Central Nervous System Whipple Disease Presenting as Hypersomnolence

Area of Science:

• Infectious Diseases
• Neurology
• Microbiology

Background:

• Whipple disease (WD) is a rare systemic bacterial infection caused by Tropheryma whipplei.
• While typically affecting the gastrointestinal tract, WD can manifest with diverse clinical presentations, including cardiac, pulmonary, and central nervous system (CNS) involvement.

Observation:

• A 58-year-old male with a history of classic WD presented with functional decline, somnolence, and recurrent hydrocephalus.
• Cerebrospinal fluid (CSF) analysis revealed a positive Tropheryma whipplei polymerase chain reaction (PCR), indicating a neurologic relapse.

Findings:

• The patient received successful treatment with intravenous ceftriaxone followed by oral trimethoprim-sulfamethoxazole (TMP-SMX).
• Central nervous system involvement in WD often occurs late in the disease or as a relapse, particularly after incomplete treatment.

Implications:

• This case underscores the importance of considering CNS involvement in patients with a history of WD presenting with neurological symptoms.
• Prompt diagnosis and appropriate antibiotic therapy are essential for managing neurologic relapses of Tropheryma whipplei infection.