Russell Syndrome 1
General: Onset between 3 months and 2 years; caused by tumors of the anterior portion of the thalamus (usually astrocytoma), optic chiasm, midcerebellar region, and midline ependymoma; erosion under the anterior clinoid processes that causes a characteristic J-shaped sella in lateral skull films.
Ocular: Lid retraction; nystagmus (horizontal, vertical, or rotatory); homonymous hemianopsia; optic nerve atrophy.
Clinical: Extreme emaciation; euphoria; pale skin.
Ciccarelli EC, Huttenlocher PR. Diencephalic tumor: a cause of infantile nystagmus and cachexia. Arch Ophthalmol 1967; 78:350.
Geeraets WJ. Ocular Syndromes. 3rd ed. Philadelphia: Lea & Febiger, 1976.
Russell A. A diencephalic syndrome: emaciation in infancy and childhood. Arch Dis Child 1951; 26:274.
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