|Year : 2020 | Volume
| Issue : 1 | Page : 68-71
Corticobasal degeneration presenting with primary progressive aphasia
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Department of Neurology, Federal University of Santa Maria, Santa Maria, Rio Grande do Sul, Brazil
|Date of Submission||15-Sep-2019|
|Date of Decision||17-Oct-2019|
|Date of Acceptance||22-Oct-2019|
|Date of Web Publication||03-Feb-2020|
Mr. Jamir Pitton Rissardo
Av. Roraima n° 1000, Cidade Universitária, Bairro Camobi, Santa Maria - RS 97105-900
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rissardo JP, Caprara AL. Corticobasal degeneration presenting with primary progressive aphasia. Curr Med Issues 2020;18:68-71
| Case Scenario|| |
A 55-year-old female presenting with difficulty hearing and speaking was admitted to our hospital. Her family history was negative for neurological disorders. Although she could perform household activities and her daily habits and personality did not change, her language ability gradually deteriorated over the years. On neurological examination, agrammatic speech with impaired sentence comprehension relatively preserved single-word comprehension, and possible apraxia of speech was observed. Furthermore, her left superior limb was rigidity the right was normal. Laboratory tests were within normal limits. A brain magnetic resonance imaging (MRI) was requested [Figure 1].
|Figure 1: Axial T1-weighted (a), axial fluid-attenuated inversion recovery (b), axial T2-weighted gradient-echo (c), coronal T2-weighted (d), and sagittal fluid-attenuated inversion recovery (e and f) images of the brain MRI|
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| Questions|| |
- What are the findings in the brain MRI?
- What is the most likely diagnosis taking into account this clinical scenario?
| Answers|| |
- Neuroimages of the brain showed asymmetric parietal and perisylvian atrophy. Axial T1-weighted (a), axial fluid-attenuated inversion recovery (b), axial T2-weighted gradient-echo (c), coronal T2-weighted (d), and sagittal fluid-attenuated inversion recovery (d and e) images of the brain MRI [Figure 2].
- Probable nonfluent/agrammatic variant of primary progressive aphasia.
Corticobasal degeneration (CBD) is a rare and progressive neurodegenerative disease, which is probably related to pathological aggregation of tau protein in neurofibrillary or gliofibrillary tangles in the human brain. It was first described in 1967 and later recognized as a polymorphous disease due to its complexity. In this context, the classical symptoms, asymmetric motor features associated with higher cortical function, were associated with multiple histological presentations. A number of clinical criteria for CBD have already been proposed, but none was validated. In 2013, a review of pathologically confirmed cases of CBD, different phenotypes associated with CBD pathology, and these phenotypes were used to create two sets of consensus criteria the probable and the possible [Table 1]
|Figure 2: Neuroimages of the brain showed asymmetric parietal and perisylvian atrophy (arrows). Axial T1-weighted (a), axial fluid-attenuated inversion recovery (b), axial T2-weighted gradient-echo (c), coronal T2-weighted (d), and sagittal fluid-attenuated inversion recovery (e and f) images of the brain MRI|
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|Table 1: Corticobasal degeneration clinical criteria - systematic review (2013)|
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Another interesting fact is that in the early stages, brain imaging may be normal. As disease progresses, abnormalities such as asymmetric cortical atrophy are observed in almost 50% of the subjects. In this way, focal atrophy predominantly involves the posterior frontal and parietal regions, and atrophy of the corpus callosum is also commonly seen.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, et al.
Criteria for the diagnosis of corticobasal degeneration. Neurology 2013;80:496-503.
Chan JL, Liu AB. Anatomical correlates of alien hand syndromes. Neuropsychiatry Neuropsychol Behav Neurol 1999;12:149-55.
[Figure 1], [Figure 2]