Journal of the Royal Society of Medicine Volume 85 September 1992

klkk

-fi.

i.. ....:e...|

i 7" j

*ton pSiit favousqa nerv sheath'origin-

^strn postvt faor

a nerve

shath origi

preparation and for S-100 protein (Figure 2). Some of the small nerve bundles within the tumour were involved. A second lesion excised one month later had similar histopathological features.

Discussion GCT is a controversial lesion with many synonyms and an unresolved histogenesis4. It was originally thought to arise from muscle and known as 'granular cell myoblastoma'2. The term 'granular cell tumour' is desirable as the neoplastic nature of the lesion is uncertainiSAi. Most leading authors on this topic accept it as being of nerve sheath origin and prefer the nomenclature 'granular cell schwannoma' which incorporates the neuroectodermal derivation24. The ultrastructure and histochemical evidence, in particular staining for the S-100 protein support an origin from Schwann cells4"6. This neuroectodermal connection suggests that GCT may be similar to lesions seen in neurofibromatosis2'. There have been no accounts of GCT occurring in patients with von Recklinghausen's disease2. GCT is more common in the black population and multiple lesions are particularly frequent in this group7. Multiple lesions otherwise are uncommon' and familial cases are extremely rare8. The lesions occur in diverse anatomical sites24'7. GCT follows a benign clinical course although a malignant variant is known to occur rarely24. The symptoms will depend on the site and size of the lesion3 but are usually asymptomatic in the skin tissue. There may be tenderness suggesting neural involvement9.

Persistent diplopia following Streptococcus suis type 2 meningiti

J S MeechamMB ChB R C WorthMRcP Countess of Chester Hospital, Liverpool Road, Chester CHI 3ST Keywords: streptococcus suis type 2 meningitis; diplopia

Streptococcus suis type 2 meningitis is a zoonosis which is uncommon in the UK. It occurs in pig handlers and handlers of pig carcasses2. Previously documented sequelae include permanent deafness and vertigo;-this case was complicated by persistent diplopia.

579

The surgical differential diagnoses are numerous and an excisional biopsy is the only confirmatory diagnostic modality. GCT is usually diagnosed first at microscopy2. The histological confirmation in addition may demonstrate the clinically malignant variants. This is of paramount importance in the surgical management of these patients. The histological diagnosis of malignancy in GCT is, however, subjective', and may in fact not always be possible as similar morphology may be seen in the benign tumour"35. The diagnosis of malignancy is often based on the clinical behaviour of the lesion especially if there is metastatic spread, usually after several years2'3. GCT do not appear to regress spontaneously. Radiotherapy has no beneficial effect. When GCT is solitary, complete surgical excision with a rim ofperipheral tissue seems most appropriate and is effectively curative24. The recurrence rate (8%) is similar in lesions that are incompletely excised and suggests that a subsequent wider clearance is not necessary'. A clinical follow-up will enable symptomatic or rapidly growing lesions to be referred for local excision8'9. References 1 Lack EE, Worsham GF, Callihan MD, et aL Granular cell tumor: a clinicopathological study of 110 patients. J Surg Oncol 1980;13:301-16 2 Enzinger FM, Weis SW. Benign tumors and tumorlike lesions of uncertain histogenesis. In: Soft tissue tumor. St Louis: The CV Mosby company, 1983 3 Hajdu SI. Miscellaneous soft tissue tumors. In: Pathology ofsoft tissue tumors. Philadelphia: Lea & Febiger, 1979:510-35 4 Ashley DJ. Supporting tissues ofthe body - voluntary muscle. In: Evans' histological appearances oftumours, 3rd edn. Edinburgh: Churchill Livingstone, 1978:50-3 5 Buley ID, Gatter KC, Kelly PM,- et aL Granulosa cell tumours revisited. An immunohistological and ultrastructural study. Histopathology 1988;12:263-74 6 Armin A, Conelly EM, Rowden G. An immunoperoxidase investigation of S-100 protein in granular cell myoblastomas. Evidence of schwann cell derivation. Am J Clin Pathol 1983;

79:37-44 7 Vance 5, Hudson RP. Granular cell myoblastoma. Clinicopathological study of 42 patients. Am J Clin Pathol 1969; 52:208-11 8 Riflkin RH, Blocker SH, Palmer JO, Ternberg JL. Multiple granular cell tumors. A familial occurrence in children. Arch Surg 1986;121:945-7 9 White SW, Gallagher RL, Rodman OG. Multiple granular-cell tumors. J Dermatol Surg Oncol 1980;6:57-61

(Accepted 5 July 1991)

Case report A 46-year-old pig farmer was admitted having been unwell for 3 days. He described a sudden onset of clumsiness and vertigo, followed after 2 days by a severe frontal headache, high fever, confusion and vomiting. He owned approximately 200 pigs, all of which were healthy, and was not involved with their slaughter. There had been no recent travel abroad. Examination showed him to be disorientated, agitated, but afebrile; there were no rashes. nitially, there was no evidence of meningeal irritation, or focal neurological abnormality.- Within 2 h of admission, there had been a marked deterioration; he was pyrexal (39°C), and had obvious nuchaI rigidity with photophobia. After immediate treatment with 2.4 g intravenous benzyl penicillin and 1.0 g intravenous chloramphenicol,' a CT scan was performed to exclude a cerebral abscess. A lumbair puncture showed turbid cerebrinal fliid (CSF) underrad pre e, with a WBC of 10x10011 (95% polymorphs) and abundant gram-positive cocci. CSF protein was 4.5 gAl and glucose 0.6 mmol/l (plasma

