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Malignant Histiocytosis and Encephalomyeloradiculopathy

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Malignant Histiocytosis and Encephalomyeloradiculopathy Gut: first published as 10.1136/gut.24.5.441 on 1 May 1983. Downloaded from Gut, 1983, 24, 441-447 Case report Malignant histiocytosis and encephalomyeloradiculopathy complicating coeliac disease M CAMILLERI, T KRAUSZ, P D LEWIS, H J F HODGSON, C A PALLIS, AND V S CHADWICK From the Departments ofMedicine and Histopathology, Royal Postgraduate Medical School, Hammersmith Hospital, London SUMMARY A 62 year old Irish woman with an eight year history of probable coeliac disease developed brain stem signs, unilateral facial numbness and weakness, wasting and anaesthesia in both lower limbs. Over the next two years, a progressive deterioration in neurological function and in intestinal absorption, and the development of anaemia led to a suspicion of malignancy. Bone marrow biopsy revealed malignant histiocytosis. Treatment with cytotoxic drugs led to a transient, marked improvement in intestinal structure and function, and in power of the lower limbs. Relapse was associated with bone marrow failure, resulting in overwhelming infection. Post mortem examination confirmed the presence of an unusual demyelinating encephalomyelopathy affecting the brain stem and the posterior columns of the spinal cord. http://gut.bmj.com/ Various neurological complications have been patients with coeliac disease by Cooke and Smith.6 described in patients with coeliac disease. These She later developed malignant histiocytosis with include: peripheral neuropathy, myopathy, evidence of involvement of bone marrow. myelopathy, cerebellar syndrome, and encephalo- on September 27, 2021 by guest. Protected copyright. myeloradiculopathy.1 2 On occasion, neurological Case report symptoms may be related to a deficiency of water soluble vitamins or to metabolic complication of A 54 year old Irish woman first presented to her malabsorption, such as osteomalacia. In most local hospital in 1970 with a two year history of instances, however, such causative factors cannot be abdominal pain; pale, watery stools; ankle swelling implicated. and weight loss. Blood tests revealed iron, folic acid Malignant complications are also well recognised and vitamin K deficiency, and low levels of serum in coeliac disease with increased prevalence of both albumin and calcium. A peroral suction biopsy of gastrointestinal adenocarcinomas and of lymphomas the third part of the duodenum showed partial with and without gut involvement.3 It has recently villous atrophy, flattening of surface enterocytes, been shown4 5 that these 'Iymphomas' consist of increased epithelial lymphocytes, and increased cells with morphological and immunohistochemical mononuclear cell infiltration in the lamina propria characteristics of histiocytes, and the condition suggesting adult coeliac disease. A gluten free diet could therefore be classified as malignant histio- was instituted and the diet supplemented with iron, cytosis. folic acid, calcium, and vitamin B12. Within weeks, This paper describes a patient with coeliac disease her pain and diarrhoea had disappeared, and her who developed a neurological syndrome resembling weight, haematological and biochemical abnorm- the encephalomyeloradiculopathy described in alities had returned to normal. The jejunal was reassessed. She Address for reprints: M Camilleri, Gastroenterology Unit, Hammersmith morphology not, however, Hospital. Ducane Road. London W12 OHS. continued to enjoy good health until seven years Received for publication 13 August 1982 later when, after a three month period of anorexia 441 Gut: first published as 10.1136/gut.24.5.441 on 1 May 1983. Downloaded from 442 Camilleri, Krausz, Lewis, Hodgson, Pallis, and Chadwick and weight loss, she developed deafness in the right neurological lesions involving the postchiasmatic ear, numbness on the right side of the face, an optic tracts; the brain stem at the level of the left absent right corneal reflex, and some unsteadiness cochlear nucleus; the posterior (and possibly lateral) of gait. The clinical features suggested a lesion in the columns of the spinal cord; and the lumbo-sacral region of the right cerebellopontine angle or nerve roots (widely and asymmetrically). The sural affecting the right side of the brain stem. Full nerve action potentials were retained, and histamine radiological studies of the skull (including CT scan flares were brisk over the feet. Computerised and vertebral and carotid angiograms) were non- tomographic scan and metrizamide myelography did contributory. not reveal any space occupying lesion in the brain, Over the next six months, the lower limbs became cerebellopontine angle, or spinal cord. The cerebro- progressively weaker. Examination revealed that spinal fluid contained 38 