SC disease pathology Flashcards
(147 cards)
1
Q
Name the six categories of SC diseases
A
Degenerative Traumatic Metabolic/toxic Infectious/inflammatory Tumor Vascular
2
Q
What is Amyotrophic lateral sclerosis? What category of disease does it fit in?
A
ALS
DEGENERATIVE
3
Q
What category does syringomyelia fit in?
A
Traumatic
4
Q
What category does disc herniation fit in?
A
traumatic
5
Q
What category does Friedreich’s ataxia fit in?
A
Degenerative
6
Q
Subacute combined system degeneration fits in what category?
A
Metabolic/toxic
7
Q
What category does Cervical spondylosis fit in?
A
Degenerative
8
Q
What category does Myelopathy an radiculopathy fit in?
A
Degenerative
9
Q
What category does Caisson disease fit in?
A
Metabolic/toxic
10
Q
What category does radiation myelopathy fit in?
A
Degenerative
11
Q
What category does extrameduallary and intramedullary fit into
A
SC tumors
12
Q
What category does spinal hemorrhage fit into?
A
Vascular
13
Q
What category does
Tabes dorsalis fit into
A
infectious/inflammatory
14
Q
Meningitis and encephalitis fits into what category
A
infectious/inflammatory
15
Q
GB syndome fits into what category
A
infectious/inflammatory
16
Q
Transverse myelitis fits into what category
A
infectious/infalmmatory
17
Q
Poliomyelitis fits into what category
A
infectious/inflammatory
18
Q
Myopathy definition
A
issue with muscle
19
Q
Myelopathy
A
issue with SC
20
Q
Myelinopathy
A
issue with myelin
21
Q
Fibrillation vs. fasiculation
A
Fibrillation: small contraction in single muscle fiber; diagnostic you cannot see it
Fasiculation: brief observable muscle twitch, you can see these they’re generally due to nerve irritability, seen in ALS
22
Q
ALS is progressive degeneration of what
Disease of what?
A
motor neurons!
specifially the chronic, progressive wasting and atrophy of anterior horn cells
Disease of the nerve cell bodies
23
Q
In ALS UMN or LMN signs?
SC, brainstem, motor cortex?
A
SC and brainstem (site of CN): LMN
Cortex: UMN
24
Q
What accounts for the spasticity you see in ALS?
A
UMN of cortex
25
LMN signs
hypotonicity, hyporeflexia, atrophy of muscle cells. also happens to the CN due to brainstem involvement so paralysis of face, swallowing issues, vision etc.
26
UMN signs
hypertonicity/spasticity, hyperreflexia
27
picture of a person at risk for ALS
male (1.5 to 1 ratio to females)
mid 50's
28
Rizuzle/rilutek
extends life for how long?
stops glutamate cytotoxicity at the motor neurons
extends life for ~6months
29
Half of ALS population live how many years after diagnosis?
3 yrs
30
During primary lateral sclerosis what tract is targeted?
CST
31
During progressive bulbar pasly what tract is targeted?
corticobulbar tract
32
loss of AHC in SC and lower Brainstem results in what?
progressive muscular atrophy
33
Demyelination and gliosis of the CST and CBT lead to what?
primary lateral sclerosis and progressive bulbar palsy
34
What is the most common form of ALS
some combination of primary lateral sclerosis and progressive bulbar palsy with progressive muscular atrophy
35
What two things deteriorate in ALS
cell nerve body, muscle itself
36
What does an ALS spinal cord look like?
it looks huge but thats because there are fewer nerves running along the side
37
Where is very noticeable atrophy in individuals with ALS early on?
hands
38
ALS is a disease of the ______ where are the three places it happens?
Nerve cell bodies
1) Betz cells: nerve cell bodies in the cortex which are the beginning of the CST; UMN as they degenerate
2) CN nuclei, they are the AHC of the brainstem; LMN as they degenerate
3) spinal nerve nuclei affecting motor neurons; LMN/flaccid paralysis as they degenerate
39
What is the deal with the corticobulbar pathway and its influence, how does it differ than what we normally see in the CN nuclei if it is also affected?
it runs with the CST but then affects the nerve cell bodies of the CNS above the level of the CNS leading to UMN signs instead of what we're used to seeing which is LMN symptoms in the face in ALS
40
Are fasiculations UMN or LMN?
