Neuropathies Flashcards

1
Q

Know:

CRPs
GB
Diabetic neuropathy
Bells and oculomotor neuropathy
Carpal tunnel syndrome

A

-CRPs
-GB
-DM neuropathy
-bells palsy
-oculomotor palsy
TEST
-MCC- camp jejuni
-budapest criteria dont need to know
-polyneuropathy- ignore hereditary
-lumbar sacral chart- dont need to know
-never assume neck pain radiating down to arm is MSK

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2
Q

complex regional pain syndrome (CRPS): definition and pathophysiology

A

Definition: Disorder of a body region characterized by pain and inflammation s/p injury, surgery, trauma, stroke, MI
-Occurs after fractures (MC), surgery
-Pain experienced is disproportionate to the degree of tissue injury and persists beyond the normal expected time for tissue healing
-Pain is more REGIONAL rather than dermatomal or peripheral nerve distribution (ex: just hands and not up dermatome)

Pathophysiology = multifactorial: pain dysregulation in the sympathetic and central nervous systems, with likely genetic, inflammatory, and psychological contributions
- Exact mechanism is unclear

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3
Q

CRPS symptoms: sensory, motor/trophic, autonomic

A

SENSORY:
-allodynia: non-painful stimuli cause pain
-hyperalgesia: painful stimuli cause exaggerated pain

MOTOR/Trophic
- weakness
- reduced range of motion
- tremor
- dystonia
- trophic: changes in hair, skin, nails

AUTONOMIC:
-skin color and temp changes (vasomotor dysfunction): hyper/hypopigmentation; hyper/hypothermia
-swelling and sweating changes (sudomotor dysfunction): hyperhidrosis/hypohidrosis, edema

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4
Q

CRPS dx and tx

A

Dx = clinical, uses the budapest criteria (dont memorize)

Tx:
- PT/OT
-Behavioral therapy

Pharmacotherapy:
-Anti-inflammatories, anticonvulsants, antidepressants, transdermal lidocaine, bisphosphonates

Interventions if refractory:
-Nerve blocks
-Spinal cord stimulation

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5
Q

A 27-year-old female with no significant medical history presents with complaint of weakness. Symptoms have been present for the past three days. She describes initial onset of bilateral leg tingling and weakness that has progressed since onset. She awoke today and had difficulty walking so presented to the emergency department (ED). She has never had these symptoms before. She recovered from a severe diarrheal illness 2 weeks ago but denies any current gastrointestinal symptoms.
Vital signs: Temperature (T) 99.0°F, heart rate (HR) 92, blood pressure (BP) 106/78, respiratory rate (RR) 18, oxygen saturation O2sat 97% on room air.
Physical exam: The patient appears fatigued but is not in any distress. Heart is regular. Breath sounds slightly diminished but otherwise clear. Lower extremity strength is 3/5 bilaterally and patellar reflexes are absent

A

Vital signs: Temperature (T) 99.0°F, heart rate (HR) 92, blood pressure (BP) 106/78, respiratory rate (RR) 18, oxygen saturation O2sat 97% on room air.
Physical exam: The patient appears fatigued but is not in any distress. Heart is regular. Breath sounds slightly diminished but otherwise clear. Lower extremity strength is 3/5 bilaterally and patellar reflexes are absent

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6
Q

guillane-barre syndrome: definition and causes

A

Definition: Autoimmune disorder causing destruction of PERIPHERAL nervous system MYELIN sheath/axons (somatic and autonomic) and cranial nerves causing an acute polyneuropathy
-Incidence of 1-2 per 100,000 per year
-All age groups, but changes increase with age
-Male > Female
- Infection → Abx against Postsynaptic ACh receptors

Preceding infection / trigger: ~5 days to 4 weeks before the onset of neurologic symptoms
-Idiopathic 40%
-Infections – EBV, CMV, mycoplasma, CAMPYLOBACTER JEJUNI (MC)*
-Immunizations 17%
-Surgery
-Immune response to preceding infection –> molecular mimicry of peripheral nerves –> body attacks our own nerves -> cross reaction in immune response –> demyelination that progressively gets worse

