A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies
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CASE REPORT
Open Access
A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies Theresa E. Pancotto1* and John H. Rossmeisl Jr2
Abstract Background: 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. Although entities that clinically mimic stiff-person spectrum disorders (SPSD) have manifested in horses, they have not been reported in dogs. Case presentation: We 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. Conclusion: 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. Keywords: Canine, Autoimmune, GAD antibodies, Stiff person syndrome
Background In 1956 the “stiff man” syndrome was initially described in 14 patients with progressive neck and back stiffness, and episodic and painful muscular spasms resulting in impaired ambulation [1]. As subsequent studies have demonstrated that the syndrome affects twice as many women as men, the condition is now commonly referred to as the gender neutral stiff person syndrome (SPS) [2–4]. SPS is a rare and presumably immune mediated CNS disorder, although the pathophysiology remains unclear [4, 5]. The clinical features associated with classical SPS include: rigidity of the axial muscles, especially the abdominal and paraspinal musculature, leading to hyperlordosis which can progress to affect the limb musculature; episodic painful reciprocal spasms of * Correspondence: [email protected] 1 Department of Small Animal Clinical Sciences (Pancotto, Rossmeisl), Virginia-Maryland College of Veterinary Medicine, Virginia Tech, Mail Code 0442, Blacksburg, VA 24061, USA Full list of author information is available at the end of the article
agonist and antagonist muscles triggered by stress, auditory, or tactile stimuli; electromyographic evidence of continuous motor unit activity at rest, and the presence of glutamic acid decarboxylase (anti-GAD) antibodies [6]. SPS is now recognized as a heterogeneous group of autoimmune disorders (stiff person spectrum disorder [SPSD]) that are classified based on clinical presentation into classic SPS, paraneoplastic SPS, and SPS variants (focal or segmental-SPS, jerking-SPS, SPS-plus, and progressive encephalomyelitis with rigidity and myoclonus [PE
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