thrive/infant sign
Feeding/Apetite Problems Child
Nausea
Vomiting
Vomiting in Children
Vomiting in infancy
Vomiting recurrent
Vomiting/effortless/without nausea
Weight loss good appetite
Ataxia
Fixed gaze/infant
Hypereactive Child
Hypereactive/on exam
Intelligence normal
Level of alertness maintained
Nystagmus
Nystagmus/Ocular dysmetria
Staggering Gait
Tremor
Tremor in Children
Euphoria/Elated/'high' behavior/Gaiety
Hyperactivity, child/signs
Short attention span
Hypersomnia/Increased need for sleep
Tall Adult
Tall/thin habitus/long body/Marfanoid
Weight Loss
Bilateral Vision Loss
Difficulty Focusing Eyes
Diplopia Double vision
Double Image Vision Confirmed
Visual Problems
True Vertigo Sign Confirmed
Clinical Presentation & Variations
Presentation/Infant FTT Emaciated with Adequate intake
Disease Progression
Course/Lethal
Course/Progressive
Onset/Infancy
Onset/Six months
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Infant
Population/Young child ('Twos')
Associated Diseases & Rule outs
Associated Disease & Complications
Amnesia
Blindness
Blindness in Children
Blindness, central
Cachexia/inanition/wasting
Diencephalic syndrome
Hydrocephalus, chronic acquired
Hypertension
Hypertension in a child
Hypoglycemia
Hypotension
Ophthalmoplegia
Vomiting Excess/Chronic
Ataxia Disorder
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/Anatomically strategic lesion (ex)
Pathophysiology/Apparent Growth hormone resistancea
Pathophysiology/Diencephalic brain lesion/disorder
Pathophysiology/Diencephalic lesion/CNS
Process
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Neoplastic (category)
PROCESS/Paraneoplastic disorder (ex)
PROCESS/Paraneoplastic neurologic disorder
PROCESS/Vegetative-Autonomic/Endocrine (category)
Synonyms
Synonym
Diencephalic syndrome of infancy, Diencephalic syndrome of infancy (disorder), Synonym/Diencephalia infantilis, Synonym/Diencephalic cachexia, Synonym/Diencephalic Syndrome of Childhood, Synonym/Diencephalic Syndrome of Emaciation, Synonym/Diencephalic syndrome of ematiation, Synonym/Diencephalic syndrome of infancy, Synonym/Paramedian Diencephalic Syndrome, Synonym/Russell syndrome, Synonym/Russell's Diencephalic Cachexia, Synonym/Russell's Syndrome
Definition

(also called Russell"s syndrome), a rare cause of failure to thrive in infancy and early childhood, first described by Russell in 1950; It is characterized by profound emaciation, normal caloric intake, absence of subcutaneous fat, locomotor hyperactivity, alertness and euphoria; It

commonly occurs in association with chiasmatic and hypothalamic astrocytoma but may be seen in association with other tumours in the suprasellar region including benign lesions such as epidermoid cyst; Posterior fossa tumours may also produce this syndrome; Tumours in children with diencephalic syndrome tend to present earlier and are larger tumours than those without the syndrome;

---[GE Health/Medcyclopaedia website 2006]--------

Diencephalic Syndrome; Diencephalic Syndrome of Childhood;

Diencephalic Syndrome of Emaciation; Paramedian diencephalic Syndrome ; Russell"s Diencephalic Cachexia;

Russell"s Syndrome; Russell"s diencephalic syndrome; Diencephalic cachexia, diencephalia infantilis, diencephalic syndrome of emaciation, diencephalic syndrome of infancy; The diencephalic syndrome is a very rare neurological disorder characterized by failure to thrive, abnormal thinness (emaciation), amnesia, intense sleepiness, unusual eye position and sometimes blindness; It is normally seen in infancy or early childhood but some cases have been reported in older children and even adults; Diencephalic syndrome is usually caused by a brain tumor such as a low-grade glioma or astrocytoma------[NORD website 2006]-----------------------------

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External Links Related to Diencephalic syndrome
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Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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