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Progressive encephalomyelitis with rigidity associated with anti-amphiphysin antibodies
  1. A Ishii1,
  2. A Hayashi2,
  3. N Ohkoshi3,
  4. S Matsuno3,
  5. S Shoji3
  1. 1Department of Neurology, Tsukuba Soai Hospital, Department of Neurology, University of Tsukuba, Tsukuba, Japan
  2. 2Department of Neurology and Rehabilitation, Juntendo University, Tokyo, Japan
  3. 3Department of Neurology, University of Tsukuba, Tsukuba, Japan
  1. Correspondence to:
 A Hayashi
 Clinical Pathology, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan; hayashimed.juntendo.ac.jp

https://doi.org/10.1136/jnnp.2003.010504

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    Progressive encephalomyelitis with rigidity (PER) is a rare disorder of unknown aetiology, characterised by muscular rigidity, abnormal postures, painful muscle spasms, and myoclonus, and is caused by inflammation in the brainstem or spinal cord.1,2 We report a case of PER with positive anti-amphiphysin antibodies in the serum and CSF.3 This association has not been previously reported and raises the possibility that PER may have an autoimmune pathogenesis similar to that of stiff person syndrome (SPS).4

    Case report

    Clinical features

    A 37 year old female presented having had symptoms of PER for about three months. Spasms began with several minutes of paroxysmal painful muscle stiffness in the left upper limb, followed by pain and muscle spasms in the upper limbs, shoulders, neck, and back. These spasms were easily evoked by light touches, conversations, and by being startled. The patient remained bedridden and showed left dominant weakness of the limbs, with contractures in the upper limbs and difficulty in relaxing the muscles. She also developed abducent nerve palsy. Her deep tendon reflexes were absent and her plantar responses were both flexor. The serum antinuclear antibody was positive (1:160); …

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