Graefe Usher syndrome MRI

MRI brain shows:

Thinning of bony calvarium with inner table scalloping, cerebral cortical atrophy marked in bilateral frontal and temporal lobes with sub dural hygroma iso intense to Csf.

Diffuse cerebellar atrophy with widening of CSF space in posterior fossa overlying bilateral cerebral convexity, CP angle cisterns isointense to CSF.

Bilateral symmetric widening of bilateral internal auditory canal, Meckel's cave.

Widened empty sella.

Associated bilateral optic nerve atrophy, increased prominence of sub arachnoid spaces around bilateral optic nerves.

Imaging diagnosis: Graefe-Usher syndrome.

Usher syndrome is characterized by partial or total hearing loss, vision loss that worsens over time. The hearing loss is sensorineural, caused by abnormalities of the inner ear. 

A rare, congenital, autosomal recessive disorder characterized by retinitis pigmentosa and sensorineural hearing loss, first described by Von Graefe in 1858.

Secondary Empty Sella MRI

Term Empty sella was first applied by Busch in 1951 to an anatomic finding of severely flattened Pituitary gland against the floor of the sella at autopsy.

Primary Empty Sella Syndrome is an anatomical variation where the wide aperture of the diaphragma sella, through which the pituitary stalk reaches pituitary. When this aperture is wide, the cardio pulmonary pulsations with time make the sella wide with flattening of the pituitary gland at the floor of sella. When an isolated finding has no clinical significance and pt's are usually asymptomatic.

MRI is investigation of choice. Midline sagittal T1 and T2 images show sella occupied by fluid isointense to Csf, infundibular stalk traversing the sellar cavity to the residual pituitary tissue which is flattened at the floor of hypophyseal fossa.
On MRI other findings like slit like ventricles, prominent subarachnoid space around the optic nerves, tortuosity of the optic nerves, compressed dural venous sinuses should be looked for, presence of which may suggest the clinical diagnosis of Idiopathic Intracranial hypertension in symptomatic patients with papillodema clinically.

Secondary empty sella syndrome occurs when the sella is empty because the pituitary gland has been damaged by either Radiotherapy or Surgery.

MRI Sagittal T2 image shows Empty Sella secondary to surgery as pt's previous clinical details reveals operative notes mentioning a pituitary adenoma excised with trans sphenoid approach.