Pick's disease

Introduction

May be deprecated term for frontotemporal dementia.

Epidemiology

• 5th-7th decade of life

Pathology

• lobar atrophy of temporal &/or frontal lobes
  • may involve basal ganglia
• atrophy is generally symmetric, but may be asymmetric
• gliosis
• neuronal loss
• Pick bodies
• amyloid plaques may be present
• neurofibrillary tangles may be present

Genetics

reported cases of autosomal dominant familial disease

Clinical manifestations

• cognitive impairment -> slowly progressive dementia
• behavior disturbance
  • hyper-oral behavior
  • bulimia
  • emotional disinhibition
  • irritability
  • persistent aimless wandering
  • personality changes
• language disturbance
  • aphasia
  • forced repetitive speech patterns
  • echolalia in later phases
  • mutism with severe disease

Laboratory

• see dementia
• autopsy confirms diagnosis

Radiology

• neuroimaging (CT or MRI) shows lobar atrophy of temporal &/or frontal lobes

Differential diagnosis

• difficult to distinguish from Alzheimer's disease

Management

• no specific treatment

More general terms

• frontotemporal dementia

Patient information

Pick's disease patient information

Database

• OMIM: https://mirror.omim.org/entry/172700