A curable pseudo-dementia related to an atypical presentation of giant cell arteritis

Area of Science:

• Neurology
• Rheumatology
• Vascular Medicine

Background:

• Giant cell arteritis (GCA) is a systemic vasculitis typically affecting large and medium arteries.
• Cranial GCA commonly presents with headache and visual disturbances, but neurological manifestations can be diverse.
• Atypical presentations, including significant cognitive decline, are less common but recognized complications.

Observation:

• A 70-year-old patient presented with rapid cognitive decline, functional impairment, weight loss, and cerebellar ataxia.
• 18FDG-PET imaging revealed metabolically active vasculitis in the brachiocephalic trunk.
• Temporal artery biopsy confirmed pan-arteritis, leading to a diagnosis of GCA with vascular involvement.

Findings:

• The patient experienced progressive cognitive improvement following treatment with corticosteroids and immunosuppressive therapy.
• This case highlights the potential for GCA to manifest with severe neurological symptoms, including cognitive impairment and ataxia.
• Published reports suggest a range of cognitive symptoms, such as memory impairment and delirium, may be linked to GCA.

Implications:

• GCA should be considered in the differential diagnosis of unexplained cognitive impairment, especially when accompanied by systemic inflammation and weight loss.
• Early recognition and treatment of GCA can lead to reversible neurological deficits and improved patient outcomes.
• This case underscores the importance of advanced imaging (18FDG-PET) and biopsy in diagnosing GCA with atypical vascular and neurological involvement.