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HAVE AN INTERESTING CLINICAL CASE?
Email the editor at jo. hartley @ adg. com. au. We pay $ 400 for each case and photos are encouraged.
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AN obese, 49-year-old man with no medical history of note presented in August 2014, with a five-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 inflammatory response.
Flexible sigmoidoscopy showed severe confluent colitis( see figure 1A), and histopathology confirmed a first presentation of active ulcerative colitis.
In view of these findings, we started intravenous methylprednisolone 30mg twice daily for seven days followed by a standard reducing course of oral prednisolone( 40mg daily, decreasing by 5mg per week).
Because he had only a part response to steroids, we started rescue therapy with cyclosporin on day five, with stool frequency, serological markers and observations all returning to normal by day 11, when he was discharged.
He re-presented one 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 cyclosporin 200mg twice daily and prednisolone 15mg daily, and had received 45 days of steroids in total.
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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( Medical Research Council Scale for Muscle Strength Grade of 4 +/ 5).
Lower-limb deep tendon reflexes 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 10th rib. Spine examination was unremarkable.
Blood results were within normal range, reducing the likelihood of an intrinsic cause of myelopathy such as infection, or vitamin B12 or copper deficiency, and he had no evidence of impaired glucose tolerance.
MRI of the spine showed an epidural mass from T1 to T11( see figure 1B), with further epidural deposits in the sacral region.
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 diffuse distribution could only represent lipomatosis.
Debulking surgery could not be done because of the extensive nature of the spinal cord compression,
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Figure 1. A: Endoscopic image of sigmoid colon demonstrating severe colitis. B: T1 weighted MRI scan of spine. Sagittal view of the cervical and thoracic spine shows spinal epidural lipomatosis with compression T1 – T9( arrow).
since he would have required a T1-L2 decompressive laminectomy followed by pedicle screw fixation 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: rapid tapering of his steroids over seven days; pregabalin for neuropathic pain; and physiotherapy and dietitian input to aid weight loss.
Interval MRI five 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.
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