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).
. 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.
. 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).
. Axial FLAIR image shows increased tegmental signal intensity compared to age matched control.
Typical Magnetic Resonance Image (MRI) of PSP presents with midbrain atrophy11 . 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. and hiperintensity22 . 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. while CBS displays asymmetrical frontoparietal and CP atrophy33 . Koyama M, Yagishita A, Nakata Y, Hayashi M, Bandoh M, Mizutani T. Imaging of corticobasal degeneration syndrome. Neuroradiology 2007;49:905-912..
This case has been clinically classified as PSP-CBS4 but definite diagnosis is through neuropathology.
References
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1Massey 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.
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2Righini 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.
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3Koyama M, Yagishita A, Nakata Y, Hayashi M, Bandoh M, Mizutani T. Imaging of corticobasal degeneration syndrome. Neuroradiology 2007;49:905-912.
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4Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009;8:270-279.
Publication Dates
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Publication in this collection
July 2014
History
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Received
13 Feb 2014 -
Reviewed
10 Apr 2014 -
Accepted
30 Apr 2014