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Neuro Clerkship > Neuropathic Disease > Flashcards

Neuropathic Disease Flashcards

1
Q

What is the most common inherited motor-sensory neuropathy?
Where geographically is it common?
Which is more common– acquired or inherited neuropathies?

A

Charcot-Marie-Tooth Disease

  • Common in rural WV
  • Acquired neuropathies are more common
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2
Q 
Demyelinating CMT-1: 
Inheritance pattern? 
Which gene/ protein is mutated? 
What is the problem?
What is are two distinct clinical features associated with the disease?

Describe the findings on a nerve biopsy?

A

AD, mutant peripheral myelin protein 22 (PMP22) gene–> demyelination and remyelination of large axonal segments
**Patients have high arches and hammer toes

Nerve biopsy will reveal “onion bulbing”

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3
Q

Which age group gets demyelinating CMT-1?
How does the weakness and wasting present? (2)
Do patients typically have sensory symptoms?

A
  • Presents in teenagers/ adults

Clinical presentation:

  • Distal –> proximal muscle wasting + weakness
  • Eventually wheelchair bound
  • Typically no numbness; patients may simply never have had sensation to lose
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4
Q

Axonal CMT-2:
Inheritance pattern?
What is the problem?
How does it differ from CMT-1?

A

AD, axonal damage

  • Slower to progress than CMT 1 (patients ambulate with orthotics)
  • Otherwise similar in clinical manifestations
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5
Q

Dejerine-Sottas Diasease (CMT-3):
Inheritance pattern?
Problem?
Clinical manifestations and age of onset?

A
  • AR, demyelinating neuropathy

- Presents in infants and toddlers with delayed / absent motor milestones

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6
Q

What does the diagnostic workup entail for the CMT diseases? (3)

  • What history should be asked?
  • What is seen on neuro PE?
  • What testing should be done?
A
  • Good genetic hx.
  • Good neuro PE: will see absent reflexes due to demyelination/ axonal injury
  • Nerve conduction study (differentiate etiology)
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7
Q

What will you seen on the nerve conduction study for a patient with demyelinating CMT?(3)–which of the findings determines severity of disease?

A
  • # 1 Decreased velocity**
  • Increased latency (determines SEVERITY)
  • Marginally decreased amplitude
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8
Q

What will you see on the nerve conduction study for a patient with axonal CMT?

A
  • # 1 Decreased amplitude**
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9
Q

How do we treat CMT?

A

Surgery for scoliosis, tendon lengthening, damage due to muscle atrophy

**There are no preventative or disease suppressing treatments

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10
Q

What is the most common nerve entrapment syndrome?

Describe the clinical presentation (2).
How is it diagnosed (2 clinical tests + 1 additional finding)?

A

Carpel Tunnel Syndrome

  • Numbness, pain over palm digits 1-3
  • Weakness in advanced disease

Diagnosed Clinically or NCS:

  • Phalen’s, Tinnels
  • Weakness in abductor pollicis brevis, Opponens pollicis (gripping)
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11
Q

How do we treat carpel tunnel syndrome? (3 conservative, 2 aggressive)

What is the only definitive treatment?

A

Treat conservatively w braces, rest, NSAIDS

Treat aggressively with corticosteroid injections or ligament release surgery (only definitive cure)

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12
Q

What causes CTS?

A

Repeated gripping

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13
Q

What is the leading cause of neuropathy in US and Europe?
What was the former leading cause?

How does the neuropathy present?

How is it diagnosed?

A

Diabetes (60-70% DM patients have neuropathy)–formerly leprosy

  • Presents with stocking-glove sensory loss, burning–> evolves to weakness
  • Diagnosed CLINICALLY or w/ NCS (rarely)
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14
Q

How do we treat DM neuropathy? (1 lifestyle, 3 drugs)

A

1 Glycemic control (prevent further damage)

  • TCAs treat sx.
  • Gabapentin + other AEDs
  • Topical capscaisin
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15
Q

Describe the pathophysiology of GBS:

  • Problem?
  • Timeline?
  • Which areas are damaged first?
A

Autoimmune demyelinating disease +/- secondary axonal loss

  • Progresses within days to weeks (2-4 weeks to plateau)
  • Proximal–> distal demyelination
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16
Q

What causes the axonal damage associated with GBS?

A

MQs scavenging damaged areas after myelin is destroyed

17
Q

Describe how GBS is considered to be a parainfectious disease.

With which vaccine was it most heavily associated?

Which infection?

