Tsai, Howden and Thomson Probable Chron's colitis mimicking ischaemic colitis ischaemic colitis on the basis of barium enema findings. In three patients there was no histological confirmation. One patient had appendicectomy; the appendix showed nonspecific chronic inflammatory changes. If the appendix had been involved in the ischaemic process distinctive histological features would have been expected." The diagnosis of "ischaernic colitis" in these patients remains speculative. This case suggests that acute Crohn's colitis may mimic ischaemic colitis radiologically. Ischaemic colitis should not be diagnosed in a young patient on the basis of the barium enema findings alone. Colonoscopy and biopsy of affected areas should be performed. Some of the previously reported cases of "ischaemic colitis" in young adults may have been misdiagnosed. REFERENCES I Boley SJ. Schwartz S. Lash J, Sternhill V. Reversible vascular occlusion of the colon. Surg. Gynecol. Obstet: 1963; 116: 53-60. 2 Clark A W, Lloyd-Mostyn RH, de Sadler MR. "Ischaemic" colitis in young adults. BMJ 1972; 4: 70-72. 3 Barcewicz PA, Welch JP. Ischaemic colitis in young adult patients. Dis. Colon. Rectum 1980; 23: 109. 4 Thomas ML. Plain films and barium studies of ischaemic bowel. Clinics in Gastroenterology. 1972; 1:3.581-595. 5 McGovern VJ, Goulston SJM. Ischaemic enterocolitis. Gut 1965; 6: 213. 0036/9330/89/0458/0407/$2.00 in USA Scot Med J 1989; 34: 407-408 © 1989 Scottish Medical Journal MALIGNANT ANGIOENDOTHELIOSIS - AN UNSUAL CAUSE OF STROKE I.M. Lennox. J. Zeeh. N. Currie, *G. Martin. **J.H. Adams Departments of Geriatric Medicine and • Pathology, Victoria Infirmary and" Department of Neuropathology, Institute of Neurological Sciences, Southern General Hospital, Glasgow. Correspondence to and reprints from: Dr lain M. Lennox, Victoria Infirmary, Glasgow Dr). Zeeh' was supported by a grant from the B. Stiftung, Melsungen, F.R.G. Abstract: We report a case of malignant angioendotheliosis in a 63-year-old female who presented with a right hemiparesis. This diagnosis should be considered when multi-focal neurological signs develop in association with a progressive deterioration ofmental state and conscious level. Key words: malignant angioendotheliosis, multiple neurological deficit. stroke. -. Case report An obese 63-year-old maturity onset diabetic with a history of arterial hypertension was admitted to hospital having suddenly developed a right hemiparesis with dysarthia 10 days previously. The patient was drowsy and showed evidence of recent memory loss, but according to her family she had previously been completely lucid, although she had complained for the past six weeks of feeling vaguely unwell. In the interval between the development of the hemiparesis and admission to hospital her conscious level had been noted to fluctuate. Her only medication was Metformin. On admission the patient was drowsy but responsive to verbal comands. Speech was dysarthric and there was flaccid paralysis of the right leg with and extensor plantar reflex. There was a mild left ptosis and a right homonymous hemianopia. Her conscious level deteriorated intermittently over the next 48 hours and she developed paralysis of her right arm, with conjugate deviation of the eyes to the right. Fundoscopy was unremarkable. The full blood count and peripheral blood film were repeatedly normal. and biochemical monitoring failed to reveal any significant metabolic upset. A scan performed one week after admission showed • • Fig. I • - • , , , ,.-• . • • • ,, ' .,. , ~ FIg.2a . .. . cr ~.J .. • .... - • \ ..~ Fig.2b 407 Lennox, Zeeh, Currie, Martin and Adams Malignant angioendotheliosis no abnormality (skull X-ray and isotope brain scan were also normal). By this time the picture had changed to a left hemiparesis with persistent flaccid weakness of the right leg. Over the next week she became unresponsive to pain and developed a pyrexia of 39°C. No obvious focus of infection was found and blood cultures were negative. Lumbar puncture yielded normal results. A course of dexamethasone did not produce a significant improvement in the clinical state and the patient eventually died in deep coma five weeks after the onset of neurological symptoms. The principal findings post mortem were widespread and severe atheroma and bronchopneumonia. The brain was of entirely normal appearance externally. In coronal slices of the cerebral hemispheres there were occasional small grey disintegrating foci in the subcortical white matter (Fig. 1). The brain stem and cerebellum were normal on section. Histological examination established that the abnormal foci in the white matter were infarcts. Several others that had not been seen macroscopically were also identified. The most striking feature, however, was the presence of many nucleated cells in small blood vessels in the subarachnoid space and in all parts of the brain (Figs 2a and 2b). The cells varied in size, had scanty cytoplasm and rather grooved hyperchromatic nuclei. Mitotic figures were identified in occasional cells. No thrombosed vessels were identified. The appearances were typical of malignant angioendotheliosis. Review of sections available from other organs of the body established the presence of similar cells in small blood vessels in the lungs. Discussion Malignant an¥ioendotheliosis is thought to be a rare form of lymphoma ,2 first described by Pfleger and Tappeiner 3 in 1959, in which the malignant cells are localised intravascularly, usually within the central nervous system, leading to a wide range of neurological signs." The skin, lungs, kidneys, adrenal glands and heart can also be affected. 