0141-0768/92 090579-02/$02.00/0 X 1992 The Royal Society of Medicine

580

Journal of the Royal Society of Medicine Volume 85 September 1992

glucose 7.8 mmol/l). Other abnormalities found on investigation were; a raised white cell count (14.6x109/1) with marked neutrophilia (13.0x 10/l), thrombocytopaenia (23x 109/l), abnormal clotting (PT 21.0 s and APPT 65.7 s) and raised liver enzymes (ALT 217 u/l and GGT 120 u/l). Treatment was continued with 2.4 g intravenous benzyl penicillin 4 hourly and 1.0 g intravenous chloramphenicol 6 hourly. Within 24 h he was clinically much improved. At this stage, culture of the CSF and blood revealed a beta-haemolytic streptococcus which was not identifiable as a member of Lancefield Group A, B, C, D, or G. Antibiotics were changed to intravenous amoxycillin 500 mg 6 hourly with probenacid 250 mg 12 hourly, in order to cover infection with enterococci. Final identification of the organism was streptococcus suis type 2, which was sensitive to amoxycillin. After 48 h, he was afebrile, with no headache, but was now complaining of double vision. The diplopia was in all directions of eye movements with no obvious ocular nerve palsy. Intravenous antibiotics were stopped after one week but continued orally for a further 2 weeks. The abnormal clotting parameters and liver enzymes normalized within 4 days and 2 weeks respectively. He was discharged from hospital, well, after 2 weeks; he remains disabled by persistent diplopia, and has been provided with a pair ofprism glasses after ophthalmological assessment.

Discussion Streptococcus suis type 2 was first identified as a cause of septicaemic infections in pigs in 19591. In humans, infections have been described, mostly as meningitis. As in the present case, nearly all infections with streptococcus suis type 2 are associated with occupational contact with pigs or pig carcasses preceding the infection2 3. This is probably explained by the occurrence of streptococcus suis type 2 both as a commensal and an opportunistic pathogen in pigs2. Breton, Mitchell and Rosendal described a carrier rate of 9.7% in a herd of apparently normal pigS4.

Meningitis is the commonest form ofpresentation although infective endocarditis has also been described5. It has been suggested that bacteria gain access to the cerebrospinal fluid compartment in association with monocytes migrating along normal pathways (particularly at the choroid plexus)6. The meningitis, itself, can be much more severe than in this case, resulting in multiple organ failure and death7. Damage to the eighth cranial nerve is the commonest sequela of the meningitis, resulting in deafness and vertigo which may be permanent and disabling2. Although there has been one reported case of blind-deafness5, diplopia as a complication has not been previously described. We presume, as no ocular nerve palsy was demonstrable, that this was a brain-stem ophthalmoplegia. Four months following his illness, diplopia remains and is both disabling and preventing him from returning to work. References 1 de Moor CE. Ein niewe streptococcus haemolyticus (Lancefield groep R). Verslagen en Mededelingen Betreffende de Volkogezondheid 1959;2:474-7 2 Lutticken R, Temme N, Hahn G, Bartelheimer EW. Meningitis caused by streptococcus suis: case report and review of the literature. Infection 1986;14:181-5 3 Kaufhold A, Lutticken R, Litterscheid S. Systemic infection caused by streptococcus suis. Dtsch Med Woschenschr 1988;113:1642-3 4 Breton J, Mitchell WR, Rosendal S. Streptococcus suis in slaughter pigs and abattoir workers. Can J Vet Res 1986; 50:338-41 5 Ho AK, Woo KS, French GL. Infective endocarditis caused by streptococcus suis serotype 2. JInfect 1990;21:209-11 6 Williams AE, Blakemore WF. Pathogenesis of meningitis caused by streptococcus suis type 2. J Infect Dis 1990;162:474-81 7 Van-Jaarsveld BC, van-Kregten E, van-Kesteren RG, RozenbergArska M, Bartelink AK. Fulminant sepsis caused by streptococcus suis. Ned TUdschr Geneeskd 1990;134:1462-4 8 Cammaert T, Verstraete W, Baeck E. Deafness-blindness caused by streptococcus suis meningitis - epidemiology and rehabilitation. Acta Otorhinolaryngol Belg 1990;44:37-41

(Accepted 24 July 1991)

Meeting reports Clinical presentations by undergraduates Keywords: case presentations; undergraduates

This Clinical Section meeting was unusual by being the first to be given over entirely to presentations by undergraduates. Four patients were seen and five were discussed later. Mr Russell Davies presented a 49-year-old man who presented in 1982 with a squamous cell carcinoma of the right retromolar trigone. This was excised combined with a functional neck dissection in May 1982 at Canniesburn Hospital, Glasgow. The defect was repaired with a free forearm flap based on the left radial artery. This was the first occasion on which this technique had been perforned in the United Kingdom. Despite nodal metastatic disease at presentation the patient remained free from recurrence with an excellent functional result 10 years after his surgery.

Mr Gerard Short presented a postmortem case from St Mary's Hospital, Paddington, of necrotising enterocolitis in a patient with AIDS. This was the first description of this condition complicating AIDS. This case demonstrated the various manifestations of AIDS and the way in which the clinical features have changed in recent years. This was followed by a discussion of the pathogenesis of necrotising enterocolitis and of the risks to health care workers inherent in the care of patients with AIDS.

Miss Katherine Jones and Miss Elizabeth Japes delivered a joint presentation from the Homerton Hospital, Hackney, of a young African woman successfully treated for a Franz' tumour of the body of the pancreas. A thorough exposition of previous reports followed and the pathogenesis ofthe condition was discussed. Franz' tumour is a papillary cystic neoplasm of the pancreas and is rare. It is usually of low grade malignancy and seems to be almost confined to young women particularly those of African origin. Possible explanations for these features were discussed.

Report of meeting of Clinical Section, 13 March 1992

0141-0768/92 090580-02/$02.00/0 © 1992 The Royal Society of Medicine