Neuromuscular Flashcards

Question

Huntington's Disease

Answer 1

DNS degenerative disease characterized by marked caudate nucleus atrophy & putamen & globus pallidus to lesser degree Autosomal dominant

Answer 2

Progressive dementia Chorea (involuntary jerking or writhing movement) Tremors Rigidity & contractures Depression, aggressive outburst, mood swings Difficulty w/ speech & swallowing

Answer 3

Treat the choreiform movements Antidepressants (Haldol) Physical, occupational, & speech therapy

Answer 4

``` Aspiration risk → RSI Prolonged response to Succinylcholine Sensitive to NDMR Avoid anticholinergics & Reglan Glycopyrrolate > Atropine ```

Answer 5

``` Myasthenia gravis Lambert-Eaton Muscular dystrophy Myotonic dystrophy Mitochondrial disorders Guillain-Barre Spinal muscular atrophy ```

Answer 6

Autoimmune destruction IgG antibodies against nAChR Inactivation postsynaptic ACh receptors at NMJ ↓receptors & function degradation complement-mediated damage to postsynaptic end plate

Answer 7

``` Diploplia & ptosis Fluctuating fatigue & weakness that improves after rest Mouth & throat muscle weakness Dyspnea w/ exertion Proximal muscle weakness ```

Answer 8

``` Cholinesterase inhibitor (Pyridostigmine) Plasmapheresis Corticosteroids Immunosuppressants & immunoglobins Thymectomy ``` S/S exacerbated by pregnancy, infection, electrolyte imbalance, surgical & psychological stress, aminoglycoside antibiotics

Answer 9

``` Aspiration risk Sensitive to NDMR Regional preferred (amide LAs) Avoid block > mid thoracic or interscalene or supraclavicular block d/t phrenic nerve paralysis RESISTANT to Succinylcholine 2mg/kg DOA ↑5-10min ```

Answer 10

Pre-synaptic defect Antibodies attach to voltage-gated Ca2+ channels on the nerve terminal ↓ACh release at the motor end plate Affects males > females

Answer 11

Proximal muscles 1° affected Weakness generally worse in the morning & improves throughout the day Respiratory & diaphragm muscles become weak ANS dysfunction - Orthostatic hypotension - Delayed gastric motility - Urinary retention

Answer 12

``` 3,4 DAP Guanidine hydrochloride Corticosteroids Immunosuppressants Plasmapheresis ```

Answer 13

Sensitive to Succinylcholine & NDMR Inadequate reversal w/ AChE High risk postop respiratory failure 60% small cell carcinoma

Answer 14

``` X-linked recessive disorder Results from abnormal protein dystrophin production Affects males > females Presents between 3-5yo Rarely live past 30yrs ```

Answer 15

Symmetric proximal muscle weakness manifested as gait disturbance "Gower sign" Fatty infiltration typically causes muscle enlargement (particularly the calves) Kyphoscoliosis Respiratory muscle weakness Cardiac muscle degeneration Impaired GI hypo-motility, airway reflexes, &cardiac conduction Cognitive impairment Pulmonary HTN

Answer 16

X-linked recessive disorder Partial dystrophin Less common than Duchenne

Answer 17

``` Present later in life (adolescence) Disease progresses more slowly Less severe Intellectual disability less common Proximal muscle weakness Prominent calf pseudohypertrophy Cardiac muscle degeneration Death r/t respiratory complications ```

Answer 18

Genetic testing ↑CK levels Muscle biopsy (rare)

Answer 19

``` Surgery Physical therapy Steroids delay disease progression Biphosphates Mystatin inhibitors Gene modification Protease inhibitors Stem cell infusions ```

Answer 20

Pre-anesthesia testing PFTs, echo, EEG, sleep apnea study Avoid Succinylcholine & volatile anesthetics Malignant hyperthermia syndrome characterized by rhabdomyolysis & hyperkalemia Avoid preop w/ benzodiazepines & opioids Positioning complications d/t kyphoscoliosis Sensitive to NDMR - prolonged DOA Local & regional anesthesia preferable Aspiration risk

Answer 21

Hereditary skeletal muscle degenerative disease Results in dysfunctional Ca2+ sequestration by SR Na+ & Cl¯ channel dysfunction

Answer 22

Weakness - facial, thoracic, intercostal, diaphragm, sternocleidomastoid, & distal limb Inability to release/relax hand grip (myotonia) Cardiomyopathy Conduction defects 1st degree AV block Dysphagia & slowed gastric emptying Endocrine dysfunction Central sleep apnea Ptosis Males (triad) - frontal balding, cataracts, & testicular atrophy

Answer 23

