Different courses of Foix-Chavany-Marie syndrome (FCMS) in children with herpes encephalitis

Case Report: The Foix-Chavany-Marie syndrome (FCMS) or operculum syndrome is characterized by dysfunction of facial and pharyngeal muscles caused by bilateral cortical opercular lesions, resulting in a disorder of expressive speech, mastication, and swallowing, whereas involuntary movements are not affected.

In herpes simplex virus encephalitis (HSVE), uni- or bilateral temporal damage is characteristic (Baskin 2007), whereas FCMS has been reported only casuistically (van der Poel 1995, Garcia-Ribes 2007, Domjan 2000, McGrath 1997, Grattan Smith 1989, and Asenbauer 1998).

We report two cases with different severities and outcomes which are as follows:

Patient 1: First HSVE at the age of 2 months, recurrence at the age of 2 years, since then epilepsy including hemifacial myoclonus, disturbance in swallowing, mental retardation, and blindness. MRI showed bilateral occipital, temporal, and opercular/precentral defects.

Patient 2: Onset of disease at the age of 9 with headache, drowsiness, and impaired swallowing while speech was almost normal; after 7 weeks insertion of a PEG tube and after 24 weeks completely oral feeding. Intermittent incontinence and suspected hemifacial seizures. The MRI (day 6) showed cytotoxic edema bilaterally in the insula/operculum as well as in the right temporobasal, temporomesial, and right frontobasal areas.

Discussion: We report two further cases with FCMS and HSVE. In both children, the hemifacial myoclonus and the dysphagia can be explained by the lesions in the facial motor area, the incontinence in patient 2 can be explained by the representation of the distal urinary tract and rectum in the insular cortex.

Most cases with FCMS and HSVE are reported with rather poor recovery and persistent impairment of voluntary facial motor coordination. Only one boy is reported without persisting impairment of speech, mastication, and swallowing (Wolf et al). Accordingly, our patient 1 had severe persisting impairment of swallowing; patient 2 recovered well and showed only mild dysarthria and dysphagia.