5 One fifth of patients have more benign chronic cutaneous presentation, but our case is typical of the rapidly progressive form of the disease, with a short prodromal illness followed by fluctuating neurological signs and progressive mental impairment. The disease is uniformly fatal and the improvement observed in some cases after steroid administration is of questionable significance.V' Brain biopsy is currently the only definitive diagnostic test available. In one case a high dose contrast delay CT scan demonstrated the pathological changes within the white matter and the authors suggest that this examination might be of diagnostic value.P In our patient the initial presentation suggested a straightforward cerebrovascular accident or transient cerebral ischaemic attack, but the development of bilateral signs and an obviously fluctuating conscious level, raised the possibility of subdural haemotoma (there was a vague history of minor head injury). In the presence of a normal CT scan and negative bacteriological/biochemical drug screening our clinical impression was that this patient was experiencing numerous minor cerebrovascular incidents, although the aetiology was unclear. In retrospect this was exactly what was happening, with neoplastic cells compromising the vascular supply and producing multifocal ischaemic damage. The reason for intravascular localisation of the malignant cells is unclear, although it has been suggested that the cells may lack the necessary surface receptors for extravascular migration. 7 Although malignant angioendotheliosis is rare, it might be potentially treatable with cytotoxic agents 2 and the diagnosis should be considered in any patient presenting with multifocal, fluctuating, neurological signs. REFERENCES 1 Bhawan J, Wolff SM, Ucci AA, Bhan AK. Malignant lymphoma and malignant angioendotheliomatosis: one disease. Cancer 1985, 55: 570-576. 2 Daniel SE, Rudge P, Scarvilli F. Malignant angioendotheliosis involving the nervous system; support for a lymphoid origin of the neoplastic cells. J. Neurol. Neurosurg. Psychiatry 1987, 50: 1173-1177. 3 Pfleger L, Tappeiner J. Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefasse (Reticuloendotheliose?) Hautarzt 1959; 10: 359-63. 4 Beal MF, Ferry JA. Case records of the Massachusetts General Hospital. Case 39. N. Engl. J. Med 1986; 315: 874-85. 5 Knight RSG, Anslow P, Theaker JM. Neoplastic angioendotheliosis; a case of subacute dementia with unusual cerebral CT appearances and a review of literature. J. Neurol. Neurosurg. Psychiatry 1987: 50: 1022-28. 6 Dolman CL, Sweeney VP, Magil, A. Malignant angioendotheliosis - a case of the missed primary? Arch. Neurol. 1979; 36: 5-7. 7 Carroll TJ, Schelper, RL, Goeken, JA Kemp JD. Neoplastic angioendotheliomatosis: Immunopathologic and morphologic evidence for intravascular malignant lymphomatosis. Am. J. Clin. Pathol. 1986; 85: 169-75. Scot Med J 1989; 34: 408-409 0036-9330/89/0598/0408/$2.00 in USA © 1989 Scottish Medical Journal ARGININOSUCCINIC ACIDURIA - AN UNDERDIAGNOSED CAUSE OF NEONATAL DEATH? l.H. White, S.J. Rose, * and D.J. Lloyd* Departments of Clinical Biochemistry and Child Health, * University of Aberdeen, Aberdeen. Abstract: We describe a case of neonatal argininosuccinic aciduria, a condition we suggest is underdiagnosed. Although the clinical presentation can be of overwhelming septicaemia, certain routine biochemical investigations are indicative ofthis inborn error of urea cycle metabolism. Key words: Neonatal argininosuccinic aciduria. Introduction R GININO SU CCINIC aciduria is a rare inborn error of the urea cycle due to a deficiency of argininosuccinate-lyase. I It is most commonly diagnosed in late infancy A Correspondence to: Dr Ian White, Department of Clinical Biochemistry, University Medical Buildings, Foresterhill, Aberdeen AB9 2ZD. 408. or childhood when the clinical symptoms of mental retardation, brittle hair and nails, convulsions, periods of coma and ataxia, and hepatomegaly become apparent. 1 Reports of neonatal diagnosis of argininosuccinic aciduria are few,2,3,4,5 which may reflect an underdiagnosis in some cases of sudden, unexplained neonatal death. Prompt diagnosis of neonatal argininosuccinic aciduria, which is