Diancephalic syndrome

Pathology

• This is usually caused by tumours in or around the hypothalamus
• It may also result from brainstem and optic pathway tumours
• It has been reported with non-tumour lesions

Causes

Tumour sites

Systemic features

Ophthalmic features

Neurological features

Differential diagnosis

Magnetic resonance imaging (MRI) brain

Synonym

References

1. La Spina M, Caruso M, Gulizia C, et al. Diencephalic syndrome: misleading clinical onset of low-grade glioma. Curr Oncol 2023; 30:8401-8410.
2. Trapani S, Bortone B, Bianconi M, Rubino C, Sardi I, Lionetti P, Indolfi G. Diencephalic syndrome in childhood, a challenging cause of failure to thrive: miniseries and literature review. Ital J Pediatr 2022; 48:147.
3. Tavares LS, Pinho RS, Aragão MM, et al. Diencephalic syndrome-like presentation of brainstem tumor: a series case-based review. Childs Nerv Syst 2023; 39:1115-1122. 
4. De Martino L, Picariello S, Triarico S, et al. Diencephalic syndrome due to optic pathway gliomas in pediatric patients: an Italian multicenter study. Diagnostics (Basel) 2022; 12:664.
5. Poussaint TY, Barnes PD, Nichols K, Anthony DC, Cohen L, Tarbell NJ, Goumnerova L. Diencephalic syndrome: clinical features and imaging findings. AJNR 1997; 18:1499-1505.
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Related checklists

• Hypothalamic hamartoma: clinical features
• Hypothalamic hamartoma: management
• Pallister Hall syndrome (PHS)
• Hypophysitis