Neurodegenerative Diseases and Myasthenia Gravis

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10 Questions

What is a typical sign of meningeal irritation associated with multiple sclerosis?

Nausea and vomiting

Which diagnostic test is superior for diagnosing multiple sclerosis?

MRI

What is the percentage of affected individuals who initially present with relapsing-remitting MS?

90

What is a characteristic of secondary progressive MS?

Progressive downhill course after each relapse

What is a treatment for subarachnoid hemorrhage?

Bed rest

What is a potential environmental factor in the development of multiple sclerosis?

Exposure to bacteria and viruses

What is a typical feature of relapsing-remitting MS?

Unpredictable recurrent episodes of neurological dysfunction

What is a characteristic of demyelinating diseases?

Damage to the myelin sheath

What is the name of the virus that has been linked to the development of multiple sclerosis?

All of the above

What is the goal of early repair after a sentinel hemorrhage?

To prevent further hemorrhage

Study Notes

Degenerative Diseases

  • Characterized by progressive neuronal death, multifactorial
  • Examples include multiple sclerosis and myasthenia gravis

Myasthenia Gravis

  • Autoimmune-mediated neuromuscular disorder
  • More common in women, incidence peaks in 20s and 30s
  • Etiology unknown, but genetic factors play a role
  • Most patients demonstrate antibodies to acetylcholine receptor
  • Seronegative patients often have antibodies to muscle-specific tyrosine kinase (MuSK)
  • Cardinal features: weakness, easy fatigability, preserved DTR
  • Cranial muscles: diplopia and ptosis

Multiple Sclerosis

  • Characterized by immune-mediated focal or patchy destruction of myelin sheaths
  • Inflammatory response and demyelination
  • Classic findings: sensory loss, visual symptoms, weakness
  • Genetic and environmental factors, including exposure to bacteria and viruses
  • 4 Clinical Types: relapsing-remitting, secondary progressive, primary progressive, and progressive-relapsing
  • Relapsing-remitting MS: 90% of affected individuals, unpredictable recurrent episodes of neurological dysfunction
  • Secondary progressive MS: relapsing disease that begins to pursue a progressive downhill course after each relapse

Arteriovenous Malformations

  • Rare in pregnancy
  • Congenital focal abnormal conglomerations of dilated arteries and veins
  • Lack capillaries and have arteriovenous shunting
  • When AVMs bleed, half do so into the subarachnoid space, half are intraparenchymal with subarachnoid extension
  • Mode of delivery: cesarean delivery, high risk of recurrent hemorrhage from an unresected or inoperable lesion

Diagnosis and Management

  • MRI: superior diagnostic test for demyelinating diseases
  • Cerebrospinal fluid analysis: characteristic multifocal white-matter plaques
  • Treatment of subarachnoid hemorrhage: bed rest, analgesia, neurological monitoring, and strict blood pressure control
  • Early repair after the sentinel hemorrhage is recommended

This quiz covers the characteristics and diagnosis of neurodegenerative diseases, including progressive neuronal death, and Myasthenia Gravis, an autoimmune-mediated neuromuscular disorder.

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