LMN
41
What two areas are fasiculations common in the body.
| Why here?
tongue, hands
motor units are so small it doesn't take much chemical change (from nerve cells degenerating causing chemical changes around the nerve cell creating irritability)
42
Are Riluzole/Rilutek and Myotrophin used to treat ALS effective?
no!
43
causes of ALS?
Unknown but suggestions include
toxicity, deficiency of nerve growth factor, excess of extracellular glutamate, autoimmune process, viral
44
Diagnostic criteria for ALS
UMN the LMN signs in 3 spinal regions OR 2 spinal region + Bulbar signs
45
Can you do a procedure to diagnose ALS?
nope just symptomology
46
do individuals with ALS have sensory loss?
no
No cognitive loss either, autonomic involvement are not a thing either (CV, sphincter)
47
Oculomotor nerve vs. optic nerve involvement in ALS
Oculomotor NOT affected
Optic can be affected
48
What is the common first signs of ALS generally?
clumsy, slurring of words, difficulty swallowing
Something that shows some type of weakness
49
____% of motor neuron loss by the time weakness is noted?
80% there is so much redundency in the system
50
When do fasciculation's and cramps tend to be a symptom in patients with ALS?
further down the line
51
Typically distal or proximal loss first with ALS?
distal!
52
is speech involved in ALS
yes eventually all motor function is lost including speech
53
when is ventilation required for individuals with ALS?
40-50% VC
54
When should supplements or feeding tube start. What % of BW loss?
10% BW loss
PEG tubes are placed relatively early in the disease process generally
55
What are the three things Friedrich's ataxia is a degeneration of?
Spinocerebellar tracts (unconscious proprioception), CST and posterior columns (DCML)
56
What is the most common hereditary ataxia?
How is it passed down?
Age of onset?
Friedreich's Ataxia
Autosomal recessive disorder
Onset 8-15 years
57
Age of onset for Freidreich's ataxia
Progression? Lose ability to walk by? WC By?
8-15 years
Variable progression
Lose ability to walk ~15 years after onset
WC by 45
58
What does spinocerebellar carry?
proprioception unconsciously
59
Pes Cavus and Kyphotocolionis are sequelae of what disease?
Friedrich's ataxia
60
Describe pes cavus
What do we need to do for it?
What are the implications?
hyperextension of MTP and flexion of the IP joints
Generally they'll be braced
Generally they cannot ambulate
61
Signs and symptoms of Freidricks ataxia
Progressive ataxia
Increasing weakness
Loss of tendon reflexes (DCML gone)
Impaired proprioception (no spinocerebellar)
DM
Scoliosis, ataxia in UE, cardiomyopathy, abnormal occular movements
Affective disorders
62
What disease generally has affective disorders associated with it?
Fredrick's ataxia
63
Degenerative disc disease with bone spurs is called what
cervical spondylosis
64
what is the most common cause of myelopathy?
Cervical spondylosis
65
Gradual onset of neck pain, radicular arm pain, LMN signs and UMN signs in legs stems from what?
cervical spondylosis
66
What two tx are common for cervical spondylosis
prophylactic as well as possible decompression laminectomy (trying to expand the space)
67
if the disc impinges on the SC what will you see in the LE
UMN signs all down the legs
68
what procedure is done on the spine to primarily remove pressure on the SC?
posterior laminectomy
69
Is radiation myelopathy progressive or transient?
Both!
Transient: 3-6 months after radiation, normally sensory >> motor problem and sensation generally resolves (Lhermite's sign; pain with flexion of neck)
Progressive: 9-15 month post radiation. Has more of a weakness component than transient. Progression stops but does not reverse
70
Cysts in the SC around the central canal (typically in the cervical area) is called what?
Syringomyelia (traumatic)
71
What two things are syringomyelia caused by?
TEST QUESTION
Congenital: hydrocephalus commonly via Chiari Malmformation
Trauma
72
Explain traumatic syringomyelia
central canal runs through SC this is carrying CSF. When the central canal enlarges it impinges on the SC and tissue is compressed affecting sensory and motor components
73
What is a syrinx formation?
enlarged central canal (where CSF flows)
74
Do syrinx formations happen in traumatic or congenital syringomyelia?
both!
