Neuromuscular Diseases Flashcards

(42 cards)

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
Inflammatory Demyelinating Polyneuropathies
-cause weakness, sensory loss, and areflexia Rapid progression -Guillain-Barre -ends by 4th weeks Slow progression -CIDP -weakness proogressing >2mon
26
Guillain-Barre: Progression
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
Guillain-Barre: Diagnosis
-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
Chronic Inflammatory Demyelinating Polyneuroathy
-CIDP -Male>female -relapsing -remitting (2/3), progressive course (1/3) -slowly progressive -Motor>sensory, proximal and distal -hypo or areflexic
29
Diabetic Neuropathies
-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
Diabetic Amyotrophy (length dependent)
-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
Hereditary Neuropathies
-insidious onset, <20yrs 1. Charcot-marie-Tooth disease 2. Hereditary Sensory Autonomic Neuropathy 3. Amyloid: abnormal protein 4. HNPP: liability to pressure palsies
32
Chatcot-Marie-Tooth
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
Neuromuscular Junction Disorders
Presynaptic: -lambert eaton -Botulism Post-Synaptic: -myasthenia gravis
34
Myasthenia Gravis
-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
Myopathies
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
Hereditary Myopathies
-muscular dystrophies -congenital myopathies -metabolic/mitochondrial -channelopathies
37
Acquired Myopathies
-toxic/drug induced -endocrine -inflammatory -systemic illness
38
Inflammatory Myopathies
Polymyositis Dermatomyositis -steroids and chemo
39
Inclusion Body Myositis
-slowly progressive weakness -proximal leg and distal arm (hands in fists) -asymmetric -muscle atrophy -dysphagia
40
Myotonic Dystrophy Type 1
-sustained contracture of muscles
41
Oculopharyngeal MD
-presents likes myastenia gravis -does not return to function
42
Emery-Dreifuss MD
-early contractures