A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies

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.Electronic supplementary materialThe online version of this article (doi:10.1186/s40734-017-0053-3) contains supplementary material, which is available to authorized users.

Interim report on the effective intraperitoneal therapy of insulin-dependent diabetes mellitus in pet dogs using “Neo-Islets,” aggregates of adipose stem and pancreatic islet cells (INAD 012-776)

“…ELISAs for GAD65 and IA2 autoantibodies were kindly carried out by Dr. Boris Fehse, University of Hamburg, Germany, using GAD65 and IA2 ELISA kits (both from Euroimmun, Lübeck, Germany, EA 1022–9601 G and EA 1023–9601 G, respectively) and the same serum samples were analyzed for allo-antibody levels. The GAD65 kit has previously been shown to cross-react with dog antibodies [30].…”

Companion animal models of neurological disease

“…Companion animals, particularly dogs, display a broad phenotypic range of abnormal involuntary movements ( Table 2 ) ( Richter et al, 2015 ; Urkasemsin and Olby, 2014 ; Podell, 2004 ; Waln and Jankovic, 2015 ; Pancotto and Rossmeisl, 2017 ; Gilliam et al, 2014 ; Bhalerao et al, 2002 ; Quitt et al, 2018 ; Lowrie et al, 2018 ; Gill et al, 2012 ; Kolicheski et al, 2017 ; Geiger and Klopp, 2009 ; Gill et al, 2011 ). These movement disorders (MD) are classified using a scheme that parallels that of humans in which the abnormal movement is first defined as hyperkinetic or hypokinetic, followed by the frequency that the abnormal movements are observed (episodic/paroxysmal or constant/continual), and then a description of the cardinal phenotypic manifestation of the abnormal movement (dyskinesia, dystonia, myoclonus, myokymia/neuromyotonia, and myotonia) ( Richter et al, 2015 ; Urkasemsin and Olby, 2014 ; Podell, 2004 ; Waln and Jankovic, 2015 ).…”

“…Etiologies of MD in companion animals are equally diverse. Examples that recapitulate human conditions ( Table 2 ) include autoimmune disorders (stiff-dog-syndrome ( Pancotto and Rossmeisl, 2017 ), canine epileptoid cramping syndrome ( Lowrie et al, 2018 )), inherited glial ( KCNJ10 , spinocerebellar ataxia with myokymia, seizures, or both ( Gilliam et al, 2014 )) or sarcolemmal ( CLCN1 , myotonia congenita ( Bhalerao et al, 2002 ; Quitt et al, 2018 )) ion channelopathies, spinal cord serotonin deficiency (Scottish terrier cramp ( Geiger and Klopp, 2009 )), glycine transporter mutations (Startle disease ( Gill et al, 2011 )), and suspected neurodegenerative diseases of subcortical circuits and basal nuclei ( Garosi et al, 2005b ).…”

“…In dogs, the majority of gliomas appear as intra-axial tumors associated with both gray and white matter located within the telencephalon or diencephalon in close contact with the lateral ventricle. In both species, most tumors appear hypointense on T1-weighted images and hyperintense on T2-weighted images ( Pancotto and Rossmeisl, 2017 ; Gilliam et al, 2014 ). Common features include contrast-enhancement, ring-like contrast enhancement, intralesional cystic regions and hemorrhage ( Young et al, 2011 ; Rodenas et al, 2011 ; Wisner et al, 2011 ; Stadler et al, 2017 ; Bentley et al, 2013 ).…”

First report on the effective intraperitoneal therapy of insulin-dependent Diabetes mellitus in pet dogs using “Neo-Islets,” aggregates of adipose stem and pancreatic islet cells

“…ELISAs for GAD65 and IA2 autoantibodies were kindly carried out by Dr. Boris Fehse, University of Hamburg, Germany, using GAD65 and IA2 ELISA kits (both from Euroimmun, Luebeck, Germany, EA 1022-9601 G and EA 1023-9601 G, respectively) and the same serum samples were analyzed for allo-antibody levels. The GAD65 kit has previously been shown to cross-react with dog antibodies [28].…”