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Neuromuscular > Neuromuscular Diseases > Flashcards

Neuromuscular Diseases Flashcards

1
Q

A-Alpha Fibers

A

-1a (spindle) and 1b (GTO) (sensory)
-fastest
-large motor axons
-Proprioception: muscle stretch (spindle) and tension (GTO)

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2
Q

A-Beta Fibers

A

-II (sensory)
-touch, pressure, vibration, joint position

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3
Q

A-Gamma Fibers

A

-II (sensory)
-gamma efferent motor axons
-intrtafusal fibers
-motor and muscle spindles

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4
Q

A-Delta Fibers

A

-III (sensory)
-sharp pain, light touch and temp

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5
Q

B Fibers

A

-III (sensory)
-SNS preganglionic motor axons

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6
Q

C Fibers

A

-IV (sensory)
-slowest
-dull aching pain, temp and sensation

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7
Q

ALS

A

-amyotrophic lateral sclerosis
-progressive fatal disease: decreases breathing, anterior horn degeneration
-degeneration of Upper and lower motor neurons
-no cognitive loss
-Average life expectancy: 3yrs
-can be sporadic (MC) or inherited

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8
Q

ALS Diagnostics

A

LMNL: weakness, atrophy
-Brainstem: jaw/face, tounge atrophy
-Cervical: neck, arm, hand, diaphram
-Lumbosacral: back, abdomen, leg/foot

UMNL: spread of reflexes and clonus
-Brainstem: clonic jaw jerk, gag reflex, spaticity
-Cervical: DTRs, hoffmann, spatic tone
-Thoracic: loss of abdominal reflexes
-Lumbosacral: ^^^

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9
Q

ALS Symptoms

A

Fatigue/SOB
-BIPAP or ventilator

Muscle Stiffness/Weakness
-dyphagia (swallowing)
-breathing

Pseudobulbar Palsy
-laughing/crying disconnect

Mood

Sialorrhea
-lots of saliva

Speech Changes

Pain
-lack of movement
-positional
-cramps/contractures

Nutrition
-maintain muscle

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10
Q

West Nile Virus

A

-virus from mosquito (july-october)
-80% asym
-20% west nile fever: pain, headache, fever, rash
-<1% Neuroinvasive: aseptic meningitis, meningoencehalitis (brain and meninges, acute flaccid paralysis (B/B)

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11
Q

Acute Flaccid Paralysis

A

-after viral illness
-acute onset and rapid of asymmetric flaccid weakness
-hypoactive or absent reflexes
-pure motor
-respiratory insufficiency
-B/B dysfuntion separates from Guillain-Barre

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12
Q

Poliomyelitis

A

-enterovirus (no in US except for vaccination)
-mostly asymptomatic limited to gut

Minor diesease
-flu like

Major disease
-CNS invasion (anterior horn)
-<1%

S/s: muscle atrophy and weakness via anterior horn

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13
Q

Post-Polio Syndrome Diagnostic Criteria

A
  1. Hx Paralytic poliomyelitis
  2. Partial or complete recovery
  3. Onset of persistent muscle weakness
  4. Symptoms for >1yr
  5. Exclude other cases
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14
Q

Post-Polio Syndrome

A

-Lower motor neuron syndrome
-joint pain w/ cold intolerance
-may have a new neurologic problem unrelated polio

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15
Q

Radiculopathy

A

-nerve root compression
-bulge compresses root
-L5-S1(MC) & C7/C8

S/s:
-pain, numbness, and tingling in dermatome
-weakness of muscles in myotome
-reduced reflex

Tx:
-improve with time and PT
-most relapse
-surgery if not responding or weakness

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16
Q

Polyradiculopathy

A

-multiple spinal nerve roots

S/s:
-motor s/s predominate, some sensory loss, pain, loss of DTR

Causes:
-inflammatory diseases, DM, infectious, neoplastic

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17
Q

Plexopathy

A

-cervical or lumbosacral plexus

S/s:
-painful, restricted to single limb, more widespread than a single nerve

Causes:
-trauma most common, cancer (pancoast tumor), thoracic outlet

18
Q

Mononeuropathy

A

-neuropathy of a single peripheral nerve

S/s:
-nerve distribution

Cause:
-compression, entrapment, trauma, rarely ischemia

Ex:
-carpal tunnel
-meralgia parathetica (britany spears)

19
Q

Polyneuropathy

A

-peripheral nerves

S/s:
-symmetric, length dependent weakness, sensory loss, Loss of DTRs
-Stocking Glove distribution

