Lecture: Neuromuscular Syndromes Flashcards

1
Q

Motor unit

A
  • Lower Motor Neuron
  • Neuromuscular Junction
  • Muscle
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2
Q

Maintenance of motor unit

A
  • Maintenance by tonic inputs from LMN
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3
Q

Denervation atrophy

A

rapid and severe (5-7 days)

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4
Q

LMN signs

A
  • Paresis to plegia
  • Muscle atrophy
  • poor/loss muscle tone
  • weakness to loss of reflexes
  • exercise intolerance
  • paretic, stiff gait
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5
Q

Ataxia and LMN dz

A
  • ataxia is an ulikely sign of LMN dz
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6
Q

LMN DZs

Tetanus

A

Happens at level of spinal cord

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7
Q

DZs of LMN

Nerve root

A

Polyradiculoneuritis

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8
Q

DZs of LMN

Nerve

A
  • (poly)Neuropathies
    • Axon
    • Myelin
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9
Q

DZs of LMN

Junction

A
  • Tick Paralysis
  • Botulism
  • Myasthenia Gravis
  • Snakes
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10
Q

DZs of LMN Muscle

A
  • Inflammatory
  • Infectious
  • Neoplastic
  • Snakes
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11
Q

Polyradiculoneuritis

Coonhound Paralysis, Guillan-Barre syndrome

A
  • Poly = multiple
  • Radiculo = root
  • Neuritis = inflammation of the nerve
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12
Q

Polyradiculoneuritis

About

A
  • Racoon saliva?
  • Demyelinating dz - rapid recovery
    • can get distal axonal degeneration
  • Root & proximal nerve disorder
  • mildly delayed nerve conduction velocity (NCV)
  • Albuminocytologic dissociation on CSF
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13
Q

Polyradiculoneuritis

CS

A
  • Acute, ascending flaccid paralysis
    • LOSS of reflexes and tone
    • motor, not sensory
    • atrophy
  • CN deficits common
    • CN VII, gag
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14
Q

Polyradiculoneuritis

TX

A
  • Variable dz course
  • may require ventilation
  • supportive care
  • aggressive physical therapy to avoid joint fibrosis
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15
Q

Polyneuropathy

A
  • Degenerative
  • Paraneoplastic
  • Endocrine
  • Genetic/heritable
  • Infectious
  • Toxic: Vincristine (microtubule disruptor)
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16
Q

Degenerative Polyneuropathies

Labrador retrievers

A
  • 10+ yo
  • Laryngeal paresis/paralysis
  • Dysphonia (bark change)
  • Esophageal dysfunction
  • Paresis worse in pelvic limbs
  • abnormal gait

*affects myelin & axons

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17
Q

Inherited polyneuropathy

A

Leonbergers

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18
Q

Endocrine Neuropathies

A
  1. Paraneoplastic - insulinoma
  2. Hypothyroidism
  3. Diabetic neuropathy - feline
    • plantigrade posture
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19
Q

Tick Paralysis

A
  • Presynaptic neuromuscular blockade
  • Dermacentor & Amblyomma most common
  • Dogs flaccid 5-9 days after tick attachement
    • cats resistent
  • +/- megaesophagus
  • In Australia
    • Ixodes holocyclus, Argasid ticks
    • MUCH Worse
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20
Q

Tetanus

about

A
  • Tetanospasmin must be cleaved to be activated
    • absorbed at the NMJ and travels retrograde to spinal cord
  • Inhibits glycine/GABA release at SPINAL CORD
    • leads to hyperactive alpha motor neurons = constant muscle contraction
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21
Q

Tetanus

CS

A
  • 5-10 days after infection
  • increased tone, stiffness, risus sardonicus, elevated tail, sawhorse stance, elevated nictitans
    • Extensors are stronger than flexors
  • external stimuli may exacerbate spasticity
22
Q

Tetanus

TX

A
  • Vaccination
  • Tetanus toxoid in horses
  • Antitoxin
  • Antibiotics and wound debridement
    • penicillin G, tetracycline, metranidazole
  • Sedatives
    • Phenothiazines, benzodiazepines, barbiturates
  • Supportive care
    • dark, quit, well padded, feeding tube
23
Q

Tetany

A
  • Hyperexcitability of membrane due to electrolyte imbalance
    • Na+, K+, Ca+, Cl-
    • Hypocalcemia
    • Hypomagnesemia
    • Strychnine
24
Q

