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Progressive Muscle Weakness Diagnosis: Amyotrophic Lateral Sclerosis (ALS)…
Progressive Muscle Weakness Diagnosis: Amyotrophic Lateral Sclerosis (ALS)
Downstream Effects
Respiratory Failure: As ALS progresses, the muscles used to breath become paralyzed. This is the most common cause of death for ALS.
To alleviate/help with these symptoms: Continuous positive airway pressure (CPAP);Bilevel positive airway pressure (BiPAP); Tracheostomy (for full-time use of automatic-respirator)
Speaking Problem: Due to the muscle's weakness, the muscles that allow us to speak eventually weakens to the point a person with ALS can't speak very well.
Someone with this symptom can try speech therapy to strengthen the muscles or find other methods of communication.
Aspiration/Aspiration Pneumonia: Muscles that control swallowing weaken with ALS. A person is at risk for aspirating these foods/liquids/mouth secretions into their lungs, potentially leading to aspiration pneumonia.
To reduce the risk of aspiration/aspiration pneumonia a feeding tube can be placed.
Malnutrition/Dehydration: When the muscles that help with swallowing are weakened, the person is at risk for malnutrition/dehydration because they aren't able to eat and drink normally.
To aid someone who is malnourished and unable to take in foods/drinks, a feeding tube can be placed.
Dementia: Many of the individuals with ALS have problems with memory and decision-making , and with time, some of them are diagnosed a form of demential called (frontotemporal dementia - Where the frontal lobe and temporal lobe shrink).
Speech therapy; Antidepressants (e.g. Trazodone) and antipsychotics (e.g. Zyprexa) are often used to reduce behavioral problems related to this disease.
Muscle Weakness: Due to the deterioration of the upper and lower motor neuron, the muscles also suffer and weaken.
Someone could try physical therapy but not a lot of improvement will happen, as it is the motor neurons that are affected.
Upstream Causes
Direct Cause: In ALS, upper and lower motor neurons slowly degenerate and shrink. This eventually leads to neuronal death. The degeneration of these neurons weakens the muscles.
Indirect Cause: This patient has a family history of ALS. Her brother died of ALS at the age of 31 and they suspect the father also died of it. It could be a possibility she got familial ALS from her father (if he had it). If it's not familial ALS, her age is a risk factor since ALS is most common between the ages of 40-mid 60's (This patient is 52). Also, genetics make people more susceptible to ALS.
Background
Physiology
Functions of Cerebrum/Cerebellum
Frontal Lobe: Involved with motor functions and personality.
Parietal Lobe: Involved with general sensory functions.
Occipital Lobe: Processes visual information.
Temporal Lobe: Involved in the sense of hearing and smell.
Insula: Involved with gustation/taste
Post-central Gyrus: Primary sensory area of the brain, which is involved in many of the body's sensory functions.
Pre-central Gyrus: Primary motor area of the brain, which regulates many of the body's motor functions.
Cerebellum: Regulates motor movements & assists with equilibrium.
The Arbor Vitae: Allows the neurons inside cerebellum to talk to each other quickly.
The vermis: Body posture and locomotion.
Basal Nuclei: Regulates muscle movement.
Corpus Callosum: Connects the two cerebral hemispheres.
Epithalamus: Controls the secretion of melatonin by the pineal gland.
Thalamus: Relay center; filters sounds and sights.
Hypothalamus: Controls the secretion of hormones (sex, blood pressure) by the pituitary gland; controls homeostasis.
Fornix: Carry information in & out of the hippocampus.
Corpora Quadrigemina: Involved in visual & auditory reflexes.
Midbrain: Regulates eye movement.
Pons: Relays information from medulla oblongata & higher brain regions.
Medulla Oblongata: Reflex centers; cardiac & breathing control centers.
The Spinal Cord
Dorsal Root Ganglion: Where sensory neuron cell bodies are found.
Dorsal Gray Horn: Contain cell bodies of interneurons.
