title: Spastic paretic hemifacial contracture (SPHC) in a patient with multiple sclerosis. A clinical, EMG and neuroimaging study.
abstract: We present the case of a 42-year-old woman with a 4-year history of definite multiple sclerosis (MS) and sustained contracture of the left hemiface with marked left deviation of the nose, deepened left nasolabial groove, narrowed palpebral fissure and weakness of the contracting muscles. Needle electromyography showed continuous resting activity of irregularly firing motor unit potentials (MUP) in the left orbicularis oculi (OO) and orbicularis oris, but not in the left frontalis. Simultaneous recording of maximal voluntary contraction of the OO on both sides showed marked preponderance on the right. Blink reflex findings were consistent with an injury in the pons, mainly in the vicinity of the left facial nucleus. A magnetic resonance imaging study showed multiple supra- and infra-tentorial white matter lesions, as well as multiple lesions at the level of the pons. Complete recovery was observed after 3 months. This condition has been described in cases of brainstem tumor as spastic paretic hemifacial contracture (SPHC). Up to now SPHC has not been explicitly associated with MS and, in this case, it could be arguably attributed to hyperexcitability of the facial neurons due to demyelination of the corticofacial fibers.
authors.affiliation: EMG Laboratory, Department of Neurology, Aeginition Hospital, University of Athens, 72-74 Vas. Sofias Avenue, 11528 Athens, Greece.
authors.forename: Ptolemeos
authors.initials: P
authors.lastname: Sarrigiannis
authors.forename: Constantinos
authors.initials: C
authors.lastname: Tsakanicas
authors.forename: Maria
authors.initials: M
authors.lastname: Anagnostouli
authors.forename: Nicos
authors.initials: N
authors.lastname: Karandreas
mesh_terms: D000328:Adult; D000588:Amines / Q000627:therapeutic use; D001418:Baclofen / Q000627:therapeutic use; D001767:Blinking / Q000502:physiology; D001921:Brain / Q000473:pathology; D002998:Clonazepam / Q000627:therapeutic use; D003509:Cyclohexanecarboxylic Acids / Q000627:therapeutic use; D004576:Electromyography; D005071:Evoked Potentials / Q000502:physiology; D018691:Excitatory Amino Acid Antagonists / Q000627:therapeutic use; D005152:Facial Muscles / Q000294:innervation / Q000503:physiopathology; D005260:Female; D018755:GABA Agonists / Q000627:therapeutic use; D018757:GABA Modulators / Q000627:therapeutic use; D000077206:Gabapentin; D019569:Hemifacial Spasm / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000150:complications*; D009474:Neurons / Q000502:physiology; D011149:Pons / Q000473:pathology / Q000502:physiology; D005680:gamma-Aminobutyric Acid / Q000627:therapeutic use
chemical_list: D000588:Amines; D003509:Cyclohexanecarboxylic Acids; D018691:Excitatory Amino Acid Antagonists; D018755:GABA Agonists; D018757:GABA Modulators; D005680:gamma-Aminobutyric Acid; D002998:Clonazepam; D000077206:Gabapentin; D001418:Baclofen