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A 59 year old man with progressive spinal cord and peripheral nerve dysfunction culminating in encephalopathy: Edinburgh advanced clinical neurology course, 1999
  1. R Al-Shahia,
  2. C P Warlowa,
  3. G H Jansenb,
  4. CJM Frijnsc,
  5. J van Gijnc
  1. aDepartment of Clinical Neurosciences, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK, bDepartment of Neuropathology, University Hospital of Utrecht, Box 85500, 350GA Utrecht, The Netherlands, cDepartment of Neurology
  1. Professor C P WarlowCharles.Warlow{at}

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Case presentation

A 59 year old, retired metal worker presented with a 3 month history of difficulty walking. He had initially developed left leg weakness, followed 1 month later by numbness of both legs, a burning sensation in his anal region and penis, constipation, urinary frequency, retention, and incontinence. At that stage he presented to another hospital with abdominal pain, but all investigations for this were normal. On examination there was some weakness of his left leg, but no objective sensory loss in the legs or perineum. The weakness progressed to involve his right leg over the next 2 months such that he needed a stick to walk.

For 6 years before presentation he had had unremitting paraesthesiae of both shins, for which no cause had been found. He had been judged unfit to work 4 years previously due to depression. Ankylosing spondylitis had been diagnosed at the age of 30, followed by proctitis 10 years later for which he had been successfully treated with sulphasalazine.

Cognitive, cranial nerve, cerebellar, and general physical examination were unremarkable. Upper arm and pectoralis muscles were atrophied, but not weak. His gait was broad based, stiff, and he dragged both feet. There was bilateral MRC grade 4 weakness of knee flexion and MRC grade 4+ weakness of the left extensor hallucis longus muscle. Jaw jerk was absent, but deep tendon reflexes in the arms and legs were brisk with clonus at the right ankle, an absent left ankle jerk, and up going plantar responses. Pain and touch sensations were diminished over both forearms, as they were below both knees with additional impairment of vibration sense.

Initial abnormal investigations were C reactive protein 43.7 mg/l (reference range (RR) 0–8 mg/l), lactate dehydrogenase 638 μmol/l (RR 300–620 μmol/l), serum angiotensin converting enzyme (SACE) 28 U/l (RR 8–21 U/l) …

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