Lecture 7 - Demyelination Ataxia

Question 1

What is ADEM (Acute disseminated encephalomyelitis)

Answer 1

Acute demyelinating illness that commonly follows an infection or vaccine (75%)

rare

MRI shows BILATERAL symmetric inflammation of the same age (meaning it started at the same time)

Question 2

ADEM is most common in what patients

Answer 2

Children

Question 3

how does ADEM look on an MRI?

Answer 3

big fluffy areas of inflammation on both sides

Question 4

What does “sensory level noted at T4” mean?

Answer 4

Sensory issues below T4

Question 5

What is Acute Transverse Myelitis?

Answer 5

Inflammation of spinal cord causing lesions

Can be first episode of multiple sclerosis (MS), espeicially if brain MRI is also abnromal

Question 6

How will acute Transverse myelitis appear on an MRI?

Answer 6

Small patchy lesions limited to two areas different vertebral segments

Question 7

How will Acute Transverse myelitis present

Answer 7

Back pain, sensory level problems at spine (example: everything under T4 doesnt work), sphincter disturbances (bowel and bladder), paraparesis

Question 8

How will optic neuritis present?

Answer 8

Pain w/ eye movement
pale and inflamed optic disc

vision loss, loss of color vision

Consensual pupillary constriction no longer works when shining light into that eye, nor does the right eye dilate when you shine light into the right eye itself

however, the L can consensually constrict the R eye still

Question 9

Optic neuritis is a common initial symptom of ________________

Answer 9

MS

w/ abnormal brain MRI, 56% had MS in 10 years

w/ normal brain MRI, 22% had MS in 10 years

Question 10

What factors increase the risk for MS?

Answer 10

Younger age, female, previous neuro symptoms, multiple MRI lesions

Question 11

What is the normal treatment for optic neuritis?

Answer 11

IV steroids

Question 12

Optic neuritis on its own is usually ___________

Answer 12

monophasic

but not if its related to MS

Question 13

How can Transverse Myelitis and Optic Neuritis be confirmed to be MS

Answer 13

Recurrent or other attacks elsewhere

Question 14

What is MS (Multiple Sclerosis)

Answer 14

Immune mediated disease of CNS

leading cause of non-traumatic disability in young adults

Question 15

Where is MS more common?

What is the peak onset age of MS?

What gender is more common?

How does it affect life expectancy?

Answer 15

North of equator, greater rates w/ greater distance

20-30

female

reduces by 7-14 years

Question 16

What are MS risk factors?

Answer 16

Note: exact cause of MS is not known

Risk factors: EBV exposure (Epstein-Barr virus)

Low sun exposure

Obesity

Smoking

Genetic risk factor (20% heritability risk increase)

Question 17

How does the demyelination happen in MS?

Answer 17

movement of auto-reactive T cells and demyelinating antibodies from the systemic circulation into the CNS through disruption of blood brain barrier

Question 18

How does MS usually progress?

Answer 18

Recurrent attacks with partial recovery, each time you acquire more and more disability

(called RRMS) Relapsing Remittent Multiple Sclerosis

Note: disability can also trend up in straight line (primary progressive MS)

Question 19

After 10 years what MS progression is more common?

Answer 19

Secondary Progressive Multiple Sclerosis

Basically it commonly starts as Relapse/remittent MS followed by a steady decline after a while (no more recover)

Question 20

What happens to brain volume due to MS?

Answer 20

Decreases

Question 21

Who has a better prognosis for MS?

Who has worse?

Answer 21

Better: Women, Caucasians, Monofocal onset, low relapse rate, low disablity at 5 years

Worse: Men, Non-white populations, Smoking/obesity, high relapse rate, high disability at 5 years

Question 22

What bodily systems can MS effect

Answer 22

Every system

CNS, Visual,Speech, throat, MSK, sensation, bowel, urinary

Question 23

What are the most common motor symptoms with MS?

What occurs later usually?

Answer 23

UMN spastic weakness 80%

spastic paraparesis is most common

Later: ataxia, tremor, incoordination, scanning speech

Question 24

What are the most common sensory symptoms (what tract) experienced with MS?

