Bilateral lower limb weakness in acute severe ulcerative colitis

Abstract

An obese 49-year-old man with no medical history of note presented in August, 2014, with a 5-week history of bloody diarrhoea, anorexia, and unintentional weight loss. He reported passing 20 bloody stools per day, with nocturnal disturbance, and on admission had pyrexia, tachycardia, anaemia, and a robust infl ammatory response. Flexible sigmoidoscopy showed severe confl uent colitis, and histopathology confi rmed a fi rst presentation of active ulcerative colitis (fi gure). In view of these fi ndings we started intravenous methylprednisolone 30 mg twice daily for 7 days followed by a standard reducing course of oral prednisolone (40 mg daily, decreasing by 5 mg per week). Because he had only a part response to steroids, we started rescue therapy with ciclosporin on day 5, with stool frequency, serological markers, and observations all returning to normal by day 11 when he was discharged. He re-presented 1 month later with progressive gait disturbance, falls, lumbar pain, and a feeling of a tight band around his torso. He denied any history of urinary retention or incontinence or bowel incontinence, but did report new erectile dysfunction. He had no history of fever or spinal trauma, and no previous neurological symptoms. At this point he was taking ciclosporin 200 mg twice daily and prednisolone 15 mg daily, and had received 45 days of steroids in total. His colitis remained in excellent clinical and biochemical remission. On examination he had a moon face typical of Cushing’s syndrome and walked with the assistance of two crutches. Tone was increased in both legs with a symmetrical pattern of pyramidal weakness (MRC Grade 4+/5). Lowerlimb deep tendon refl exes were pathologically brisk bilaterally (knee jerk 3+, ankle jerk 4+) with non-sustained bilateral ankle clonus. Joint position sense was impaired distally in the lower limbs and plantar responses were equivocal. He had reduced sensation to light touch and pinprick below T10, and reduced vibration sensation to the tenth rib. Spine examination was unremarkable. Blood results were within normal range, reducing the likelihood of an intrinsic cause of myelopathy such as infection, vitamin B12, or copper defi ciency, and he had no evidence of impaired glucose tolerance. MRI of the spine showed an epidural mass from T1 to T11, with further epidural deposits in the sacral region (fi gure). The mass was completely suppressed on short T1 inversion recovery (STIR) imaging and was uniformly bright on both T1 and fast spin echo T2 images. This appearance is consistent only with fat, and in view of its diff use distribution could only represent lipomatosis. Debulking surgery could not be done because of the extensive nature of the spinal cord compression, since he would have required a T1-L2 decompressive laminectomy followed by pedicle screw fi xation and direct excision of the lipomatosis, which would have carried a very high risk of blood loss and morbidity as well as exacerbation of his lumbar spinal back pain. We therefore opted for conservative management: a rapid tapering of his steroids over 7 days; pregabalin for neuropathic pain; and physiotherapy and dietitian input to aid weight loss. Interval MRI 5 months later showed part resolution of the spinal lipomatosis with compression extending from T6 to T9 and no intrinsic cord signal abnormality. He continued to improve gradually, and in October, 2015, had only residual decreased sensation in his toes and normal power throughout, although he continued to walk with the aid of walking sticks. Spinal epidural lipomatosis is the pathological overgrowth of physiological fat tissue in the extradural space leading to spinal cord compression. It is uncommon, and has been reported in infl ammatory bowel disease only twice previously. Spinal epidural lipomatosis can be a complication of long-term corticosteroid treatment (55·3% of cases) but rarely presents acutely or in as short a period of treatment as in our patient. 24·5% of cases are attributed to obesity alone and the increase in background extradural fat in obese people might predispose obese patients to more rapid onset of corticosteroid-induced spinal epidural lipomatosis. 17% of cases have no identifi able cause and we are unaware of any previous reports of ciclosporin associated with spinal epidural lipomatosis. 25% of reported cases of spinal epidural lipomatosis associated with steroid use are treated conservatively with removal of exogenous steroids and weight loss. Surgical laminectomy with debulking has been more frequently reported as an alternative treatment (75% of cases) and has a similar reported response rate to conservative management (77%). Few cases have been reported of patients with spinal epidural lipomatosis as Lancet 2016; 388: 101–02