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7
Q

guillain barre syndrome sx

A

2-4 weeks after a preceding illness:
- 1st sx: Sensory disturbance: paresthesia/pain in back and limbs (54%, mild compared to weakness); stocking glove distribution
- Progressive ascending SYMMETRIC FLACCID weakness (+/- diaphragm once its C4) and loss of DTRs (94%)
-Cranial nerve involvement: fascial weakness, dysphagia, dysarthria, ophthalmoplegia - (46%)
-Autonomic dysfunction: tachycardia/bradycardia, dysrhythmias, wide variations in blood pressure, postural hypotension, urinary retention, constipation, facial flushing, anhydrosis, hypersalivation) may be present
-Severe respiratory weakness requiring mechanical ventilation: 30%**
-no brain localizing findings or fever at onset
-Symptoms peak in ~2-4 weeks, and recovery varies from weeks to a year

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8
Q

guillain barre syndrome dx and tests to run/workup

A

Dx:
-Clinical evaluation: bilateral limb paresis, hyporeflexia, sensory involvement
-LP/CSF: Albumino-cytologic dissociation: (HIGH protein (after 1 week) but normal WBC count), non-emergent
-EMG/NCS: acute polyneuropathy with either demyelinating or axonal features
-Neuroimaging: rule out spinal cord compression / cauda equina (mimic)

Negative inspiratory force (NIF) testing used to predict impending respiratory failure
-Normal NIF is >60cm of water
-Abnormal NIF <30cm (or dropping from baseline); consider PPV or intubation

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9
Q

what makes dx of guillian barre doubtful

A

-Sensory level (decrease or loss of sensation below a spinal cord root level as determined by neurologic examination) -> Points to spinal cord injury
-Marked, persistent ASYMMETRY of weakness
-Severe and persistent bowel and bladder dysfunction: conus medularis
-More than 50 white cells in the CSF: should have normal WBC

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10
Q

guillain barre syndrome ddx not important

A

-Other acute polyneuropathies: severe vitamin B1 deficiency, acute arsenic poisoning, n-hexane (glue sniffing neuropathy), vasculitis, Lyme disease, tick paralysis (mostly in children), porphyria, sarcoidosis, leptomeningeal disease, paraneoplastic disease, and critical illness.
-Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
-Continues to progress or has relapses for greater than eight weeks.
-Diseases of the spinal cord, neuromuscular junction, and muscle.
-Distinguish from cauda equina syndrome, which is often associated with bowel/bladder disturbance, and confirmed with MRI L/S spine

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11
Q

guillain barre syndrome tx

A

-Primarily supportive
-Monitor respiratory status, prevent immobility consequences, pain management

Tx indication: IVIG or Plasmapharesis
-non-ambulatory within 4 weeks of onset
- ambulatory patients not improving within 4 weeks of sx onset

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12
Q

peripheral neuropathy: definitioin and mononeuropathy vs polyneuropathy vs radiculopathy

A

Definition: Any disorder of the PNS including radiculopathies, mononeuropathies, polyneuropathy

Mononeuropathy:
- damage to a single nerve, usually due to a local cause such as trauma, compression, or entrapment.
-Example: Carpal tunnel syndrome

Polyneuropathy:
- damage to terminal branches of multiple nerves
- result: symmetrical and distal distribution

Radiculopathy:
- damage to a nerve root; distribution corresponds to the dermatome

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13
Q

polyneuropathy: causes and main categories

A

Categories: Divided into axonal and demyelinating types.
-MC = Diabetic polyneuropathy (axonal)
-Other common: alcohol abuse, and HIV infection

Systemic causes, predominantly axonal neuropathy:
-Critical Illness Polyneuropathy: a/w prolonged intubation and poor nutritional status
-Vitamin deficiencies
-Amyloidosis
-Hypothyroidism
-Lyme disease
-Toxic (predominantly axonal): Alcohol, Chemotherapy, heavy metals

Hereditary (demyelinating): Charcot-Marie-Tooth types 1A, 1B, and X-linked (ignore)

Idiopathic: 25%

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14
Q

polyneuropathy sx

A

-Symmetric distal sensory loss, burning, or weakness.
-MC: Chronic axonal polyneuropathies (eg, DM or uremia)
-Axon Length Injury: longer axons are affected first, resulting in symptoms that begin in the LOWER extremities
-Sensory symptoms precede motor symptoms
-Slowly progressive sensory loss and dysesthesias such as numbness, a burning sensation and pain in the feet, and mild gait abnormalities.
-As the syndrome progresses, mild weakness of the lower legs and hand symptoms may begin, resulting in the classic “stocking and glove” distribution of sensory loss.

