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Neuromuscular management > Lecture 7 - Demyelination Ataxia > Flashcards

Lecture 7 - Demyelination Ataxia Flashcards

1
Q

What is ADEM (Acute disseminated encephalomyelitis)

A

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)

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2
Q

How does ADEM usually progress

A

usually monophasic, but can reoccur

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2
Q

ADEM is most common in what patients

A

Children

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3
Q

how does ADEM look on an MRI?

A

big fluffy areas of inflammation on both sides

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4
Q

What does “sensory level noted at T4” mean?

A

Sensory issues below T4

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5
Q

What is Acute Transverse Myelitis?

A

Inflammation of spinal cord causing lesions

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

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6
Q

How will acute Transverse myelitis appear on an MRI?

A

Small patchy lesions limited to two areas different vertebral segments

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7
Q

How will Acute Transverse myelitis present

A

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

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8
Q

How will optic neuritis present?

A

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

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9
Q

Optic neuritis is a common initial symptom of ________________

A

MS

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

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

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10
Q

What factors increase the risk for MS?

A

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

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11
Q

What is the normal treatment for optic neuritis?

A

IV steroids

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12
Q

Optic neuritis on its own is usually ___________

A

monophasic

but not if its related to MS

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13
Q

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

A

Recurrent or other attacks elsewhere

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14
Q

What is MS (Multiple Sclerosis)

A

Immune mediated disease of CNS

leading cause of non-traumatic disability in young adults

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15
Q

Where is MS more common?

What is the peak onset age of MS?

What gender is more common?

How does it affect life expectancy?

A

North of equator, greater rates w/ greater distance

20-30

female

reduces by 7-14 years

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16
Q

What are MS risk factors?

A

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)

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17
Q

How does the demyelination happen in MS?

A

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

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18
Q

How does MS usually progress?

A

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)

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19
Q

After 10 years what MS progression is more common?

A

Secondary Progressive Multiple Sclerosis

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

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20
Q

What happens to brain volume due to MS?

A

Decreases

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21
Q

Who has a better prognosis for MS?

Who has worse?

A

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

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22
Q

What bodily systems can MS effect

A

Every system

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

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23
Q

What are the most common motor symptoms with MS?

What occurs later usually?

A

UMN spastic weakness 80%

spastic paraparesis is most common

Later: ataxia, tremor, incoordination, scanning speech

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24
Q

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

A

75% of patients expeirence sensory issues

most common spinothalamic lesions, resulting in dysesthesias, pain

DCML involvement is less often

25
Q

What is the sensory sensation that often happens with MS?

A

Lhermitte’s phenomenon

electric sensation passing down neck and limbs upon flexion of neck

26
Q

What are common brainstem symptoms of MS?

A

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

27
Q

What are the most common cognitive problems caused by MS?

A

Cognitive symptoms affect 60% of MS patients

usually have “subcortical dementia” which affects information processing, visuospatial, memory, and executive function

28
Q

What kind of conditions make fatigue worse for MS patients?

A

Heat

Note: systemic fatigue very common

29
Q

What is the most common diagnostic test for MS?

A

MRI of brainstem and spinal cord

CSF fluid tap to look for inflammatory changes

“evoked potentials” of visual, auditory, somatosensory

30
Q

What does MS look like on a brain MRI?

A

diffuse inflammation and damage throughout

31
Q

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

A

Help with inflammation

but cannot fix nerves that are already damaged

32
Q

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

A

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)

33
Q

What does the “Mcdonald criteria” for MS consists of

A

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

34
Q

The 2017 MS diagnostic criteria consists of what

A

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

35
Q

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

A

Steroids: IV or PO
or Plasma exchange

36
Q

What is the difference between old vs new MS meds?

A

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

37
Q

What is Progressive Multifocal Leukoencephalopathy (PML)

A

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

38
Q

What are treatment goals associated w/ MS

A

Prevent longterm disability

no consensus on approach to therapy

39
Q

If both optic discs are enlarged what is likely wrong?

If only one is enlarged?

A

Both: Increased intracranial pressure

one: Optic neuritis

40
Q

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

A

Spasticity

41
Q

MS patients also commonly have sexual dysfunction and spastic and flaccid bladder

what is the difference between spastic and flaccid bladder

A

Flaccid bladder - cant get urine out (leads to retention)

spastic bladder- overactive, frequency urgency

42
Q

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

A

Ampyra (D-Alfampridine)

43
Q

What is neuromyelitis optica (devic’s disease)

A

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

44
Q

What is the treatment for acute NMO (neuromyelitis optica)

maintenance?

A

Acute attacks: Steroids, plasma exchange

Maintenance: medications

45
Q

What is myelin oligodendrocyte glycoprotein antibody disease? (MOG)

A

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

46
Q

MOGAD vs AQP4+NMOSD

A

Basically these are both MOGAD

However, the regular MOGAD affects the lower spinal cord whereas the AQP4+ affects the upper spinal cord

47
Q

How does ataxia present?

A

Disturbance in smooth preformance of voluntary motor acts

Error in rate, range, force, and duration

48
Q

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

A

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

49
Q

What are the clinical manifestations of cerebellar dysfunction

A

limb/trunk/gait ataxia
ipsilateral to lesions of the cerebellar hemisphere

impaired stance (wide)

intention tremor

Dysmetria

Dysdiadochokinesia

Impaired checking response

hypotonia

speech deficits: mutism, scanning, dysarthria

Nystagmus/abnormal saccades

50
Q

What does scanning speech sound like?

A

uneven time and inflection points

no problem w/ word findng or speech production

51
Q

Acute causes of ataxia

A

Intoxication

Vascuar lesions (strokes)

Trauma

Infections

52
Q

Subacute causes of ataxia (days to weeks)

A

Brain tumors

Alcoholic-nutritional thiamine or vitamin E deficiency

Related to cancer (paraneoplastic)

Demyelinating cause (MS)

53
Q

Causes of chronic ataxia (years)

A

Friedreich ataxia (common in kids)

Spinocerebellar ataxia (SCA)

Other cerebellar degeneration

Hereditary metabolic diseases

54
Q

Rostral vermis syndrome is usually in what population?

What are the features?

A

Chronic alcoholics

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

55
Q

Caudal vermis syndrome is usually present in what population?

What are the features?

A

Typically in children w/ medulloblastoma

Features:

axial dysequilibrium and staggering gait

little to no limb ataxia

sometimes spontaneous nystagmus and rotated postures of head

56
Q

cerbellar hemispheric syndrome is usually present in who?

What are the features?

A

People w/ infarcts, tumors, abscess

Features: incoordination of ipsilateral movements, particularly fine motor control

57
Q

What are the causes of pancerebellar syndrome?

What are the features?

A

Causes: infection, hypoglycemia, hyperthermia, paraneoplastic, toxic-metabolic, hereditary

features: Bilateral cerebellar signs affecting limbs trunk and cranial musculature

58
Q

What is the clincial presentation of friedreich ataxia?

A

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”

59
Q

Why is it important to identify friedreich ataxia early?

is friedreich ataxia autosomal dominant or resessive?

what chromosome is affected?

A

New medication can slow progression by 50%

recessive

chromosome 9

Neuromuscular management (19 decks)

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