VERIFIED ANSWERS/ GET IT 100% ACCURATE
ALS - correct answer- Amyotrophic—atrophy of the
muscle fibers
Lateral sclerosis—hardening of the anterior and lateral
corticospinal tracts as motor neurons degenerate, replaced by
gliosis
General
◾Progressive lose ability to speak, swallow, move extremities,
and use respiratory muscles
◾Involves both UMNs and LMNs, characterized by muscle
wasting and progressive paralysis as a result of destruction of
motor neurons in the brainstem and in the anterior horn of the
spinal cord
-begins in the brain at the cfrontal lobe for motor function to
dorsal horn?
Lower motor neuron- Anterior horn cells to ...
◾Some muscles become weak and atrophic, others with
spasticity and hyperreflexia
◾Increased incidence of cognitive and behavioral changes 2/2
frontal lobe changes
◾Death most often 2/2 respiratory failure
,-Chest wall the issue with ALS not alveoli
Pathophysiology
◾Genetic mutation that mediates motor neuron degeneration;
question of environmental influences
◾Marked degenerative changes in anterior horn cells of the
spinal cord, motor nuclei in the brain stem (especially CN VII and
XII), corticospinal tracts, and areas of frontal cortex
Cranial nerve- facial (7), hypoglossal (12)
Corticospinal tract- starts on cortex, ends in the spine
ALS S&S - correct answer- 2 main categories:
◾Limb onset
◻ Focal motor weakness UE or LE, start with muscle twitching or
cramps
◻ UMN degeneration ⟶ spasticity, hyperreflexia, decreased
strength
◻ LMN degeneration ⟶ flaccidity, paralysis, muscle atrophy
◻ Eventual weakness/atrophy usually asymmetrical, includes
respiratory
◻ B&B not affected directly, but constipation and urgency occur
,◾Bulbar onset—weakness of muscles innervated by motor
nuclei of
brainstem (muscles of jaw, face, tongue, pharynx, larynx)
◻ Problems speaking, swallowing, dysphagia, managing oral
secretions
◻ Nocturnal hypoventilation, dyspnea, orthopnea
◾Cognitive/behavioral symptoms—frontal lobe involvement,
deficits in
◻ Attention, concentration, executive function, pseudobulbar
affect (PBA)
-Pseudobulbar affect- cry for no reason, cry almost hysterically.
Arthropod-Borne - correct answer- ◾Transmitted by
mosquitoes, ticks, flies
◾WEE, EEE, WNV all spread by mosquitoes
Western equine encephalitis (WEE)
Eastern equine encephalitis (EEE)
West nile virus (WNV)
◾Degenerative changes in neurons + inflammation
◾Seasonal prevalence late summer/early fall
◾Arthropod ⟶ non-human host ⟶ arthropod ⟶ human
, ◾Incubation 3-8 days
◾Present with flu-like symptoms: fever, HA, listlessness, N/V;
generally improve over a few weeks; some may present with
signs of meningitis
◾WNV, EEE attack anterior horn cells ⟶ flaccidity
◾Diagnostics same as for meningitis/encephalitis
◾No specialized treatment, supportive and symptomatic
◾May need control cerebral edema, anticonvulsants, analgesics,
antipyretics
-Treat symptomatically
Autonomic Symptoms/Intervention Strategies - correct
answer- Cardiovascular
◾Neurogenic orthostatic hypotension
-drop in 20 mm Hg in systolic blood pressure, 10 in diastolic.
Dizzy when they stand up
◾SNS baroreceptors affected by Lewy bodies, preventing normal
vasomotor tone response
◾Target to treat the dizziness, blurry vision
Interventions:
◾Educate patient/caregiver to manage orthostasis