POLYNEUROPATHIES Flashcards

1
Q

What is triad of polyneuropathies?

A

Stocking glove distribution, hyporeflexia, distal weakness

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2
Q

Are most poly’s axonal or demyelinating?

A

Axonal; length dependent/distal to proximal gradient

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3
Q

What type of gradient do demyelinating polys have?

A

proximal to distal ie GBS, IDP, porphyria and DM polyradiculopathy

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4
Q

What are some signs of demyelinating poly?

A

Global areflexia/hyporeflexia, hypertrophic nerves and mm weakness w/o atrophy

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5
Q

What reflexes are lost usually with axonal poly?

A

Normally only the ankle jerk

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6
Q

What are some acquired demyelinating polys?

A

GBS/AIDP, CIDP, MMN, HIV, Diptheria and toxic

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7
Q

What are NCS findings of acquired demyelinating polys?

A

Patchy demyelination with conduction block and increased temporal dispersion; occur in noncommon entrapment sites. ie: forearm

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8
Q

What are some inherited demyelinating polys?

A

HSMN/CMT and HNPP- uniform slowing

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9
Q

What could be causing assymetric findings on NCSs?

A

mononeuropathy multiplex (vasculitis) or CIDP

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10
Q

Describe GBS/AIDP.

A

rapid ascending paralysis, distal paraesthesias in fingers and toes same time, hypo/areflexia, bulbar weakness (dysarthria,dysphagia)

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11
Q

What is the best prognostic indicator for GBS/AIDP?

A

Distal CMAP at 3-4wks; <20% LLN=poor prognosis

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12
Q

Describe CIDP clinical findings.

A

Acquired, 50-60yo, prox and distal mms, slow progressive wks to months, gait ataxia; +Rhomberg, areflexia/hyporeflexia,

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13
Q

Describe CIDP NCS/EMG findings.

A

decreased CV with prolonged latencies, assymetric CB/temporal dispersion. EMG- decreased recruitment with large MUAPs, +paraspinals, will improve with plasma xchange or corticosteroids

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14
Q

Describe GBS/AIDP findings.

A

Delayed F-waves and sural sparing (abnormal median w/normal sural)

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15
Q

Describe multifocal motor neuropathy MMN.

A

progressive assymetric weakness and atrophy;

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16
Q

What are some medical condition polys?

A

Diabetic, Uremic(renal), ETOH and malignant

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17
Q

What are some inflammatory or infectious polys?

A

GBS, leprosy/Hansen’s disease, Diptheric

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18
Q

What are some metabolic polys?

A

toxic (lead, arsenic and chemo)

19
Q

What are some inherited polys?

A

CMT/HSMN and HNPP

HNPP is hereditary but has multifocal multiplex pattern…no uniform slowing in all segments like CMT

20
Q

What are the demyelinating polys of CMT/HSMN?

A

CMT I, CMT III/Dejerene Sottas, CMT IV/Refsum’s disease

21
Q

What is another name for CMT III and who does it affect?

A

Dejerine Sottas—-infants

22
Q

What is another name for CMT IV and what does it affect?

A

Refsum’s disease—ataxic neuro

23
Q

What are axonal polys of CMT/HSMN?

A

CMT II and CMT V-Spinocerebellar degeneration

24
Q

Poly Edx protocol is?

A

3 or more limbs, 2 motor, 2 sensory, 2 F-waves each limb, EMG all 4 limbs prox and distal

25
Q

Uniform demyelinating, mixed sensorimotor polys

A

CMT I, III and IV

26
Q

Segmental demyelination, motor>sensory polys

A

GBS/AIDP, CIDP or HNPP

27
Q

Axon loss, motor>sensory polys

A

GBS, CMT II and V, lead and vincristine

28
Q

Axon loss, mixed sensorimotor polys

A

ETOH, RA, SLE, metals (arsenic, gold, silver), AIDS

29
Q

Mixed axon loss, demyelinating polys

A

DM and uremic(renal)

30
Q

Describe ETOH neuropathy.

A

insidious onset of slow progressive sensorimotor poly

31
Q

Describe clinical findings of ETOH neuropathy.

A

N/T, paraesthesia/burning pain/ reduction to all sensory modalities in stocking glove distribution, worse in LE vs UE, AJ absent

32
Q

Describe NCSs/EMG findings in ETOH neuropathy.

A

CMAP tib/peroneal, Sural SNAP- decreased amp and CV EMG- distal lower limb; fibs and decreased recruitment

33
Q

Describe Polydiabetic neuropathy/Distal symmetric sensory polyneuropathy (DSPN).

A

Length dependent; sensory loss toes-legs-fingers-proximal, stocking glove/sensory loss to all modalities

34
Q

Describe NCSs/EMG findings of polydiabetic neuropathy.

A

Snaps Key! decreased CV, amplitudes, sural/plantar/H-reflex, CMAPs slow EMG- decreased MUAP recruitment

35
Q

DM amyotrophy=’proximal DM neuropathy’=diabetic polyradiculoneuropathy

A

Older pnts with type II DM, prox LE assymetric weakness may have had severe wt loss starts unilaterally w/severe pain in back, hip and thigh f/b days-wks atrophy and weakness of proximal and distal mms of affected leg

36
Q

What are EMG findings of DM amyotrophy/diabetic polyradiculoneuropathy?

A

SNAPs absent or decreased amplitude, CMAP decreased in affected mms; CV normal or mild slow EMG-fibs in femoral/obturator and paraspinals with decreased recruitment

37
Q

Describe amyloid neuropathy aka family amyloid polyneuropathy ‘FAP’.

A

protein ‘amyloid’ deposited in tissues and nerves (this causes nerve to degenerate) affects peripheral sensory, motor and autonomic protein deposits in endoneurium leads to axonal loss and demyelination

38
Q

What are NCSs/EMG findings of amyloid neuropathy?

A

SNAPs decreased amps with mild slow CV, CMAP less affected. EMG- fibs in weak distal mms with decreased recruitment

39
Q

What is the treatment for multifocal motor neuropathy?

A

IVIG, does not respond to plasma xchange or predisone

40
Q

Describe HNPP.

A

Can be assymetric, gene disorder of chromosome 17/PMP 22, which causes decreased myelin formation. These patients are susceptile to pressure palsies.

Have prolonged latencies and decreased CVs across common entrapment sites.

41
Q

Differeniate between CIDP and MMN.

A

MMN you have asymetric findings only in motor nerves. Typically in the hand but could involve one foot or one hand.

CB and temporal dispersion across non common entrapment sites.

42
Q

What does it mean if you find demyelination across a non common entrapment sites?

A

Red flag make you think that the immune system is attacking the nerve which is causing the demyelination.

Possible its an autoimmune issue, cause normal people don’t get demyelination at non common entrapment sites.

43
Q

What is this type of distribution and what type of polyneuropathy is it most common to?

A

Stocking glove distribution and most common in axonal ‘length dependent’ polyneuropathies

44
Q

What is this type of distribution and what disorders would have this presentation?

A

Multiplex distribution which is assymetric.

Mononeuropathy Multiplex and Multifocal Motor Neuropathy