Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
, 4 (8), 455-60

Mapping the Progression of Progranulin-Associated Frontotemporal Lobar Degeneration

Affiliations
Case Reports

Mapping the Progression of Progranulin-Associated Frontotemporal Lobar Degeneration

Jonathan D Rohrer et al. Nat Clin Pract Neurol.

Abstract

Background: A 55-year-old woman was followed over a 13-year period as part of a longitudinal study of people at risk for familial dementia. She was a member of a family with an autosomal dominant familial dementia that fulfilled consensus criteria for frontotemporal lobar degeneration. The patient was initially asymptomatic but developed progressive behavioral and cognitive decline characterized by apathy, impaired emotion recognition, mixed aphasia and parietal lobe dysfunction.

Investigations: Clinical assessments, neuropsychometry, volumetric brain MRI, and genetic mutation screening.

Diagnosis: Progranulin-associated frontotemporal lobar degeneration.

Management: Explanation of the patient's condition and genetic counseling for her family.

Figures

Figure 1
Figure 1
Timeline showing clinical assessments and progression of neuropsychological and neuroimaging deficits in a person at risk of frontotemporal lobar degeneration.
Figure 2
Figure 2
Series of five registered T1-weighted MRI images of a patient with progranulin-associated frontotemporal lobar degeneration. (A) Eight and a half years before symptom onset. (B) Eighteen months before symptom onset. (C) Six months before symptom onset. (D) Six months after symptom onset. (E) Eighteen months after symptom onset.
Figure 3
Figure 3
Sagittal and coronal MRI images of a patient with progranulin-associated frontotemporal lobar degeneration, with voxel-compression-mapping overlay over the time periods (A) from 6 months before to 6 months after symptom onset, and (B) 6–18 months after symptom onset. Red represents 20% or greater expansion of voxels and blue represents 20% or greater contraction of voxels.

Similar articles

See all similar articles

Cited by 17 PubMed Central articles

See all "Cited by" articles

Publication types

Feedback