Progressive supra-nuclear palsy

Clinical History

A 49-year-old male patient presented with over 2 years history of:
1. Difficulty in moving eyes and lack of control over the eyes.
2. Stiffness in both upper and lower limbs.
3. Difficulty with speech and swallowing.
4. Mild cognitive impairement.
5. No h/o past vascular insult/stroke.

Imaging Findings

Plain MRI findings:

• The mid-sagittal T1WI shows that the rostral midbrain tegmentum appears small in size with a concave profile (Fig 1) instead of normal convex profile (Fig 2).
• Pons appears normal.

• Mild dilatation of the third ventricle is noted(Fig 3).

• Increased signal intensity is noted in the periaqueductal grey matter on T2WI and FLAIR sequences.(Fig 4)

Discussion

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a neurodegenerative disease that affects cognition, eye movements, and posture. The disease usually develops after the sixth decade of life.
The onset of PSP is insidious and usually a prolonged phase of vague fatigue, headaches, arthralgias, dizziness and depression occurs. Patients also develop subtle personality changes, memory problems, and pseudobulbar symptoms.
The cardinal manifestations of PSP are supranuclear ophthalmoplegia, pseudobulbar palsy, prominent neck dystonia, Parkinsonism, behavioural, cognitive, and gait disturbances that cause imbalance and frequent falls.
Although the full constellation of symptoms occurring in a progressive fashion over time is characteristic, the vertical gaze palsy is the most distinctive single clinical feature.
Diagnosis:
In PSP there is pronounced atrophy of the mid brain [1] ( mesencephalon) which accounts for the typical upward gaze paralysis. Although in early stages MRI may offer little help; in advanced cases it serves as a powerful tool to differentiate it from other diseases especially Alzheimers and Parkinsonism diseases.
Normally the upper border of the mid brain is convex .On MRI sagittal images the atrophy of the mid brain in PSP results in a concave upper border of the midbrain with typical “Humming Bird Sign” [2] (Hallmark Feature. Atrophy of the upper brainstem is an acknowledged radiological and pathological feature of PSP. Also it has been described atrophy of the superior cerebellar peduncles in PSP.Other features on MRI includes dilatation of the third ventricle due to midbrain atrophy [1] and also increase in the signal intensity of the peri-aqueductal grey matter on T2WI, FLAIR and PD sequences consistent with gliotic changes.MRI parkinsonism index [3] can be used to differentiate PSP from parkinsons disease and also from parkinsons variant of Multisystem Atrophy(MSA-P).The index value is larger in patients with PSP than it is in those with parkinsons disease and MSA-P.
Treatment of PSP is challenging and only a few patients respond to dopaminergic or anticholinergic drugs and responses often are short-lived and incomplete. No medication is effective in halting the progression of PSP.
Message:
Imaging is indicated as adjunct to diagnose progressive supra-nuclear palsy and also to rule out stroke / normal pressure hydrocephalus / Alzheimers /Parkinsonism .
MRI is the imaging modality of choice in neuro-imaging cause of its multiplanar imaging capabilities and better differentiation of brain structures.
“Humming Bird Sign” [2] seen on mid sagittal image is due to atrophy of the superior part of midbrain and is pathognomic of PSP.

Differential Diagnosis List

Final Diagnosis

Progressive supra-nuclear palsy.

URL:	https://www.eurorad.org/case/9389
DOI:	10.1594/EURORAD/CASE.9389
ISSN:	1563-4086