Miscellaneous Neurologic Disorders - Exam 4 Flashcards
What is multiple sclerosis? What are the 4 characteristic features?
An autoimmune disorder resulting in damage to the neurons of the CNS
aka the body attacks myelin sheaths
chronic inflammation
demyelination
gliosis (plaques or scarring)
neuronal loss
What is the proposed etiology behind MS?
an environmental agent or event (eg, viral or bacterial infection, exposure to chemicals, lack of sun exposure) occurs in the presence of a genetic predisposition to immune dysfunction resulting in an autoimmune attack on the CNS neurons
In the pathophys behind MS: Insult to immune system results in development of _______ (inflammatory T cells, B cells, and macrophages) that cross the BBB. An inflammatory reaction occurs ______ around the axon resulting in what is know as an _____. The body attempts to ______ leads to a build up of rubbery or hardened _______
autoreactive lymphocytes
breaking down the myelin
MS “lesion”
remyelination
plaques (sclerosis)
MS more common in males or females? what is the typical age of onset? MC ethnicity?
more common in females
between 20-40 years of age
RRMS - average age 28-31
PPMS - average age 40
white people in the northern US
What are the risk factors for MS?
genetics
low vit D
EBV exposure
smoking
How does MS present? **What is the important phrase to remember here? What are some sensory symptoms a pt might complain about?
abrupt or insidious “attacks” that range from asymptomatic to severe that are separated in time and space and s/s are increased when the body temperature increases
sensory symptoms include: paresthesias, hypethesia (decreased sensation, numbness), unpleasant sensation, pain, shock-like sensation radiating down the back of the legs
s/s rarely follow dermatomes and sometimes the patient describes them as “weird”
What is it called when the s/s worsen by increased body temp?
Uhthoff Phenomenon
What are shock-like sensation radiating down back of legs called? Where do they RARELY present?
Lhermitte’s symptoms
rarely down the arms
What are some eye conditions that are associated with MS?
optic neuritis: usually unilateral, blurred vision, pain with EOM movements and central field disturbances, decreased color perception
RAPD: unequal response to pupillary constriction with swinging light test
optic disc may be normal or atrophied
How will the motor symptoms of MS present?
weakness: worse with exercise, facial weakness that may resemble Bell’s palsy
spasticity: if due to UMN lesions
hyperreflexia
+babinski sign
intention tremor
dysarthria: slurred speech
What cranial nerve is involved? Name 2 other s/s that can also be seen on the face with MS.
Trigeminal neuralgia - CN V
same s/s as idiopathic TN but often bilateral, occurring before age 50
facial myokymia: involuntary twitching or contraction of the facial muscles
glossopharyngeal neuralgia: intense electric shock-like pain in the posterior pharynx, tongue or ear triggered by swallowing or without warning
How does the trigeminal neuralgia seen with MS differ from idiopathic TN?
MS: bilateral and before the age of 50
idiopathic TN: unilateral and after the age of 40
What is facial myokymia? What CN? What condition?
involuntary twitching or contraction of the facial muscles
CN VII
seen in MS
What is glossopharyngeal neuralgia? What condition is it associated with?
intense electric shock-like pain in the posterior pharynx, tongue or ear triggered by swallowing or without warning
MS
What are the GU complaints seen with MS?
detrusor hyperreflexia
detrusor sphincter dyssynergia: difficulty starting and stopping urine stream, hesitancy, urinary retention, overflow incontinence, recurrent infections
bowel changes: constipation or fecal urgency/incontinence
can also have sexual dysfunction in both men and women
Can also see ______, ______ , _____ and _____ with MS
mild cognitive dysfunction
depression
fatigue
vertigo
Describe the timing of MS symptoms? They are usually precipitated by _____ or _______
Neurological symptoms lasting 10-120 seconds and occuring 5-40 x/d
Precipitated by hyperventilation or movement
What do they contribute the paroxysmal symptoms of MS to? Give some MS paroxysmal symptoms
Thought to be a result of spontaneous discharges from neurons at the edge of demyelinated plaques
Lhermitte symptoms, tonic contractions, dysarthria, ataxia, sensory disturbances
these are usually self limiting after weeks to months
What are the 5 types of MS? Which 2 are associated with the very early stages? Which 3 describe the pattern of symptoms?
very early:
Clinically isolated syndrome (CIS)
Radiologically Isolated Syndrome (RIS)
types of MS patterns:
RRMS
SPMS
PPMS
What is clinically isolated syndrome? How long does it have to last?
the first MS attack
must last for at least 24 hours, is characteristic of multiple sclerosis but does not yet meet the criteria for a diagnosis of MS
What is Radiologically isolated syndrome (CIS)?
evidence of MS on brain MRI before “clinical attack” is present
What is the MC type of MS? describe the pattern of symptoms. What is happening in between attacks?
Relapsing-remitting MS (RRMS)
discrete attacks of neurological dysfunction that evolves over days-wks (relapse), followed by partial or complete recovery over wks to months (remission)
between attacks patients are neurologically stable
What is secondary progressive MS?
starts as RRMS², at some point in the course of the disease a deterioration in function occurs unassociated with the acute attacks
aka quit seeing improvement
What is primary progressive MS?
a steady decline in function from disease onset and LACKS the relapse-remission characteristics of other forms of MS
What is the diagnostic criteria for MS? What is the timing?
2 or more episodes of symptoms and 2 or more signs that reflect pathology in different areas anatomically. lesions on MRI can act as the 2nd sign if necessary
Symptoms must last > 24 hours and must be separated by at least a month in recurrence
What will the lesions on brain MRI with gadolinium contrast look like for a pt with MS?