mg protein per 100 ml with the cranial nerve signs had receded somewhat but an oligoclonal pattern on electrophoresis. Eighteen there was now weakness of both lower limbs. leucocytes were also detected per mm3 of CSF (56% Tendon reflexes and perception of pinprick were lymphocytes, 33% polymorphs, and 11% absent below the knees. The plantar responses were histiocytes). equivocal, possibly extensor. The visual evoked Her neurological status continued to deteriorate, responses were normal, as was the spinal fluid. The so that by May 1980, when she was referred to haemoglobin was 14-2 g/dl, albumin 40 g/l, calcium Hammersmith Hospital, she was severely 2.45 mmol/l, red cell folate 64 ng/ml (normal incapacitated with deafness in the right ear, 160-640), and urine D-xylose excretion after five weakness of both lower limbs, impaired sensation hours was 16 mmol (N: 33). The clinical diagnoses at (all modalities) in L5-S5 dermatomes, and reduced this time were coeliac disease with peripheral bowel and bladder sensation. She had no complaints neuropathy, although the possibility of multiple referable to the gastrointestinal tract. sclerosis had been considered at one stage. She was The clinical diagnosis was revised to encephalo- treated with a course of ACTH and prednisolone myeloradiculopathy complicating coeliac disease.6 and improved markedly. Within six months she was A detailed dietary assessment revealed that gluten fully ambulant. had been ingested consistently during the past 10 This remission was not sustained, however, and years. Haemoglobin was 13 g/dl, serum albumin 45 six months later (January 1980) there was rapid g/l, B12 level 300 ng/l, and serum and red cell folates http://gut.bmj.com/ deterioration with weakness in both legs (R>L) and (while on supplements) were 94 and 4656 ,ug/1, numbness and burning pain in the right leg and foot. respectively. Jejunal biopsy performed while on a For the first time, she also had saddle anaesthesia gluten restricted (though not completely gluten free) and loss of anal, urethral, and vaginal sensation. She diet showed partial villous atrophy, infiltration of was referred to the National Hospital for Nervous epithelium by lymphocytes, increased cellularity of Diseases, London, where detailed examination and the lamina propria, and crypt hyperplasia (Fig. 1). extensive investigations suggested multiple discrete Intestinal function tests revealed reduced urinary on September 27, 2021 by guest. Protected copyright. Fig. 1 Photomicrograph of jejunal biopsy showingpartial i villous atrophy and increased '6 * chronic inflammatory infiltrate i: in lamina propria (H and E, x80, original magnification). :L' Gut: first published as 10.1136/gut.24.5.441 on 1 May 1983. Downloaded from Malignant histiocytosis and encephalomyeloradiculopathy complicating coeliac disease 443 excretion of a 25 g D-xylose load (16 mmol or 2-4 g o 2 in five hours), increased faecal fat excretion (36 mmol (10 g/day), and marginally low vitamin B12 0 - absorption (8% on a Schilling test performed with intrinsic factor). These findings were still consistent with poorly-treated coeliac disease. Although there -23 * Right ° were no ocular or mucocutaneous signs of oLeft " nutritional deficiencies, assays were performed for B , vitamins (thiamine, biotin, pyridoxine, B12) and |-4." trace elements (copper, zinc, magnesium). The J J A S O N D J'F serum levels were all within the normal range. 1980 " 1981 Clinical management was based on the assumption Fig. 2 Graph ofthe change in musclepower in two lower that the neurological disorder was a nutritional limbs. Each point is mean ofthe power recorded in all complication of active coeliac disease and accord- muscle groups tested in each limb, compared with the ingly a therapeutic trial of high dose vitamin and musclepower on presentation. Letters on horizontal axis mineral therapy together with a strict gluten free indicate month starting with June 1980. diet and intensive physiotherapy was instituted. After two weeks without obvious improvement, prednisolone 30 mg/day was added to this regime, tomy at which no evidence of malignancy was found but there was no improvement in neurological macroscopically or on biopsies of the intestine and symptoms and signs, or in gut morphology or liver. function. Four weeks postoperatively, she developed ankle Between June and October 1980 there was weight swelling, deterioration of the weakness and wasting loss of 6 kg, a progressive fall in haemoglobin, in the lower limbs, and general lethargy. The serum cholesterol, and albumin levels (Table) and haemoglobin level had fallen to 9.4 g/dl and the for the first time, there was evidence of hypo- albumin to 24 g/l, and both the total leucocyte and splenism on the blood film (Howell-Jolly bodies). platelet counts were falling rapidly. A repeat bone Splenic hypofunction
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