advanced:
- proximal numbness: affecting the intercostal nerves (the next longest nerve fibers after the arms), sensory loss over the STERNUM
-The top of the head may be affected with further progression

  • Damage in distal vessels -> reduced blood flow in the distal arteries which feed the nerves -> nerve damage
    Stocking glove loss of sensation
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15
Q

polyneuropathy: axonal neuropathy exam

A

-Wasting of the intrinsic muscles of the feet or lower leg (late: hands)
-Distal loss of sensation to pin prick, light touch, vibration, cold, and proprioception may also occur.
-Reflexes become hypoactive or absent distally, usually at the ankles initially.
-Early on can be identified w/ detailed sensory examination of the lower extremities

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16
Q

polyneuropathy: demyelinating neuro exam

A

-Generalized weakness is the rule in patients with symptoms of a more fulminant polyneuropathy.
-Distal muscles more affected > proximal
-Sensation is also reduced
-Large myelinated fibers are most damaged, resulting in abnormalities of VIBRATORY testing and PROPRIOCEPTION! that are often out of proportion to loss of pin prick or temperature sensation.
-Hypo or absent reflexes

17
Q

polyneuropathy work up and tx

A

NCS/EMG:
-Slow nerve conduction velocity *
-Dispersion of evoked compound action potentials
-Conduction block (decreased amplitude of muscle compound action potentials on proximal compared with distal nerve stimulation)

Labs: depends on whether axonal or demyelinating
-Wait for EMG/NCS; eg, would not order TFTs if electrophysiology is demyelinating.

Nerve biopsy = rare

Treatment:
- Glycemic control
- treatment of pain: Gabapentin, TCAs

18
Q

77-year-old man with progressive numbness and tingling in his feet for four months. The tingling has spread up to his bilateral knees and his fingertips of both hands, more prominent in the first three fingers. His symptoms are symmetrical. He felt his feet are heavy and tight, and he lacks energy.
PMH: Type II DM for 4 years which is well controlled, HTN, Hypercholesterolemia. Recently diagnosed B12 deficiency and he gets IM injections.
EXAM: Strength is full. He has decreased pinprick and light touch in a stocking distribution up to bilateral knees. There is decreased pinprick and light touch in the first three fingers. Absent joint position sense in the great toes.
Ankle reflexes are absent and the rest are 2+.
Diagnosis?
Neuropathy, but secondary to what?
Diabetic?
Vitamin B12 deficiency?
Other etiology?

A

PMH: Type II DM for 4 years which is well controlled, HTN, Hypercholesterolemia. Recently diagnosed B12 deficiency and he gets IM injections.
EXAM: Strength is full. He has decreased pinprick and light touch in a stocking distribution up to bilateral knees. There is decreased pinprick and light touch in the first three fingers. Absent joint position sense in the great toes.
Diagnosis?
Neuropathy, but secondary to what?
Diabetic?
Vitamin B12 deficiency?
Other etiology?
EMG/NCS: Moderate axonal sensorimotor polyneuropathy
Blood work:
Normal: Metabolic panel, thyroid function, folate, CPK, SPEP, IPEP, RPR
+ ANA
Hemoglobin A1c = 7.1
B12 = 197, repeated >2000
ESR : 87
DIABETIC NEUROPATHY?
Muscle biopsy done and consistent with vasculitis
Don’t assume DM is always the cause!

19
Q

diabetic neuropathy types

A

Distal symmetric polyneuropathy (MC)
-Classic “glove-stocking” pattern of distal sensory loss

Autonomic neuropathy:
-Diagnosis of exclusion
- sx: postural hypotension, gastroparesis, enteropathy (Diarrhea or Constipation)

Polyradiculopathies: multiple nerve roots damaged, distribution along dermatomes
-Diabetes frequently injures the NERVE ROOTS at thoracic or high lumbar regions -> axonal degeneration
- common features: older, have coexisting peripheral polyneuropathy, and have WEAKNESS and ATROPHY in the distribution of one or more contiguous nerve roots with frequent territorial expansion

Focal mononeuropathies:
-Cranial mononeuropathies: MC is CN III (oculomotor palsy)
-Peripheral mononeuropathies: MC is carpal tunnel syndrome

mononeuropathy multiplex:
- Asymmetric involvement of multiple peripheral nerves

20
Q

diabetic neuropathy review

A

-MC symptoms of diabetic neuropathy include pain, burning, tingling, or numbness in the toes or feet and extreme sensitivity to light touch.
-Worst at rest and improve with activity, such as walking.
-Diabetic neuropathy usually affects both sides of the body !!!
-Symptoms are usually noticed first in the toes, then may gradually move up the legs; if the mid-calves are affected, symptoms may develop in the hands. Over time, the ability to sense pain may be lost, which greatly increases the risk of injury.