Acute MS lesions: larger with ill-defined margins. (lasts appx 1 month)
Chronic lesions: smaller with sharper margins
When would you use evoked potentials as a dx tool in MS? If the test is +, what will it show?
indicated for asymptomatic patients and are most effective in assessment of clinically uninvolved pathways
a marked delay in latency of a specific stimulation which is diagnostic in an asymptomatic patients
When would you order a CSF analysis in an MS pt? What would you expect to find?
Indicated in patients with CIS or an atypical presentation (e.g. MRI is nondiagnostic)
Oligoclonal bands present in 90-95%
Increased levels of intrathecal synthesized IgG (70–90%)
mildly elevated WBC (< 75 cells/μL)
CSF protein is normal to elevated
What are the some red flag findings that would make you think this is NOT MS?
symptoms localized to the posterior fossa (ataxia and imbalance), craniocervical junction (neck pain, headache, balance problems, voice changes, difficulty swallowing, respiratory problems or sleep apnea, motor speech issues such as difficulty articulating, compression of the spinal cord, spasticity, twisted or rotated neck) or spinal cord (sensory and motor symptoms only)
patient is <15 or >60 years of age
clinical course is progressive from onset
patient has NEVER experienced visual, sensory, or bladder symptoms
diagnostic findings (e.g., MRI, CSF, or EPs) are atypical
What is the tx for acute exacerbations? What is the pt does not respond to the first treatment?
glucocorticoids: High-dose IV or oral methylprednisolone: reduce severity and shorten duration of attacks
Plasma exchange to get rid of the antibodies
What is the first line tx for disease modifying treatment in MS? What is their MOA?
Monoclonal antibodies
function to decrease the AI response
**What do you need to screen for before starting a pt on monoclonal antibodies? **Why?
screen for immunity to Hepatitis A,B,C and VZV
**BBW: risk of progressive multifocal leukoencephalopathy (PML) an opportunistic viral infection (John Cunningham [JC] virus) of the brain that usually leads to death or severe disability
Need to assess if a pt has ______ before starting monoclonal antibodies. Do you want to result to be positive or negative? Why?
JC antibodies
patients who are seronegative are less likely to develop PML
**Which monoclonal antibody is the only agent indicated for PPMS? What are the rest of the agents indicated for?
ocrelizumab (Ocrevus)
rest are indicated for RRMS
What is the 2nd line tx for disease modifying therapy in MS?
Fumarates and Sphingosine 1-phosphate receptor (S1PR) modulators
______ MOA not fully known - modulates proinflammatory and anti-inflammatory cytokines. Has anti-oxidative properties. what do they end in?
fumarates
fumarates
_____ MOA binds to the lymphocyte surface (within the lymph nodes/spleen) preventing the release of lymphocytes into the PNS in turn preventing migration to the CNS. What do they end in?
Sphingosine 1-phosphate receptor (S1PR) modulators - end is “mod”
What is the 3rd line tx in MS? What is the MOA?
Platform Injection Therapy:
Glatiramer Acetate
Interferon β
Works in a variety of ways to suppress the immune system
What is used in MS to treat the ataxia/tremor? What is used to treat severe spasticity and spasms?
Clonazepam, propranolol, primidone
Thalamotomy and deep-brain stimulation - mixed results when utilized
severe: Baclofen oral or intrathecal pump if severe
______ is MS specific med that is used to treat the symptomatic LE weakness when it interferes with ambulation. What is the MOA?
dalfampridine (Ampyra)
K+ channel blocker
What factors favor a BETTER prognosis in MS?
ON or sensory symptoms at onset
fewer than two relapses in the first year of illness
minimal impairment after 5 years
What factors favor a WORSE prognosis in MS?
truncal ataxia
action tremor
pyramidal symptoms
primary progressive disease
What are the 4 pt education points needed in MS?
- maintaining an optimistic outlook
- a healthy diet
- regular exercise without excessive overheating as tolerated. swimming is often well-tolerated because of the cooling effect of cold water
- correct vitamin D deficiency
A patient with RRMS presents with complaints of increased weakness and double vision that began this morning. On exam you note a temperature of 103.2. Work-up reveals the patient is positive for Influenza A. Should we start high dose steroids to treat the symptoms of her acute MS?
not recommended to tx because the pt is experiencing “pseudo-exacerbation” due to the increase in body temperature not actually a true MS acute episode. Tx the flu and MS symptoms should improve
What is the key description of cerebral palsy? What is the underlying cause?
An umbrella term used to describe a group of disorders that cause permanent, NON-PROGRESSIVE motor dysfunction affecting muscle tone, posture, and/or movement
Results from an insult to the developing brain during birth, delivery, or in the perinatal period
______ is the leading cause of childhood disability. _____ are congenital and _____ are acquired
cerebral palsy
70-90% are congenital
10-30% are acquired
What are the risk factors for cerebral palsy? which ones are modifiable?
Genetic abnormality
toxic or infectious etiology
vascular insufficiency
Modifiable risks: maternal smoking, maternal heavy alcohol use, maternal obesity
Gross motor delay in first year of life
Abnormal muscle tone - most often hypotonia followed by spasticity
Definite hand preference before 12 months of age
Asymmetric crawling or failure to crawl
Growth disturbances (weight/height)
Hyperreflexia
Some cases - persistent primitive reflexes
What am I?
cerebral palsy
What are the different ways CP is classified? Which on is the MC?
Based upon predominant impairment AND location of disability
spastic- MC 70-80%
dyskinetic
ataxic
AND (see picture)
Name 3 additional tests used in CP. Why would you order each?
What is Plexiform neurofibroma?
tumor formation on a nerve plexus, can lead to disfigurement
**What is the criteria to dx neurofibromatosis type 1?
Requires at least 2 of the 7 NIH criteria
