GSS, Gerstmann Straussler Scheinker syndrome
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Clinic
- It is a prion miasm
- Typicaly includes progressive Cerebellar ataxia + Pyramidal tract signs + Pseudobulbar paralysis + myoclonus, + Absent tendon reflexes. [1] + Dysesthesia
Parkinsonism
- Tremor
- Spasticity: Rigid muscle tone and progressive muscle stiffness and weakness, causing abnormal, writhing movements
- Bradykinesia: abnormal slowness of movement [2]
- Myoclonus
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
- Paralysis of certain cranial nerves
- Pseudobulbar paralysis
- Dysphagia
- Dysarthria / Speech difficult
- Emotional lability / Pseudobulbar affect
- Hyper Reflexia
- Spasm
Remedies
- ↑ 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.
- ↑ NORD
- ↑ 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.