20
Q

DM managment and neuropathic pain: pharm tx

A

First line:
- pregabalin*, gabapentin

others:
- TCAs - amitriptyline
- SSRI: duloxitine

-There is no cure for diabetic neuropathy, but treatment can improve painful symptoms and prevent complications:
-Control blood sugar levels
-Lifestyle changes (esp diet and exercise)
-Foot care
-Pain control : Anti-seizure meds (Pregabalin!!!! [1st line], Gabapentin!,), TCAs (Amitriptyline!), SSRI (Duloxetin!)

21
Q

bells palsy

A

-Acute peripheral facial nerve (CN VII!) palsy
-We only call it Bells if its idiopathic
-Risk increases during pregnancy:3rd trimester - first postpartum week.
-Etiology: HSV activation (MC!!!!), then zoster

Presentation:
-Sudden onset (usually over hours) of inability to close the eye, disappearance of the nasolabial fold, and mouth drawn to the unaffected side.
-May also have decreased tearing, hyperacusis, and/or loss of taste sensation on the anterior two-thirds of the tongue
-Onset is acute, over a day or two; the course is progressive, reaching maximal clinical weakness within three weeks
-Recovery or some degree of function is present within six months
- Can give steroids to help hasten recovery

22
Q

bells palsy dx

A

-consider serologic testing for LYME disease if indicated

23
Q

bells palsy ddx

A

Lyme disease
Sarcoidosis
HIV infection
Herpes zoster, Epstein Barr virus infection
Otitis media
Guillain-Barré syndrome
Sjögren syndrome
Tumor

24
Q

bells palsy prognosis and tx

A

Prognosis: related to severity of lesion.
-Clinically incomplete lesions tend to recover.
-Prognosis favorable if some recovery is seen within the first 21 days.
-*In severe cases, the cornea may be at risk bc of poor eyelid closure and reduced tearing!!!
-!Eye patch at night, Artificial tears, Lacrilube ointment to lids

Treatment: early treatment (w/i 3 days)
-!!Prednisone 60 to 80 mg/day for one week.
-For severe facial palsy at presentation, early combined therapy with Prednisone plus Valacyclovir (1000 mg three times daily for HSV) for one week.
-Follow-up is essential for eye care, (avoid exposure keratopathy), and psychological support

24
Q

Diabetic neuropathy: managment

A

There is no cure for diabetic neuropathy, but treatment can improve painful symptoms and prevent complications:
-Control blood sugar levels
-Lifestyle changes (esp diet and exercise)
-Foot care

Pain control for neuropathic pain:
- Anti-seizure meds (Pregabalin!!!! [1st line], Gabapentin!,)
- TCAs (Amitriptyline!)
- SSRI (Duloxetine)

25
Q

74 yo man with DM with pain over RIGHT eyebrow x 3 days and diplopia on looking to LEFT.
Exam shows partial deficit in adduction of RIGHT eye, and inability to supraduct or infraduct (look up or down) left eye.
Pupils are symmetric and reactive.

A

Eye is down and out: other muscles are working
- Ptosis: CN III raises eyelid
- Isolated CN III: worried about brain compression: trauma, bleed, aneurysm, tumor
- Can also be DM!!! Vasculopathy - more painful
- Spared pupil with vasculopathy: tends to affect the inside of the nerve and pupillary tends to run on the outside and has collateral circulation
- Helps guide dx

26
Q

oculomotor nerve palsy

A

-Oculomotor nerve palsies often present with ptosis and a “down and out eye”
-Is it an “ischemic” or “medical” 3rd nerve palsy (3NP)?
-Dilated and nonreactive: indicates a space-occupying lesion/posterior communicating artery aneurysm compressing CNIII (due to compression of external parasympathetic fibers)
-Spared pupil indicates ischemic/inflammatory VASCULOPATHY: pupillary fibers run externally on nerve and have more collateral blood supply, making them less susceptible to ischemia than central nerve fibers
-Consider microvascular vasculopathy from DM (this will usually be painful)

