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52 year old female diagnosed with Amyotrophic Lateral Sclerosis…
52 year old female diagnosed with Amyotrophic Lateral Sclerosis
Background information
Anatomy
spinal cord
dorsal median sulcus
columns of myelinated axons on the outer surface of the spinal cord, surrounding the gray matter horns
ventral grey horn
region of grey matter that is anterior in the spinal cord and contains the cell bodies of motor neurons traveling to skeletal muscles and glands
ventral median fissure
the bundle of motor neurons that leaves the anterior side of the spinal cord to travel to muscles and glands
white matter funiculi
bundle of sensory neurons that enters the posterior side of the spinal cord
central canal
the middle open space that contains cerebrospinal fluid
lateral gray horn
region of gray matter thats lateral in the spinal cord and has autonomic motor neurons
dorsal grey horn
region of grey matter that is posterior in the spinal cord and contains the cell bodies of interneurons
dorsal root ganglion
an enlargement along the dorsal root of a spinal nerve where sensory neuron cell bodies are found
dorsal root
narrow groove on the posterior side of the spinal cord
ventral root
a slightly wider groove on the anterior side of the spinal cord
Specific types of neurons/neuron structures found in spinal cord
lateral grey horn
visceral motor (autonomic) neurons
ventral grey horn
somatic motor neurons
dorsal grey horn
interneurons
cerebrum
postcentral gyrus
central sulcus
precentral gyrus
longitudinal fissure
gyri
sulci
Ascending tracts
SPECIFIC EXAMPLES
Spinothalamic tract
Spinocerebellar tract
Descending tracts
SPECIFIC EXAMPLES
Corticospinal tract
vestibulospinal tract
Physiology
Cerebrum
found anterior to the central sulcus; its the location of the primary motor area of the brain and regulates many body motor functions
found posterior to the central sulcus; its the location of the primary sensory area of the brain which is involved in many of the body's sensory functions
raised bumps on the surface of the cerebrum
separates the parietal lobe and frontal lobe
small indentations in the surface of the cerebrum
very deep indentation that divides the cerebrum into two halves
specific neurons/structures in the spinal cord
dorsal grey horn
interneurons that receive input from somatic sensory neurons
interneurons that receive input from visceral sensory neurons
lateral grey horn
visceral motor (autonomic) neurons
they control involuntary actions like heart rate, blood pressure, digestion
they conduct impulses to the smooth muscle, cardiac muscle, and glands
ventral grey horn
controls voluntary actions and conducts impulses to skeletal muscles for movement
functions of neurons in ascending tracts(sensory tracts)
2nd order- the interneuron in the dorsal horn of the spinal cord
3rd order- neuron in the thalamus that connects with the cerebral cortex
1st order- neuron that has receptors and brings the sensory info to the spinal cord
functions of neurons in descending tracts(motor tracts)
upper motor neuron- in the brainstem
lower motor neuron- the neuron the synapses with the muscle and goes to the NMJ
process of movement
precentral gyrus
location of primary motor area, contains the motor "map" called the motor homunculus that shows us how the size of a body part indicates how many different motor neurons travel there
basal nuclei
helps regulate muscle movement by preventing unwanted movements
cerebellum
assists the precentral gyrus with skeletal muscle movements. It enables smooth coordinated movements. Collects motor info. about what should be happening, and compares that to sensory info. about what is happening
descending spinal tracts
direct motor tract- I make the decision on what i'm moving
indirect motor tract- the decision to move comes from the brain stem
Ascending tract examples
spinothalamic tract
is a sensory pathway from the skin to the thalamus
spinocerebellar tract
is a nerve tract originating in the spinal cord and terminating in the same side (ipsilateral) of the cerebellum
Descending tract examples
corticospinal tract
is a white matter motor pathway starting at the cerebral cortex that terminates on lower motor neurons and interneurons in the spinal cord, controlling movements of the limbs and trunk
vestibulospinal tract
this is an indirect motor tract, and it helps us stay balanced
neurons found in ascending tracts
1st order
2nd order
3rd order
neurons found in descending tracts
upper motor neuron
lower motor neuron
UPSTREAM CAUSES
DIRECT
In this disorder motor neurons deteriorate and die and can no longer do their job of communicating and controlling the muscles which causes all of the symptoms of ALS- muscle weakness, difficulty walking and doing normal daily activities, slurred speech, muscle cramps, twitches, and tripping/dropping things more often than usual
INDIRECT
This patient did have a family history of ALS and since genetic testing confirmed she had it as well, that tells us this is most likely genetically inherited
Risk factors include
Heredity. Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease
Age. ALS risk increases with age, and is most common between the ages of 40 and the mid-60s.
Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70
Genetics. Some studies examining the entire human genome found many similarities in the genetic variations of people with familial ALS and some people with noninherited ALS. These genetic variations might make people more susceptible to ALS.
lifestyle factors include
Smoking. Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause.
Environmental toxin exposure. Some evidence suggests that exposure to lead or other substances in the workplace or at home might be linked to ALS. Much study has been done, but no single agent or chemical has been consistently associated with ALS.
Military service. Studies indicate that people who have served in the military are at higher risk of ALS. It's unclear what about military service might trigger the development of ALS. It might include exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion
DOWNSTREAM EFFECTS
complications going forward
SPEAKING
Most people with ALS develop trouble speaking. This usually starts as occasional, mild slurring of words, but becomes more severe. Speech eventually becomes difficult for others to understand, and people with ALS often rely on other communication technologies to communicate.
EATING
People with ALS can develop malnutrition and dehydration from damage to the muscles that control swallowing. They are also at higher risk of getting food, liquids or saliva into the lungs, which can cause pneumonia. A feeding tube can reduce these risks and ensure proper hydration and nutrition.
BREATHING
Over time, ALS paralyzes the muscles you use to breathe and without treatment is fatal
treatment includes-a device to help you breathe at night, similar to what someone with sleep apnea might wear. For example, you may be given continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) to assist with your breathing at night.
Some people with advanced ALS choose to have a tracheostomy, which is a surgically created hole at the front of the neck leading to the windpipe (trachea) for full-time use of a respirator that inflates and deflates their lungs.
DEMENTIA
Some people with ALS have problems with memory and decision-making, and some are eventually diagnosed with a form of dementia called frontotemporal dementia.
Unfortunately there is no cure for this fatal disease