“Case Reports in Neurology” is a new open access journal. The articles are available online only and at no charge.
A report was published recently on a case of “rapidly progressive” corticobasal syndrome. “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.”
After three months, the woman died. Upon brain autopsy, it was determined that she had CJD (Creutzfeldt-Jakob disease), sometimes called the human version of “mad cow disease.” (In the US, special precautions are taken when someone with a rapidly progressive dementia donates his/her brain. Presumably the same is true in Portugal, where this woman lived.)
A few people here have reported this same outcome — CJD is found upon brain autopsy. But I think all the people here whose loved ones had a confirmed CJD diagnosis had received clinical diagnoses of PSP rather than CBD.
The authors seem to imply that they used MRI scan to diagnose this woman while alive with CJD. They describe how an MRI is helpful in diagnosing CBD: “On MRI, CBD patients may present asymmetric frontoparietal and midcallosal atrophy. Additionally, on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images, they may also show putaminal hypointensity and subtle subcortical periventricular hyperintensity. On DWI, extensive hyperintensity in the frontoparietal white matter, especially on the predominant side of cortical atrophy, has been described. These features are especially evident in the early course of CBD.”
I’ve copied the abstract below.
The link to get to the article (PDF) online is very long:
http://content.karger.com/ProdukteDB/pr … elNr=32982\
It might be easier to go first to the journal’s home page here:
http://content.karger.com/ProdukteDB/pr … roduktNr=2\
Then click on the “May – August” issue. Then click on the article that begins
on page 185.
Case Reports in Neurology. 2011 May;3(2):185-190. Epub 2011 Aug 23.
Rapidly Progressive Corticobasal Degeneration Syndrome.
Valverde AH, Costa S, Timoteo A, Ginestal R, Pimentel J.
Department of Neurology, Hospital Prof. Dr. Fernando Fonseca, EPE, Amadora, EPE/Hospital de Santa Maria, Lisbon, Portugal.
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).
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).
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.
PubMed ID: #21941496 (see pubmed.gov for this abstract only)