Abstract

We present here a case of a 59 year old woman who presented with many medical problems prompting referral and investigation under many medical specialties over several years. These included bowel motility problems, dysphagia secondary to oesophageal achalasia and cardiac problems with atypical chest pain and an abnormal ECG. She was referred to Neurology when she developed a 2 year progressive history of difficulty walking with the main problem being her inability to support her body weight and her body moving forwards towards the ground with abnormal forward flexion (camptocormia) on standing. Her sitting was not affected. On examination she had obvious camptocormia with forward stooping on walking. She had some mild 4+/5 proximal muscle weakness and wasting and mild neck flexion/extension weakness but brisk reflexes. She was investigated during an inpatient admission with a normal MRI spine and normal screening bloods. A CK was 342iu/l. An EMG showed an active myopathic process suggestive of an inflammatory myopathy or inclusion body myositis. She went onto have a quadriceps muscle biopsy which confirmed active polymyositis and she was commenced on corticosteroid therapy with steroid sparing immunosuppression. We present her e this case of a rare presentation of a rare disease with photography and a brief review of the literature.