-Diabetes (Most Common)
-thyroid disease
-toxic
-infections

20
Q

Axonal Polyneuropathy

A

-killing the axon
-grows slowly

S/s:
-Distal>proximal
-Leg>arms
-Muscle wasting

Ex:
-Diabetes
-Toxicity
-Alcohol

21
Q

Demyelinating Polyneuropathy

A

-weakness without atrophy
-length dependent
-patchy
-asymmetric

Ex:
-compression/stretch
-Guillain-Barre
-Charcot-Marie-Tooth

22
Q

Small Fibers Neuropathy

A

-reduced ability to feel pain and temp
-allodynia
-neuropathic pain
-discoloration of skin
-autonomic dysfunction

23
Q

Large Fibers Neuropathy

A

-numbness, tightness, balance
-walking on foam

24
Q

Autonomic Neuropathy

A

-involuntary

S/s:
-orthostatic hypotension
-arrhythmias
-constipation, urinary retention, erectile (1st sign)
-abnormal sweating
-gastroparesis

25
Q

Inflammatory Demyelinating Polyneuropathies

A

-cause weakness, sensory loss, and areflexia

Rapid progression
-Guillain-Barre
-ends by 4th weeks

Slow progression
-CIDP
-weakness proogressing >2mon

26
Q

Guillain-Barre: Progression

A

Acute Inflammatory Demyelinating Polyneuropathy

Progression:
1. Tingling, parasthesias
2. Severe radicular back pain
3. Weakness
-legs & arms (32%)
-legs then arms (56%)
-arms to legs (12%)
-facial involvement (70%)
-dysphagia (40%)

27
Q

Guillain-Barre: Diagnosis

A

-Acute Inflammatory Demyelinating Polyneuropathy
-symmetric weakness, facial
weakness
-sensory loss variable
-areflexia in after 1 week
-CSF

Tx:
-suportive care
-plasma exhange (IVIG)
-ROM, strengthening, transfers

28
Q

Chronic Inflammatory Demyelinating Polyneuroathy

A

-CIDP
-Male>female
-relapsing -remitting (2/3), progressive course (1/3)
-slowly progressive
-Motor>sensory, proximal and distal
-hypo or areflexic

29
Q

Diabetic Neuropathies

A

-65% of neuropathies (length dependent)
-distal and symmetrical (LE before UE)
-stocking glove pattern
-may produce atrophic apearance
-controlled by DM control

does not cause weakness

30
Q

Diabetic Amyotrophy (length dependent)

A

-Diabetic Lumbosacral Radiculoplexus Neuropathy
-cause weakness
-associated with large weight loss
-sharp aching pain in hip and thigh >leg
-Pain > weakness of thigh and knee > weakness distally

31
Q

Hereditary Neuropathies

A

-insidious onset, <20yrs

  1. Charcot-marie-Tooth disease
  2. Hereditary Sensory Autonomic Neuropathy
  3. Amyloid: abnormal protein
  4. HNPP: liability to pressure palsies
32
Q

Chatcot-Marie-Tooth

A

Hereditary Motor and Sensory Neuropathy

-group of genetic neuropathies

S/s:
-distal weakness and atropy
-length dependent sensory loss
-foot deformities: pes cavus, pes plannus, hammer toes
-decreased reflexes

Clinical Phenotypes:
-early onset of walking
-normal walking before 15mo
-slow runner
-s/s 5-20 years old
-onset of symptoms after 20

33
Q

Neuromuscular Junction Disorders

A

Presynaptic:
-lambert eaton
-Botulism

Post-Synaptic:
-myasthenia gravis

34
Q

Myasthenia Gravis

A

-autoimmune
-improvement with rest
-young women and older men

S/s:
-fluctuation, fatigueable
-asymmertic ptosis, diplopia
-chewing issues
-hoarseness
-limb weakness

Triggers:
-drugs
-heat illness
-emotional; updset
-heat
-menstral cycle

35
Q

Myopathies

A

Exam:
-proximal weakness
-sensation usually intact
-DTRs: normal or reduced

Positive Signs (added)
-myalgia
-cramps/contractures
-hyperthrophy
-stiffness

Negative Signs: (taken away)
-weakness
-fatigue
-atrophy
-exercise intolerance

36
Q

Hereditary Myopathies

A

-muscular dystrophies
-congenital myopathies
-metabolic/mitochondrial
-channelopathies

37
Q

Acquired Myopathies

A

-toxic/drug induced
-endocrine
-inflammatory
-systemic illness

38
Q

Inflammatory Myopathies

A

Polymyositis
Dermatomyositis
-steroids and chemo

39
Q

Inclusion Body Myositis

A

-slowly progressive weakness
-proximal leg and distal arm (hands in fists)
-asymmetric
-muscle atrophy
-dysphagia

40
Q

Myotonic Dystrophy Type 1

A

-sustained contracture of muscles

41
Q

Oculopharyngeal MD

A

-presents likes myastenia gravis
-does not return to function

42
Q

Emery-Dreifuss MD

A

-early contractures

Neuromuscular (21 decks)

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