Setting: University hospital-based acute rehabilitation.
Patient: 75-year-old woman with Stiff-Person Syndrome (SPS) with a recent fall and Colles fracture.
Case Description: Four months prior to admission, the patient was diagnosed with SPS, negative for anti-GAD antibodies. Diagnosis was based on a 3-year history of progressive rigidity leading to frequent falls and fractures. Anxiety and fear of falling limited her mobility, and she sustained a sacral pressure ulcer during acute hospitalization. On admission, history was remarkable for unsteady gait and muscle cramps exacerbated when startled or excited. Examination was remarkable for rigidity in her axial and limb muscles. She presented at the maximal assist level for transfers and toileting and moderate assist level for grooming and ambulation using a platform walker (right arm in cast). She was unable to tolerate titration of diazepam due to sedation, or baclofen due to hypotension.
Results: During acute rehabilitation, rigidity was treated with titration of dantrolene (from 25 to 50 mg four times daily) in addition to maximal tolerated doses of diazepam (1 mg qAM/2 mg qPM) and baclofen (20mg TID). The addition of dantrolene reduced rigidity and improved range of motion, both subjectively per patient and objectively by exam. Functional gains stalled with dose decrease and resumed with dose increase. She had pronounced gains in grooming to the supervision level, modest gains in transfers and toileting to the moderate assist level, but remained at the moderate assist level for ambulation. Progress was limited due to a change to non-weight bearing status of her right arm. Anxiety and depression were improved with buspirone, paroxetine, and psychological counseling.
Discussion: SPS results in significant activity of daily life and ambulatory dysfunction as exemplified by her pressure ulcer and multiple falls. Although GABA agonists are the preferred treatment for SPS, the adverse effects of high doses can increase the risk of falls. Dantrolene reduced muscle rigidity and improved function without sedative or hypotensive effects.
Conclusion: Dantrolene is a useful additional treatment for SPS rigidity.
Vasudevan, MD, John M.; Fetouh, MD,, S. Kamal; Ankam, MD, Nethra S.; and Schreiber, DO, MA, Adam L., "Utilization of Dantrolene in Stiff-Person Syndrome: A Case Report" (2009). Department of Rehabilitation Medicine Faculty Papers. Paper 12.