594 Proc. roy. Soc. Med. Volume 68 September 1975 no ameebe or cysts. ESR 34 mm, WBC 12 500/ mm3, phosphatase normal. Barium enema normal. This illness was considered to be due to incomplete eradication of the amoeba possibly with superimposed intestinal amoebiasis. He was therefore restarted on metronidazole 800 mg three times daily for 10 days, with clinical improvement. Discussion The long latent period which may exist between the intestinal infection and hepatic involvement (up to fifty years in a case described by Paulley 1961) has not been explained. Few cases of hepatic amcebiasis have concurrent amoebic dysentery and only some 25% will give a past history of it (SepuJlveda et al. 1959). Examination of the fmces yields cysts and trophozoites in 17-50% of cases but much depends on the skill and patience of the examiner. Radioactive liver scanning, when available, has largely replaced liver aspiration for confirmation of the diagnosis, and in following treatment. Serology, including immunofluorescence, hlemagglutination and complement fixation, should be used whenever the diagnosis is suspected. Positive results are obtained in 60-80% of cases of symptomatic intestinal amoebiasis and 95-98 % of cases of hepatic amoebiasis (Stamm 1970); serology is thus useful in excluding the diagnosis of amoebic liver abscess. However, antibodies persist for a long time after cure and a positive result may be due to past infection. Aspiration of an amoebic abscess is unjustifiable for purely diagnostic reasons. It should be reserved for cases with a palpable mass, persistent localized tenderness, a markedly raised right hemidiaphragm or failure to respond to drug therapy (Powell 1971). Delay in diagnosis increases the morbidity and mortality (Wright 1966) and hepatic ameebiasis should be considered as a differential diagnosis in anyone who has ever been in the tropics even witbout a past history of dysentery. REFERENCES

Paulley J W (1961) British Medical Journal i, 462 Powell S J (1971) In: Management and Treatment of Tropical Diseases. Ed. N G Maegraith and H M J Gilles. Blackwell, Oxford; p 3 Stamm W P (1970)Lancet ii, 1355 Sepulveda B, Jurich H, Bassols F & Muhioz R (1959) American Journal of Digestive Diseases 4, 43 Wright R (1966) British Medical Journa li, 957

Dr V W Johnson (New Cross Hospital, Wolverhampton) asked if calcification of the liver was a common finding in amoebic liver disease. Dr Jones replied that it was an extremely uncommon

finding.

22 Relapsing Meningoencephalitis ? Cerebral Sarcoidosis L J Findley MB MRCP (for F Clifford Rose MB FRCP) (Department of Neurology, Charing Cross Hospital, London W6 8RF) D B, man aged 36, right-handed. Airduct erector History: Presented in March 1973 complaining of left-sided headaches for three weeks, photophobia and subjective right-sided weakness. On examination there were no objective physical signs. Symptoms followed mild head trauma to the left parietal region. He was investigated for possible subdural hematoma and all investigations, including skull and chest X-rays, skull echo-encephalogram, EEG and left carotid arteriogram, were normal. ESR 15 mm in 1 hour (Westergren). CSF not examined. Discharged well after two weeks. Relapse I (April 1973). Following rapid deterioration he complained of drowsiness, headache and paresthesia over left side of face. On examination: meningism associated with right-sided hyperreflexia. ESR 70 mm in 1 hour; h[emoglobin, differential white count, serum proteins and immunoglobulins normal. CSF at normal pressure: total protein 60, glucose 60 mg/100 ml; lymphocytes 70/mm3; bacterial cultures sterile. Lange colloidal gold curve and CSF Wassermann reaction negative. Continued to deteriorate after admission and was treated as tuberculous meningitis with streptomycin 1 g daily, isoniazid 150 mg twice daily and para-aminosalicylic acid 6 g twice daily. Steroids were given as dexamethasone 2 mg three times daily. After starting treatment he improved steadily and was symptomatically and clinically quite well within four weeks. June 1973: CSF normal; steroids discontinued. July 1973: Cultures for mycobacteria negative;

streptomycin discontinued. Relapse 2 (August 1973). He complained of severe cervical pain, parmsthesix in hands and feet and incontinence of urine. On examination there was weakness and wasting of intrinsic muscles of both hands. Investigations confirmed CSF changes similar to those of first illness (total protein 70, glucose 60 mg/ 100 ml; lymphocytes 70/mm3, light microscopy and bacterial cultures negative). ESR 13 mm in 1 hour; the following investigations were also normal: hemoglobin, full count and differential leukocyte count, serum total and

differential proteins, serum immunoglobulins, serology for syphilis, toxoplasma dye test and complement-fixation test, brucella agglutination reaction, CSF culture for cryptococcus, viral cultures and antibody studies, leptospira complement-fixation test, urinary and serum calcium

