Diffuse neurofibrillary tangles with calcification (DNTC)
Neurodegenerative diseases
Diffuse neurofibrillary tangles with calcification (DNTC) is a rare condition characterized by the following clinical manifestations:
- Early-onset in life
- Higher prevalence in females
- Slowly progressive cortical dementia, initially manifesting with memory impairment
- Various neuropsychiatric symptoms may precede or accompany the dementia syndrome (hallucinations and delirium can occur relatively early in the disease course)
Calcification is a critical imaging feature that distinguishes this disease and is detectable in the early stages, despite normal thyroid and parathyroid function with normal serum calcium and phosphate levels.
Frontotemporal degeneration
Atrophy is localized in the frontotemporal lobes, with reduced cerebral blood flow in these regions on perfusion single-photon emission computed tomography (SPECT) imaging.
Calcificaction
Calcification involves the globus pallidus and the dentate nucleus.