Progressive supranuclear palsy

Case contributed by Dr Utkarsh Kabra

Presentation

Forgetfulness, parkinsonian symtoms

Patient Data

Age: 80 years
Gender: Female

Disproportionate atrophy of midbrain is seen with concavity of its superior and dorsolateral margins.
Midline sagittal midbrain to pons area ratio is ~ 0.12 (reduced; normal value is ~ 0.24). Mid brain to pons width ratio is approx. 0.4 (reduced).
Convexity sulci and lateral ventricles are prominent, likely age related atrophy.

Chronic lacunar infarcts, confluence and discrete area of ischemic demyelination are seen in pons, bilateral lentiform nuclei, periventricular, deep and subcortical white matter.
Focal microhemorrhage is seen in left cerebral peduncle.
Linear hypointense area is seen in pons in midline with blooming on GRE images, possibly representing a developmental venous anomaly or capillary telangiectasia.

Case Discussion

Disproportionate atrophy of midbrain is seen with concavity of its superior and dorsolateral margins.
Midline sagittal midbrain to pons area ratio is ~ 0.12 (reduced; normal value is ~ 0.24). Mid brain to pons width ratio is approx. 0.4 (reduced).
 

These findings along with clinical history of the patient suggest possibility of progressive supranuclear palsy. The above measurements are important to objectively assess for the disease in a patient with clinical suspicion as the so called 'humming bird' sign of progressive supranuclear palsy can be seen in a lot of patients of older age group.

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