Prosopagnosia as the Presenting Symptom of Whipple Disease.

Abstract

Whipple disease is a rare, chronic multisystem infectious disease. The central nervous system (CNS) is secondarily involved in 43% of patients; 5% of patients have isolated or primary CNS involvement. The most frequent CNS symptoms are cognitive changes. Prosopagnosia is an inability to recognize familiar faces, in a person who does not have vision impairments or cognitive alterations. This relatively rare condition is usually related to vascular, traumatic, degenerative, or infectious lesions. We report a 54-year-old woman who presented subacutely with fever, headache, and seizures that led to a diagnosis of infectious meningoencephalitis. She improved temporarily on broad-spectrum antibiotics, but then developed a chronically evolving cognitive impairment with associative prosopagnosia as the major complaint. She had a history of sporadic abdominal pain and mild sacroiliac arthralgia. After a negative duodenal biopsy, we confirmed primary CNS Whipple disease by polymerase chain reaction and brain biopsy. We treated the patient with ceftriaxone for 15 days and then co-trimoxazole for 2 years. At 8-year follow-up, she had no further impairments, but continuing prosopagnosia. To our knowledge, this is the first description of isolated prosopagnosia in a patient with primary CNS Whipple disease. Because CNS Whipple disease can lead to serious, irreversible lesions if not promptly treated, clinicians must suspect the diagnosis, treat with long-term antibiotics, and follow patients carefully to prevent recurrence.

• Authors: Miguel Tábuas-Pereira, Margarida Vicente, Filomena Coelho, Isabel Santana
• Journal: Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology (Cogn Behav Neurol) Vol. 29 Issue 2 Pg. 100-6 (06 2016) ISSN: 1543-3641 [Electronic] United States
• PMID: 27336807 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Bacterial Agents
• Ceftriaxone

Topics

• Anti-Bacterial Agents (therapeutic use)
• Ceftriaxone (therapeutic use)
• Cognition Disorders (diagnosis, etiology)
• Female
• Humans
• Middle Aged
• Prosopagnosia (diagnosis, drug therapy, etiology)
• Whipple Disease (complications, diagnosis)