J Mov Disord.  2017 Sep;10(3):145-148. 10.14802/jmd.17025.

‘Hummingbird’ Sign in a Patient with Guam Parkinsonism-Dementia Complex

• Yeo T
• Tan LC

• KMID: 2395012
• DOI: http://doi.org/10.14802/jmd.17025

Abstract

We present a case of a 71-year-old male Chamorro patient from Guam who presented with progressive supranuclear palsy (PSP)-Richardson's syndrome. Considering his strong family history of parkinsonism and a PSP phenotype, he was clinically diagnosed with Guam parkinsonism-dementia complex (PDC). Magnetic resonance imaging (MRI) of the brain revealed prominent midbrain atrophy with preserved pontine volume, forming the "˜hummingbird' sign, which has not been described before in Guam PDC. Molecular analysis of the chromosome 9 open reading frame 72 gene (C9orf72) showed only 6 GGGGCC repeats. We discuss the clinico-pathological similarities and differences between PSP and Guam PDC, and highlight the topography of neuropathological changes seen in Guam PDC to explain the appearance of the "˜hummingbird' sign on MRI.

Keyword

Guam parkinsonism dementia complex; progressive supranuclear palsy; MRI; hummingbird sign; mesial temporal sclerosis

MeSH Terms

Aged
Atrophy
Brain
Chromosomes, Human, Pair 9
Guam*
Humans
Magnetic Resonance Imaging
Male
Mesencephalon
Open Reading Frames
Parkinsonian Disorders
Phenotype
Supranuclear Palsy, Progressive