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[Foix-Chavany-Marie syndrome in a child caused by a head trauma]
A Laurent-Vannier1, G Fadda, P Laigle
1Service de Rééducation des Pathologies Neurologiques Acquises de l'Enfant, Hôpital National de Saint-Maurice.
Insights
Traumatic Foix-Chavany-Marie syndrome (SFMC) in a child resulted in anarthria and orofacial motor deficits due to brain injury. Recovery of motor abilities was limited despite intensive rehabilitation.
Area of Science:
- Neurology
- Neuroscience
- Traumatic Brain Injury
Background:
- Foix-Chavany-Marie syndrome (SFMC) is a rare neurological condition characterized by suprabulbar palsy.
- It involves bilateral opercular lesions, affecting voluntary control of orofacial muscles.
- SFMC can present in various forms, including classical, subacute, developmental, reversible, and neurodegenerative types.
Observation:
- A 10-year-old boy sustained a traumatic brain injury from a traffic accident, leading to coma and subsequent development of SFMC.
- Clinical examination revealed anarthria (inability to speak), masticatory diplegia, drooling, and automatic-voluntary dissociation of facial movements.
- Neurological deficits included impaired voluntary control of cranial nerves V, VI, IX, X, and XI, with preserved reflex swallowing.
Findings:
- Neuroimaging confirmed frontal fracture and ischemic-hemorrhagic lesions, with subdural and extradural hematomas surgically evacuated.
- The patient exhibited hallmark clinical features of SFMC, including deficits in voluntary orofacial motor control.
- Despite intensive rehabilitation, the boy recovered only limited orofacial motor abilities, indicating a poor prognosis for this acute acquired syndrome.
Implications:
- This case highlights the severe and persistent deficits associated with traumatic SFMC in children.
- Understanding the distinction between anarthria and aphasia/apraxia is crucial for effective speech and swallowing rehabilitation.
- The case underscores the importance of early diagnosis and tailored rehabilitation strategies for pediatric traumatic brain injury patients with SFMC.
Abstract:
We report the case of a traumatic Foix-Chavany-Marie syndrome (SFMC) which is the cortico-subcortical type of suprabulbar palsy. A 10-year-old boy was brain injured in a traffic accident in August 1996. He was found comatous (initial GCS = 6) without any focal neurological deficit. The hemodynamic situation was stable even though he presented two wounds of the scalp and a hemoperitoneum that required intensive perfusions. The initial CT scan elicited a frontal fracture, ischemo hemorrhagic lesions of the right frontopolar and anterior temporal cortex. On the second day, he developed on the left side a subdural collection and a extradural hematoma which was surgically withdrawn. The comatous state ended on the ninth day. On examination, The child was awake and alert, able to understand spoken and written language but unable to speak. There was masticatory diplegia: the mouth was half open, the patient was drooling, chewing was impossible. The most striking feature was the automatic voluntary dissociation which might be observed on laughing, crying and yawning. The patient was unable to initiate swallowing but reflex swallowing was preserved once food was placed into the pharynx. The child had a deficit of voluntary control of muscles supplied by nerves V, VI, IX, X, XI. These clinical features are the hallmarks of SFMC. The first case was reported in 1837 by Magnus. The syndrome was described by Foix Chavany et Marie in 1926, and called SFMC by Weller (1993). His literature review of 62 SFMC allowed the differentiation of five clinical types: the classical and most common form associated with cerebrovascular disease, a subacute form caused by central nervous system infections, a developmental form, a reversible form in children with epilepsy and a rare type associated with neurodegenerative disorders. Bilateral opercular lesions was confirmed in 31 of 41 patients who had CT or MRI performed, and by necropsy in 7 of 10 patients. As previously reported, the outcome was poor for this boy who recovered very limited orofacial motor abilities. The medical functional readaptation was long et tedious and took in consideration the fact that the speech disturbance was anarthria and not an aphasic or an apraxic one and the age of onset of this acute acquired syndrome.