Myelin Disorders

• What are the three peripheral myelin diseases mentioned?

  Guillain Barre Syndrome, CIDP, Charcot-Marie-Tooth disease
• What is the cause of Guillain Barre Syndrome?

  Autoimmune response attacking peripheral nerves
• What is the incidence of Guillain Barre Syndrome?

  ~2 in 100,000
• How does Guillain Barre Syndrome typically present?

  Rapid onset muscle weakness without sensory disturbance
• What is the treatment for Guillain Barre Syndrome?

  Plasma exchange and intravenous immunoglobulin
• What is the prognosis for Guillain Barre Syndrome?

  The majority recover within a year
• How does the prognosis of Guillain Barre Syndrome differ with age?

  Worse outlook in older individuals
• What is the cause of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)?

  Immune origin with inflammation and autoimmune destruction
• What is the incidence of CIDP?

  ~1 in 100,000 in the UK
• How does CIDP progress?

  Slower progression of sensory and motor symptoms
• What is the treatment for CIDP?

  Steroids and intravenous immunoglobulin
• What is the prognosis for young adults with CIDP?

  Can fully recover if they respond to treatment
• What is the cause of Charcot-Marie-Tooth disease?

  Hereditary defects in genes for myelin and axons
• What is the incidence of Charcot-Marie-Tooth disease?

  40 in 100,000
• When does Charcot-Marie-Tooth disease typically occur?

  In the first or second decade of life
• How does Charcot-Marie-Tooth disease progress?

  Slower progression of sensory and motor symptoms
• Where do symptoms of Charcot-Marie-Tooth disease usually begin?

  In the feet and legs
• What is the treatment for Charcot-Marie-Tooth disease?

  Physiotherapy and surgery
• What is the prognosis for Charcot-Marie-Tooth disease?

  Not life-threatening but emotionally impactful
• What are the key features of Guillain Barre Syndrome, CIDP, and Charcot-Marie-Tooth disease?

  • Guillain Barre Syndrome: Autoimmune, acute, motor symptoms
  • CIDP: Immune, chronic, motor and sensory symptoms
  • Charcot-Marie-Tooth disease: Hereditary, chronic, motor and sensory symptoms
• What are the genetic abnormalities associated with central myelin disorders?

  General lesions and leukodystrophies
• What is the cause of multiple sclerosis?

  Immune mediated inflammatory demyelination
• What diagnostic methods are used for multiple sclerosis?

  MRI, lumbar puncture, neurological exams
• What are the classifications of multiple sclerosis?

  RRMS, SPMS, PPMS, PRMS
• What is relapsing remitting MS?

  Most common form with defined attacks and remissions
• What characterizes secondary progressive MS?

  Slow worsening without relapses or remissions
• What is primary progressive MS?

  Very uncommon with slow worsening from the start
• What is progressive relapsing MS?

  Rare form with slow worsening and relapses
• What happens during remyelination in MS?

  New myelin sheaths are thinner and shorter
• What is the age of onset for multiple sclerosis?

  20 - 40 years old
• How does gender affect MS incidence?

  Twice as common in females
• How does location affect MS incidence?

  Higher incidence further from the equator
• What is the effect of moving before puberty on MS risk?

  It affects your risk of MS
• What are the treatments for multiple sclerosis?

  ABC, Vitamin D, stem cell therapy
• What is the role of Vitamin D in MS treatment?

  Modulates the immune system
• What is the process of stem cell therapy for MS?

  Ablate immune system, harvest stem cells, inject
• Which glial cells myelinate axons in the peripheral and central nervous system?

  Schwann cells and oligodendrocytes
• What is the outcome of stem cell treatment?

  Rebuilds the immune system
• What are the key features of multiple sclerosis?

  • Cause: Immune and inflammatory mediated demyelination
  • Types: RRMS, SPMS, PPMS, PRMS
  • Incidence: 20-40 years old, twice as common in females
  • Diagnosis: Peripheral symptoms, MRI for lesions
  • Treatment: ABC, Vitamin D, stem cell therapy
  • Prognosis: Not fatal but progressively debilitating
• What is the name of the region between the sections of myelin?

  Node