75
Chiari Malformation is associated with what disease
syringomyelia
76
Explain chiari malformation
posterior skull is deformed, CB sinks posterior and presses on the skull. This stops CSF from flowing down SC as it should --> hydrocephalus and enlargement of ventricles --> syrinx in the SC --> pressure eventually kills neural tissue in brain and SC
77
Posterior decompression/decompression laminectomy is done for what?
relieving the pressure caused by a chiari malformation
its the bone flap taken off the posterior aspect of the skull
78
Where does the ventriculoperitoneal shunt travel?
Tx for what?
ventricles --> neck --> abdomen
Syringomyelia
79
Where in the body does syringomyelia generally stem from?
C-spine
80
Complication of trauma, meningitis, hemorrhage, tumor or arachnoiditis can all be a cause of what even months or years after the initial injury?
acquired/traumatic Syringomyelia
81
most common syringomyelia sx? Why?
pain in the area of the injury as well as pain and temperature loss. Touch generally in tact.
Its a central issue, think about central cord, ALS crosses in the middle. DCML would be in tact
82
If you were to see CST issues due to syringomyelia, how and where would you see it?
weakness and wasting in hands and scapula
83
Two common medical tx for syringomyelia?
1) posterior/decompression laminectomy
| 2) shunt
84
What areas of the SC are lost in subacute combined degeneration?
Posterior columns: DCML
| Lateral columns: CST and spinocerebellum
85
Etiology of subacute combined degeneration?
inability to absorb vitamin B12 = pernicious anemia
86
Loss of what sensations?
light touch, vibration, proprioception, weakness, tingling in hands and feet, stiff limbs, irritable, drowsy, confused
87
tx for subacute combined degeneration?
replacement therapy, replace B12
88
Common population you see Subacute combined degeneration in?
elderly bc they don't absorb B12
89
Etiology of Caisson disease
accumulated nitrogen that cannot be exhaled forming bubbles in blood and tissues
90
2 pathologies of Caisson disease and is it central or peripheral?
AGE: central damage
DCS: peripheral damage
91
Explain AGE as part of Caisson disease
occlusion of epidural venous sinus by nitrogen bubbles leading to impaired venous return and patchy hemorrhagic infarcts in SC
92
Explain sx of DCS as part of Caisson disease
severe reaction?
recovery?
joint pain, skin rash, edema
Severe reaction: 10-30 min after surfacing paresthesias and paralysis LE>UE
Recovery: over weeks in 60-80% especiallyi f they're in a compression chamber
93
Air embolisms cause what?
infarct in the SC or brain
94
Inflammation of the leptomeninges (two most internal layers; arachnoid and pia) and underlying subarachnoid cerebrospinal fluid is what?
meningitis
95
What are the two most internal layers of the leptomeninges?
What disease is this in relation to?
arachnoid and pia matter
Meningitis
96
Bacteria colonize in the nasopharynx or enteroviruses are common causes for what disease?
meningitis
97
Brain inflammation caused by a virus is what?
encephalitis
98
Meningitis and encephalitis lead to ____ which leads to _____
inflammation --> pressure which can cause damage quickly
99
Whats the deal with CSF and meningitis?
Just like syrincomyelia CSF may not be able to exit causing back pressure down the line.
100
What is Tabes Dorsalis a degeneration of?
Specifically in what are?
dorsal columns/dorsal spinal roots = loss of DCML
Specifically in the lumbosacral area
101
What disease is associated with sharp leg pain, ataxia, urinary incontinence, absent knee and ankle reflexes?
Tabes dorsalis (inflammatory/disease issue)
Side note: develops 10-20 years after syphillis
102
What does poliomyelitis attack?
AHC and sometimes motor nuclei of the brainstem
103
Loss of AHC in poliomyelitis leads to UMN or LMN?
LMN asymmetrical weakness with prominent atrophy overtime
104
What disease do you get systemic illness prior to neurologic symptoms?
poliomyelitis
105
What happens to muscle fiber and motor unit ration in poliomyelitis
more muscle fibers per motor unit
surviving AHC's sprout to re-innervate orphaned fibers creating a ton of muscle fibers per motor units
Unaffected muscle fibers hypertophy due to overload
106
MMT grading came about due to what disease?
polio
107
Normal MMT grade could still have how much %AHC loss?
60%
There is such a high degree of redundancy in the system
108
Pes cavus and scoliosis are common in what two diagnosis?
Friedrich's ataxia and Polio
109
Whats the big picutre in post polio for PT
these people have worked hard all their life with these overworked motor units! We don't want them overdoing it! You need a definite stop so they do not overexercise.