Lateral Gray Horn: Contains visceral motor neurons. (Autonomic/Involuntary: Heart rate, breathing)
Ventral Gray Horn: Contains somatic motor neurons, traveling to skeletal muscles & glands.
Central Canal: Middle open space that contains CSF.
Dorsal Root: Bundle of sensory neurons.
Ventral Root: Bundle of motor neurons that travel to muscles and glands.
White Matter Funiculi: Columns of myelinated axons on the outer surface of the spinal cord, surrounding the gray matter horns.
Ascending Tracts: Sensory information that is sent to the brain.
2nd Order Neuron: Interneuron that processes the information received.
e.g. The Spinothalamic Tracts: 1st order neuron (dorsal root ganglion) goes to and enters spinal cord; 2nd order neuron travels up the spinal cord (dorsal horn) until it reaches the thalamus; 3rd order neuron sends the information from the thalamus to the cerebral cortex.
3rd Order Neuron: Neuron in the thalamus that connects with the cerebral cortex.
1st Order Neuron: Has receptors and brings the information to the spinal cord.
Descending Tracts: Motor direction sent to the skeletal muscles.
Upper Motor Neuron: Neuron located in the brain/brainstem.
e.g. The Corticospinal Tract: Upper motor neuron is located in the pre-central gyrus of the brain cortex and it travels down the spinal cord. The lower motor neuron goes from the ventral gray horn and synapses with the muscle (e.g. NMJ).
Lower Motor Neuron: Neuron that synapses with the muscle (goes to NMJ)
Functions of Organs and Organ Systems Affected
Nervous System: Detects and responds to stimuli.
Neurons: Receive, process and transmits information.
Respiratory System: Does gas exchange.
Lungs: Allows the process of gas exchange (respirations)
Muscular System: Assist with movements and heat production.
Muscles (Skeletal, Smooth, and Cardiac): Assist with movement.
The Process of Movement
The contraction of the body's skeletal muscles is initiated by the neurons in the pre-central gyrus (upper motor neurons). This is where the motor homunculus is located. The upper motor neuron sends the message (time to contract) to the lower motor neuron, which travels from the ventral gray horn to the NMJ. This is a descending tract.
The cerebellum assists the pre-central gyrus with skeletal muscle movements by collecting motor information on what should be happening. It compares that to sensory information about what is happening. It will enable smooth, coordinated movements. If any corrections are needed, it will send out the orders to do so.
The basal nuclei also helps regulate skeletal muscle movement. The specific job of the basal nuclei is to prevent unwanted movements.
Anatomy
Cerebrum/Cerebellum
The Cerebellum
The Arbor Vitae
The Vermis
The cerebellar Hemispheres
Basal Nuclei
5 Lobes
Occipital Lobe
Temporal Lobe
Parietal Lobe
The Insula
The Frontal Lobe
Diencephalon
Hypothalamus
Pituitary Gland
Fornix
Thalamus
Intermediate Mass of the Thalamus
Epithalamus
Pineal Gland
Corpus Callosum
Brain Stem
Midbrain
Pons
Corpora Quadrigemina
Medulla Oblongata
Spinal Cord
Post-central Gyrus
Central Sulcus
Pre-central Gyrus
Longitudinal Fissure
The Spinal Cord
White Mater Funiculi
Ascending Tracts
Sensory Information
2nd Order Neuron
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3rd Order Neuron
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1st Order Neuron
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Descending Tracts
Motor Directions
Upper Motor Neuron
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Lower Motor Neuron
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Ventral Gray Horn
Lateral Gray Horn
Dorsal Gray Horn
Dorsal Root Ganglion
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Interneurons
Visceral Motor Neurons
Somatic Motor Neurons
Central Canal
Dorsal Median Sulcus; Ventral Median Fissure
Dorsal Root; Ventral Root
Spinal Nerve
Organs and Organ Systems Affected
Nervous System
Neurons
Respiratory System
Lungs
Muscular System
Skeletal Muscles
Cerebrum
Cerebellum
Diencephalon
Brain Stem
