Difference between revisions of "Oculomasticatory myorhythmia"

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1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. Ann Neurol. 1986;20:677-83.  
 
1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. Ann Neurol. 1986;20:677-83.  
 +
 
2. Revilla FJ, de la Cruz R, Khardori N, Espay AJ. Teaching NeuroImage:
 
2. Revilla FJ, de la Cruz R, Khardori N, Espay AJ. Teaching NeuroImage:
 
Oculomasticatory myorhythmia: pathognomonic phenomenology of Whipple disease.
 
Oculomasticatory myorhythmia: pathognomonic phenomenology of Whipple disease.
 
Neurology. 2008;70:e25.  
 
Neurology. 2008;70:e25.  
 +
 
3. De Jonghe P, Martin JJ, Budka H, Ceuterick C. Cerebral manifestations of
 
3. De Jonghe P, Martin JJ, Budka H, Ceuterick C. Cerebral manifestations of
 
Whipple's disease. Acta Neurol Belg. 1979;79:305-13.  
 
Whipple's disease. Acta Neurol Belg. 1979;79:305-13.  
 +
 
4. Ratnaike RN. Whipple's disease. Postgrad Med J. 2000;76:760-6.
 
4. Ratnaike RN. Whipple's disease. Postgrad Med J. 2000;76:760-6.
 +
 
5. Hausser-Hauw C, Roullet E, Robert R, Marteau R. Oculo-facio-skeletal
 
5. Hausser-Hauw C, Roullet E, Robert R, Marteau R. Oculo-facio-skeletal
 
myorhythmia as a cerebral complication of systemic Whipple's disease. Mov Disord.
 
myorhythmia as a cerebral complication of systemic Whipple's disease. Mov Disord.
 
1988;3:179-84.  
 
1988;3:179-84.  
 +
 
6. Simpson DA, Wishnow R, Gargulinski RB, Pawlak AM. Oculofacial-skeletal
 
6. Simpson DA, Wishnow R, Gargulinski RB, Pawlak AM. Oculofacial-skeletal
 
myorhythmia in central nervous system Whipple's disease: additional case and
 
myorhythmia in central nervous system Whipple's disease: additional case and
 
review of the literature. Mov Disord. 1995;10:195-200.
 
review of the literature. Mov Disord. 1995;10:195-200.
 +
 
7. Louis ED, Lynch T, Kaufmann P, Fahn S, Odel J. Diagnostic guidelines in
 
7. Louis ED, Lynch T, Kaufmann P, Fahn S, Odel J. Diagnostic guidelines in
 
central nervous system Whipple's disease. Ann Neurol. 1996;40:561-8.
 
central nervous system Whipple's disease. Ann Neurol. 1996;40:561-8.
 +
 
8. Rajput AH, McHattie JD. Ophthalmoplegia and leg myorhythmia in Whipple's
 
8. Rajput AH, McHattie JD. Ophthalmoplegia and leg myorhythmia in Whipple's
 
disease: report of a case. Mov Disord. 1997 Jan;12(1):111-4. PubMed PMID:
 
disease: report of a case. Mov Disord. 1997 Jan;12(1):111-4. PubMed PMID:
  
 
[[Category:Neuro-ophthalmology]]
 
[[Category:Neuro-ophthalmology]]

Latest revision as of 15:43, 27 January 2018

Oculomasticatory myorhythmia (OMM) is an unusual combination of eye and jaw movements that is considered pathognomonic of CNS involvement by Whipple’s disease. OMM consists of synchronous vergence eye movements and contractions of the masticatory muscles. The eye movements are pendular vergence movements referred to as pendular vergence oscillations. The video demonstrates one of the first patients reported with OMM in the paper in which the term was introduced. Unusual facial and eye movements had been reported previously in Whipple’s disease, but the seminal 1986 publication by Schwartz et al reported two patients and characterized the disorder in detail, including eye movement recordings and masseter EMG.(1)

The index case was a 46 year old man with a history of seronegative rheumatoid arthritis who gradually lost the ability to read and developed excessive somnolence, urinary incontinence and weight loss. On admission to the McGuire VA Medical Center in Richmond, Va, he appeared cachectic and lethargic. The eyes oscillated in smooth, rhythmic convergence movements with divergence back to primary position at about 1 Hz. Rotation induced horizontal eye movements were full but compensatory saccades were absent. There was paralysis of both voluntary and reflex vertical eye movements. Rhythmic opening and closing of the jaw was synchronous with the eye movements. The eye and jaw movements were continuous during the day and persisted in sleep. Revilla, et alhave also provided a video of OMM.(2)

Because of the history of seronegative RA and previous reports of unusual facial and eye movements, CNS Whipple’s disease was suspected.(3) Initial small bowel biopsy was negative but inadequate. A second, fluoroscopically guided biopsy revealed the features of Whipple’s disease. Small bowel biopsy is normal in about 30% of patients with Whipple’s disease.(4) After antibiotic therapy there was improvement in mental status and lessening of the myorhythmia. When seen in clinic 10 years later, he was alert and conversant and had no obvious neurologic deficit. The eye and jaw movements persisted but were subtle. He complained bitterly of burning mouth that proved recalcitrant to all treatment attempts, including IV vitamin replacement. He had made several visits to the ER by EMS because of the burning mouth.

Evidence continues to mount that OMM is pathognomonic of CNS Whipple’s disease. OMM accompanied by synchronous movements of the extremities has been referred to as oculofacioskeletal myorhythmia.(5,6) Either OMM or oculofacioskeletal myorhythmia was present in 20% of a series of 84 patients, and was always accompanied by a supranuclear vertical gaze palsy.(7) In one reported case, there was supranuclear ophthalmoplegia and myorhythmia of one leg.(8)

Video courtesy of Dr. John B. Selhorst

References

1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. Ann Neurol. 1986;20:677-83.

2. Revilla FJ, de la Cruz R, Khardori N, Espay AJ. Teaching NeuroImage: Oculomasticatory myorhythmia: pathognomonic phenomenology of Whipple disease. Neurology. 2008;70:e25.

3. De Jonghe P, Martin JJ, Budka H, Ceuterick C. Cerebral manifestations of Whipple's disease. Acta Neurol Belg. 1979;79:305-13.

4. Ratnaike RN. Whipple's disease. Postgrad Med J. 2000;76:760-6.

5. Hausser-Hauw C, Roullet E, Robert R, Marteau R. Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple's disease. Mov Disord. 1988;3:179-84.

6. Simpson DA, Wishnow R, Gargulinski RB, Pawlak AM. Oculofacial-skeletal myorhythmia in central nervous system Whipple's disease: additional case and review of the literature. Mov Disord. 1995;10:195-200.

7. Louis ED, Lynch T, Kaufmann P, Fahn S, Odel J. Diagnostic guidelines in central nervous system Whipple's disease. Ann Neurol. 1996;40:561-8.

8. Rajput AH, McHattie JD. Ophthalmoplegia and leg myorhythmia in Whipple's disease: report of a case. Mov Disord. 1997 Jan;12(1):111-4. PubMed PMID: