stiff person syndrome

18 Jan

a.k.a. zebra I knew nothing about until I saw a person with it recently

Stiff-person syndrome (SPS) = an uncommon disorder characterized by progressive muscle stiffness, rigidity, and spasm involving the axial muscles, resulting in severely impaired ambulation (and worst case scenario, impairedĀ respiration)

 

found IgG antibody elevations in the cerebrospinal fluid of the majority of patients with SPS. These antibodies were found to target GABA-ergic neurons and their nerve terminals. (hence the spasms)

 

often associated with type 1 diabetes mellitus (high concentration of anti-GAD antibodies also recognizes pancreatic beta cells)

 

RARE ZEBRA. GOOD LUCK:

  • Over the past 25 years, approximately 150 cases have been reported in the medical literature
  • Women appear to be affected more often than men
  • symptoms usually begin between the third and seventh decades of life.

 

TREATMENT:

  • benzos (e.g. valium)
  • baclofen
  • immunosuppresants (e.g. prednisone, bigger guns)
  • benzos
  • benzos

 

References: uptodate.com article; picture

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