Dados do Trabalho

Título

Foix-Chavany-Marie Syndrome: A Case Report

RESUMO

Clinical case: J.J.S, male, 61 years old. This patient was referred with an insidious condition of vocal alteration, difficulty in swallowing and sporadic choking since childhood. From the age of 40, he noticed an intensification of symptoms, presenting lower and slurred speech associated with significant dysphagia for solids and liquids. Neurological examination showed involvement of multiple cranial nerves with hypoesthesia and diparesis facial, reduction of the nausea reflex, dysphagia, dysarthrophonia and paresis of the tongue musculature. Despite the paralysis of the orofacial voluntary muscles, they are preserved when evoked by emotion, such as the act of smiling. He also had mild cognitive impairment, as evidenced by cognitive tests. On MRI, bilateral atrophy was observed in the opercular regions. Discussion: Foix-Chavany-Marie syndrome, also known as opercular syndrome, occurs due to bilateral cortical injury in the anterior opercular region. It is a rare suprabulbar palsy condition with dissociation in voluntary and spontaneous (emotional) movements. The clinical presentation reflects bilateral paralysis of voluntary movements of muscles innervated by NNCC V, VII, IX, X and XII, as in this case. However, the initial involvement of impairment in chewing, tongue movement and speech apraxia, but without language impairment, stands out. The main cause is vascular, but cases caused by neuroinfections, traumatic brain injury, neuroinflammatory, demyelinating and neurodegenerative diseases have already been described. On MRI, asymmetric cortical atrophy is seen, predominantly in the lower frontal region, especially in the opercular region, as in this case. Final Comments: This case shows that progressive loss of orofacial voluntary motor skills and apraxia of speech with preservation of emotionally evoked movements are the hallmarks of this rare syndrome. The exact diagnosis of the cause is not always possible and the treatment involves a multidisciplinary team. Due to the significant involvement of the facial muscles, malnutrition is the main consequence and alternative feeding methods are necessary.

Palavras Chave

Opercular; Foix-Chavany-Marie; Syndrome; facial