578 SA MEDIESE TYDSKRIF DEEL 63 9 APRIL 1983

skull radiographs may reveal other causes for theophthalmoplegia, such as meningioma of the sphenoid wing. Araised ESR is rare and is more typical of temporal arteritis. eTmay demonstrate parasellar tumours and suprasellar extensionof pituitary gland tumours. Arteriography is recommended toexclude internal carotid aneurysm and parasellar tumours.Segmental narrowing and slight irregularities of theintracavernous portion of the carotid artery have been reportedin a few cases. 7 Orbital venography has been performed bySondheimer and Knapp, who demonstrated completeipsilateral occlusion of the superior ophthalmic vein and partialobliteration of the cavernous sinus. However, these venographicfindings are nonspecific and may be found in parasellarmeningiomas and pituitary rumours. Lakke6 reported theoccurrence of pleocytosis in THS, and other authors; havesuggested that it may reflect an aseptic inflammatory response inthe cavernous sinus. However, the cerebrospinal fluid appears to

-be normal in the majority of case of THS. Surgical explorationis not necessary if there is a good response to steroid therapy.

Differential diagnosisTemporal arteritis may have features similar to those ofTHS.

In the former the age of onset is usually later than in THS, andthere is a clear predominance of females over males, which is notthe case in THS. The ESR is rarely elevated in THS. Paralysis ofthe 3rd cranial nerve is the most common ocular finding in THS,whereas in temporal arteritis blindness is characteristic. Diabeticophthalmoplegia, intracavernous carotid aneurysms,

nasopharyngeal tumours, tumours of the orbit andophthalmoplegic migraine are the most common entities to beconsidered in the differential diagnosis. Orbital myositis mayeasily be mistaken for THS, but the presence of chemosis of thebulbar conjunctiva and exophthalmos favours the formerdiagnosis.

Treatment

High doses of steroids evoke a good response in 24-48 hours.Long-term steroid therapy does not appear to be beneficial, butsteroid therapy may be repeated if there are recurrences.

REFERENCES

l. Tolosa E. Perianeritic lesions of the carotid siphon with the clinical rea(\lres ofacarotid infraclinoidal aneur~"Sm.J Neural Neuromrg Psychiatry 1954; 17: 300­302.

2. HUn! WE, Meagher IN, LeFe\'er HE, Zeman W. Painfulophthalmoplegia. Itsrelation to indolent inflammation of the cavernous sinus. Neurology (Minneap)1961; 11: 56-62.

3. Smith JL, Taxdal DSR. Painful ophthalmoplegia: the Tolosa-Hunrsyndrome.Ophrhalmologica 1966; 61: 1466-1472

4. Mathew NT, Chandy J. Painful ophthalmoplegia. J /I;eurol Sei 1970; 11: 243­247.

5. Terrence CF, Samaha FY. The Tolosa-Hunt syndrome in children. Dev MedChild Neural 1973; 15: 506-509.

6. Lakke JP. Superior orbital fissure syndrome: report of a case caused by localpachymeningitis. Arch Neural 1962; 7: 289-300.

7. Scharz NJ, Farmer P. Tolosa-Hunt syndrome: the pathology of painfulophthalmoplegia. In: Smith JL, ed. Nellro-oprhalmology, vol. 6. St Louis: CVMosby, 1972: 102-112.

8. Sondheimer FK, Knapp J. Angiographic findings in the Tolosa-Huntsyndrome: painful ophthalmoplegia. Radiology 1973; 106: 105-112.

The operculum syndromeA case report

A. SANDYK

Summary

A 48-year-old hypertensive man with signs andsymptoms of pseudobulbar paralysis was Ultimatelyfound to be sLiffering from the operculumsyndrome. Recognition of this syndrome. isimportant in that: (a) it may be caused by a localizedcortical lesion amenable to treatment; (b) some ofthe symptoms carry a good prognosis as opposed tqthose in pseudobulbar paralysis; and (c) theoperculum syndrome usually has a-more favourableprognosis since there is no associated mentalimpairment or sphincter disturbances.

S Afr Med J 1983: 63: 578-579.

Department of Medicine, Hillbrow Hospital, JohannesburgR. SANDYK, .\01.0. (BON1'.~

Date received: 9 March 1982.

The operculum insulae is a small region of the telencephalicmantle which covers the insula (Reili). Its connections andfunctions have been only partially clarified, but the clinicalsymptomatology of opercular lesions has been well documented;however, studies of these lesions since 1837 have been almostexclusively carried out by the French; the syndrome appears tohave escaped Anglo-Saxon anention.

Case report

A 48-year-old hypertensive man presented with an 18-monthhistory of dysphagia, dysarthria and dysphonia. Hishypertension was longstanding and he had been on variousmedications in the past. He had been well until 2 years previouslywhen he had had an acute cerebrovascular accident which lefthim with mild right-sided hemiparesis and mild expressivedysphasia.

On examination there were no abnormal cardiovascular,respiratory or abdominal signs. The peripheral pulses werepresent and there were no carotid bruits. His blood pressure was175/ 105 mmHg, and the pulse rate 72/min and regular. He hadno neck stiffness and was well orientated in time and space.

