DEGENERATIVE CONDITIONS OF THE NERVOUS
SYSTEM
1.ALS targets what? (2): -corticospinal tracts
-anterior horn cells
2.ALS - mixed UMN and LMN:
3.ALS is degeneration of what? (2): -corticospinal tracts
-neurons in the motor cortex and brainstem
4.ALS is degeneration of anterior horn cells where? (2): -spinal cord
-motor cranial nerve nuclei in the brainstem
5.ALS is also known as what disease?: Lou Gehrig's disease
6.ALS is massive loss of anterior horn cells in the SC and motor cranial
nerve nuclei in the brainstem which causes what? (2): muscle atrophy and
weakness in affected areas (amyotrophic)
7.ALS is also caused by demyelination and gliosis of what tracts? (2): -
corti- cospinal tracts
-corticobulbar tracts
(UMN symptoms= lateral sclerosis)
8.for ALS the etiology is unknown, but what are some proposed
mechanisms?
(4): -viral mechanism
-excitotoxicity
-autoimmune response
-protein misfolding disorders
9.ALS etiology
-excitotoxicity= overstimulation of nerve cells due to excessive amounts of
glutamate, may result in cell death
-protein misfolding disorders= proteins adopt an abnormal shape, leading to
functional impairment and potential disease development:
10.for ALS etiology, excitotoxicity can occur from damage from
acclamation of what?: free radicals in the nervous system
11.for ALS etiology, autoimmune response, what was found in some
pa- tients?: antibodies to motor neurons
12.what are the known risk factors for ALS? (5): -disease-causing
mutations (SOD1, alsin)
-clusters (ex- western pacific...exposure to something in the
environment)
-family history
-age
-gender (male > female)
13.ALS is the most common what?: motor neuron disease
14.what is the mean age of onset for ALS?: 57 years old (2/3 diagnosed
, DEGENERATIVE CONDITIONS OF THE NERVOUS
SYSTEM
between ages 50-70)
, DEGENERATIVE CONDITIONS OF THE NERVOUS
SYSTEM
15.for ALS, 95% of cases are sporadic, but approximately 5% are what?:
famil- ial relationship
16.to diagnose ALS, there is a clinical presentation of what?: motor
system changes
17.to diagnose ALS, we need to rule out other pathologies and there is no
specific lab test, but approximately 70% of patients have elevated what?:
CPK levels
(creatine phosphokinase= when this is high it means there has been
injury or stress to muscle tissue, the heart, or the brain)
18.what is considered the cardinal sign of ALS and may be caused by LMN
or UMN loss?: muscle weakness
19.for diagnosis of ALS, the EMG shows spontaneous what? (2): fibrillation
and fasciculation
20.-fasciculation= spontaneous discharge of motor unit potentials,
occasion- ally seen in normal muscles
-fibrillation= short duration of spontaneous firing/potentials produced by sin
gle muscle fibers:
21.frequent/arhythmical fasciculation is indicative of what disease?:
motor neuron disease
22.motor neuron disease= rare condition that progressively damages parts
of the nervous system and this leads to muscle weakness, often with
visible wasting:
23.fibrillations represents unstable what?: muscle fiber cell membrane
(in my- opathies or peripheral myopathies)
24.the signs and symptoms of ALS is it has an insidious onset and what
type of weakness?: asymmetrical weakness
25.for signs and symptoms of ALS
-weakness is the most frequent initial symptom, it spreads over time and
they start to lose function (ex- ascending stairs, fine motor coordination)
-may have 80% motor neuron loss before patient complains of weakness:
26.for ALS, at onset, individuals may notice difficulty with fine motor
move- ments (buttoning, pinching, or writing) or may notice foot "slapping"
or in- creased frequency of tripping while walking:
27.for ALS, individuals with bulbar onset may noticed changes in what,
diffi- culty with what, or decreased what?: -changes in their voice
-difficulty moving the tongue
-decreased ability to move the lips or open or close the mouth
28.for ALS signs and symptoms, 70-80% develop what vs what onset?:
devel- op limb onset vs bulbar onset
SYSTEM
1.ALS targets what? (2): -corticospinal tracts
-anterior horn cells
2.ALS - mixed UMN and LMN:
3.ALS is degeneration of what? (2): -corticospinal tracts
-neurons in the motor cortex and brainstem
4.ALS is degeneration of anterior horn cells where? (2): -spinal cord
-motor cranial nerve nuclei in the brainstem
5.ALS is also known as what disease?: Lou Gehrig's disease
6.ALS is massive loss of anterior horn cells in the SC and motor cranial
nerve nuclei in the brainstem which causes what? (2): muscle atrophy and
weakness in affected areas (amyotrophic)
7.ALS is also caused by demyelination and gliosis of what tracts? (2): -
corti- cospinal tracts
-corticobulbar tracts
(UMN symptoms= lateral sclerosis)
8.for ALS the etiology is unknown, but what are some proposed
mechanisms?
