A common symptom is a progressive loss of coordination that may present as unsteadiness of gait, difficulty walking, and clumsiness. [rarediseases.org]

Stanley Prusiner, Cold Spring Harbor Laboratory Press: 2004, pp. 673-775 Research study by Julie Moreno, Ph.D., Colorado State University, "The Role of Cellular Chaperones and the Unfolded Protein Response in GSS Syndrome," presented at the CJD Foundation [cjdfoundation.org]

• Pain

  Neurologists, psychiatrists, psychologists, pain specialists, social workers and other healthcare professionals may need to systematically and comprehensively plan treatment. Psychosocial support for the entire family is essential as well. [rarediseases.org]

• Movement Disorder

  Additional symptoms that sometimes occur include seizures, paralysis (palsy) of certain cranial nerves, and movements disorders like jerky, muscle spasms (myoclonus) or slow, writhing, involuntary movements (athetosis). [rarediseases.org]

• Nystagmus

  May also include: dysarthria (difficulty speaking), nystagmus (movement of the eyes), hearing problems, spasticity, visual disturbances including double vision, and dementia. [cjdfoundation.org]

  Some affected individuals develop rapid, involuntary eye movements (nystagmus) and have difficulties judging distance or scale (ocular dysmetria), and problems with vision. Sometimes, eye problems can progress to blindness. [rarediseases.org]

• Dysarthria

  May also include: dysarthria (difficulty speaking), nystagmus (movement of the eyes), hearing problems, spasticity, visual disturbances including double vision, and dementia. [cjdfoundation.org]

  Signs & Symptoms The initial symptoms associated with Gerstmann-Sträussler-Scheinker disease are progressive loss of coordination and mild difficulty with speech (dysarthria). [rarediseases.org]

• Encephalopathy

  […] typeAmyloidosis cerebral with spongiform encephalopathy; Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system; Encephalopathy subacute spongiform Gerstmann-Straussler type; GSSD; Gerstmann Straussler Scheinker syndrome [rarediseases.info.nih.gov]

  Introduction GSS is classified as a transmissible spongiform encephalopathy (TSE) or a prion disease. Prion diseases are caused by the accumulation of misfolded prion protein in the brain. [rarediseases.org]

• Cerebellar Ataxia

  National Center for Advancing Translational Sciences Home Browse by Disease Gerstmann-Straussler-Scheinker Disease Gerstmann-Straussler-Scheinker Disease Other Names: Amyloidosis cerebral with spongiform encephalopathy; Cerebellar ataxia, progressive [rarediseases.info.nih.gov]

• Clumsiness

  A common symptom is a progressive loss of coordination that may present as unsteadiness of gait, difficulty walking, and clumsiness. [rarediseases.org]

Clinical Testing and Workup Molecular genetic testing can confirm a diagnosis. [rarediseases.org]

Standard Therapies Treatment The treatment of GSS is directed toward the management of specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

[…] system; Encephalopathy subacute spongiform Gerstmann-Straussler type; GSSD; Gerstmann Straussler Scheinker syndrome; Gerstmann-Straussler-Scheinker syndrome; Subacute spongiform encephalopathy, Gerstmann-Straussler type About the Disease Diagnosis & Treatment [rarediseases.info.nih.gov]