Funduscopy revealed stage II hypertensive retinopathy but theoptic disc appeared normal. Frontal lobe reflexes wereexaggerated and the glabellar tap was positive. Pupillaryresponses were present and his eye movements were full, withsome limitation of upward gaze. The corneal reflex was presentbilaterally. There was mild bilateral ptosis. Voluntarymovements of the facial muscles were frozen and he was unableto show his teeth on command but was able to smile and laugh ata joke. He was unable to wrinkle his forehead on upward gaze orto close his eyelids tightly on command. However, the eyelidsclosed easily when tested for the presence of the corneal reflex orduring sleep. Hearing and understanding were intact. His jawmuscles were weak bilaterally and he was drooling from one sideof his mouth. There was no masseter atrophy and his jaw jerk wasbrisk. His tongue was small and immobile and there was noatrophy or fasciculations. Chewing and swallowing were weakand the gag reflex was absent. His speech was dysarthric anddysphonic and he had mild expressive dysphasia. Thesternomastoid muscles were weak bilaterally. Muscle tone wasincreased bilaterally, perhaps slightly more on the right and apositive Babinski sign was present on the right. Hoffmann'sreflex was negative bilaterally. His gait was slow with no ataxia.There was no mental impairment and no sphincter disturbance.Pathological crying and laughing were not observed. There wasno sensory loss and his cerebellar functions were intact.

The cerebrospinal fluid was normal. Computed tomography(CT) revealed prominent basal cisterns, marked tissue loss in theregion of the sylvian fissure and insulae bilaterally, several low­density areas in the left frontal and right occipital regions(multiple infarcts) and moderately dilated ventricles. The tissueloss involved predominantly the operculum insulae (Fig. I).

In summary, the patient had: (i) signs of pseudobulbar palsyinvolving the 5th, 7th and 9th through 12th cranial nerves,associated with marked automatovoluntary dissociation, absenceof mental impairment and sphincter disturbances and absence ofpathological crying and laughing; (ii) marked dysarthria,dysphonia and expressive dysphasia; (iii) facial weaknessincluding the upper portion of the face and forehead muscles;(iv) no signs of muscle denervation and no palatal and pharyngealreflexes; and (v) had previous cerebrovascular accidents andmarked tissue loss of both operculum regions as evidenced onCT.

Discussion

The opercular syndrome, being the cortical type ofpseudobulbar palsy, includes the clinical manifestations ofpseudobulbar palsy of the 5th, 7th and 9th through 12th cranialnerves. There are, however, specific features that distinguish itfrom pseudobulbar palsy. The operculum syndrome may followunilateral lesions, while a transient and predominantly distalpyramidal-type paresis of the arm on the same side as the cranialnerve involvement invariably accompanies it. Lesions of theparietal operculum may also cause hemi-anaesthesia. However,the dissociation between the discrete sensory symptoms and themarked motor deficit of the 5th cranial nerve suggests asupranuclear location of the lesion. A further remarkable featureis that the central-rype facial palsy includes the upper portion ofthe face. Mental symptoms or sphincter impairment neveroccur, in contrast with pseudobulbar palsy, and pathologicalcrying and laughing are rare. The patient's history often reveals acerebrovascular accident (in the great majority of cases eitherembolic or thrombotic) leading to well-definedencephalomalacia. The patients are usually hypertensive andover 50 years of age. The opercular syndrome ultimately resultsfrom an acute attack (although it may appear gradually) and thesigns and symptoms characteristically regress within a few days.

It has been suggested that ischaemia in the area of the smallersylvian branches of the middle cerebral artery is the immediatecause l but other causes have been reported, e.g. astrocytoma andmeningo-encephalitis. l

SA MEDICAL JOURNAL VOLUME 63 9 APRIL 1983 579

Fig. 1. eT reveals prominent basal cisterns, marked tissue loss inthe region of the sylvian fissure and insulae bilaterally andmoderately dilated ventricles. The tissue loss involvespredominantly the opercular regions bilaterally.

Recognition of the operculum syndrome not only offers anopportunity for the study of cortical functions but is also ofpractical importance since it may point to a localized corticallesion (such as a tumour) in the operculum area as opposed to thebilateral lesion of pseudobulbar palsy, and since some of thesymptoms, such as impaired deglurition (salivation, difficultywith eating), tend to regress rapidly in contrast to theirprolonged presence in patients with pseudobulbar palsy. Finally,the operculum syndrome usually has a better prognosis thanpseudobulbar palsy since there is no associated mentalimpairment or sphincter disturbance. The operculumsyndrome, mainly analysed by the French school of neurology,still requires detailed investigations which may well becorrelated with the extent of the lesion visualized on CT.

REFERENCE

I. Bruyn GW, Garhier Je. Theoperculumsmdrome. In: Vinken PJ, Bruyn GW,eds. Handbook a/Clinical Nellrology. vol. 2. Amsrerdam: Norrh-Holland, 1969:776-783.