Postgrad Med J: first published as 10.1136/pgmj.53.625.704 on 1 November 1977. Downloaded from 704 Case reports such a disorder-the leuco-erythroblastic blood activity to a varying degree and also interferes with picture and histological evidence of haemopoiesis the control of cell release from the bone marrow. in the spleen, but the histological appearances of the This would not be surprising with an oat cell carci- bone marrow and biopsies were not those of myelo- noma whose capacity for synthetizing various poly- fibrosis. The amount of bone marrow was excessive peptides with physiological properties is well known. in both patients, despite extensive metastases in one In addition this would support the suggestion of them (Case 1) and the absence of haemolysis that marrow fibrosis is not the initial event in the or bleeding suggests that this cell proliferation was pathogenesis of agnogenic myeloid metaplasia. due to something other than simple compensatory hyperplasia. The monocytosis, present in both Acknowledgment patients, could be a further manifestation of an ab- We thank Professor M. J. S. Langman for his help in the normal marrow proliferation for, although blood preparation of this manuscript. monocyte counts are higher on average in patients with cancer, they rarely reach the level that was References recorded in the first case (Barrett, 1970), and a mono- BARRETT, COL. O'NEILL, JR (1970) Monocytosis in malignant cytosis has been described in other myeloprolifera- disease. Annals of Internal Medicine, 73, 991. tive states (Maldonado and Hanlon, 1965). CHERVENICK, P. (1973) Increase in circulating stem cells in Thus fibrosis of the bone patients with myelofibrosis. Blood, 41, 67. marrow is not an essen- DAMESHEK, W. & GUNZ, F. (1964) Leukemia, Second tial feature of the myeloproliferative disturbance Edn, Grune and Stratton, New York. that has previously been described in patients with KIELY, J.M. & SILVERSTEIN, M.N. (1969) Metastatic car- metastatic cancer. Furthermore, the failure to find cinoma simulating agnogenic myeloid metaplasia and tumour in the marrow of one of myelofibrosis. Cancer. New York, Philadelphia, etc., the present patients 24, 1041. (Case 2) suggests that bone secondaries are not MALDONADO, J.E. & HANLON, D.G. (1965) Monocytosis: a essential either. One explanation for this type of current appraisal. Proceedings of Staff Meetings of the Protected by copyright. haematological complication of malignancy and of Mayo Clinic, 40, 248. the myeloid metaplasia that has been WARD, H.P. & BLOCK, M.H. (1971) The natural history of described agnogenic myeloid metaplasia and a critical evaluation by others, is that some tumours elaborate a factor of its relationship with the myeloproliferative syndrome. which stimulates fibroblastic, myeloid or erythroid Medicine. Baltimore, 50, 357. Postgraduate Medical Journal (November 1977) 53, 704-708. Papilloedema associated with respiratory failure 1. F. PYE R. L. BLANDFORD http://pmj.bmj.com/ M.A., M.B., M.R.C.P. M.B., M.R.C.P. Department of Neurology, University Hospital of Wales, Heath Park, Cardiff Summary Case history on September 28, 2021 by guest. A case of papilloedema secondary to respiratory A 49-year-old housewife presented in January failure is reported which caused considerable diag- 1976 with a 4-year history of progressively severe nostic difficulty and led to extensive neurological right frontal headache exacerbated by coughing investigation. Neurological complaints of headache and lying down and which was frequently worse in and visual impairment overshadowed respiratory the mornings. For 6 months vision had been in- symptoms and were associated with gross haemor- distinct, with difficulty in reading newsprint, and rhagic papilloedema. The case is compared with pre- one momentary episode of absolute visual failure vious reports and the pathogenesis of papilloedema in had occurred during December 1975. The patient's respiratory failure is reviewed briefly. The similarity general health was good apart from long standing between the pathogenesis of this condition and benign chest symptoms of wheezing and dyspnoea on hills intracranial hypertension is discussed. and stairs, which she attributed to a chest deformity Postgrad Med J: first published as 10.1136/pgmj.53.625.704 on 1 November 1977. Downloaded from Case reports 705 dating from childhood. She had smoked regularly only non-specific intermittent bilateral excess of theta fifteen cigarettes per day for 30 years and had activity. The gamma cerebral scan was normal. recently taken salbutamol 4 mg t.d.s. Shortly after admission to hospital, the patient On examination she was a moderately obese alert developed bilateral pitting ankle oedema without lady, able to give a good account of herself. She other evidence of congestive failure. Chlorthalidone had a dorso-cervical