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Article Excerpt
Foix-Chavany-Marie syndrome (FCMS), also known as the anterior operculum syndrome, is considered the cortical type of pseudobulbar palsy, which is characterized by severe dysarthria and bilateral paralysis of the facial, lingual, pharyngeal, and masticatory muscles with automatic-voluntary dissociation. Although FCMS appears to be a well-defined clinical entity, there are a few equivocal points. First, the distinction between FCMS and the noncortical type of acute pseudobulbar palsy is not clearly defined. Second, there is some confusion in the diagnostic classification of the developmental, reversible, and neurodegenerative forms of FCMS or similar syndromes. Third, some ambiguity exists regarding the terminologies used for certain symptoms of FCMS. In this article, two FCMS cases are presented, along with reviews of the literature on FCMS. The purpose of this study is to draw attention to this syndrome and to discuss its clinical characteristics, associated lesions, and prognosis.
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Foix-Chavany-Marie Syndrome (FCMS), also known as the anterior operculum syndrome, is characterized by severe dysarthria and bilateral central voluntary paralysis of the lower cranial nerves with preserved automatic, involuntary, and emotional innervation. The name of this syndrome originates from case reports by Foix, Chavany, and Marie (1926) and by Foix and Chavany (1926). Historically, this syndrome was mainly reported by French authors (Alajouanine & Thurel, 1933; Cambier, Viader, Paquelin, Poullot, & Pariser, 1983; Chateau et al., 1966; Schob, Gruner, & Mamo, 1956), and it was around 1980 that case reports of this syndrome began to appear in international literature (Biller, Asconape, Challa, Toole, & McLean, 1981; Ferrari, Boninsegna, & Beltramello, 1979; Mariani, Spinnler, Sterzi, & Vallar, 1980; Sandyk & Brennan, 1983).
Weller (1993) provided a comprehensive review of reports on FCMS and classified FCMS into five clinical types:
1. the classical and most common form associated with cerebrovascular diseases;
2. a subacute form caused by central nervous system infections;
3. a developmental form, probably most often related to neuronal migration disorders;
4. a reversible form in children with epilepsy; and
5. a rare type associated with neurodegenerative disorders.
It has been suggested that FCMS most often develops after staged cerebrovascular accidents.
Typical FCMS patients present with severe dysarthria and dysphagia. They are mute or can only produce inarticulate sounds; however, they can comprehend conversations and communicate through writing or gestures. The salient aspect of this syndrome is the automatic and voluntary dissociation of the bulbar muscles. These patients are unable to perform voluntary movement of the bulbar muscles on command or by imitation; however, the automatic movements of these muscles while smiling, crying, or yawning under natural circumstances are possible.
FCMS is considered to be the cortical type of pseudobulbar palsy (Alajouanine & Thurel, 1933; Bruyn & Gathier, 1969). Generally, a bilateral abnormality in the anterior operculum is the neuroanatomical cause of this syndrome. FCMS can be clinically distinguished from the noncortical type of pseudobulbar palsy by its acute onset, prominent dissociation between the voluntary and automatic movements of the bulbar muscles, decreased gag reflex, and absence of emotional lability. FCMS can also be distinguished from bulbar palsy syndrome by the absence of abnormal eye movements, lower motor neuron signs, and the presence of automatic voluntary dissociation (Mao et al., 1989).
While FCMS appears to be a well-established clinical entity, there are a few equivocal points regarding its differential diagnosis. First, the distinction between this syndrome and the noncortical type of pseudobulbar palsy is not clearly defined. Second, there is no agreement with regard to the diagnostic classification of the developmental, reversible, and neurodegenerative forms of FCMS or similar syndromes. Third, some ambiguity exists regarding the terminologies used for certain symptoms of FCMS. For example, some authors use the term apraxia to indicate the loss of voluntary movements in this syndrome, whereas others do not.
Although FCMS patients present with profound dysarthria and dysphagia, it appears that this syndrome has not been discussed completely in the field of speech and language pathology; therefore its prognosis and therapeutic outcome remain unclear. The purpose of this study is to draw attention to this syndrome and to discuss its clinical characteristics, associated lesions, and prognosis by presenting two cases and reviewing the literature.
CASE REPORTS
Case 1
A 79-year-old, right-handed female was admitted for the sudden onset of inability to speak. Seven years earlier she had suffered from a stroke that resulted in mild speech and language disorders. With the exception of dysgraphia, recovery from this stroke was almost complete. On neurological examination, tendon reflexes were normal and she was completely ambulant, with no signs of paresis in all extremities. She was completely mute and showed profound dysphagia necessitating nasogastric tube feeding. A CT scan was obtained on the first admission day, and it revealed a low density area involving the left insula. Another CT scan that was obtained on the third admission day revealed another low density area involving the right operculum (Figure 1). A right cerebral infarct was assumed to have caused this episode.
The patient was referred to the author (speech and language therapist) 3 weeks after the onset of the inability to speak. On examination, she was alert and cooperative but unable to communicate verbally. The aural comprehension of conversation appeared to be normal since she could respond adequately to the examiner's questions by nodding, through other gestures, and writing. She correctly followed commands such as "put the pencil on the side of the scissors." When she was asked about the problems she was facing, she wrote "I cannot speak" and "I cannot eat food" in Japanese with mild paragraphia. Her face appeared atonic, she could not open her mouth completely and showed complete inability to protrude her tongue or pucker her lips. Tongue atrophy or fibrillation was not observed. She was speechless and could only phonate rough and breathy sounds. Despite the severe impairments in voluntary control of the bulbar muscles, the phonation and movements of the facial muscles appeared normal when she laughed or cried under natural circumstances. Emotional lability was not observed.
[FIGURE 1 OMITTED]
Speech therapy was provided on a weekly basis with the aim of improving the voluntary control of the jaw, lips, and tongue to enable the production of speech sounds. The voluntary control of the jaw and lips improved gradually, whereas that of the tongue did not show any improvement. She was able to produce vowel sounds with distortion at the seventh week after the onset of the inability to speak and labial sounds with distortion at the tenth week postonset. The phonations were still rough and breathy. Dysphagia improved without specific treatment,...
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