What is the most common form of MS? Describe.

'relapsing remitting'

patient develops periodic loss of neurological function

In multiple sclerosis, when a patient's symptoms worsen with an increase in body temperature this is called Uhthoff's phenomenon.

due to poor electrical conduction along demyelinated axons

a new neurologic disability that lasts > 24 hrs...

can be recurrence of an OLD symptom separated by a YEAR or so....

or a new symptom

both involve onset of a new neurologic disability...

MS relapse onset = SUBACUTE

stroke = ACUTE

a disease that is separated by time & space

time = 2 relapses at diff pds in a person's life

space = 2 different areas of the CNS (ex. optic neuritis + transverse myelitis)

(inflammatory)

protein may be normal

a) IgG index ELEVATED

b) oligoclonal bands

c) Myelin Basic Protein ELEVATED

d) also, MAY see elevated lymphocytes but never neutrophils

perivascular cuff (monoctyes within a blood vessel)

T cells

B cells

Macrophages

microglia

(but NO neutrophils)

hyper-intense (very white) areas represent MS plaques. they are usually periventricular, ovoid within the deep white matter. (can also be in spinal cord)

can inject gadolinium - doesn't normally enter brain parenchyma due to BBB but with MS, this has broken down & it can be visualized with MRI

- cause is uncertain but may scientists belive that a virus acts as a trigger in genetically-susceptible people --> autoimmune response

- TH1s are activated, stimulate Bcells & macrophages --> antibodies and demyelinating damage

- TH2s exert anti-inflamm effects to regulate the immune response

a) relapsing remitting MS (RRMS) - exacerbations followed by complete or incomplete recovery... often with slow, inconsistent accumulatio of disability

b) primary progressive MS - no recovery stages... often present with spasticity, weakness, sensory disturbance, urinary symptoms...difficult to treat

c) secondary progressive MS - pt started wout with RRMS then at one point develops progressive disease

1. Optic neuritis - aches behind eye, pain on eye mvmt... retina usually looks normal... may have central vision loss

2. Myelitis - more often sensory, band-like pressure around torso... + Lhermitte's sign (on neck flexion, pt feels electrical signal down back to legs & fingers)... bladder, bowel symptoms... acute dystonias (limbs move rhythmically)

3. Brainstem - nystagmus, trigem neuralgia, Bell's palsy...

4. Cerebellum - ataxia, tremor, eye mvmt abnormalities

In MS patients...

If your pt has a plaque in the cervical spinal cord,

nexk flexion --> electrical signal will radiate down pt's back to legs & fingers

SUBACUTE onset (between acute - stroke-like -  and chronic - Parkinson's-like)

symptoms usually last for hours or days

best for evaluating the progression of symptoms in an MS patient

(gadolinium does not normally pass BBB into brain parenchyma, therefore presence of gadolinium in parenchyma suggests level of BBB breakdown)

1. Clinical signs & symptoms

2. MRI (possibly with gadolinium)

3. Spinal Tap

4. Evoked Potentials (visual etc)

5. Labs (CBC, CMP, ESR, RPR, cholesterol profile, B12, ANA, ACE, lyme)

IgGs of similar size.

They ARE normally found in serum.

They ARE NOT usually found in CSF.

If found in your pt's CSF, they are indicative but not specific for MS... could also be lyme disease, syphillis, or lupus...

Test for demyelination along nerve fibers conducting visual images to the occipital cortex (test for MS)

- light flashed in front of pt

- watch for the P100 (the wave that results from the light, recorded from the occipital cortex)

- normal P100 = 100 msec

- if the optic nerve is demyelinated, P100 will be delayed or completely absent

What is a 'somatosensory evoked response'?

What is a 'brainstem evoked response'?

both used to measure demyelination (slower conduction along nerves)

somatosensory -- time it takes for stimulus to travel from finger or toe to sensory cortex

brainstem -- how long it takes for a click to be heard from hear to the auditory cortex

If the patient has OPTIC NEURITIS (as many MS patients do.. this is accompanied by unilateral vision loss & pain on eye mvmt...),

then you will likely see an APD (Afferent Pupillary Defect) on the affected side. This is due to demyelination of the Optic Nerve on that side.

(ex. L RAPD = demyel. of L Optic Nerve)

Can be used to diagnose a patient at his or her FIRST ATTACK (bc MRI can show plaques, suggesting that there was at least one more attack at another point in time... therefore this current presentation is in fact a RELAPSE.. and 2 lesions in 2 different parts of the CNS = sufficient to satisfy "time and space" requirements for MS Dx)

(Traditionally, at first attack the clinician would wait until the pt's 2nd attack to diagnose MS. Now MRI speeds up the diagnosis process.)

only ONE!