``` Procainamide Phenytoin Mexiletine Baclofen Dantrolene Carbamazepine Cardiac pacemaker ```

Answer 24

Avoid Succinylcholine Aspiration risk Volatile anesthetics may produce exaggerated myocardial depression High volatile anesthetic concentrations potential to abolish myotonia Questionable d/t association w/ MH Anesthesia & surgery could aggravate cardiac conduction problems via ↑vagal tone Neostigmine & Physostigmine aggravate myotonia Sensitive to respiratory depressant Maintain normothermia & avoid shivering PFTs & EKG Transthoracic pacing readily available

Answer 25

Heterogenous groups Skeletal muscle energy metabolism disorders Mitochondria produce energy required by skeletal muscle cells through the oxidation-reduction reactions of the electron transport chain & oxidative phosphorylation → ADP

Answer 26

Abnormal fatigue w/ sustained exercise Skeletal muscle pain & progressive weakness Hearing loss & impaired vision Balance & coordination problems Seizures Learning deficits Organ problems - heart, liver, kidney, brain

Answer 27

``` Symptomatic Admin metabolites & cofactors Sodium bicarbonate Dichloroacetate Ketogenic diet ```

Answer 28

``` Prone to acidosis & dehydration Lactate level (avoid LR) Avoid continuous infusion Propofol Avoid Succinylcholine Maintain normothermia Use NDMR & LAs w/ caution Avoid Bupivacaine Avoid prolonged tourniquets ```

Answer 29

Immunologic assault on myelin in the peripheral nerves particularly lower motor neurons AP unable to be conducted Motor endplate does not receive the incoming signal Upward progression 2wks Full recovery 4wks w/ some permanent paralysis remaining

Answer 30

``` Flaccid paralysis Begins in distal extremities & ascends bilaterally Intercostal muscle weakness Facial & pharyngeal weakness Sensory deficits Autonomic dysfunction common Cognition intact ```

Answer 31

Plasmapheresis IVIG Steroids not useful

Answer 32

``` Avoid Succinylcholine NDMR sensitivity ↑DVT risk Aspiration risk Exaggerated response to indirect sympathomimetics (A-line) Assess Na+ levels Slow position changes Avoid barbiturates → CV depression (autonomic dysfunction) Postop ventilation ```

Answer 33

Deletions or mutations in survival motor neuron gene on chromosome 5q13 SMN gene involved in RNA complex formation & trafficking out nucleus Loss SMN function promotes apoptosis lower motor neurons - affects anterior spinal cord horn

Answer 34

``` Most severe Infantile spinal muscular atrophy Autosomal recessive disorder Manifests w/in 3mos Rapidly progressive leading to death from respiratory complications usually by age 3 ``` Difficulty sucking, swallowing, breathing, atrophy & fasciculation in tongue & limb muscles

Answer 35

Autosomal recessive inheritance Begins 6mos-1yo Progressive more slowly than infantile form Able to survive into adulthood

Answer 36

Juvenile form Develops after age 2 Weakness proximal limb muscles w/ relative sparing bulbar muscles (cranial nerves)

Answer 37

``` Spinraza Zolgensma 2019 Evrysdi 2020 PT Surgery Antibiotics ```

Answer 38

Pulmonary consultation Difficult intubation d/t secretions, muscle weakness, contractures, & aspiration risk Avoid Succinylcholine Varying sensitivity to NDMR (longer DOA) Regional controversial - neuraxial difficult/unreliable Cautious w/ opioids Postop respiratory support

Answer 39

Mixed motor neuron disease - upper & lower motor Rapidly progressive motor neuron degeneration in corticospinal tract (1° descending upper motor neurons) & lower motor neurons in anterior spinal cord gray matter Astrocytic gliosis replaces the affected motor neurons

Answer 40

``` Spasticity Hyperreflexia & loss coordination Muscle weakness Fasciculations Atrophy often begins in hands Orthostatic hypotension Resting tachycardia Sensation remains intact Cognition WNL Bladder & bowel function not typically affected ```

Answer 41

``` Riluzole (NDMA receptor antagonist) - Only drug that reduces mortality Edaravone ↓ADLs decline Spasmolytics Analgesics ```

Answer 42

``` Avoid Succinylcholine Hyperkalemia risk ↑NDMR sensitivity Aspiration risk Consider postop mechanical ventilation ↑sensitivity to respiratory depressants Autonomic dysfunction w/ hemodynamic instability risk (A-line) Avoid spinal anesthesia d/t demyelination Induction → PEA ```

Neuromuscular Flashcards

(66 cards)