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NEUROLOGY 2008;70:e25
© 2008 American Academy of Neurology
Resident and Fellow Section

Teaching NeuroImage: Oculomasticatory myorhythmia

Pathognomonic phenomenology of Whipple disease

Formula

Fredy J. Revilla, MD, Rafael de la Cruz, MD, Nancy Khardori, MD, FACP and Alberto J. Espay, MD, MSc

From the Department of Neurology (F.J.R., A.J.E.), Movement Disorders Center, University of Cincinnati, OH; Division of Infectious Diseases (R.d.l.C.), Capitol Community Health Center, Springfield, IL; and Department of Medicine (N.K.), Division of Infectious Diseases, Southern Illinois University, Springfield.

Address correspondence and reprint requests to Dr. Alberto J. Espay, University of Cincinnati, 231 Albert Sabin Way, MSB 4503, Cincinnati, OH 45267-0525 alberto.espay{at}uc.edu.

A 41-year-old patient developed diplopia, imbalance, and weight loss. Examination showed pendular vergence oscillations of the eyes and synchronous contractions of the masticatory but not palatal muscles, i.e., oculomasticatory myorhythmia (OMM; figure). There was complete supranuclear vertical and, to a lesser extent, horizontal gaze palsy. The remainder of the examination was unremarkable. Brain MRI was normal. OMM is pathognomonic of Whipple disease.1 In its presence, neither jejunal biopsy nor blood or CSF PCR of Tropheryma whippelii is necessary for the initiation of trimethoprim-sulfamethoxazole.2 This patient became symptom free after 6 months of treatment. Video footage of the typical presentation should assist clinicians in recognizing this highly treatable neurologic disorder.


Figure 117
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  		Figure Divergent and convergent ocular oscillations and attempts to upgaze and downgaze

Episodes of divergent (A) and convergent (B) ocular oscillations can be appreciated in primary gaze (the corneal light is displaced laterally from A to B). Note the elevation of the inferior eyelid crease (horizontal arrow, A to B), indicating contraction of the levator labii muscles, synchronous with the convergent ocular movements. Attempts to upgaze (C) and downgaze (D) are ineffective.

 

Disclosure: The authors report no conflicts of interest.


   REFERENCES
Top.
 REFERENCES
 

  1. Schwartz MA, Selhorst JB, Ochs AL, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. Ann Neurol 1986;20:677–683.[Medline]
  2. Louis ED, Lynch T, Kaufmann P, Fahn S, Odel J. Diagnostic guidelines in central nervous system Whipple's disease. Ann Neurol 1996;40:561–568.[Medline]




This Article
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Right arrow Articles by Espay, A. J.
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Right arrow Nystagmus

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