INTRODUCTION: Corticobasal syndrome (CBS) has a heterogeneous clinical 
presentation with no specific pathologic substratum. Its accurate diagnosis is a 
challenge for neurologists; in order to establish CBS definitively, postmortem 
confirmation is required. Some clinical and radiological features can help to 
distinguish it from other neurodegenerative conditions, such as 
Creutzfeldt-Jakob disease (CJD).
CLINICAL CASE: A 74-year-old woman presented with language impairment, 
difficulty in walking and poor attentiveness that had begun 10 days before. 
Other symptoms, such as asymmetrical extra-pyramidal dysfunction, limb dystonia 
and 'alien limb' phenomena, were established over the next 2 months, with rapid 
progression. Death occurred 3 months after symptom onset. Laboratory results 
were normal. Initially, imaging only showed restricted diffusion with bilateral 
parieto-occipital gyri involvement on DWI-MRI, with unspecific EEG changes. An 
autopsy was performed. Brain neuropathology confirmed sporadic CJD (sCJD).
CONCLUSIONS: CBS is a heterogeneous clinical syndrome whose differential 
diagnosis is extensive. CJD can occasionally present with clinical 
characteristics resembling CBS. MRI detection of abnormalities in some sequences 
(FLAIR, DWI), as previously reported, has high diagnostic utility for sCJD 
diagnosis - especially in early stages - when other tests can still appear 
normal. Abnormalities on DWI sequencing may not correlate with neuropathological 
findings, suggesting a functional basis to explain the changes found.