Abstract
BackgroundThe stiff person syndrome (SPS) is a rare and debilitating autoimmune disorder with an unknown pathogenesis and variable clinical presentation that can present a diagnostic challenge. Although entities that clinically mimic stiff-person spectrum disorders (SPSD) have manifested in horses, they have not been reported in dogs.Case presentationWe describe a 2-year-old beagle dog presented for progressive attacks of muscular rigidity and lordosis with superimposed spasms of the appendicular muscles triggered by tactile stimulation which resulted in marked gait impairment. Resting electromyography revealed continuous motor unit activity in the axial musculature. Compared to age-matched healthy beagle dogs, this patient had elevated glutamic acid decarboxylase antibody concentrations in serum and cerebrospinal fluid.ConclusionThis dog presented with phenotypic, electrodiagnostic, and immunologic criterion consistent with an SPSD, including elevated anti-GAD antibody titers, which we have termed the “stiff dog syndrome (SDS)”. Durable clinical improvement was achieved with symptomatic and immunosuppressive treatments including baclofen, gabapentin, prednisone, and intravenous immunoglobulin.
Highlights
The stiff person syndrome (SPS) is a rare and debilitating autoimmune disorder with an unknown pathogenesis and variable clinical presentation that can present a diagnostic challenge
This case illustrates the existence of a condition, which we refer to as the “stiff dog syndrome (SDS)”, similar to SPS in humans, and emphasizes the heterogeneous clinical manifestations of spectrum disorders (SPSD) variants and the diagnostic challenges associated with this condition
Cerebrospinal fluid was collected from the cerebellomedullary cistern and was grossly, cytologically, and biochemically unremarkable (WBC 1 cell/uL; RBC 0 cell/uL; protein 24.9 mg/dL); though the IgG index was elevated and anti-GAD antibodies were observed in CSF and serial serum samples obtained from the patient (Table 1)
Summary
This dog presented with phenotypic, electrodiagnostic, and immunologic criterion consistent with an SPSD, including elevated anti-GAD antibody titers, which we have termed the “stiff dog syndrome (SDS)”. Durable clinical improvement was achieved with symptomatic and immunosuppressive treatments including baclofen, gabapentin, prednisone, and intravenous immunoglobulin
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