STIFF PERSON SYNDROME WITH ATYPICAL FEATURES AND A FAVOURABLE OUTCOME WITH STEROIDS

Türker H., Cengiz N., Gungor L., Onar M.

MARMARA MEDICAL JOURNAL, cilt.18, sa.1, ss.24-27, 2005 (ESCI) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 18 Sayı: 1
  • Basım Tarihi: 2005
  • Dergi Adı: MARMARA MEDICAL JOURNAL
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus
  • Sayfa Sayıları: ss.24-27
  • Anahtar Kelimeler: Stiff person syndrome, Stiff man syndrome, Stiff person syndrome and steroids
  • Ondokuz Mayıs Üniversitesi Adresli: Evet

Özet

Stiff Person Syndrome (SPS) is a rare and disabling disorder characterised by continuous motor unit activity causing severe rigidity and episodic spasms in axial and limb muscles. It deteriorates the quality of life and causes a serious burden in the patient's life. Here we describe a male patient with stiff person syndrome having atypical features like pyramidal signs and generalised convulsions. Benzodiazepines, baclofen and IVIG did not alter the clinical status whereas oral methylprednisone had a dramatic effect and resulted in a favourable outcome.