(having 1-3,4-10, or >10 lesions puts you at 90% risk of developing MS)

Internuclear Opthalmoplegia...

cause?

clinical presentation?

Often seen in pts with what disease?

cause = demyelination or damage to the MLF (Median Longitudinal Fasciculus) which connects CN III on one side to CN VI on the other side (for example, so that R eye aDducts when L eye aBducts to look left)

clinical presentation = failure of aDuction of one or both eyes (ex. looking Left, L eye aBducts just fine, but R eye fails to fully aDduct. This results in nystagmus of the L eye) The lesion is the MLF on the side of the aDduction deficit. (in this example, it would be the R MLF)

often seen in pts with = MULTIPLE SCLEROSIS

- optic neuritis

- INO (internuclear ophthalmoplegia)

- nystagmus

- partial myelitis

- cerebellar syndromes

- vision

- sexual dysfunction or incontinence

5 areas of the spinal cord (that we need to know...)

type of information carried in each area?

where is each located within the cord?

1. Corticospinal tract - UMNs (lateral cord)

2. Posterior columns - vibration, postion sense (dorsal cord)

3. Spinothalamic tract - pain, temp (anterolateral cord)

4. Anterior Horn Cells - LMNs (anterior horn of the gray area)

5. Roots - axons of LMNs, sensory info

Pt presents with.... lesion is where in spinal cord?

hyper-reflexia

spasticity

+Babinski sign

weakness

Pt presents with.... lesion is where in spinal cord?

flaccid weakness

hypo-reflexia

fasciculations

Pt presents with.... lesion is where in spinal cord?

+ Rhomberg sign

loss of vibration sense

Pt presents with.... lesion is where in spinal cord?

loss of pain & temperature sense

Pt presents with.... lesion is where in spinal cord?

stabbing pain

numbness

hypo-reflexia

Root

(stabbing pain = "lancinating")

1. spinal cord parenchyma

2. pia matter

3. subarachnoid space

(arachnoid)

4. subdural space

5. dura

6. epidural space (contains fat)

7. bone (vertebral body)

What does "leptomeningeal" mean?

within the subarachnoid space

(could be leptomeningeal disease)

Within the spinal cord parenchyma

(could be intramedullary disease)

cord = gray or black

CSF = white

Cannot feel sensation at or below a certain level

ex below T4 (nipple)

or

below T10 (umbilicus)

Bladder control is primarily...

and deficits may be seen in...

CNS-controlled (brain & spinal cord)

deficits in bladder control are seen in spinal cord lesions (esp because the nerves are so long - axons begin in frontal lobes of brain to synapse in the thoracic & sacral areas of the spinal cord!)

1. acute lesions - urinary retention (bladder doesn't empty completely) with some overflow incontinence

2. chronic lesions - small spastic bladder that doesn't empty completely --> spasms (even at low urine volume) & urge incontinence

a hemi-cord syndrome (in pure form, HALF - R or L - of the spinal cord is knocked out)

Hemisection of the cord damages...

1. UMN of Corticospinal tract

2. Dorsal Column

3. Spinothalamic tract

So presentation is... (all below level of lesion)

1. IPSI spastic paralysis

2. IPSI loss of vibration, proprioception sense

3. CONTRA loss of pain & temp sense

flaccid paraplegia (complete paralysis of lower half of body) with a complete sensory level to all modalities

(indicative of full-blown spinal cord compression)

A. early

- Motor - hyperreflexia, spasticity, Babinksi, trouble walking

- Sensory - root irritation --> IPSILAT allodynia

- Spinothalamic tract - subtle at this point... CONTRALAT lower extrem loss of pain, temp

- Urinary urgency is common

B. middle

- WEAKNESS of LEGS

- Definite loss of bladder & potentially bowel control

- may have Partial Brown Sequard syndrome

C. late (full-blown compression)

- if acute --> spinal cord shock (flaccid paraplegia with complete sensory level to all modalities)

- if chronic --> spastic paraparesis (condition of spasticity AND weakness of extrems), definite sensory level

(extradural = epidural)

1. disc disease

2. metastatic tumor

3. abscess

1. Neurinoma

2. Meningioma

1. syringomyelia

2. glioma

3. myelitis

Methylprednisolone

1. Lung

2. Breast

3. Prostate

4. Kidney

5. Thyroid

lbp-kt

If you are clinically suspicious of a spinal cord compression,

1. Tx with steroids (Dexamethasone)

2. Arrange a spinal MRI with gadolinium

SPINAL TAP.

