title: 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.affiliation: *Neurology Department and †Infectious Diseases Department, Hospital and University of Coimbra, Coimbra, Portugal ‡Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
authors.forename: Miguel
authors.initials: M
authors.lastname: Tábuas-Pereira
authors.forename: Margarida
authors.initials: M
authors.lastname: Vicente
authors.forename: Filomena
authors.initials: F
authors.lastname: Coelho
authors.forename: Isabel
authors.initials: I
authors.lastname: Santana
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002443:Ceftriaxone / Q000627:therapeutic use; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D006801:Humans; D008875:Middle Aged; D020238:Prosopagnosia / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D008061:Whipple Disease / Q000150:complications* / Q000175:diagnosis
chemical_list: D000900:Anti-Bacterial Agents; D002443:Ceftriaxone