Demyelinating

Question 1

A 30 year-old female presented with with double vision.

Answer 1

Multiple sclersois - Dawson’s fingers

Question 2

A 30 year-old female presented with with double vision.

Answer 2

Multiple sclerosis - Juxtacortical lesions

Question 3

A 30 year-old female presented with with double vision.

Answer 3

Multiple sclerosis - infratentorial lesions

Question 4

A 30 year-old female presented with with double vision.

Answer 4

Multiple sclerosis - lesions in corpus callosum

Question 5

A 50 year-old man developed personality changes and subtle left-sided weakness.

Answer 5

Multiple sclersois - Tumefactive lesion

• Clinical presentation is atypical for MS:
  
  • Aphasia
  • Visual field deficits
  • Cognitive and personality changes
  • Neglect syndrome
  • Weakness and sensory deficits
• Biopsy is often taken for correct diagnosis

Question 6

A 24 year-old man presented with left arm weakness.

Answer 6

Multiple sclerosis - Balos

Question 7

A 34 year-old patient presented with double vision and left arm weakness.

Answer 7

Multiple sclerosis - incomplete ring-enhancing lesions

Question 8

A 29 year-old woman presented with blurry vision and pain in her left eye for 3 days.

Answer 8

Multiple sclerosis - left optic neuritis

• Other conditions with optic neuritis:
  
  • ​ADEM (acute disseminated encephalomyelities)
  • SLE, Sjogren’s, Bechet’s
  • Sarcoidosis
• ​Treatment
  
  • ​3 days of IV solumedrol

Question 9

A 45 year-old female with cognitive impairment, ataxia.

Answer 9

Multiple sclerosis - black holes

• Older lesions appear hypointense on T1WI

Question 10

A 34 year-old woman presented with several days of severe, progressive blindness.

Answer 10

Neuromyelitis optica (NMO)

• Bilateral inflammation of optic nerves, chiams, and longitudinally extensive myelitis
• Criteria for diagnosis
  
  • Optic neuritis + myelitis
  • 2 of 3 supportive criteria
    
    • MRI evidence of spinal cord involvement
    • MRI of brain nondiagnostic of MS
    • NMO-IgG positive
• Epidemiology
  
  • Women > men
  • Africans > caucasians
• Treatment
  
  • IV steroids or plasmapheresis
  • MS treatment WORSENS NMO

Question 11

A 45 year-old male with MS developed cognitive slowing and abulia.

Answer 11

Progressive multifocal leukoencephalopathy (PML)

• “Milky-way” pattern on imaging
• Medications that can cause PML:
  
  • Natalizumab, fingolimod, dimethyl fumarate

Question 12

An 8 year-old child presented with encephalopathy and seizures.

Answer 12

Acute disseminated encephalomyelitis (ADEM)

• Pathogenesis
  
  • Central immune-mediated demyelinating inflammatory disorder affecting white matter of brain and spinal cord
  • Occurs during or after systemic illness or vaccination
    
    • ​Measles infection is highest risk
  • Typically found in children
• Signs and symptoms
  
  • Headache, lethargy, myalgia, malaise, fever, vomiting
  • Encephalopathy for 3-4 weeks
    
    • Seizures, neuropathies, weakness, hemiparesis, sensory deficits, ataxia, aphasia, paresthesias, altered mentals tatus
• Diagnosis
  
  • Perivascular inflammation in cerebral hemispheres, brainstem, cerebellum, spinal cord, optic nerves
  • CSF is normal
• Treatment
  
  • High dose IV steroids
  • Then oral steroids + IVIG