GSS, Gerstmann Straussler Scheinker syndrome: Difference between revisions

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=== Clinic ===
=== Clinic ===


* '''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 list|miasm]]
* It is a [[prion]] [[Miasm list|miasm]]
=== Sign / Symptoms ===
Common symptoms are
* Progressive ataxia
* Pyramidal signs
* Dementia
* 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'''
=== Types ===
* Four clinical phenotypes are recognized:
*# Typical GSS
*# GSS with areflexia and paresthesia
*# Pure dementia GSS
*# [[CJD, Creutzfeldt Jakob Disease|CJD]]-like GSS
=== Diseases ===
*Typicaly includes progressive '''[[Cerebellar ataxia]]''' + [[Pyramidal system|Pyramidal tract signs]] + [[Pseudobulbar palsy|Pseudobulbar paralysis]] + [[myoclonus]], + [[Hypo reflexia|Absent tendon reflexes]]. <ref>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.</ref> + '''[[Dysesthesia]]'''
*Typicaly includes progressive '''[[Cerebellar ataxia]]''' + [[Pyramidal system|Pyramidal tract signs]] + [[Pseudobulbar palsy|Pseudobulbar paralysis]] + [[myoclonus]], + [[Hypo reflexia|Absent tendon reflexes]]. <ref>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.</ref> + '''[[Dysesthesia]]'''




=== [[Parkinsonism]] ===
 
==== [[Parkinsonism]] ====
*''[[Tremor]]''
*''[[Tremor]]''
*''[[Spasticity]]: Rigid muscle tone and progressive muscle stiffness and weakness, causing abnormal, writhing movements''
*''[[Spasticity]]: Rigid muscle tone and progressive muscle stiffness and weakness, causing abnormal, writhing movements''
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=== Initial symptoms  ===
==== [[Athetosis miasms|Athetosis]] ====
* Paralysis: '', [[Paraplegia]] / [[Paraparesis]]''
* Visual disturbances/  Blindness
* '''Seizures'''
* Movements disorders such as Jerking, '''Myoclonus'''
*Deafness: Less commonly, '''hearing loss''' or deafness will develop
 
 
=== [[Athetosis miasms|Athetosis]] ===
*Slow, writhing, involuntary movements
*Slow, writhing, involuntary movements


=== Depression ===
==== Depression ====
 
* '''Hypomimia''' or '''Masked''' '''faces''': Reduced degree of facial expression / Emotion
* '''Hypomimia''' or '''Masked''' '''faces''': Reduced degree of facial expression / Emotion
* [[bradyphrenia]]  
* [[bradyphrenia]]  




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




=== [[Cerebellar ataxia miasms|Cerebellar Ataxia]] ===


==== [[Cerebellar ataxia|Cerebellar Ataxia]] ====
* [[Nystagmus]]
* [[Nystagmus]]
* Progressive [[Ataxia]] / Incoordination may present as unsteadiness, difficulty '''walking''', and '''clumsiness''' /Awkwardness
* Progressive [[Ataxia]] / Incoordination may present as unsteadiness, difficulty '''walking''', and '''clumsiness''' /Awkwardness
* '''Ocular dysmetria: Difficulties judging distance or scale'''
* '''Ocular dysmetria: Difficulties judging distance or scale'''
* Slurred speech which progress to severe Spastic [[Dysarthria]]  with [[Upper Motot Neuron|UMN]] origin
* Slurred speech which progress to severe Spastic [[Dysarthria]]  with [[Upper motor neuron, UMN|UMN]] origin






=== [[Pyramidalism|Pyramidal signs]] / [[Upper Motot Neuron|UMN]] ===
=== [[Pyramidal system|Pyramidal signs]] / [[Upper motor neuron, UMN|UMN]] ===


==== [[Corticospinal tract]]  ====
==== [[Corticospinal tract]]  ====


* [[Spasticity miasms|Spasticity]] / ''[[Spastic Paralysis]]''  
* [[Spasticity]] / ''Spastic Paralysis''
* [[Hyper reflexia|Hyper Reflexia]]
* [[Hyper reflexia|Hyper Reflexia]]


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* '''Paralysis''' of certain '''cranial nerves'''  
* '''Paralysis''' of certain '''cranial nerves'''  
* [[Pseudobulbar palsy|Pseudobulbar paralysis]]
* [[Pseudobulbar palsy|Pseudobulbar paralysis]]
** [[Dysphagia remedies|Dysphagia]]
** [[Dysphagia]]
** [[Dysarthria]] / Speech difficult
** [[Dysarthria]] / Speech difficult
** [[Pseudobulbar affect|Emotional lability]] / [[Pseudobulbar affect]]
** [[Pseudobulbar affect|Emotional lability]] / [[Pseudobulbar affect]]
** [[Hyper reflexia|Hyper Reflexia]]
** [[Hyper reflexia|Hyper Reflexia]]
** [[Spasm miasms|Spasm]]
** [[Spasm]]


=== Remedies ===
=== Remedies ===

Revision as of 03:57, 25 September 2023

Clinic

  • 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

Sign / Symptoms

Common symptoms are

  • Progressive ataxia
  • Pyramidal signs
  • Dementia
  • 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


Types

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


Diseases


Parkinsonism

  • Tremor
  • Spasticity: Rigid muscle tone and progressive muscle stiffness and weakness, causing abnormal, writhing movements
  • Bradykinesia: abnormal slowness of movement [2]
  • Myoclonus


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

  3. Phos, Agar, Caust, Lach, Con
  4. 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.
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