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ALS with bulbar involvement (Diagnostic Testing (Maximum inspiratory…
ALS with bulbar involvement
Protein aggregates accumulate in UMN/LMN
Degeneration/ demyelination of the nerves
degeneration and loss of upper/lower motor neurons in brain and spinal cord
secondary degeneration of mm fibers
patchy distribution distal weakness
distal involvement > proximal
weakness of bulbar musculature
progressive inspiratory and expiratory muscle weakness
reduced lung and thorax compliance
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ineffective cough and airway clearance
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dysphagia
Aspiration PNA
dysarthria
Slurred speech, hoarseness
Thinning of the anterior and lateral spinal cord
As nerves continue to degenerate and die, muscles attached die
UMN signs and symptoms
Spasicity
hyperreflexia
pathological reflexes
LMN signs and symptoms
Fasiculations
hyporeflexia
muscle cramping
fatigue
weakness/atrophy
Decreased function
Increased falls
respiratory muscle weakness
orthopnea
hypoventilation
morning HA and lethargy
dyspnea
dysphagia
dysarthria
Diaphragm less innervated, begins to die
Oxidative stress, DNA damage, inability to repair DNA
Impaired oligodendrites and Schwann Cells
Astrocytes create excess damaging factors
Cytotoxicity
Medical Management
Riluzole
Slow disease progression
Edarvone
Reduces decline in daily function
baclofen, diasopene
spasticity control
Trihexyphenidyl
aid in swallowing ability
gabapentin
pain control
PEG tube for feeding
Diagnostic Testing
Maximum inspiratory pressure
Maximum Expiratory Pressure
Forced Vital Capacity
speech and swallowing evaluation
nerve conduction studies
ABGs
alveolar arterial gradient
serum blood count
spinal tap
myelogram
mm biopsy
Idopathic
Inherited
EMG
MRI
Blood and urine tests
http://erj.ersjournals.com/content/34/2/444#ref-10
https://www.uptodate.com/contents/the-evaluation-diagnosis-and-treatment-of-the-adult-patient-with-acute-hypercapnic-respiratory-failure#H468120