Convergent-Divergent Nystagmus as a Manifestation of Oculopalatal Tremor

Galvez-Ruiz, Alberto; Roig, Carles; Muñoz, Silvia; Arruga, J

doi:10.3109/01658107.2011.609606

Cited by 8 publications

(7 citation statements)

References 12 publications

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“…Palatal myoclonus is characterized by rhythmical movements of the soft palate that are typically produced by contractions of the tensor or levator veli palatine; they may be synchronous with pharyngeal, laryngeal, neck, diaphragm, and other branchial muscles, occurring at a frequency of 2 to 3 Hz, similar to that observed in myorhythmia . Convergent‐divergent and pendular nystagmus has also been reported in association with PM . The rhythmic movements of PM are thought to originate from dysfunction or lesions affecting the Guillain‐Mollaret triangle (GMT) (Fig.…”

Section: Phenomenology Of Myorhythmia and Differential Diagnosismentioning

confidence: 96%

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Myorhythmia: Phenomenology, etiology, and treatment

2014

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Myorhythmia is defined as repetitive, rhythmic, slow (1-4 Hz) movement affecting chiefly cranial and limb muscles. When occurring in the limbs it may be oscillatory and jerky, whereas oculo-masticatory myorhythmia, typically associated with Whipple's disease, is a slow, repetitive, often asymmetrical, facial and ocular movement. Thus, myorhythmia overlaps phenomenologically with tremor and segmental myoclonus. Although often present at rest, it must be differentiated from parkinsonian or dystonic tremor. Recognition of this movement disorder is important because it is usually associated with lesions involving the brainstem, thalamus, or other diencephalic structures with potentially treatable etiologies. In addition to Whipple's disease, myorhythmia has been described in patients with cerebrovascular disease, listeria encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, steroid-responsive encephalopathy associated with autoimmune thyroiditis, multiple sclerosis, and other disorders. In addition to our own experience, we have systematically reviewed the medical literature, focusing on the phenomenology, pathophysiology, and etiology of this poorly recognized movement disorder. In this review, we aim to highlight the clinical features that differentiate myorhythmia from other movement disorders. Treatment should be directed against the underlying etiology.

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Section: Phenomenology Of Myorhythmia and Differential Diagnosismentioning

confidence: 96%

“…[37][38][39] Convergentdivergent and pendular nystagmus has also been reported in association with PM. 40 The rhythmic movements of PM are thought to originate from dysfunction or lesions affecting the Guillain-Mollaret triangle (GMT) (Fig. 2).…”

Section: B a I Z A B A L -C A R V A L L O E T A Lmentioning

confidence: 99%

Myorhythmia: Phenomenology, etiology, and treatment

2014

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“…Patients have mainly vertical pendular oscillations of the eyes with varied combinations of torsional and horizontal components ( 21 – 24 ) (Video S2 in Supplementary Material). The nystagmus can sometimes take the form of convergent–divergent nystagmus ( 20 , 25 ). This pendular nystagmus is of quite large mean amplitude (8°), high peak velocity (16°/s), and demonstrates irregularity ( 24 ) (Figure 1 ).…”

Section: Clinical Features Of Pt and Optmentioning

confidence: 99%

Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

2017

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Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor (PT) or oculopalatal tremor (OPT). This syndrome manifests as a synchronous tremor of the palate (PT) and/or eyes (OPT) that may also involve other muscles from the branchial arches. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number and size of astrocytes, severe fibrillary gliosis, and demyelination. It appears on MRI as an increased T2/FLAIR signal intensity and enlargement of the inferior olive. There are two main conditions in which hypertrophic degeneration of the inferior olive occurs. The most frequent, studied, and reported condition is the development of PT/OPT and hypertrophic degeneration of the inferior olive in the weeks or months following a structural brainstem or cerebellar lesion. This “symptomatic” condition requires a destructive lesion in the Guillain–Mollaret pathway, which spans from the contralateral dentate nucleus via the brachium conjunctivum and the ipsilateral central tegmental tract innervating the inferior olive. The most frequent etiologies of destructive lesion are stroke (hemorrhagic more often than ischemic), brain trauma, brainstem tumors, and surgical or gamma knife treatment of brainstem cavernoma. The most accepted explanation for this symptomatic PT/OPT is that denervated olivary neurons released from inhibitory inputs enlarge and develop sustained synchronized oscillations. The cerebellum then modulates/accentuates this signal resulting in abnormal motor output in the branchial arches. In a second condition, PT/OPT and progressive cerebellar ataxia occurs in patients without structural brainstem or cerebellar lesion, other than cerebellar atrophy. This syndrome of progressive ataxia and palatal tremor may be sporadic or familial. In the familial form, where hypertrophic degeneration of the inferior olive may not occur (or not reported), the main reported etiologies are Alexander disease, polymerase gamma mutation, and spinocerebellar ataxia type 20. Whether or not these are associated with specific degeneration of the dentato–olivary pathway remain to be determined. The most symptomatic consequence of OPT is eye oscillations. Therapeutic trials suggest gabapentin or memantine as valuable drugs to treat eye oscillations in OPT.

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“…We found 24 publications on OPT for a total of 82 reported cases [1,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30]. We compiled them with our series of 8 patients, totaling 90 cases of OPT.…”

Section: Resultsmentioning

confidence: 99%

Oculopalatal Tremor: Variations on a Theme by Guillain and Mollaret

Jang

Borruat²

2014

Eur Neurol

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Background: Oculopalatal tremor (OPT) is a delayed complication of a brainstem lesion, characterized by involuntary contractions of the soft palate associated with a synchronized ocular pendular nystagmus. MRI reveals inferior olivary nucleus hypersignal/hypertrophy (IONH). Our objective was to refine the clinical profile of patients with OPT and to report a few oddities in both presentation and evolution. Methods: We performed a retrospective study of patients diagnosed with OPT and a literature search. Results: From our database, we retrieved 5 men and 3 women with a diagnosis of OPT. Eighty-two patients with OPT were retrieved from the literature and were compiled with our series. The average age was 54 years and there was a male predominance. Brainstem vascular lesion was the most common etiology (80%). Prominent vertical pendular nystagmus was found in 90%. Dissociated nystagmus was mostly associated to unilateral contralateral IONH on MRI, while bilateral symmetrical nystagmus was due to a bilateral IONH in the majority of cases. Three oddities were found amongst our 8 patients: prominent nystagmus ipsilateral to IONH; disappearance of IONH on MRI despite persisting nystagmus, and asymptomatic OPT. Conclusion: The clinical profile of OPT is rather stereotyped. Rarely do patients deviate from the classical description of OPT.

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Convergent-Divergent Nystagmus as a Manifestation of Oculopalatal Tremor

Cited by 8 publications

References 12 publications

Myorhythmia: Phenomenology, etiology, and treatment

Myorhythmia: Phenomenology, etiology, and treatment

Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

Oculopalatal Tremor: Variations on a Theme by Guillain and Mollaret

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