Nsp Week 1: Demyelinating Diseases (Reyna)

Front 1

Where does the spinal cord terminate in neonates, and where does it terminate in adults?

Back 1

Neonates = L3
Adults = L1

Front 2

List the arteries that supply the spinal cord. Which artery contributes the most?

Back 2

1. Anterior spinal artery <-- 2/3
2. R & L Posterior spinal arteries
3. Radicular arteries

Front 3

Which segment of the spinal cord is the most fragile, in terms of blood supply?

Back 3

T4 - T8

Front 4

Voluntary motor activity is transmitted through which spinal tract?

Back 4

Corticospinal tract

Front 5

Which portion of the spinal cord transmits touch, proprioception, and vibration sensations?

Back 5

Dorsal column (Gracilis and Cuneate tracts)

Front 6

Which spinal tract transmits pain and temperature information?

Back 6

Spinothalamic tract

Front 7

List some classic signs associated with UMN disease

Back 7

1. Weakness
2. Sensory loss
3. Spasticity
4. Brisk DTRs (deep tendon reflexes-- hyperreflexia)
5. Spastic bladder
6. Detruser-sphincter dyssynergia

Front 8

List some classic signs associated with LMN disease

Back 8

1. Weakness
2. Atrophy
3. Hypesthesia/ dysesthesia
4. Radicular pain
5. Decreased DTRs (hyporeflexia)

Front 9

List some etiologies of myelopathy

Back 9

1. Vascular (ischemia, hemorrhage)
2. Neoplastic
3. Structural or congenital
4. Myelitis (infectious, inflammatory, autoimmune, metabolic, trauma, atrophy)

Front 10

Do spinal cord infarctions preferentially involve white or gray matter?

Back 10

Gray matter

Front 11

Are spinal cord infarctions more common in the anterior or posterior column?

Back 11

Anterior column
(anterior spinal artery supplies 2/3 of spinal cord)

Front 12

Which portion of the spinal cord remains intact in motor neuron diseases?

Back 12

Posterior column

Front 13

Which portions of the spinal cord are affected by subacute combined degeneration?

Back 13

Posterior and lateral portions

Front 14

Which syndrome results from loss of sensation and motor function that is caused by the lateral hemisection of the spinal cord?

Back 14

Brown-Sequard syndrome

Front 15

Describe the ipsilateral and contralateral signs/symptoms seen in Brown-Sequard Syndrome

Back 15

(half of the spinal cord is damaged)

Ipsilateral:
1. Weakness
2. UMN signs
3. Proprioceptive deficits

Contralateral
1. Superficial sensation deficits

Front 16

Describe the signs/symptoms associated with Anterior Spinal Artery Syndrome

Back 16

Bilateral:
1. Weakness
2. UMN signs
3. Superficial sensory deficites

*Preserved proprioception
(does not affect posterior column, so Dorsal column-Medial lemniscus pathway is preserved)

Front 17

Syringomyelic syndrome typically occurs in which portion of the spinal cord?

Describe the different signs associated with upper and lower extremities

Back 17

Cervical spinal cord

Upper extremities = LMN signs (flaccid)
Lower extremities = UMN signs (spastic)

Front 18

Which syndrome is associated with "suspended" superficial sensory loss?

Back 18

Syringomyelic syndrome
(expansion of the central canal; Loss of pain/temperature sensations with preservation of light touch, proprioception and vibration)

*Usually involves upper arms and spares the legs (but can involve the legs)

Front 19

Is upper extremity or lower extremity weakness more prominent in Syringomyelic syndrome?

Back 19

Upper extremity weakness

Front 20

Flaccid arms and spastic legs may be evidence of which spinal cord syndrome?

Back 20

Syringomyelic syndrome

Front 21

What is the term for a fluid-filled, neuroglial cavity within the spinal cord or brainstem?

Back 21

Syrinx

Front 22

Describe the two general causes of central canal expansion (syrinx).

Back 22

1. Chiari malformation-- congenital. Occurs in younger people. Cerebral tonsils are pushed down (due to small/ misshapen skull), pressing against spinal canal.

2. Trauma or tumor-- usually occurs in older people

Front 23

Describe the effects of a complete cord transection on the bladder

Back 23

Initially the bladder will be FLACCID, and then it will become SPASTIC

Front 24

Autonomic dysreflexia may occur from a complete cord transection if the lesion is above which spinal nerve level?

Back 24

T6 or higher

Front 25

List some signs associated with Conus Syndromes

Back 25

1. Early sphincter signs
2. Flaccid bladder (urinary retention)
3. Flaccid bowels (constipation; megacolon)
4. Decreased anal tone
5. "Saddle anesthesia"
6. Impotence

Front 26

Give an example of an acute, acquired central myelin disease

Back 26

Acute Transverse Myelitis (ATM)

Front 27

Give an example of a chronic, acquired central myelin disease

Back 27

Multiple sclerosis

Front 28

Give an example of an acute, acquired peripheral myelin disease

Back 28

Guerin beret syndrome (GBS)

Front 29

Give an example of a chronic, acquired, peripheral myelin disease

Back 29

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Front 30

Give 2 examples of hereditary myelin diseases of the central and peripheral nervous system

Back 30

Central --> Leukodystrophies
Peripheral --> Charcot-Marie-Tooth

Front 31

Describe the spinal cord involvement in acute transverse myelitis (ATM)

Back 31

Usually large cross-sectional involvement over multiple segments. May be diffuse, involving up to whole length of the cord

Front 32

What is cause of inflammation seen in Acute Trasverse Myelitis (ATM)?