27
Q

mononeuropathies definition and MC type; sx

A

-Damage or dysfunction of a singular peripheral nerve
-Most common: Carpal tunnel syndrome, cubital tunnel syndrome, peroneal nerve
-Causes: nerve compression against a tight or constrictive surface, entrapment of nerve in a narrow space, repetitive actions causing trauma to nerve, infections, radiation, cold

Symptoms:
-Sensory: Numbness, tingling, pain!!
-Motor: Weakness, loss of coordination, long standing can cause atrophy!!

28
Q

carpal tunnel syndrome: overview

A

Most frequent compressive focal mono neuropathy
-Prevalence : 1-5%; F > M (3:1)
-Risk factors: obesity, female
-Associated conditions: diabetes, pregnancy, rheumatoid arthritis, hypothyroidism, connective tissue diseases!!
-Role of repetitive hand/wrist use and workplace factors in the development of CTS is controversial

sx:
Pain or paresthesia’s (numbness and tingling) in median nerve distribution (first 3 ½ fingers)
-Symptoms typically worse at night;
-Typical hx: shakes hand out on awakening to help with pain
-Sx may be at wrist, entire hand, or radiate proximally to as high as the shoulder.
-Sx often provoked by activities that involve flexing or extending the wrist.
-severe CTS: weakness or clumsiness of hand.

29
Q

carpal tunnel syndrome: motor/sensory exam

A

Motor exam: normal
-Late stages: weakness of thumb abduction and opposition, and atrophy of the thenar eminence.

Sensory exam: deceased sensation in median distribution!, but spares the thenar eminence (palmar sensory cutaneous nerve arises proximal to the wrist and passes over, rather than through, the carpal tunnel)

Nerve conduction studies (NCS):
- slowed conduction velocities across the carpal tunnel and normal conduction elsewhere.

EMG: r/o other causes: polyneuropathy, plexopathy, and radiculopathy

30
Q

test for nerve entrapment neuropathy

A

-Test sensation of the nerves:
-INDEX FINGERPAD = Median nerve
-DORSAL WEBSPACE OF THUMB/INDEX = Radial nerve
-PINKY FINGERPAD = Ulnar nerve

31
Q

tinel’s test

A

+ if shooting, aching, worsening numbness with repeated tapping

Try to reproduce numbness and tingling -> 60% sensitive, still used test

32
Q

carpal tunnel tx

A

-NSAIDs are first line*
-Volar wrist splint nightly: Neutral position

severe:
-Glucocorticoid injections
-Surgical decompression

33
Q

cubital tunnel: def, sx, test

A

Ulnar nerve mononeuropathy - fingerpad of pinky

Symptoms:
- numbness and tingling of the ulnar side of the hand, decreased grip strength
-SEVERE: Clawing deformity of 4th and 5th digits

Tinel test: Tap the ulnar nerve over the medial epicondylar groove to reproduce symptoms of paresthesias

34
Q

saturday night palsy findings

A

Radial nerve palsy from compression in the axilla from sleeping on a chair (alcohol), crutch use, or “honeymoon” palsy

EXAM:
- Weakness causing WRIST DROP (inability to extend the finger, thumb, wrist) and numbness over the 1st dorsal interosseous

35
Q

phalen maneuver

A
36
Q

Carpal tunnel syndrome: DDx what other ds mimics it?

A

Ddx: Cervical radiculopathy, (C6 or C7 nerve root involvement commonly mimics CTS)

Cervical radiculopathy :
- neck pain, the exacerbation of sx with neck movement
- radiation of pain from the neck into the shoulder and arm
- reduced reflexes mediated by the C6/C7 nerve roots (ie, biceps, brachioradialis, and triceps)
- weakness of proximal arm muscles involving elbow flexion, extension, and arm pronation, and sensory loss in the palm or forearm outside the region of sensory abnormalities caused by CTS.

CTS Symptoms: Localized to the hand, affecting the median nerve distribution (excluding the thenar eminence).

Cervical Radiculopathy Symptoms: Broader, involving the neck, shoulder, and upper arm.