Answer 24

75% of patients expeirence sensory issues

most common spinothalamic lesions, resulting in dysesthesias, pain

DCML involvement is less often

Question 25

What is the sensory sensation that often happens with MS?

Answer 25

Lhermitte's phenomenon electric sensation passing down neck and limbs upon flexion of neck

Question 26

What are common brainstem symptoms of MS?

Answer 26

Nystagmus, diplopia, facial weakness, vertigo, dysphasia and **Trigeminal neuralgia** internuclear opthalmoplegia- due to lesion in medial longitudinal fasiculus- inability to adduct ipsilateral eye note: trigeminal nerve effected because oligodendrocytes myelinate the first bit of the nerve in the CNS

Question 27

What are the most common cognitive problems caused by MS?

Answer 27

Cognitive symptoms affect 60% of MS patients usually have "**subcortical dementia**" which affects information processing, visuospatial, memory, and executive function

Question 28

What kind of conditions make fatigue worse for MS patients?

Answer 28

Heat Note: systemic fatigue very common

Question 29

What is the most common diagnostic test for MS?

Answer 29

**MRI of brainstem and spinal cord** CSF fluid tap to look for inflammatory changes "evoked potentials" of visual, auditory, somatosensory

Question 30

What does MS look like on a brain MRI?

Answer 30

**diffuse** inflammation and damage throughout

Question 31

What do MS medications help with? What do they not help with?

Answer 31

Help with inflammation but cannot fix nerves that are already damaged

Question 32

When testing CSF in MS patients, what will you see?

Answer 32

**WBC** elevated **protein** may be high increased **immunoglobulin** production (**OCB**)oligoclonal bands- presence of 2 or more antibody clons (indicates damaged blood brain barrier or immunoglobulin production in the brain)

Question 33

What does the "Mcdonald criteria" for MS consists of

Answer 33

Includes: * **Prior** attacks, * **new** lesion on subsequent MRI, * MRI with active or enhancing lesions and coexisting inactive lesions * CSF **oligoclonal banding** **Evidence of atleast 2 areas damage at different times**

Question 34

The 2017 MS diagnostic criteria consists of what

Answer 34

2 different clincal attacks with 2 different locations at 2 different times 1 attack with MRI showing lesions, enhancing and non-enhancing (meaning one is progressing and one is old) 1 attack with MRI, and repeat MRI with new lesions 1 month later

Question 35

What is the most common treatment of **acute exacerbations** of MS?

Answer 35

Steroids: IV or PO or Plasma exchange

Question 36

What is the difference between old vs new MS meds?

Answer 36

Older medications: -very safe but not as effective , mainly injections Newer medications - more risk of infection but more effective - by mouth (PO) or by monthly shot

Question 37

What is Progressive Multifocal Leukoencephalopathy (PML)

Answer 37

**Severe** demyelinating disease of CNS due to **JC virus** infecting **oligodendrocytes** note: 86% of the population have a asymptomatic infection of JC virus the risk comes due to immunosupression associated with treatment of other autoimmune disorders (people with MS are at risk) usually **fatal within 1 year** if not caught can be treated by stopping immunosuppressants will present like an MS attack at first

Question 38

What are treatment goals associated w/ MS

Answer 38

Prevent longterm disability no consensus on approach to therapy

Question 39

If **both** optic discs are enlarged what is likely wrong? If only **one** is enlarged?

Answer 39

**Both**: Increased intracranial pressure **one**: Optic neuritis

Question 40

_______ is common for patients with MS, especially in the lower extremities

Answer 40

Spasticity

Question 41

MS patients also commonly have sexual dysfunction and spastic and flaccid bladder what is the difference between spastic and flaccid bladder

Answer 41

Flaccid bladder - cant get urine out (leads to retention) spastic bladder- overactive, frequency urgency

Question 42

What medication works at the neuromuscular junction and is shown to increase walkingspeed in patients by an average of 25%

Answer 42

Ampyra (D-Alfampridine)

Question 43

What is neuromyelitis optica (devic's disease)

Answer 43

presents like transverse myelitis combined with **bilateral** optic neuritis **spinal cord transverse myelitis longitudinally extensive lesion across more than 3 vertebrae levels** more severe than MS diagnosed through antibody testing **NMO IgG**

Question 44

What is the treatment for acute NMO (neuromyelitis optica) maintenance?

