J Neurol (1991) 238 : 337-339 Journal of Neurology © Springer-Verlag 1991 A case of unilateral opercular syndrome associated with a subcortical lesion Lucio Posteraro 1, Fabrizio Pezzoni 2, Emilio Varalda 2, Gloria Fugazza 2, and Anna Mazzucchi 1 1Sezione di Neuropsicologia, Clinica Neurologica, Universit~t di Parma, Strada del Quartiere, 4, 1-43100 Parma, Italy 2Fondazione Clinica del Lavoro, Centro Medico di Riabilitazione, Montescano (Pavia), Italy Received May 10, 1990 / Received in revised form November 28, 1990 / Accepted January 21, 1991 Summary. A patient w h o d e v e l o p e d a unilateral opercular s y n d r o m e following a cerebrovascular accident is described. C o m p u t e d t o m o g r a p h y s h o w e d that the lesion did not affect the opercular cortex, but involved d e e p white m a t t e r and the h e a d of the c a u d a t e nucleus of the left hemisphere. Persistent h y p o p h o n i a and transient aphasia were associated. C o m p a r i s o n with previous cases is discussed. Key words: 0 p e r c u l a r syndrome - Aphasia - H y p o p h o n i a - Computed tomography Introduction T h e opercular s y n d r o m e , also k n o w n in F r e n c h literature as labio-facio-glosso-pharyngo-laryngo-brachial paralysis, is generally r e g a r d e d as the cortical type of pseudobulbar paralysis [4]. T h e clinical picture appears in its m o s t characteristic f o r m w h e n bilateral lesions are present [1, 5, 8, 14, 15]. Occasionally a unilateral type of the s y n d r o m e has also b e e n described [2, 3, 10, 11, 12]. In the latter case, the classical picture includes an impairm e n t of function of the lower cranial nerves, usually beginning f r o m the fifth, which is often a c c o m p a n i e d by a transient and p r e d o m i n a n t l y distal p y r a m i d a l - t y p e paresis of the a r m on the same side as the a p p a r e n t cranial nerve involvement. A c c o r d i n g to B r u y n and G a t h i e r ' s description [4], there are two types o f unilateral opercular s y n d r o m e : (a) an anterior type, in which m o t o r deficits p r e d o m i n a t e in some cases associated with aphasia, or Pierre Marie's anarthria; (b) a posterior type, due to i n v o l v e m e n t of the parietal o p e r c u l u m , in which symptoms of impaired sensation and sensory deficit are found. The s y n d r o m e is generally attributed to the d a m a g e (most frequently vascular, but also neoplastic or traumatic) of the o p e r c u l a r cerebral cortex. O n the whole, however, d o c u m e n t a t i o n is still scarce and further investigations are required. In particular, to our k n o w l e d g e unilateral Offprint requests"to."A. Mazzucchi cases are very few and n o n e have b e e n studied with recent imaging techniques. In the present case, a deep unilateral subcortical lesion without cortical i n v o l v e m e n t was revealed by c o m p u t e d t o m o g r a p h y (CT), in a patient w h o also p r e s e n t e d with neuropsychological deficits and persistent h y p o p h o n i a . Case report The patient was a 67-year-old housewife, right handed, with 3 years of education. She was in good health until August 1988, when she suddenly developed right hemiparesis and aphasia, and was admitted to a Department of Medicine. A C T scan, obtained 2 days later, showed a haemorrhage affecting the internal capsule and basal ganglia of the left hemisphere. Three weeks after the stroke, the patient was admitted to the Rehabilitation Medical Centre of Montescano (Pavia). Neurological examination Upon admission to the Rehabilitation Medical Centre, the neurological examination showed whispering speech and poor verbal comprehension. There was right facial weakness, more evident in the area corresponding to the lower branch of the seventh nerve. The patient showed dysphagia, mild dysarthria and severe hypophonia, which made her speech incomprehensible. No paralysis of the vocal cords was found in laryngoscopy. The soft palate deviated to the left side. There was weakness of the ipsilateral trapezius and sternocleidomastoid muscles. The tongue deviated to the right side on protrusion. There was also a right hemiparesis with inability to stand and walk. Computed tomography The second CT scan, obtained 7 weeks after onset, showed an area of decreased density involving deep white matter of the left hemisphere, and reaching the head of caudate nucleus (Fig. 1). Neuropsychological examination One month after the onset of the stroke, the patient was given aphasia examination. Spontaneous speech showed few anomias and was poor and hypophonic. Description of an event was