Movement Disorders and Oculomotor Abnormalities in Whipple's Disease: An Updated Systematic Review
Errikos Maslias1, Ruben Anker2, Philip Euskirchen3,4
1Service of Neurology, Department of Clinical Neurosciences, Lausanne University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland.
Tremor and Other Hyperkinetic Movements (New York, N.Y.)
|November 17, 2025
Summary
Movement disorders and oculomotor abnormalities are key indicators of central nervous system Whipple's disease (CNS-WhD). Early recognition of these symptoms, particularly supranuclear gaze palsy and myorhythmia, can improve outcomes for this treatable infection.
Area of Science:
- Neurology
- Infectious Diseases
- Ophthalmology
Background:
- Whipple's disease (WhD) is a rare multisystemic infection caused by *Tropheryma whipplei*.
- Central nervous system (CNS) involvement occurs in up to 50% of WhD cases, often presenting with neurological symptoms.
- Movement disorders (MDs) and oculomotor abnormalities, such as oculomasticatory myorhythmia (OMM) and oculofacioskeletal myorhythmia (OFSM), are critical diagnostic clues but are often underrecognized.
Purpose of the Study:
- To update the understanding of MDs in CNS-WhD.
- To build upon previous reviews by incorporating recent case data.
- To highlight the diagnostic importance of specific neurological and oculomotor signs.
Main Methods:
- Systematic literature search of MEDLINE, EMBASE, and Cochrane Library (01/2017-05/2025).
- Inclusion of English-language human studies.
- Screening of titles, abstracts, and full-text by two independent reviewers using Rayyan.ai.
Main Results:
- 19 new articles (22 cases) were added, totaling 168 CNS-WhD patients with MDs or oculomotor abnormalities.
- Supranuclear gaze palsy (SGP) was the most frequent sign (58%), followed by myorhythmia and ataxia (40% each).
- Pathognomonic OMM/OFSM occurred in 25% of cases. MRI abnormalities were found in 87%, and brain tissue PAS staining showed high diagnostic yield.
Conclusions:
- Oculomotor abnormalities and MDs, especially SGP and myorhythmias, are crucial diagnostic indicators for CNS-WhD.
- These signs are important even when systemic symptoms are absent.
- Increased diagnostic awareness is vital for improving outcomes in this life-threatening yet treatable condition.
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