Back 32

Immune mediated

Front 33

How quick is the onset of Acute Transverse Myelitis (ATM)?

Back 33

Usually within hours

(Can also be subacute, occuring over a period of days)

Front 34

Describe the clinical presentation of Acute Transverse Myelitis (ATM).

Back 34

1. Usually monophasic
2. Often after URI (1/3 cases)
3. Paraparesis/ tetraparesis
4. Bowel/ bladder dysfunction
5. Brisk deep tendon reflexes
6. Bilateral Babinski signs
7. Sensory level

Front 35

Describe the differing etiologies of Acute Transverse Myelitis seen in children, young adults, and older adults

Back 35

Children --> post-infectious

Young adults --> Multiple sclerosis

Older adults --> Mass lesions, infection (VZV), Ischemia

Front 36

What is the general treatment for acute spinal cord injury?

Back 36

STEROIDS!

Front 37

How should a traumatic myelopathy be treated?

Back 37

Methylprednisolone (bolus IV)

Front 38

How should an acute, compressive myelopathy be treated?

Back 38

Dexamethasone IV

Front 39

How should acute transverse myelitis be treated?

Back 39

Methylprednisolone

Front 40

Is multiple sclerosis more common in males or females?

Back 40

Females > Males (4:1)

Front 41

Most cases of multiple sclerosis occur in what age group?

Back 41

15 - 45 yo

Front 42

List 4 common presenting syndromes associated with MS

Back 42

1. Visual problems (optic neuritis- blurry vision, pain w/ eye movement, difficulty differentiating colors)
2. Brain stem syndrome (vertigo)
3. Cerebellar syndrome (ataxia)
4. Transverse myelitis

Front 43

What can be found in the CSF to aid in the diagnosis of MS?

Back 43

1. Few WBCs (<50)
2. Increased protein
3. Oligoclonal bands (OCB)**
4. Elevated IgG synthesis and index**

**Specific demyelinating markers

Front 44

What can be used to check the thickness of the retinal layer in patients with MS?

Back 44

Optical Coherence Tomography (OCT)

Front 45

List the 4 classifications of Multiple Sclerosis.

Which is the most common?

Back 45

1. Relapsing/ remitting (MOST COMMON)
2. Secondary progressive
3. Primary progressive
4. Relapsing progressive

Front 46

Describe what the typical brain lesions associated with MS look like and where they are located

Back 46

Oval, hyperintensity lesions
Typical locations:
1. juxtacortical
2. periventricular
3. Around corpus callosum
4. Deep white matter.

Front 47

Optic neuritis is associated with which CNS disease?

Back 47

Multiple sclerosis

Front 48

What is Uhthoff's phenomenon and which disease is it associated with?

Back 48

Associated with MS
*Reoccurence of an old lesion (and associated symptoms) due to HEAT.

*Patients with MS are very heat sensitive

Front 49

How are acute exacerbations of MS treated?

Back 49

1. Steroids
2. IVIg
3. PLEX

Front 50

What is the first and second lines of treatment for MS?

Back 50

First line:
1. Interferons, glatiramare acetate (injectables)
2. Fingolimod (oral)

Second line:
1. Natalizumab (monoclonal Ab)
2. Cellcept, MTX (cytotoxic agents)

Front 51

What are some favorable prognostic factors for MS patients?

Back 51

1. Early visual/ sensory symptoms
2. Onset <40 yo
3. Female
4. Relapsing forms

Front 52

What are some unfavorable prognostic factors for MS patients?

Back 52

1. Early cerebellar or motor symptoms
2. Onset > 40 yo
3. Male
4. Progressive forms

Front 53

List 4 other demyelinating diseases that are associated with or similar to MS

Back 53

1. Balo concentric sclerosis
2. Marburg's disease
3. Neuromyelitis Optica (NMO)
4. Acute Demyelinating Ecephalomyelitis (ADEM)

Front 54

Detection of which antibody may be helpful in the diagnosis of Neuromyelitis Optica?

Back 54

NMO-IgG antibody- aquaporin 4

Front 55

Which disease is a demyelinating, necrotizing disease of the optic nerve and spinal cord?

Back 55

Neuromyelitis Optic (NMO)

Front 56

What is the treatment for Neuromyelitis Optica?

Back 56

1. Rituximab
2. CellCept

Front 57

Which demyelinating disease is more common in children, following a viral illness?

Back 57

Acute Demyelinating Encephalomyelitis (ADEM)

Front 58

What is the treatment for Acute Demyelinating Encephalomyelitis (ADEM)?

Back 58

Steroids
(usually 100% recovery)