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Updated: Aug 23, 2025
Simultaneous Isolation of Principal Central Nervous System-Resident Cell Types from Adult Autoimmune Encephalomyelitis Mice
Published on: October 6, 2023
Isolated central nervous system Whipple disease
Pablo Barbero-Aznarez1, Ramon Perez-Tanoira2, Daniel Aguirre-Mollehuanca3
1Department of Neurosurgery, Instituto Clavel, San Francisco De Asis University Hospital, Madrid, Spain.
Background:
Whipple disease (WD) is an infection caused by Tropheryma whipplei, which might present in three different forms: classical, localized, and isolated in the central nervous system (CNS).
Methods:
We report the result of a systematic review of the literature on WD unusually presenting with exclusively neurological symptoms, including two previously unpublished cases. A description of two cases with isolated CNS WD was performed, as well as a literature search in Cochrane, Scielo, and PubMed.
Results:
Two male adult patients presented with exclusively neurological symptomatology. Both magnetic resonance imaging (MRI) showed an intracranial mass suggestive of brain tumor. The histopathological examination was consistent with WD, with no systemic involvement. In the review of the literature, 35 cases of isolated CNS WD were retrieved. The median age at diagnosis was 43.5 (IQR 31.5-51.5). In 13 patients, the MRI showed a brain mass consistent with a brain tumor. The most common finding in the biopsy was the periodic-acid Schiff-stained foamy macrophages. Only five cases presented the pathognomonic sign of oculomasticatory myorhythmia. Thirteen cases had an adverse outcome that resulted in death during follow-up, whereas another 13 improved. The other nine patients remained stable or presented moderate improvement.
Conclusion:
Isolated CNS WD is a rare disease that should be considered among the differential diagnosis of CNS mass lesions. Brain biopsy is necessary to establish the diagnosis. It is stressed in the literature that an extended antibiotic course is required to prevent relapses and to control the disease.
Insights
Whipple disease (WD) can affect the central nervous system (CNS) exclusively, mimicking brain tumors. Early diagnosis via brain biopsy and prolonged antibiotics are crucial for managing this rare neurological infection.
Area of Science:
- Neurology
- Infectious Diseases
- Pathology
Background:
- Whipple disease (WD) is an infection caused by *Tropheryma whipplei*.
- WD can manifest in classical, localized, or isolated central nervous system (CNS) forms.
Purpose of the Study:
- To systematically review literature on WD presenting solely with neurological symptoms.
- To describe two new cases of isolated CNS WD.
Main Methods:
- Systematic literature search of Cochrane, Scielo, and PubMed databases.
- Inclusion of two previously unpublished cases of isolated CNS WD.
- Analysis of clinical presentation, neuroimaging, histopathology, and outcomes.
Main Results:
- Two adult males presented with neurological symptoms and intracranial masses on MRI, diagnosed as WD via biopsy.
- Literature review identified 35 cases of isolated CNS WD; median age 43.5 years.
- Periodic-acid Schiff-stained foamy macrophages were common; oculomasticatory myorhythmia was rare. Outcomes varied, with 13 deaths and 13 improvements.
Conclusions:
- Isolated CNS WD is a rare condition that must be considered in the differential diagnosis of CNS mass lesions.
- Brain biopsy is essential for diagnosis.
- Extended antibiotic therapy is critical for disease control and relapse prevention.
