Michael M McDowell1, Xiao Zhu1, Marion A Hughes2, Raymond F Sekula3. 1. Department of Neurosurgery, University of Pittsburgh Medical Center, 200 Lothrop Street, B-400, Pittsburgh, PA, 15213, USA. 2. Department of Radiology, Division of Neuroradiology, University of Pittsburgh Medical Center, Pittsburgh, PA, 15213, USA. 3. Department of Neurosurgery, University of Pittsburgh Medical Center, 200 Lothrop Street, B-400, Pittsburgh, PA, 15213, USA. sekularf@upmc.edu.
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.
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.
Authors: Parthasarathy D Thirumala; Xuhui Wang; Aalap Shah; Miguel Habeych; Donald Crammond; Jeffrey R Balzer; Raymond Sekula Journal: Br J Neurosurg Date: 2015-06-22 Impact factor: 1.596
Authors: Manish S Sharma; Douglas Kondziolka; Aftaab Khan; Hideyuki Kano; Ajay Niranjan; John C Flickinger; L Dade Lunsford Journal: Neurosurgery Date: 2008-10 Impact factor: 4.654