Botulism

about

A
  • Presynaptic neuromuscular blockade
  • Ingest preformed toxin
    • destroyed at 100 degrees
    • resistant to proteases
  • Toxin imported, blocks presynaptic release of ACh from nerve terminal
25
Botulism CS
* 12 hrs-6 days * progressive, symmetric ascending paresis/paralysis * reflexes and tone are lost * CN VII, megaesophagus, decreased gag and jaw tone * +/- mydriasis, KCS, constipation, urinary retention
26
Botulism DX
* Very difficult * detection of organism in ingesta, serum, vomit
27
Botulism Prognosis
* Improvement in 1-3 weeks * must regenerate new motor endplates * 2-4 months in mammalian species
28
Myasthenia Gravis Congenital
* Insufficient receptors * Negative AChRAb titer * rare * jack/parsons russel terriers * signs from birth * generalized weakness
29
Myasthenia Gravis Acquired
* Autoantibodies against nicotinic acethylcholine receptors on post-synaptic membrane * receptor is blocked, causing muscle weakness
30
Acquired Myasthenia Gravis three forms
* Focal: typically esophageal muscle weakness * dogs only; cats have smooth muscle * Generalized: exercise intolerance * Fulminant: 1/4 of the generalized patients * Grave
31
Myasthenia Gravis CS
* Exercise intolerance * progressively stiff, stilted gait, crap walking, crouched * Postural rxns should be intact * Reflexes present/ withdrawels can tire * No CN deficits * may have weak gag, ptyalism, ptosis * Megaesophagus
32
Acquired Myasthenia Gravis Distribution
* Bimodal * osteosarc is also bimodal * \< 5 yrs old: immune mediated * \> 7 yrs old * para/pre-neoplastic * treat/remove primary source to ease tx
33
Myasthenia Gravis DX
* Tensilon test: edrophonium * ultra-short acting acetylcholinesterase inhibitor * **Acetylcholine receptor antibody titer** (GOLD STANDARD) * Electrodiagnostics: repetitive nerve stimulation (RNS)
34
Myasthenia Gravis TX
* Acetylcholinesterase inhibitor * increases ACh at the NMJ * Immunosuppression * stops Ab production * Treatment of underlying dz * eliminates trigger
35
Generalized Myopathy
* Immune-mediated * Infectious * neospora caninum, Hepatozoon americanum * Pre-/Para-neoplastic * lymphoma * Dermatomyositis * Congenital * muscular dystrophies
36
Generalized polymyositis
* Generalized weakness, stilted gait, dysphagia, muscle atrophy * Myalgia is rare * DO NOT LOSE REFLEXES * Boxers and Newfies * Boxers: preneoplastic * Newfies: breed specific
37
Masticatory myositis DX Acute Chronic TX
Type 2M and Type 1 variant muscle fibers * Autoantibodies to type 2M fibers (only expressed in masticatory mm) Serum 2M antibody titer (easy dx) Acute * pain opening mouth * mild exophthalmia Chronic * masticatory m atrophy * not painful to open mouth * fibrosis of masticatory mm TX * immunosuppression
38
Extraocular Myositis
Acute phase: ze eyeballz don't move
39
Neospora caninum
* Prenatal exposure * Polyradiculoneuritis and polymyositis * encephalomyelitis * Muscle atrophy * Contracture of Pelvic Limbs \*Spay the mom
40
Endocrine Myopathies
* Type 2 myofibers more affected * Hypothyroidism-dogs * Hyperthyroidism-cats * Glucocorticoid excess-dogs * Electrolyte derangements
41
Cushing's Myopathy
Stiff, not flexing, hypertrophic, stiff muscles
42
Cat anatomical anomaly
No nuchal ligament
43
Feline Hyperthyroidism
* T3 & T4 effects on sarcoplasm * CS * Paresis * ventroflexion * tremors * abnormal gait
44
Feline: Toxoplasma gondii
Brain and systemic infection
45
Rattlesnake Envenomation | (pit vipers)
* Mentation, paresis/plegia, rigidity, CP deficits
46
Southern Pacific, Timber, Western Diamondback envenomation
* Myokymia * Ca interference on nerve membrane * looks like a moving nerve under skin I think
47
Mojave rattlesnake envenomation
* Neurotoxins * inhibits Ach release at presynaptic terminal of NMJ leading to complete neuromuscular blockade
48
Coral Snake envenomation | (Elapid snake)
* Venom has many components * postsynaptic alpha-neurotoxins * neuromuscular blockade * Generalized LMN weakness * respiratory paralysis
49
Congenital myopathies
* Muscular dystrophy: golden retrievers * Myotonia congenita * Hyperkalemic periodic paralysis
50
Exercise Induced Collapse
* labrador retrievers * collapse, hyperthermia, loss patellar reflexes * DNM1 mutation
51
Myoclonus
* Sudden contraction-relaxation of muscles * LMN abnormality * Distemper