A
  • 75% cases preceded by infection, immunization, or surgery
  • 1970s swine flu vaccine
  • # 1 Campylobacter jejuni
  • EBV, CMV, Lyme, HIV, Hepatitis
18
Q

Describe the clinical presentation of GBS (5):

A
  1. RAPIDLY ASCENDING PARALYSIS feet–> torso
  2. Absent DTRs
  3. Mid-lower back pain/ paresthesia
  4. ANS instability (KILLS**)
  5. Respiratory failure
19
Q

Describe 3 severe complications associated with GBS related ANS instability

A
  • Thyroid storm
  • Rapidly undulating BP
  • Respiratory failure
20
Q

What is the prognosis for GBS?

A

80-90% patients return to their previous baseline

5-10% die–respiratory failure

21
Q

How do we treat GBS (3)?

Which is more dangerous?

A

Most effective = Combined:

  1. IVIG for 10 days
  2. PLEX: 5 treatments, every other day
  3. Monitor in ICU for change; may need ventilation

PLEX is more dangerous bc ANS instability and severe HypoTN may result

22
Q

What will you find on the nerve conduction study for a patient w GBS?

A

NORMAL FINDINGS–Wallariean degeneration takes longer to occur, therefore the study will be worthless most of the time…

23
Q

Describe the pathophysiology of CIDP:

A

Abs + T cells + MQs invade interstitial/ perivascular endoneurium–>
SEGMENTAL DEMYELINATION of peripheral nerves

24
Q

Describe the clinical findings associated with CIDP:

  • Time course of disease?
  • Type of Deficits (2)?
  • CSF findings?
  • EMG/NCS findings?
A
  1. Insidious and slowly progressive; may relapse/ remit
  2. Sensory + Motor deficits
  3. ANS dysfunction: orthostasis, incontinence, arrythmias
  4. CSF = ^ protein
  5. EMG/ NCS shows demyelination (decreased velocity)
25
Q

What is seen on nerve biopsy of patient with CIDP?

A

Onion bulbing due to continuous demyelination and demyelination

26
Q

How do we treat CIDP?

A

Immunosuppression/modulation

  • Steroids, Azathioprine, Mycophenolate, Cytoxan
  • PLEX and/ or IVIG for unresponsive cases
27
Q

Amyotrophic Lateral Sclerosis:

  • What is the problem here?
  • When is the peak age onset?
  • Which gene is associated with the familial variant? Inheritance pattern?
A
  • Neurodegeneration of anterior motor neurons
  • Peak onset 65-70 yoa
  • AD inheritance, mutant SOD gene
28
Q

Describe the clinical presentation of a patient with ALS:

  • Sensory exam?
  • Motor sx (2)
  • Bulbar involvement?
  • Time course?
A
  • NORMAL sensory exam
  • Upper + Lower motor neuron signs w asymmetric onset
  • Bulbar involvement
  • Ventilatory failure + frontotemporal dementia 3 yrs following onset
29
Q

How do we dx ALS?

A

Clinical and EMG findings to rule out c-spine injury/ reversible cause

30
Q

What is the treatment for ALS– which is FDA approved (2)?

A
  • Riluzole (Rilutek) only FDA approved treatment, ^ life by 3 mos
  • Supportive care, i.e. PEG tube, vent, etc.
31
Q

List 3 hereditary neuropathies:

A
  • CMT
  • Amyloidosis
  • Mitochondrial disease
32
Q

List 5 potential causes of autoimmune neuropathy:

A
  • MGUS
  • Sjogrens
  • Sarcoidosis
  • RA
  • Vasculitis
33
Q

Two causes of infectious neuropathy?

A
  • HIV

- Lyme (BL Bells palsy)

34
Q

3 nutritional/ metabolic causes of neuropathy?

A
  • B12 deficiency (subacute combined degeneration)
  • B6 excess (sensory neuropathy)
  • ETOH abuse
35
Q

4 industrial exposures that can cause neuropathy?

A
  • arsenic
  • mercury
  • lead
  • organophosphates
36
Q

Drugs that can cause neuropathy (6):

A
  • colchicine
  • paclitaxel
  • vincristine
  • metronidazole
  • hydrazine
  • NO
37
Q

What is the Miller Fischer Variant of GBS– How does it present?

A

Anti GQ1B antibodies present in most cases– Presents with:

  1. facial weakness
  2. dysarthria
  3. areflexia
38
Q

Describe the clinical presentation of B12 deficiency:

  • Age/ circumstance (4)
  • clinical sx (3)
  • Dx workup (2)
A
  • 60+ yoa, pernicious anemia, gastric bypass, NO abuse
  • Numbness, neuropathic pain, gait disturbance
  • Check serum B12 (LOW), methylmalonic acid (HIGH)

Neuro Clerkship (13 decks)

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