Answer 44

Acute attacks: Steroids, plasma exchange Maintenance: medications

Question 45

What is myelin oligodendrocyte glycoprotein antibody disease? (MOG)

Answer 45

Basically neuromyelitis optica (NMO) but for children and young adults (but all ages can be impacted) Can be **monophasic** or **relapsing** usually has optic neuritis, transverse myelinitis, and ADEM like CNS lesions Has **good recovery** if treated like NMO

Question 46

MOGAD vs AQP4+NMOSD

Answer 46

Basically these are both MOGAD However, the regular **MOGAD** affects the **lower** spinal cord whereas the **AQP4+** affects the **upper** spinal cord

Question 47

How does ataxia present?

Answer 47

Disturbance in smooth preformance of voluntary motor acts Error in rate, range, force, and duration

Question 48

Quick overview of cerebellum: Vermis is for: Intermediate zone is for: Lateral hemisphere is for: Flocculonodular lobe is for: Answer choices: Proximal coordination distal coordination balance and vestiboccular reflex motor planning for extremities

Answer 48

Vermis is for: Proximal coordination Intermediate zone is for: distal coordination Lateral hemisphere is for: motor planning for extremities Flocculonodular lobe is for: balance and vestiboccular reflex

Question 49

What are the clinical manifestations of cerebellar dysfunction

Answer 49

limb/trunk/gait ataxia ipsilateral to lesions of the cerebellar hemisphere impaired stance (wide) intention tremor Dysmetria Dysdiadochokinesia Impaired checking response **hypo**tonia speech deficits: mutism, scanning, dysarthria Nystagmus/abnormal saccades

Question 50

What does scanning speech sound like?

Answer 50

uneven time and inflection points no problem w/ word findng or speech production

Question 51

Acute causes of ataxia

Answer 51

Intoxication Vascuar lesions (strokes) Trauma Infections

Question 52

Subacute causes of ataxia (days to weeks)

Answer 52

Brain tumors Alcoholic-nutritional thiamine or vitamin E deficiency Related to cancer (paraneoplastic) Demyelinating cause (MS)

Question 53

Causes of chronic ataxia (years)

Answer 53

Friedreich ataxia (common in kids) Spinocerebellar ataxia (SCA) Other cerebellar degeneration Hereditary metabolic diseases

Question 54

Rostral vermis syndrome is usually in what population? What are the features?

Answer 54

Chronic alcoholics features: -wide base stance/gait ataxia gait impaired arm coordination infrequent nystagmus/hypotonia,dysarthria

Question 55

Caudal vermis syndrome is usually present in what population? What are the features?

Answer 55

Typically in children w/ medulloblastoma Features: axial dysequilibrium and staggering gait little to no limb ataxia sometimes spontaneous nystagmus and rotated postures of head

Question 56

cerbellar hemispheric syndrome is usually present in who? What are the features?

Answer 56

People w/ infarcts, tumors, abscess Features: incoordination of ipsilateral movements, particularly **fine motor control**

Question 57

What are the causes of pancerebellar syndrome? What are the features?

Answer 57

Causes: infection, hypoglycemia, hyperthermia, paraneoplastic, toxic-metabolic, hereditary features: **Bilateral cerebellar signs** affecting limbs trunk and cranial musculature

Question 58

What is the clincial presentation of friedreich ataxia?

Answer 58

Begins in childhood or young adulthood loss of ambulation after 10-15 years areflexia foot deformities scoliosis cardiomyopahty glucose intolerance life expectancy: 40 usually presents w/ "proprioceptive gait w/ foot slap to increase sensation"

Question 59

Why is it important to identify friedreich ataxia early? is friedreich ataxia autosomal dominant or resessive? what chromosome is affected?

Answer 59

New medication can slow progression by 50% recessive chromosome 9

Question 60

How does ADEM usually progress

Answer 60

usually monophasic, but can reoccur