inadequate (55% correct). Repetition was insufficient (words 50%, non- 338 Fig.1. CT scans showing an area of decreased density in depth of left hemisphere (outlined in scans 4 and 5) Table 1. First neuropsychological assessment (30-40 days from onset) Test ~ Score WAIS (full-scale IQ) WAIS (performance IQ) WAIS (verbal IQ) 86 89 84 Verbal fluency with phonological cue (TPP) Verbal fluency with semantic cue (ALP) Equivalent score Percentiles 4.75 l 5-10% 2.25 1 5-10% Bucco-facial apraxia test (ABF) Utilization apraxia test (AU) ideo-motor apraxia test (AIM, right hand) Ideo-motor apraxia test (AIM, left hand) 64/72 Raven's PM 38 14/48 Threshold value 12/20 16/20 14/14 14/14 Not performed because of hemiparesis Follow-up The patient received rehabilitation treatment for her motor and speech disorders during the time she spent in hospital. Deficits in the cranial nerve areas and hemiparesis persisted for some weeks and subsequently tended to slow improvement. Follow-up of hypophonia and articulatory disorders showed parallel improvement. Two months after onset aphasia completely disappeared; all the patient's scores on aphasia examination were above 80% correct. Seven months after the stroke, neurological examination revealed mild facial weakness, occasional dysphagia for liquids, light hypophonia, deviation of the soft palate to the left side, diffuse right hyper-reflexia, and a mild motor deficit to the right arm. There was mild weakness of the right trapezius and sternocleidomastoid muscles. The tongue still deviated to the right side on protrusion, even though its movements in all directions were good. Neuropsychological examination revealed only mild signs of diffuse cognitive deterioration, without persistence of "focal" deficits (Table 2). Discussion 54/72 1 5-10% " For more information and normative data about these tests see Spinnler and Tognoni [I3] words 20%, sentences 50% ). Oral naming showed several anomias (45% correct). Comprehension of both spoken (100%) and written (80%) sentences was sufficient. Reading aloud (words 60%, non-words 60%, sentences 60%) and writing to dictation (words 40%, non-words 40%, sentences 10%) were impaired. Token Test [7] score was 26.5/36. Neuropsychological assessment also included a battery of standardized tests [13]: the results are shown in Table 1. It is worth noting that the patient also showed buccofacial apraxia, whereas there was no ideational or ideomotor apraxia to the left arm. I n the territory of the lower right cranial nerves the patient showed a persistent i n v o l v e m e n t , which a p p e a r e d to be of central origin. A p h a s i a a n d right hemiparesis, which recovered quickly, were associated. T h e clinical picture s h o w n by this p a t i e n t seems to m a t c h the clinical features of the w e l l - k n o w n a n t e r i o r type of o p e r c u l a r s y n d r o m e [4], the so-called labio-facio-glosso-pharingol a r y n g o - b r a c h i a l paralysis described by F r e n c h authors. Some c o m p a r a t i v e features with respect to previous cases m a y be noted. O p e r c u l a r s y n d r o m e is classically ascribed to direct i n v o l v e m e n t of the o p e r c u l a r cerebral cortex. I n s t e a d , in this case C T revealed a lesion deeply located in the left h e m i s p h e r e w i t h o u t a p p a r e n t involvem e n t of the overlying cortex. This finding suggests that such a clinical picture m a y also be i n d u c e d by lesions de- 339 Table 2. Second neuropsychological assessment (210 days from onset) Test a Score Threshold value Equivalent score Percentiles Token Test 30/36 29/36 2 10-25% TPP ALP 7.25 5.25 1 2 5-10% 10-25% ABF AU AIM (right hand) AIM (left hand) Constructional apraxia 14/20 14/14 70/72 66/72 12/14 Raven's PM 38 Acalculia test Word span Block tappirtg test Logic memory Attention test 20/48 12 3 5 0 28/60 16/20 14/14 54/72 54/72 F i n a l l y , it is i m p o r t a n t to n o t e t h e s e v e r e h y p o p h o n i a , which was a p e r s i s t e n t f e a t u r e in this case. P e r s i s t e n t h y p o p h o n i a is g e n e r a l l y r e g a r d e d as a d i s o r d e r t y p i c a l l y c o r r e l a t e d with b i l a t e r a l c e r e b r a l d a m a g e [5], w h i l e o n l y a m i l d a n d t r a n s i e n t h y p o p h o n i a g e n e r a l l y r e s u l t e d to b e a s s o c i a t e d with u n i l a t e r a l d a m a g e . This case s e e m s to suggest t h a t u n i l a t e r a l lesion in the d o m i n a n t h e m i s p h e r e can also cause a s e v e r e a n d p e r s i s t e n t h y p o p h o n i a . T h e specific m e c h a n i s m s