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Neurodegenerative biopercular disease: a rare case of Foix–Chavany–Marie syndrome
Archives of Clinical Neuropsychology (2002)
  • Nancy B Isenberg, Providence St. Joseph Health
  • All Authors Horowitz TL, Giacino JT, Isenberg N
Foix–Chavany–Marie syndrome (FCMS) is a rare type of pseudobulbar palsy resulting from bilateral
cortical lesions in the anterior opercular regions. The primary and most debilitating feature is the loss
of voluntary control over the face, tongue, and swallowing muscles, resulting in dysarthria or anarthria,
and dysphagia. Automatic movements, such as eating, yawning, and smiling are preserved. Pathological
crying or laughing is also a common symptom. The most common cause is multiple strokes affecting
the corticobulbar tracts bilaterally. However, a neurodegenerative cause has also been described, with
focal atrophy beginning in the anterior opercular regions, and additional nonmotor cognitive deficits
becoming apparent a few years into the course of the disease. We present the case of a 78-year-old
female with symptoms consistent with neurodegenerative FCMS, but with rapid progression of both
motor and cognitive symptoms over the course of 1 year. Neuroimaging and neurologic findings are
presented and discussed. Extensive neuropsychological testing was conducted. Results were indicative
of adequate language comprehension, with the exception of difficulty processing syntactically complex
language. Also, the appearance of agrammatism and literal paraphasias in writing was reminiscent of a
Broca’s aphasia. Mild executive dysfunction was present. Memory functions were adequate. This case
will be compared and contrasted with the extant literature on this debilitating condition.
Publication Date
Citation Information
Nancy B Isenberg and All Authors Horowitz TL, Giacino JT, Isenberg N. "Neurodegenerative biopercular disease: a rare case of Foix–Chavany–Marie syndrome" Archives of Clinical Neuropsychology Vol. 12 Iss. 8 (2002) p. 724
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