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January 3, 2017 E-Pearl of the Week: Foix-Chavany-Marie syndrome

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January 3, 2017

Foix-Chavany-Marie syndrome  

Foix-Chavany-Marie syndrome (FCMS) or anterior opercular syndrome is a rare neurological syndrome that is caused by developmental or acquired bilateral cortical lesions of the anterior opercula. The typical clinical manifestations of this syndrome include severe dysarthria, aphasia (expressive, receptive or both), generalized facial weakness, orofacial apraxia, and difficulty chewing and swallowing, without mental impairment. The common etiologies include stroke in the regions of bilateral opercula, infections of the central nervous system such as HSV and CMV, neoplasms, trauma and epileptic disorders. MRI of the brain often shows bilateral opercular lesions, although unilateral lesion has been reported. Management usually involves treating the underlying cause in addition to a multidisciplinary approach to speech and feeding difficulties.

References

  1. Moragas Garrido M, Cardona Portela P, Martínez-Yélamos S, Rubio Borrego F. Heterogeneous topography of Foix-Chavany-Marie syndrome. Neurologia 2007; 22: 333-336.
  2. Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe V. Foix-Chavany-Marie syndrome in a child caused by a head trauma. Rev Neurol (Paris) 1999; 155: 387-390.
  3. van der Poel JC, Haenggeli CA, Overweg-Plandsoen WC. Operculum syndrome: unusual feature of herpes simplex encephalitis. Pediatr Neurol 1995; 12: 246-249.

Derek Y. Yuan, MD, MS, Resident Physician - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY. Dr. Yuan reports no disclosures.

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