scoliosis, a short neck and low 50 mg was added to her maintenance salbutamol. hairline. The cardiovascular system was normal, In view of the essentially negative findings, but per- pulse 80/min, sinus rhythm with a blood pressure sisting headache, pneumoventriculography was of 18-6/10-6 kPa. In the chest there were scattered attemped via biparietal burr holes. This demon- inspiratory and expiratory rhonchi with some coarse strated a tense brain, but an attempt to cannulate bilateral basal crepitations. The most impressive the ventricles failed. Therefore, pneumoencephalo- clinical sign was bilateral high grade papilloedema graphy via the lumbar route was attempted, but with flame shaped haemorrhages. Corrected visual after premedication with 10 mg papaveretum, the acuity was RIGHT 6/9 and LEFT 6/6. The only other patient developed marked cyanosis and became very abnormal findings were some impairment of alter- drowsy. Treatment with nalorphine, oxygen, doxa- nate motion rate in the fingers and finger/nose ataxia pram, physiotherapy and later ampicillin and on the left, together with heel/toe ataxia and a ten- cloxacillin produced a gradual improvement in her dency to stumble to the left. respiratory state, although a temporary relapse occurred 4 days later. Serial arterial blood gas Investigations determinations are listed in Table 1. During this Hb 15-2 g/dl; PCV 47-1; red cell indices and white period, despite preservation of visual acuity, there blood cells were normal. Blood urea, electrolytes, was a marked deterioration in the fundal appearances calcium and phosphate, normal. Arterial blood with the development of bilateral subhyaloid haemor- gases: pH7-34, Pco2 6 5kPa, Po2 5 9kPa, HCO3 24 5 rhages (Fig. 1). Subsequently, lumbar pneumo- Protected by copyright. mmol/l. Chest X-ray showed multiple congenital encephalography showed small lateral ventricles abnormalities of the rib cage especially in the right (Fig. 2), centrally sited about the midline, a normal upper zone, and a high dorso-cervical scoliosis fourth ventricle and no evidence of a space-taking convex to the right. There was cardiomegaly with lesion or cerebellar ectopia. The CSF was normal, some congestive changes in both lung fields. Only the protein being 0-1 1 g/l. six vertebrae demonstrated in the cervical spine, and there was well marked disc degeneration at Progress C5/6. Skull X-rays showed a minor degree of basilar Dexamethasone 2 mg q.d.s. was commenced and invagination in a globular skull, hypoteliorism but intermittent 24%4 oxygen continued. The headache no evidence of raised pressure. The visual fields improved but at the time of discharge and again showed bilateral enlarged blind spots. The ECG at review 6 weeks later there had been no appreciable showed pulmonale, right axis deviation and early change in the fundal appearances. Treatment at right ventricular hypertrophy. The EEG revealed the time of discharge consisted of bendrofluazide TABLE 1. Blood gases http://pmj.bmj.com/ Pco2 Po2 HCO3 Date pH (kPa) (kPa) (mmol/l) 10.2.76 7-34 6-5 59 24-5 11.2.76 (after papaveretum) 7-27 6-9 5-6 21 5 11.2.76 (after 24% 02) 7-34 8-6 6-3 30-0 on September 28, 2021 by guest. 12.2.76 7 49 7-3 7-4 39-0 13.2.76 7-46 6-3 8-0 32-0 15.2.76 (no 02 for 7 hr) 7-43 8-6 7-3 38-5 16.2.76 (after 24% 02) 7-46 6-3 8-0 32-0 20.2.76* (after 28% 02) 7-42 6-4 - 300 24.2.76* (off 02) 7-44 4-3 - 23-5 5.3.76 7.49 5-3 8-4 31-5 10.8.76 7 40 5-3 8-0 24-5 * P02 values were unreliable and are therefore omitted. Postgrad Med J: first published as 10.1136/pgmj.53.625.704 on 1 November 1977. Downloaded from 706 Case reports FIG. 1(a) and (b). Optic fundi showing bilateral high grade papilloedema and a triangular subhyaloid haemorrhage on the left. Protected by copyright. http://pmj.bmj.com/ on September 28, 2021 by guest. FIG. 2. Lumbar air encephalogram: Townes' view with small lateral ventricles symmetrically sited about the midline. Postgrad Med J: first published as 10.1136/pgmj.53.625.704 on 1 November 1977. Downloaded from Case reports 707 10 mg daily with potassium supplements, salbutamol, and Kaye, 1954; Westlake, Simpson and Kaye, prethcamide and dexamethasone 2 mg t.d.s. The 1955; Sieker and Hickam, 1956; Conn et al., 1957). steroids were gradually withdrawn over a 2-week In many ofthese reported cases, respiratory symptoms period. were prominent and neurological symptoms super- Three months after discharge the patient no vened later with the development of headache, longer complained of headache and commented mental confusion, tremor, twitching and altered that exercise tolerance had improved significantly consciousness. The appearance of papilloedema following abstinence from cigarettes. On examina- was often associated with gross hypercapnia and tion, the papilloedema had completely subsided with arterial Pco2 levels of 12 kPa or more.