1. Subcutaneous immunoglobulin for maintenance therapy in stiff-person syndrome: One-year follow-up in two patients
- Author
-
G.M. Minicuci, Vincenzo Donadio, Roberto D'Angelo, Rita Rinaldi, E. Fileccia, Rocco Liguori, L. Bartolomei, Fileccia E., Rinaldi R., Minicuci G.M., D'Angelo R., Bartolomei L., Liguori R., and Donadio V.
- Subjects
0301 basic medicine ,Spasm ,medicine.medical_specialty ,Injections, Subcutaneous ,Modified Ashworth scale ,Azathioprine ,Subcutaneous immunoglobulin ,Stiff-Person Syndrome ,03 medical and health sciences ,0302 clinical medicine ,Maintenance therapy ,medicine ,Humans ,Immunologic Factors ,Intravenous immunoglobulin ,Genetics (clinical) ,biology ,business.industry ,Immunoglobulins, Intravenous ,Stiff persons syndrome ,Middle Aged ,medicine.disease ,Surgery ,Immuno-modulation ,body regions ,030104 developmental biology ,Neurology ,Pediatrics, Perinatology and Child Health ,Cohort ,biology.protein ,Female ,Rituximab ,Immunotherapy ,Neurology (clinical) ,Antibody ,business ,030217 neurology & neurosurgery ,Stiff person syndrome ,Follow-Up Studies ,medicine.drug - Abstract
Stiff person syndrome is a rare condition characterised by prolonged stiffness with superimposed muscle spasms. Immunotherapy relies mainly on intravenous immunoglobulin, steroids and plasma exchange. Azathioprine or rituximab are other possible options. We describe two patients who showed a good clinical response with intravenous immunoglobulin and persistence of the clinical improvement after shifting to equivalent dosage of subcutaneous immunoglobulin. Both patients received a diagnosis of stiff person syndrome based on their clinical symptoms (episodes of stiffness and spasms) and presence of antiglutamic acid decarboxylase. Treatment with intravenous immunoglobulin was started with improvement of symptoms as reported by patients and confirmed also by the spasm frequency scale and modified Ashworth scale. After clinical stabilisation in order to avoid the hospitalisation required for intravenous immunoglobulin treatment a switch to subcutaneous immunoglobulins was made. After one year of follow-up from the switch, the patients show clinical stability. Their scores on the modified Ashworth scale, spasm frequency scale and on the 10 Meter Walking Test were also stable. Subcutaneous formulation of immunoglobulin could be as effective as intravenous immunoglobulin in the maintenance treatment of Stiff person syndrome, although studies involving a larger cohort of patients are needed in order to confirm our anecdotal experience.
- Published
- 2020
- Full Text
- View/download PDF