GSS, Gerstmann Straussler Scheinker syndrome

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Clinic


Parkinsonism


Initial symptoms

  • Paralysis: , Paraplegia / Paraparesis
  • Visual disturbances/ Blindness
  • Seizures
  • Movements disorders such as Jerking, Myoclonus
  • Deafness: Less commonly, hearing loss or deafness will develop


Athetosis

  • Slow, writhing, involuntary movements

Depression

  • Hypomimia or Masked faces: Reduced degree of facial expression / Emotion
  • bradyphrenia


Dementia

  • Cognitive dysfunction leading to bradyphrenia
  • Cognition impairment such as problems in thinking, remembering, reasoning, imagining and processing thoughts which leads to Dementia[3]
  • In my research, GSS is the best remedy of Alzheimer disease, because of Memory weakness + Psychosis/ Behavioral change + Impaired Concentration + Depression


Cerebellar Ataxia

  • Nystagmus
  • Progressive Ataxia / Incoordination may present as unsteadiness, difficulty walking, and clumsiness /Awkwardness
  • Ocular dysmetria: Difficulties judging distance or scale
  • Slurred speech which progress to severe Spastic Dysarthria with UMN origin


Pyramidal signs / UMN

Corticospinal tract

Corticobulbar tract

Remedies

  1. Phos, Agar, Caust, Lach, Con
  2. Plb, Arg-n, Cocc, Calc, Sul, Stram , Nux-v
  1. Zhao MM, Feng LS, Hou S, Shen PP, Cui L, Feng JC. Gerstmann-Sträussler-Scheinker disease: A case report. World J Clin Cases. 2019 Feb 6;7(3):389-395. doi: 10.12998/wjcc.v7.i3.389. PMID: 30746381; PMCID: PMC6369391.
  2. NORD
  3. NORD Gratefully acknowledges Pawel P. Liberski, MD, PhD, Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland, for assistance in the preparation of this report.