23

Fig 1 Granuloma from Kveim test biopsy. x 275

Clinical Section

595

as a greatly delayed absorption of the isotope. This, and the AEG findings, were thought to be consistent with a communicating hydrocephalus. Relapse 5 (March 1974): The patient complained of pain on movement of his right eye and of loss of vision over a period of three days. Papilleedema on right with a small hemorrhage at inferior margin of optic disc. ACTH 40 units twice daily produced complete remission within seven days but subjective improvement occurred within 24 hours. In April 1974 a similar episode occurred affecting the left eye. This again responded promptly to ACTH 40 units twice daily. July 1974: The patient has remained well. The only physical signs are mild nystagmus on right lateral gaze and minimal pallor of left optic disc. Visual acuity and visual fields normal. A check Kveim test was carried out using highly selective antigen K12. Biopsy revealed a solitary follicle of epithelioid cells without giant cells and which was clear on light polarization. After the intradermal injection of antigen the patient had received a two-week course of ACTH but was in complete remission at the time of the biopsy. In view of this, the Kveim test was considered to be

studies, chest X-ray and myelogram. Mantoux skin test in dilution, 1 :1000 and 1:100 negative. Previous immune status not known. EEG in this relapse, as in all others, showed diffuse slow wave (theta) activity. Steroids reintroduced as prednisolone 10 mg four times daily. Progressive improvement; within one month he was in complete symptomatic and clinical remission. EEG had reverted to normal. Steroids withdrawn. Kveim test (antigen K19) unequivocally positive equivocally positive. (Fig 1). Biopsy showed noncaseating granulomata Discussion with epithelioid cells and lymphocytes; giant This relapsing illness affecting the central nervous cells visible in many sections; no refractile system would be consistent with disseminated material on light polarization. Scalene node sclerosis or a remitting encephalomyelitis. There biopsy, muscle biopsy (right deltoid) and skin are many unusual features for a diagnosis of biopsy (right upper arm), however, were normal. disseminated sclerosis: (1) Absence of progressive No evidence of systemic sarcoidosis; skeletal neurological deficits following several severe relapses. (2) Extreme responsiveness to steroids radiological survey showed no abnormality. Relapse 3 (December 1973): Occipital headaches, and ACTH. (3) AEG and cysternogram findings photophobia and weakness of left leg. On that would be consistent with a communicating examination he showed facial asymmetry, hori- hydrocephalus. (4) Anergy to tuberculin and zontal nystagmus on lateral gaze, and marked positive Kveim reaction. If the diagnosis is sarcoidosis, entirely confined left-sided cerebellar signs with ataxic gait. CSF normal. Response to adrenocorticotrophin gel to the CNS, then its rarity should be emphasized. 40 units twice daily for a week was excellent and In a review of 2500 cases of sarcoidosis, Zeman he was symptomatically and clinically normal (1958) could find only 18 cases entirely confined to the CNS. Urich (1969) stated that 'despite within three weeks. Relapse 4 (January 1974): Complained of severe claims to the contrary, there is no conclusive occipital headaches, diplopia and weakness of evidence of sarcoidosis confined to the CNS'. both hands. On examination he had horizontal In world literature only 25 cases have been nystagmus of each abducting eye with impaired reported. The ocular manifestations are not uncommon adduction and bilateral severe cerebellar signs. CSF examination showed normal pressure, (James 1959); the involvement of any structure of protein, glucose, and 14 lymphocytes/mm3. Air the eye may occur. Isolated involvement of thie encephalogram demonstrated moderate sym- optic nerve is rare. Salvesen (1935), Koumrouyan metrical ventricular dilatation with a paucity of (1950) and Longcope (1941) have described air over the cerebral hemispheres. Treatment patients with sarcoidosis in association with optic started again with ACTH 40 units twice daily; neuritis. Fine & Flocks (1953) mention a very complete neurological remission obtained within rapid response to steroids. Papilleedema (James fourteen days. In remission and on steroids an et al. 1967) occurs secondary to raised intraisotope cysternogram showed abnormal pene- cranial pressure or from direct infiltration of the tration of isotope into the third ventricle as well optic nerve, but it also occurs as a noninfiltrating

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effect of systemic sarcoidosis without CNS involvement (Blain etal. 1965).

