Overlapping MRI findings in progressive supranuclear palsy – corticobasal syndrome

Sobreposição de achados na RM da paralisia supranuclear progressiva – síndrome corticobasal

A 59 year old woman presented with an atypical parkinsonian syndrome with clinical and neuroimaging features of corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP). CBS manifestations were slurred/scanning speech and asymmetrical right signs: levitation phenomena, pseudo-hemiparetic gait and hypertonia/hyperreflexia. PSP signs were staring face and oculomotor apraxia. Neuroimaging revealed midbrain atrophy with asymmetrical cerebral peduncle (CP), tegmental hiperintensity (Figure 1 to 4) and frontal and midbrain hypometabolism (Figure 5).

Figure 1
. Sagital T1 weighted image at the midline shows reduction of anteroposterior midbrain diameter (1.4 cm – Normal Range (NR)>1.8 cm) and tegmental size reduction (0.95 cm – NR>1.15 cm); Age matched control midbrain.

Figure 2
. Axial T2 weighted image in high frontoparietal convexity showing no cortical asymmetry.

Figure 3
. Axial T2 weighted image at midbrain level displays anteroposterior midbrain size reduction (1.2 cm – NR>1.8 cm) and marked cerebral peduncle asymmetry (right CP 1.53 cm and left CP 1.26 cm).

Figure 4
. Axial FLAIR image shows increased tegmental signal intensity compared to age matched control.

Figure 5
. Technetium SPECT showing symmetrical frontal lobe and midbrain hypoperfusion.

Typical Magnetic Resonance Image (MRI) of PSP presents with midbrain atrophy¹ and hiperintensity² while CBS displays asymmetrical frontoparietal and CP atrophy³.

This case has been clinically classified as PSP-CBS⁴ but definite diagnosis is through neuropathology.

References

• ¹ Massey LA, Jäger HR, Paviour DC, et al. The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy. Neurology 2013;80:1856-1861.
• ² Righini A, Antonini A, De Notaris R, et al. MR imaging of the superior profile of the midbrain: differential diagnosis between progressive supranuclear palsy and Parkinson disease. AJNR Am J Neuroradiol 2004;25:927-932.
• ³ Koyama M, Yagishita A, Nakata Y, Hayashi M, Bandoh M, Mizutani T. Imaging of corticobasal degeneration syndrome. Neuroradiology 2007;49:905-912.
• ⁴ Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009;8:270-279.

Publication Dates

• Publication in this collection
  July 2014

History

• Received
  13 Feb 2014
• Reviewed
  10 Apr 2014
• Accepted
  30 Apr 2014