Neuromuscular Diseases Flashcards
A-Alpha Fibers
-1a (spindle) and 1b (GTO) (sensory)
-fastest
-large motor axons
-Proprioception: muscle stretch (spindle) and tension (GTO)
A-Beta Fibers
-II (sensory)
-touch, pressure, vibration, joint position
A-Gamma Fibers
-II (sensory)
-gamma efferent motor axons
-intrtafusal fibers
-motor and muscle spindles
A-Delta Fibers
-III (sensory)
-sharp pain, light touch and temp
B Fibers
-III (sensory)
-SNS preganglionic motor axons
C Fibers
-IV (sensory)
-slowest
-dull aching pain, temp and sensation
ALS
-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
ALS Diagnostics
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: ^^^
ALS Symptoms
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
West Nile Virus
-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)
Acute Flaccid Paralysis
-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
Poliomyelitis
-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
Post-Polio Syndrome Diagnostic Criteria
- Hx Paralytic poliomyelitis
- Partial or complete recovery
- Onset of persistent muscle weakness
- Symptoms for >1yr
- Exclude other cases
Post-Polio Syndrome
-Lower motor neuron syndrome
-joint pain w/ cold intolerance
-may have a new neurologic problem unrelated polio
Radiculopathy
-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
Polyradiculopathy
-multiple spinal nerve roots
S/s:
-motor s/s predominate, some sensory loss, pain, loss of DTR
Causes:
-inflammatory diseases, DM, infectious, neoplastic
Plexopathy
-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
Mononeuropathy
-neuropathy of a single peripheral nerve
S/s:
-nerve distribution
Cause:
-compression, entrapment, trauma, rarely ischemia
Ex:
-carpal tunnel
-meralgia parathetica (britany spears)
Polyneuropathy
-peripheral nerves
S/s:
-symmetric, length dependent weakness, sensory loss, Loss of DTRs
-Stocking Glove distribution
-Diabetes (Most Common)
-thyroid disease
-toxic
-infections
Axonal Polyneuropathy
-killing the axon
-grows slowly
S/s:
-Distal>proximal
-Leg>arms
-Muscle wasting
Ex:
-Diabetes
-Toxicity
-Alcohol
Demyelinating Polyneuropathy
-weakness without atrophy
-length dependent
-patchy
-asymmetric
Ex:
-compression/stretch
-Guillain-Barre
-Charcot-Marie-Tooth
Small Fibers Neuropathy
-reduced ability to feel pain and temp
-allodynia
-neuropathic pain
-discoloration of skin
-autonomic dysfunction
Large Fibers Neuropathy
-numbness, tightness, balance
-walking on foam
Autonomic Neuropathy
-involuntary
S/s:
-orthostatic hypotension
-arrhythmias
-constipation, urinary retention, erectile (1st sign)
-abnormal sweating
-gastroparesis
Inflammatory Demyelinating Polyneuropathies
-cause weakness, sensory loss, and areflexia
Rapid progression
-Guillain-Barre
-ends by 4th weeks
Slow progression
-CIDP
-weakness proogressing >2mon
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%)
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
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
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
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
Hereditary Neuropathies
-insidious onset, <20yrs
- Charcot-marie-Tooth disease
- Hereditary Sensory Autonomic Neuropathy
- Amyloid: abnormal protein
- HNPP: liability to pressure palsies
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
Neuromuscular Junction Disorders
Presynaptic:
-lambert eaton
-Botulism
Post-Synaptic:
-myasthenia gravis
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
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
Hereditary Myopathies
-muscular dystrophies
-congenital myopathies
-metabolic/mitochondrial
-channelopathies
Acquired Myopathies
-toxic/drug induced
-endocrine
-inflammatory
-systemic illness
Inflammatory Myopathies
Polymyositis
Dermatomyositis
-steroids and chemo
Inclusion Body Myositis
-slowly progressive weakness
-proximal leg and distal arm (hands in fists)
-asymmetric
-muscle atrophy
-dysphagia
Myotonic Dystrophy Type 1
-sustained contracture of muscles
Oculopharyngeal MD
-presents likes myastenia gravis
-does not return to function
Emery-Dreifuss MD
-early contractures
