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Opsoclonus-Myoclonus syndrome
  1. Marta Esteves Guedes,
  2. Ana Catarina Almeida,
  3. Maria Sara Patricio,
  4. João Marques Costa
  1. Ophthalmology Department, Hospital Egas Moniz, Lisbon, Portugal
  1. Correspondence to Dr Marta Esteves Guedes, marta.ce.guedes{at}gmail.com

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Description

Opsoclonus-Myoclonus syndrome is a rare dyskinesia due to an underlying infectious, metabolic or neoplastic process.1 It is frequently caused by neuroblastoma in children and breast or lung carcinoma in adults. It is characterised by high amplitude, multidirectional, arrhythmic and conjugate ocular saccadic intrusions without inter-saccadic latency causing oscillopsia2 and it is associated with myoclonus of axial and/or apendicular musculature3 and ataxia. Autoimmune dysfunction of Purkinje cells in the dorsal vermis and subsequent disinhibition of oculomotor fastigial region seems to be the most probable mechanism.4 A 53-year-old female, diagnosed with breast carcinoma 2½ years before the first ophthalmologic assessment. She had refused mastectomy and had nausea, vomiting and cerebellar gait ataxia followed by dysarthria, opsoclonus-related oscillopsia (could not read) and cervical myoclonus. The syndrome was exacerbated by tactile, sound or bright light stimuli and reduced by convergence (video 1). The patient was submitted to left radical mastectomy and monthly chemotherapy (adriamicin 60 mg/m2 and ciclophosphamide 600 mg/m2). Due to persistence of the opsoclonus-myoclonus syndrome with severe gait ataxia, intravenous immunoglobulin (IVIG), 2 g/kg, was started. Progressive improvement of oscillopsia and complete remission of opsoclonus was evident. Six months after initiating therapy, she had complete remission of the myoclonic component. Mild broad base gait is at the moment, possible without aid. We report a severe Opsoclonus-Myoclonus syndrome starting 2½ years after breast carcinoma. Mastectomy and adjuvant chemotherapy were essential in stabilising the underlying pathologic mechanism but the immune-modulating action of IVIG seems to be helpful since clinical improvement was observed after initiating therapy with complete remission after 6 months.

Video 1

Opsoclonus-Myoclonus video: high amplitude multidirectional and irregular saccadic intrusions, without interssaccadic latency, exacerbated by bright light stimuli and reduced by convergence. Vestibulo-ocular reflex and opto-kinetic nystagmus are present. We can see concomitant cervical axial musculature myoclonus.

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

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