r e m a i n to b e e x p l a i n e d . References 4 > 50% 2 10-25% 1 4 0 1 5-10% > 50% < 5% 5-10% 74 For more information and normative data about these tests see Spinnler and Tognoni [13] a s t r o y i n g fibres c o n n e c t i n g cortical a n d s u b c o r t i c a l structures, w i t h o u t cortical d a m a g e . It c o u l d b e a s k e d w h e t h e r a l e s i o n a l e x t e n s i o n to t h e c o r t e x m i g h t h a v e e s c a p e d t h e r e s o l u t i o n of CT. This p o s s i b i l i t y , h o w e v e r , a p p e a r s unlikely c o n s i d e r i n g t h a t the visible l e s i o n was d e e p a n d n o t close to the c o r t e x . A p a r t f r o m this, it w o u l d b e surprising for such a s e v e r e a n d p e r s i s t e n t s y n d r o m e to b e c a u s e d b y a c o r t i c a l lesion n o t d e t e c t a b l e o n CT. A further question regarding clinico-pathological correlations is w h e t h e r i n v o l v e m e n t o f t h e c a u d a t e n u c l e u s o r o f p a t h w a y s c o n n e c t i n g t h e b a s a l g a n g l i a a n d c e r e b r a l cortex was t h e m a i n cause o f t h e clinical m a n i f e s t a t i o n s . In this c o n n e c t i o n t h e i m p o r t a n t r o l e of t h e s e s t r u c t u r e s in p r o c e s s i n g m o t o r activities such as p h o n a t i o n a n d s p e e c h [6, 9] s h o u l d b e c o n s i d e r e d . T h e c o n c o m i t a n t p r e s e n c e in this case a n d in p r e v i o u s cases o f n e u r o p s y c h o l o g i c a l d i s o r d e r s raises the q u e s t i o n w h e t h e r t h e y a r e an a s s o c i a t e d c o m p o n e n t o f this synd r o m e . In the p r e s e n t case, l a n g u a g e d i s o r d e r s r e c o v e r e d q u i c k l y while c r a n i a l n e r v e deficits, d y s p h a g i a a n d h y p o p h o n i a p e r s i s t e d . M o r e o v e r , o n l y a v e r y few cases with n e u r o p s y c h o l o g i c a l a b n o r m a l i t i e s h a v e p r e v i o u s l y b e e n r e p o r t e d . It t h e r e f o r e s e e m s l e g i t i m a t e to c o n c l u d e t h a t n e u r o p s y c h o l o g i c a l a b n o r m a l i t i e s in o p e r c u l a r synd r o m e a r e d u e to c h a n c e i n v o l v e m e n t o f a d j a c e n t struct u r e s , a n d a r e n o t an intrinsic a n d n e c e s s a r y c o m p o n e n t . 1. Alajouanine TH, Thurel R (1933) La diplegie faciale cerebrale. Forme corticale de la paralysie pseudobulbaire. Rev Neurol (Paris) 40 : 441-458 2. Alajouanine TH, Boudine G, Pertuiset B, Pepin B (1959) Le syndrome unilateral de l'opercule rolandique avec atteinte controlaterale du territoire des V, VII, IX, XI et XIIe nerfs craniens. Rev Neurol (Paris) 101 : 168-171 3. Boudin G, Pepin W, Wiart JP (1960) Le syndrome operculaire unilateral d'origine vasculaire. Rev Neurol (Paris) 103 : 65 4. Bruyn GW, Gathier JC (1969) The operculum syndrome. In: Vinken P J, Bruyn GW (eds) Handbook of clinical neurology, vol 2. North-Holland, Amsterdam, pp 776-783 5. Cappa S, Guidotti M, Papagno C, Vignolo LA (1987) Speechlessness with occasional vocalizations after bilateral opercular lesions: a case study. Aphasiology 1:35-39 6. Damasio A, Damasio H, Rizzo M, Varney N, Gersh F (1982) Aphasia with nonhemorrhagic lesions in the basal ganglia and internal capsule. Arch Neurol 39 : 15-20 7. DeRenzi E, Faglioni P (1978) Normative data and screening power of a shortened version of the Token Test. Cortex 14: 41-49 8. Foix C, Chavany JA, Marie J (1926) Diplegie facio-linguomasticatrice d'origine cortico-souscortical sans paralysie des membres. Rev Neurol (Paris) 33:214:219 9. Jurghens U, Kirzinger A, Cramon D yon (1982) The effects of deep-reaching lesions in the cortical face area on phonation. A combined case report and experimental monkey study. Cortex 18 : 125-140 10. Pertuiset B, Perrier F (1960) Le syndrome operculaire unilateral (rolandique inferieur) d'origine vasculaire. Rev Neurol (Paris) 103 : 63-64 11. Rebucci GG, Gambetti P, Bottazzi G (1964) Le syndrome unilateral de l'opercule rolandique. A p r o p o s de trois observations cliniques. Acta Neurol Belg 64 : 1267-1278 12. Schott B, Boulliat G, Cotte L, Vauterin C (1961) Le syndrome operculaire bilateral et unilateral. Lyon Med 206 : 365-378 13. Spinnler H, Tognoni G (1987) Standardizzazione e taratura italiana di test neuropsicologici. Ital J Neurol Sci 6 [Suppl 8] : 5-120 14. Thurel R (1929) Les syndromes pseudobulbaires. Thesis, Paris 15. Villa G, Caltagirone C (1984) Speech suppression without aphasia after bilateral perisylvian softenings (bilateral rolandic operculum damage). Ital J Neurol Sci 5 : 77-83