Progressive supranuclear palsy (PSP)
Gradual problems with speech, gait, eye movements and cognition.
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Sagittal midline image demonstrates atrophy of the midbrain which is concave at its cranial margin (hummingbird sign). This concavity is also evident laterally on axial images (morning glory sign). These changes are disproportionate to the pontine volume with a pontine: midbrain ratio of 0.13 (normal ~0.24). Superior cerebellar peduncles are thinned.
The patient has problems with balance and gait which have been gradually getting worse. Speech is slurred and some cognitive problems. Hypomimia, hypophonia, and bradyphrenia. 23 on Mini Mental Status Examination.
Eye movements demonstrated markedly slowed saccadic velocities and hypometric saccades in all directions and the saccadic velocity was particularly slowed with vertical gaze. Amplitude appeared preserved. There were saccadic intrusions with pursuit eye movements.
All other cranial nerves were normal.
There was both prominent axial and appendicular rigidity with cog-wheeling seen at both wrists. Glabellar tap reflex was positive but grasp, pout, and palmomental reflex was absent. Jaw jerk was not exaggerated.
Many neurodegenerative diseases are difficult to establish with certainty. In this case, combination of clinical presentation with atypical parkinsonism, eye movement abnormalities and characteristic MRI features make the diagnosis of progressive supranuclear palsy (PSP) highly likely.
Case courtesy of Dr Raju Yerra.