Tau dementia

Case contributed by Dr Pierre Wibawa

Presentation

55 year old farmer presents with a 2 year history of cognitive decline that initially started with depression, reduced speech, difficulty operating machinery, myoclonus and limb stiffness. CSF results show p-tau 129 (normal less than 75), t-tau 923 (normal less than 357), ABeta1-42 of 529 (normal more than 603).

Patient Data

Age: 55
Gender: Male
MRI

Moderate biparietal and left frontotemporal atrophy with relative preservation of hippocampi. Mild atrophy of left basal ganglia structure with bilateral T2 subcortical hyperintensities.

Nuclear medicine

SPECT Tc-99 displaying decreased metabolism

Bilateral hypometabolism in the frontal, parietal and temporal lobes with left-side and anterior predominance.  

Case Discussion

Tau protein is an important component in the assembly of neuronal microtubules, which facilitates intraneuronal transporting. In Alzheimer's disease (AD), tau protein is hyperphosphorylated (p-tau) and , histiologically, aggregated in neurofibrillary tangles1. The hyperphosphorylation of tau exists in a variety of dementia and movement disorders, and these are subsumed under 'tauopathies'. It can also co-exist with amyloid depositions. In clinical practice, the distinction of pathological, clinical and radiological finding can be complex, as findings may overlap. 

This man presents to various health services with a range of symptoms consisted of limb apraxia, non-fluent aphasia, frontal behaviour changes, myoclonus, visuospatial deficits, depression to extrapyramidal symptoms. He has attracted various cross-sectional diagnoses of AD, progressive non-fluent/agrammatic aphasia(PNFA) and Dementia with Lewy Body(DLB). However, the initial presentation and the course of the disease suggest corticobasal degeneration (CBD)2. Clinically, CBD may present like DLB. For example, such patients may present with extrapyramidal symptoms, mood disorders and cognitive decline. Frontotemporal Dementia, PNFA, AD and CBD lies in the spectrum of tauopathies. Radiology findings of tauopathies can be unspecific and not correlate with the clinical phenotype, as demonstrated in the SPECT scan of this case. Novel tau-specific PET tracers or ligands are available in the research settings and may potentially be useful in determining subtypes of tauopathies3

In such cases where the cross-sectional presentation differs over time, a cerebrospinal fluid investigation can increase the accuracy of diagnosis. Although the Amyloid/p-tau ratio in our case is consistent with late-onset Alzheimer's, the patient presents with early onset dementia and the CSF tau is disproportionately greater than amyloid4.

Coincidentally, this man has a history of significant head injury, which may have contributed to the course of his tau dementia. However, the association between tau and head injury remains debatable, and neuropathological examination is the current gold-standard for diagnosis tau-related dementia that is due to chronic traumatic encephalopathy5

PlayAdd to Share

Case information

rID: 54132
Case created: 23rd Jun 2017
Last edited: 29th Jun 2017
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.