Opercular syndrome: A case report and review
A 9-year-old male presented with unclear and slurred speech, drooling of saliva and inability to protrude tongue and difficulty swallowing since early childhood. He was born with emergency caesarean section (due to fetal distress) and birth weight was 2.5 kg. He developed seizures at 6 hours of life due to perinatal asphyxia, for which he was admitted in neonatal intensive care unit for a week. He had motor and cognitive delay with involvement of speech domain greater than social and motor domain. At the age of 6 years; he developed seizures which were brief, tonic-clonic, involving right side of body with loss of consciousness. Seizures were poorly controlled on multiple anti-epileptic drugs regimen consisting of sodium valproate, lamotrigine, and clobazam. He had no history of trauma, surgery. Family history was not significant.\nHe had dysarthria with nasal twang in voice, mouth half open and not able to close volitionally. He was unable to bring out the tongue beyond lower incisor. Hearing and understanding were normal. There was no tongue tie or any structural defect of tongue. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on volitional control but automatic and emotional motor movements were well preserved. He was able to blink, close his eyes completely while sleeping, and move the jaw and facial muscles with spontaneous emotional responses like laughing or crying. There was no wasting or fasciculations of the tongue. There was hypertonia in all four limbs and deep tendon reflexes were exaggerated with normal jaw jerk along with extensor bilateral plantars. Electroencephalography (EEG) findings consisted of focal left fronto-temporal epileptiform discharges and magnetic resonance imaging (MRI) brain revealed bilateral fronto-temporal and parietal cortical and subcortical region encephalomalacia, with involvement of opercular region []. Induced sleep EEG did not reveal any continuous spike and wave discharges as seen in Continuous Spike and Wave discharges in Sleep (CSWS) or Landau-Kleffner syndrome (LKS).\nBased on above clinical findings, a diagnosis of acquired bilateral opercular syndrome due to perinatal asphyxia was considered.