(bc cancer cells can still enter subarach space, cause a meningitis & spinal cord injury)

mets --> begin in VERTEBRAL BODY

spinal abscesses --> begin in DISC SPACE (then eventually expands to cause cord compression)

How can an infection in the spinal column cause cord compression?

Most common infectious agent?

Which pts particularly susceptible?

infection --> ABSCESS FORMATION

Staph aureus

- esp in IVDA pts

1. FEVER

2. pain on percussion

3. elevated white count

4. elevated ESR

1. IVDA

2. HIV

3. immunosuppression

infection of the bone or bone marrow

(can be seen in spine)

on MRI - irregular vertebral body end plates, can --> compression of cord

neurologic syndrome of osteomyelitis resulting from TB infection of the vertebrae

(often causes marked kyphosis - hunchback)

Spinal meningioma -

most common location?

typical demographic?

thoracic spine, intradural extramedullary

middle-aged women

What is the most common intradural extramedullary mass?

How to diagnose?

Nerve sheath tumors - schwannomas, neurofibromas

Dx - imaging shows "dumbbell" shape low density (dark) mass

in (intramedullary) central cord lesions...

spinothalamic tracts are affected first because they CROSS in the center of the spinal cord.

However, the SACRAL area pain & temp sensation may be spared since these are represented most LATERALLY in the spinal cord.

A. Early

- loss of pain & temp (often across the torso)

B. Mid

- spreads to arms

- may have Horner's

- Dissociated Sensory Level (dec pain, temp sensation BUT vibration, position sense are PRESERVED until late)

- below lesion, spastic paraparesis, hyperreflexia, Babinskis

C. Late

- everything's worse but may be SACRAL SPARING (retains pain, temp bc sacral fibers so lateral)

(syringomyelia)

a large expanding space in the spinal cord

can be result of trauma or tumor

3 examples of how the vascular supply can affect the spinal cord

1. aneurysm

2. AVM

3. infarct

ANTERIOR 2-3rds of cord = Artery of Adamkiewicz (aka great radicular artery)

POSTERIOR 1-3rd = Posterior Spinal Artery

SO infarction etc of the Artery of Adamkiewicz will show...

a) spinothalamic (loss of pain, temp + sensory level)

b) corticospinal (weakness)

c) but INTACT POSTERIOR COLUMN FUNCTION (vibration, joint position sense) - bc supplied by the Posterior artery

gray circles within vertebral bodies

(= lumens of vessels involved in the AVM)

acute neurologic condition that reflects focal inflammation of the spinal cord

-acute or subacutely developing motor, sensory, & sphincter dysfunction

- spinal segmental level of sensory disturbance with a well-defined UPPER limit

- MRI shows no compression

- can be the first sign of MS

Tx = IV steroids

symptoms...

Symptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficits. Severe backpain may occur in some patients at the onset of the disease. Thesymptoms and signs depend upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis and sensory loss below the level of the lesion. In other cases, such loss is only partial.

Get NMO titers - serum OR CSF

(Ag = aquaporin 4)

NEUTROPHILS may be present (plus lymphos)

- NO oligoclonal bands (bc SO hyperacute)

What is 'NMO'? Define.

Neuromyelitis Optica

Optic Neuritis (usually bilateral)

+

Spinal cord involvement of 3 or more levels (Transverse Myelitis)

ACUTE - "S-FLAC"

1. spinal shock

2. flaccid paralysis

3. complete sensory loss, all modalities

4. loss of bladder, bowel, sexual function

5. autonomic malfunction

CHRONIC  - all of above WITHOUT shock, WITH spastic paralysis

'Subacute combined degeneration'

- caused by?

- Describe.

- caused by B12 deficiency - pernicious anemia

- involves BOTH sides of spinal cord & 2 tracts...

1. corticospinal

2. posterior column

- (Not a lesion or compression - a myelopathy from within the spinal cord)

Presentation - all BILATERAL

1. spastic weakness of lower extrems

2. dec sensation to vibration & position, +Rhomberg

ataxia

Babinksis

hyperreflexia

may also cause peripheral neuropathy, dementia

Tx - B12 injections

human T-lymphotropic virus type 1

infection with this virus --> spastic paraparesis & spinal cord disease

causes "HAM/TSP" = HTLV-1-associated myelopathy/tropical spastic paraparesis

corticospinal tract > posterior columns

+

CSF shows oligoclonal bands & inc IgG (like MS)

Test for syphillis

Patient may be experiencing tabes dorsalis - loss of position & vibration below a certain level with Rhomberg sign... could be result of a complication of syphillis

(ALS = Lou Gherig's Disease)

1. anterior horn cells (LMNs)

+

2. corticospinal tracts (UMNs)

bilaterally...

causes flaccid + spastic weakness,

no sensory signs