110
How long after the original disease does post polio come about?
30-40 years.
111
What disease usually occurs at levels T4-T6 or T10-12?
transverse myelitis
112
What disease? non specific sensory loss, symmetrical severe muscle weakness, loss of reflexes and bowel and bladder control (think of th elevels it affects)
Transverse myelitis
113
Prognosis for transverse myelitis (inflammatory process following another infection)
self limiting w/good recovery
114
Is GB acute or long lasting?
acute!!
115
what kind of paralysis do you have with GB syndrome?
what two parts of the nerve are affected?
flaccid motor paralysis
entire length of the nerve: Nerve root and peripheral nerve
116
What is the assumed etiology of GB sndrome?
autoimmune overreaction to a virus,50% of people have recent viral infections
117
weakness in legs spreading to the arms (ascending paralysis) in a symmetrical pattern
Asymmetric sciatic pain
is indicative of what disease?
GBS
118
Male vs. female, which is more common in GBS?
same
119
Polyradiculoneeuropathy associated with what disorder?
GB syndrome, causes flaccid motor paralysis
120
Do people with GB syndrome recover?
yes they do but they often have residual symptoms
121
The older you are, is the prognosis better or worse for GB?
worse!
122
Where does weakness normally start for GBS?
legs spreading to arms symmetrically
123
Sensory paresthesia or numbness in what pattern in GBS?
stocking glove distribution
124
Maximum weakness in GBS within how many weeks?
one week
125
Muscle weakness may include muscles of _____ in GBS leading to ____ in ____-% of people
muscles of respiration leading to ventilation in 20-30% of patients
126
50% of pts with GBS have what two things?
autonomic signs (affecting cardiac rhythm) CN involvement
127
What sx is common throughout GBS entire recovery process
pain: deep and aching in large muscle groups
128
When does the recovery process of GBS begin?
within 2-3 weeks OF THE ACUTE PROCESS meaning 2-3 weeks after their sx have stopped progressing
129
True or false: PT can help speed up the GBS recovery process?
False! you can't speed it up but you can stop shortening of tissues decrease pain etc.
130
Plasmapheresis and Intravenous immunoglobulin (IVIG) used in what disease
GBS
Plasmapheriesis: Shown earlier recovery of walking if given within the first week (it takes mulitiple days)
IVIG: more of a steroid
131
Do PT's have a role in acute phase of GBS?
no! ROM perhaps
132
Would someone with GBS show hyper or hypo reflexive deep tendon reflexes
hypo: diminished or absent
133
several days to two weeks but no worsening of symptoms is what phase of GBS?
plateau phase
Generally you see asymmetric sciatic pain here
134
Time frame of recovery for GBS depends on what generally/
the extent of the inital paralysis
it may take 1-2 years to reach best state and some may have perimant residual weakness as well as fatigue being a continual problem
135
Poorer prognosis in GBS for what three things?
- rapidly progressing sx
- need for ventilatory support
- over the age of 70
136
Very general outcomes for GBS
people get better, half walk unassisted within three months, majority don't have neurologic sequelae but are fatigued, many test with some weakness and fatigue but pts feel they are getting stronger due to compensations etc.
137
CIPD is what
chronic inflammatory demyelinating polyneuropathy
A type of GBS
138
What is the pattern of CIPD
_____ rather than _____
symmetrical or asymmetrical?
Lasting disability/
its a type of GBS following a pattern or RELAPSE rather than PROGRESSIVE
Asymmetrical
Yes some have lasting disability
139
medical tx for CIPD
corticosteroids, and plasmapheresis over months instead of days like normal GBS
140
Where is an intrameduallry tumor
on the SC itself
141
Where is an extramedullary intradural tumor
inside the dura but not adhered to the SC itself
142
Where is an extramedullary, extradural tumor?
totally outside of the dura, not attached to the SC at all, THIS IS THE MOST SERIOUS
143
Where is extradural tumor generally coming from?
metastatic
144
Sxs from bony instability can be from which of the three kinds of tumors?
extradural
Can have a decompression or radiation to reduce size
145
Intramedullary tumor generally medically tx how?
surgery, they are less aggressive and benign
146
Most likely artery to have an infarct?
anterior spinal artery
147
What does the anterior artery serve?
anterior 2/3 of the spinal cord
ALS, CST impacted. DCML in tact. UMN below the level of the lesion.
