GSS, Gerstmann Straussler Scheinker syndrome

GSS is an extremely rare, fatal neurodegenerative disease
It also classified with the transmissible spongiform encephalopathies (TSE) due to the causative role of prion protein.
It is a prion miasm


Entities	Sign / Symptoms	Diseases
Dysarthria		Pseudobulbar paralysis
Emotional lability / Pseudobulbar affect
Hyper Reflexia
Spasm
	Absent tendon reflexes. ^[1]
Myoclonus		Parkinsonism Tremor Spasticity: Rigid muscle tone and progressive muscle stiffness and weakness, causing abnormal, writhing movements Bradykinesia: abnormal slowness of movement ^[2] Myoclonus
Spasticity
Ext, Tremor
Athetosis	Slow, writhing, involuntary movements
Depression	Hypomimia or Masked faces: Reduced degree of facial expression / Emotion	Depression
Bradyphrenia		Depression
Cognition impairment such as problems in thinking, remembering, reasoning, imagining and processing thoughts.^[3] leading to bradyphrenia
Nystagmus		Cerebellar Ataxia
Spastic Dysarthria UMN origin
Extremities Incoordination
Ataxia	Progressive Ataxia / Incoordination may present as unsteadiness, difficulty walking, and clumsiness /Awkwardness
Ocular dysmetria	Difficulties judging distance or scale

Common symptoms are

Pyramidal signs
Symptoms start with Slowly developing dysarthria and cerebellar truncal ataxia and then to progressive dementia
In the early stages people exhibit clumsiness and walking difficulties, then ataxia comes
Loss of memory can be the first symptom of GSS.
Extrapyramidal and pyramidal symptoms may mimic spinocerebellar ataxia
Myoclonus is less frequently seen than in CJD
Many patients also exhibit nystagmus, visual disturbances, and even blindness or deafness.
Paralysis: Paraplegia / Paraparesis
Seizures

Four clinical phenotypes are recognized:
1. Typical GSS
2. GSS with areflexia and paresthesia
3. Pure dementia GSS
4. CJD-like GSS

Typicaly includes progressive Cerebellar ataxia + Pyramidal tract signs + + , + + Dysesthesia

In my research, GSS is the best remedy of Alzheimer disease, because of Memory weakness + Psychosis/ Behavioral change + Impaired Concentration + Depression

↑ 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.

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