Facial spasms, but not hemifacial spasm: a case report and review of literature

Childs Nerv Syst. 2016 Sep;32(9):1735-9. doi: 10.1007/s00381-016-3057-7. Epub 2016 Mar 16.

Abstract

Introduction: Facial spasms represent a complicated array of neurological motor disorders with unique diagnostic and treatment algorithms. Due to the rarity of many of these disorders in the pediatric population, special care must be taken in identifying subtle differences in presentation of these disorders.

Methods: We present a case of a 3-year-old boy diagnosed with a brainstem ganglioglioma, Chiari 1 malformation, and a 2-year history of left-sided facial spasms. Stereotyped facial contractions and subtle eye deviation occurred every 10 s, with downward movement rather than upward elevation of the eyebrow.

Results: MRI revealed absence of a clear compressive vessel of the centrally-myelinized portion of the facial nerve, and EMG of the left facial nerve demonstrated no abnormal motor response or evidence of "lateral spread." Given these findings, a diagnosis of hemifacial seizures was made. Microvascular decompression was not recommended, and botulinum toxin injection was not pursued; however, the patient has remained refractory to antiepileptic drugs, possibly due to biochemical alteration by his ganglioglioma. He may eventually require surgical debulking should his symptoms progress.

Conclusion: Hemifacial spasm is a well-recognized disorder, but similar conditions can, at times, imitate its appearance. While our patient presented with facial spasms, his clinical history, examination, and radiographic and electrophysiological findings were more consistent with hemifacial seizures secondary to a brainstem lesion, rather than hemifacial spasms. It is important to distinguish the two entities, as misdiagnosis and inappropriate diagnostic or therapeutic measures may be taken inadvertently.

Keywords: Brow-lift sign; Hemifacial seizures; Hemifacial spasm; Pediatric.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / diagnostic imaging*
  • Brain Stem / diagnostic imaging*
  • Child, Preschool
  • Facial Muscles / diagnostic imaging
  • Hemifacial Spasm / diagnostic imaging*
  • Hemifacial Spasm / etiology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Spasm / diagnostic imaging