GSS, Gerstmann Straussler Scheinker syndrome

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

• Typicaly includes progressive Cerebellar ataxia + Pyramidal tract signs + Pseudobulbar paralysis + myoclonus, + Absent tendon reflexes. + Dysesthesia

Parkinsonism

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

• Spasticity / Spastic Paralysis
• Hyper Reflexia

Corticobulbar tract

• Paralysis of certain cranial nerves
• Pseudobulbar paralysis
  • Dysphagia
  • Dysarthria / Speech difficult
  • Emotional lability / Pseudobulbar affect
  • Hyper Reflexia
  • Spasm

Remedies

3. Phos, Agar, Caust, Lach, Con
4. Plb, Arg-n, Cocc, Calc, Sul, Stram , Nux-v