Addendum (December 1974): Ten months after last relapse the patient remains asymptomatic and clinically well. REFERENCES Blain J G, Riley W & Logothetis J (1965) Archives ofNeurology 13,307 Fine M & Flocks M (1953) Archives of Ophthalmology 50, 358 James D G (1959) American Journal of Internal Medicine 26, 331-339 James D G, Zatouroff M A, Trowell J & Rose F C (1967) British Journal of Ophthalmology 51, 526 Koumrouyan H (1950) Acta oto-laryngologica 38,45 Longcope W J (1941) Journal ofthe American Medical Association 117, 1321 Salvesen H A (1935) Acta medica Scandinavica 86, 127 Urich H (1969) Practitioner 202, 632 Zeman W (1958) Handbuch der Speziellen Pathologischen Anatomie und Histologie. Springer, Berlin; p 1100

Dr V W Johnson (New Cross Hospital, Wolverhampton) said this appeared to be a rare syndrome and wondered if the patient would prove to have a reticulosis as neurological complications and sarcoid changes were known to occur in Hodgkin's disease particularly. Dr L J Findley replied that the history of relapses and remissions with responsiveness to steroids and positive Kveim reaction was consistent with sarcoidosis.

Dr Graham Neale (Royal Postgraduate Medical School, Hammersmith Hospital) said that the diagnosis of cerebral sarcoidosis was always difficult to make with certainty but asked if it was not true that CSF protein was invariably elevated if the meninges were involved. (Scadding J G, 1967, Sarcoidosis. Eyre & Spottiswoode, London; p 287.) Dr Findley replied that from the literature sarcoid involving the CNS did not invariably produce an elevation of CSF protein. Dr F Clifford Rose said that this patient was of interest because of two neuro-ophthalmological syndromes usually characteristic of multiple sclerosis. The first was optic neuritis, i.e. unilateral blurred vision associated with pain on eye movement which improved in most cases to normal or near normal vision, with or without steroid therapy. In several hundred personal cases, he had found no cause other than multiple sclerosis (Rose 1972). The secona was ataxic nystagmus, i.e. unilateral nystagmus on abduction with weakness of adduction of the opposite eye. This was accepted as being due to a lesion of the medial longitudinal fasciculus of the brain stem (the MLF syndrome) and the commonest cause, especially when bilateral was multiple sclerosis. ff this was the diagnosis in this case, it was difficult to explain the pain, the raised ESR and the positive Kveim test. In answer to a question by Dr J Lewis, Dr Rose replied that visual evoked responses were helpful in indicating a lesion of the optic pathways but not the pathological type. REFERENCE Rose F C (1972) In: The Optic Nerve. Ed. I S Cant. Kimpton, London; pp 217-219

Meeting 11 October 1974

Cases Rheumatoid Arthritis Followed by Systemic Lupus Erythematosus F P Bresnihan MB MRCP (for B M Ansell m FRcp) (MRC Rheumatism Unit, Canadian Red Cross Memorial Hospital, Taplow, Maidenhead, Berks) L M, man aged 62 History: Presented in April 1972 with a history of polyarithritis for four months accompanied by morning stiffness, severe enough to stop him working as a bricklayer. A clinical diagnosis of rheumatoid arthritis was made. This was substantiated by an ESR of 51 mm in one hour (Westergren), Latex test positive, differential agglutination titre (DAT) 1:32, antinuclear antibodies (ANA) negative, and metatarsal erosions. In August 1972, as he failed to respond to salicylates, he entered our gold/cyclophosphamide trial (Gumpel et al. 1973), receiving cyclophosphamide. After eight weeks he improved but there was a tendency to neutropenia, which led to discontinuation of cyclophosphamide in November 1973. In December 1973 he was admitted to hospital with consolidation of the right lung base. He failed to produce purulent sputum and continued to have a relative neutropenia. Antinuclear antibodies remained absent. There was also a slow response to antibiotics, but he remained generally unwell with progressive weight loss and a high ESR (over 100 mm). In March 1974, he was readmitted with consolidation in the left lung. He now had anemia (Hb 8.6 g/100 ml), and a raised alkaline phosphatase (141 iu). Investigations for underlying neoplasia were negative. He continued to deteriorate with further weight loss, alopecia, psychotic episodes, intermittent fever, skin rash, thrombocytopenia and minimal synovitis. A strongly positive ANA, raised DNA binding capacity (28%), and lowered serum C3 (60 mg/ 100 ml) were found. Systemic lupus erythematosus was diagnosed and prednisone started with benefit. Discussion

Rheumatoid arthritis and systemic lupus erythematosus have several features in common. Though differentiation of one from the other is usually possible, patients with clinical, histological or autopsy evidence of both diseases simultaneously (Kantor et al. 1969) are occasionally seen. This patient demonstrated two distinct