{
  "abstract": "Stiff person syndrome is a very rare but well publicised disease. It is an autoimmune disorder often associated with diabetes mellitis type I, presenting more in females and causing significant morbidity. It can be seen in GAD, Glycine and Ampiphysin antibody positive syndromes. Patients tend to present with progressive stiffness, pain and cramping. Focal stiff person syndrome is more rare, presenting with focal limb or trunk only involvement before generalising in some but not all cases.We present a 55 year old lady of indigenous descent with a background of anti-GAD positive, latent autoimmune diabetes in adulthood (LADA), along with a longstanding sensory neuropathy. She had a 3 month history of progressive left>>>right leg stiffness with jolting pain. She had noticed anxiety and nervousness amplifying her symptoms, causing her to fall at a school outing. Her clinical exam demonstrated normal cranial nerves, upper limbs and trunk musculature. Her lower limbs had profound tone and spasticity Left>>Right, with spontaneous subtalar inversion and upgoing plantars. Her GAD antibody was strongly positive but other serology was unremarkable. Her MRI brain and whole cord was unremarkable. CSF was unremarkable. Her NCS demonstrated a length dependent sensory axonal neuropathy. Her EMG demonstrated continuous motor unit activity in agonist and antagonist muscles despite attempts to relax the muscle, as well as agonist muscles whilst activating antagonists. A diagnosis of a focal Stiff Person syndrome was made. She responded very well to low dose Baclofen.We present a rare phenotype of a rare disease.",
  "authors": [
    {
      "affiliations": [
        "Sunshine Coast University Hospital, Caloundra, QLD, Australia",
        "Griffith University, Sunshine Coast, QLD, Australia"
      ],
      "name": "Andrew Clarke"
    },
    {
      "affiliations": [
        "Sunshine Coast University Hospital, Caloundra, QLD, Australia",
        "Griffith University, Sunshine Coast, QLD, Australia"
      ],
      "name": "Antony Winkel"
    }
  ],
  "title": "3695 You’d be stiff to miss it",
  "uid": "9d7b2ca5-c627-5e0a-85f8-9c7d92cdf7eb"
}