{
  "abstract": "A 77-year-old female presented with a 12-month history of progressive lower limb weakness and difficulty walking, with subsequent upper limb weakness and swallowing difficulties, but without significant myalgia. She had a background of ischaemic heart disease and hypertension. Medications included atorvastatin. She was a non-smoker and did not drink alcohol.On examination cranial nerves were intact. Upper and lower limbs showed symmetrical predominantly proximal weakness, but in an overall pseudo-pyramidal distribution. Tone was normal. Reflexes were absent in the upper limbs but present in the lower limbs. She had a waddling gait and required two crutches.Cervical spine magnetic resonance imaging showed mild degenerative changes. Nerve conduction studies were normal, but electromyography demonstrated myopathic changes. Routine biochemistry/hematology, autoimmune screen, paraneoplastic screen and myositis panel were normal. CK and HMG-CoA reductase antibody (HMGCR-Ab) were markedly raised. MRI thighs showed muscle oedema. She was treated for HMGCR-Ab-associated immune-mediated necrotising myopathy (IMNM) with intravenous methylprednisolone then a prednisolone tapering regimen and mycophenolate mofetil. Atorvastatin was stopped. At four months, she had minimal residual lower limb weakness and CK had normalised.Our case highlights an unusual HMGCR-Ab IMNM presentation without myalgia. Muscle weakness distribution on clinical examination should not receive undue emphasis in isolation.laura.white19@doctors.org.uk",
  "authors": [
    {
      "affiliations": [
        "Lancaster Medical School, Lancaster University",
        "Department of Neurology, Lancashire Teaching Hospitals NHS Foundation Trust"
      ],
      "name": "White Laura M"
    },
    {
      "affiliations": [
        "Lancaster Medical School, Lancaster University",
        "Department of Neurology, Lancashire Teaching Hospitals NHS Foundation Trust"
      ],
      "name": "Maskery Mark P"
    },
    {
      "affiliations": [
        "Lancaster Medical School, Lancaster University",
        "Department of Neurology, Lancashire Teaching Hospitals NHS Foundation Trust",
        "Data Science Institute, Lancaster University"
      ],
      "name": "Emsley Hedley CA"
    }
  ],
  "title": "27 A case of HMG-Co-A reductase antibody-associated immune-mediated necrotising myopathy presenting with pseudo-pyramidal weakness",
  "uid": "733b3bde-af79-513a-8aa8-9a338e11de84"
}
