Degenerative anterior biopercular syndrome initially misdiagnosed as a psychogenic disorder

Abstract

The anterior biopercular syndrome or Foix-Chavany-Marie syndrome (FCMS) originally described in 1926 is defined by diminished or absent voluntary control of facial, pharyngeal, lingual, and masticatory muscles while reflectory movements are preserved [2, 8]. In the original description, the syndrome occurred after bilateral damage of the anterior opercular area surrounding the insula caused by stroke. Other causes of the syndrome are infections of the nervous system, cortical dysplasias, tumors, trauma, and, but exceedingly rare, degenerative disease [4, 7, 8]. A 70-year-old Caucasian female presented with a progressive dysarthrophonia, swallowing problems, and hypomimia. According to her daughter, the history of speech problems started 2 years earlier and were characterized by slurred speech and hoarseness, which slowly progressed into mutism. Communication was possible by writing. Writing was without any grammatical or orthographic errors and her reading as well as her auditory comprehension were intact. The first neurological examination in an outpatient clinic 2 years earlier had revealed an alert woman without behavioral disturbances and as the patient was incidentally able to smile and laugh while she was unable to do so when she was asked to smile voluntarily, a probable psychogenic speech disorder was diagnosed and therapy with antidepressants was started. On admission to our hospital 1 year later, the patient’s face showed a dystonic-like deformation, which made us think of an ancient Greek theatre mask and she was unable to imitate or produce on request any oral–verbal expression but a growling. The patient was unable to open her mouth, to blow her cheeks, or to frown. Dysphagia and sialorrhea were present. In stark contrast, the patient was observed to laugh while listening to a joke (she started laughing when the examiner started to blow his cheeks during the clinical examination procedure) and she was able to frown when testing vertical eye-movements. The masseter muscle reflex was brisk and primitive reflexes like snout-, graspingand palmo-mental-reflex were present as was Myerson’s sign positive. Due to her dysphagia, the patient had suffered a remarkable weight loss of more than 15 kg. Neuropsychological assessment ruled out both aphasia and dementia, nor were there signs of muscle weakness, Parkinsonism, fasciculation, ataxia, or apraxia. Electrophysiological investigations including nerve conduction studies, blink reflex, motor and sensory evoked potentials were all normal. Electromyography of the M. interosseus dorsalis I, M. vastus medialis, M. tibialis anterior, and the tongue muscles did not reveal any M. Dafotakis (&) Department of Neurology, RWTH Aachen, Aachen, Germany e-mail: mdafotakis@ukaachen.de