(4): -viral mechanism
-excitotoxicity
-autoimmune response
-protein misfolding disorders
9.ALS etiology
-excitotoxicity= overstimulation of nerve cells due to excessive amounts of
glutamate, may result in cell death
-protein misfolding disorders= proteins adopt an abnormal shape, leading to
functional impairment and potential disease development:
10.for ALS etiology, excitotoxicity can occur from damage from
acclamation of what?: free radicals in the nervous system
11.for ALS etiology, autoimmune response, what was found in some
pa- tients?: antibodies to motor neurons
12.what are the known risk factors for ALS? (5): -disease-causing
mutations (SOD1, alsin)
-clusters (ex- western pacific...exposure to something in the
environment)
-family history
-age
-gender (male > female)
13.ALS is the most common what?: motor neuron disease
14.what is the mean age of onset for ALS?: 57 years old (2/3 diagnosed
, DEGENERATIVE CONDITIONS OF THE NERVOUS
SYSTEM
between ages 50-70)
, DEGENERATIVE CONDITIONS OF THE NERVOUS
SYSTEM
15.for ALS, 95% of cases are sporadic, but approximately 5% are what?:
famil- ial relationship
16.to diagnose ALS, there is a clinical presentation of what?: motor
system changes
17.to diagnose ALS, we need to rule out other pathologies and there is no
specific lab test, but approximately 70% of patients have elevated what?:
CPK levels
(creatine phosphokinase= when this is high it means there has been
injury or stress to muscle tissue, the heart, or the brain)
18.what is considered the cardinal sign of ALS and may be caused by LMN
or UMN loss?: muscle weakness
19.for diagnosis of ALS, the EMG shows spontaneous what? (2): fibrillation
and fasciculation
20.-fasciculation= spontaneous discharge of motor unit potentials,
occasion- ally seen in normal muscles
-fibrillation= short duration of spontaneous firing/potentials produced by sin
gle muscle fibers:
21.frequent/arhythmical fasciculation is indicative of what disease?:
motor neuron disease
22.motor neuron disease= rare condition that progressively damages parts
of the nervous system and this leads to muscle weakness, often with
visible wasting:
23.fibrillations represents unstable what?: muscle fiber cell membrane
(in my- opathies or peripheral myopathies)
24.the signs and symptoms of ALS is it has an insidious onset and what
type of weakness?: asymmetrical weakness
25.for signs and symptoms of ALS
-weakness is the most frequent initial symptom, it spreads over time and
they start to lose function (ex- ascending stairs, fine motor coordination)
-may have 80% motor neuron loss before patient complains of weakness:
26.for ALS, at onset, individuals may notice difficulty with fine motor
move- ments (buttoning, pinching, or writing) or may notice foot "slapping"
or in- creased frequency of tripping while walking:
27.for ALS, individuals with bulbar onset may noticed changes in what,
diffi- culty with what, or decreased what?: -changes in their voice
-difficulty moving the tongue
-decreased ability to move the lips or open or close the mouth
28.for ALS signs and symptoms, 70-80% develop what vs what onset?:
devel- op limb onset vs bulbar onset
