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Botulinum toxin treatment of facial myoclonus in suspected Rasmussen encephalitis

Nina Browner1, Shaheda N Azher, Joseph Jankovic

  • 1Department of Neurology, Cleveland Clinic Foundation, Cleveland, Ohio, USA.

Movement Disorders : Official Journal of the Movement Disorder Society
|June 8, 2006
PubMed

Insights

Rasmussen encephalitis (RE) can cause involuntary movements. This case study shows successful treatment of continuous myoclonus in a 26-year-old woman using botulinum toxin A injections.

Area of Science:

  • Neurology
  • Neuroscience
  • Movement Disorders

Background:

  • Rasmussen encephalitis (RE) is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere.
  • It typically presents in childhood with seizures, hemiparesis, and cognitive decline.
  • Involuntary movements, such as myoclonus, can be a challenging symptom in RE patients.

Observation:

  • A 26-year-old woman presented with a 3-year history of progressive, continuous myoclonus affecting the left face and arm, accompanied by left spastic hemiparesis.
  • Brain MRI revealed right hemisphere and basal ganglia atrophy.
  • EEG showed diffuse slowing with sharp waves, and FDG-PET indicated hypometabolism in the right cerebral hemisphere, basal ganglia, and thalamus.

Findings:

  • The patient exhibited symptoms consistent with Rasmussen encephalitis, including progressive hemiparesis and focal involuntary movements.
  • Neuroimaging and metabolic studies confirmed significant right cerebral hemisphere dysfunction.
  • Continuous myoclonus significantly impacted the patient's quality of life.

Implications:

  • This case highlights the potential for Rasmussen encephalitis to manifest in adulthood.
  • Botulinum toxin A injections provided a targeted and effective treatment for disabling myoclonus in this RE patient.
  • Further research into the long-term efficacy and broader application of botulinum toxin A for RE-associated myoclonus is warranted.

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