MS is a disease of (X) cells, causing (central/peripheral) de-myelination.
X = oligodendrocytes
Central only! (doesn’t affect Schwann cells)
Role of (X) supplementation and deficiency has been found to play a role in MS.
X = vitamin D
Vit D supplementation prevents and slows the progression of the
disease, while deficiency worsens the disease
Migration studies support the view that there is (X) trigger is in the pathogenesis of MS.
X = environmental (low risk in Japan and almost no cases in Inuit populations)
List the 4 MS phenotypes and star the one seen most commonly at onset of MS in patient.
- Pure relapsing-remitting (RRMS)* (85%)
- Secondary-progressive (SPMS)
- Primary-progressive (PPMS; 10%)
- Progressive relapsing (PRMS; 5%)
(X) phenotype of MS develops in many patients that initially had pure relapsing-remitting (RRMS).
X = secondary-progressive (SPMS)
What’s characterized as benign MS?
No progression for 15 y
What’s Clinically Isolated Syndrome (CIS)?
One neurological episode
or “relapse” of MS
List three clinical symptoms of visual pathways seen in MS relapse.
- Diplopia (INO or CN 3, 4, 6 issue)
- Optic neuritis (painful movement, vision affected)
- Nystagmus
MS clinical symptom: Localized SC inflammation that involves the full thickness of the cord and produces symptoms related to the sensory/motor innervation at that level.
Transverse myelitis
MS diagnosis is based on either (X) or (Y).
X = Multiple white matter lesions separated in time and space (relapsing disease) Y = Progressive disease from onset with typical clinical findings
McDonald criteria for (X) findings in (Y) disease.
X = dissemination in time and space of lesions Y = MS
MS: list the ways that MRI lesions meet McDonal criteria for “disseminated in time”
- Both enhancing and non-enhancing lesions in same MRI OR
2. Appearance of new lesions after initial MRI
MS: list the ways that MRI lesions meet McDonal criteria for “disseminated in space”
At least one T2 lesion in 2 out of the 4 typical locations for MS
List the four typical locations for lesions in MS.
- Periventricular
- Juxtacortical
- Infratentorial
- SC
T/F: There are no definitive laboratory test or MRI features for MS.
True - other diseases can mimic MS (consider alternative diagnoses, even after making MS diagnosis)
Progressive MS diagnosis: (X) year(s) of disease progression plus 2/3 of which criteria?
X = 1
- Lesions disseminated in space in brain
- Lesions disseminated in space in SC
- Oligoclonal bands in in CSF
Neuromyelitis Optica (NMO) was initially thought to be part of (X) disease, but is now considered distinct. What are the symptoms?
X = MS
Optic neuritis and transverse myelitis
T/F: Neuromyelitis Optica has better prognosis than MS.
False
(X) Ab in (serum/CSF) is seen in 80% of patients with Neuromyelitis Optica (NMO)
X = Aquaporin-4
Serum or CSF
List some clinical symptoms that can act as hints for Neuromyelitis Optica.
- Poor visual recovery
- Bilateral optical neuritis
- Transverse myelitis
- Hiccups, N/V (area postrema affected)
List two “injectable” disease-modifying drugs for MS that have been around for over 20 y. These are administered via which route?
- IFN-beta (IM, SC)
2. Glatiramer acetate (SC)
What is the mechanism of the disease-modifying drugs for MS (IFN-beta, Glatiramer acetate).
Th1 (bad) to Th2 (good) shift
List some oral disease-modifying drugs for MS.
- Fingolimod
- Teriflunomide
- Dimethyl Fumarate
MS medication: Fingolimod MOA
Sphingosine R blocker (inhibits T cell migration from lymph nodes)
(X) drug improves walking speed in 30-40% of patients with MS.
X = Dalfampridine
Tremor that improves with EtOH.
Essential tremor
Most common movement disorder.
Essential tremor
Essential tremor (present/absent) at rest and (present/absent) during action. Family Hx positive in (X)% of patients.
Absent; present
X = 50 (AD)
Essential tremor has been correlated to activity in (X) parts of brain.
X = sensorimotor area and cerebellum
Essential tremor: more (symmetric/asymmetric) than the tremor of PD. (X) key PD symptoms are not seen in this tremor.
Symmetric;
X = bradykinesia and rigidity
Essential tremor: usually involves (upper/lower) limbs in addition to:
Upper
Head (no-no) and voice
Essential tremor: which two meds mainly used (solo or in combo).
- Primidone (old antiepileptic)
2. Propranolol
The target for the treatment of essential tremor with Deep Brain Stimulation is the (X).
X = ventrointermediate
nucleus (VIM) of the thalamus
Parkinsonism comprised of which constellation of signs on PE?
- Rigidity
- Bradykinesia (slow mvmt)
- Gait abnormalities/postural instability
- Tremor
Difference between Parkinsonism and PD.
PD is Parkinsonism with no known cause
Parkinsonism can have which etiologies?
- Drug/med or toxic cause
- Vascular
- Post-traumatic
- NPH (Normal P hydrocephalus)
Mean survival in PD after onset is (X) years and most common causes are:
X = 20+
pulmonary infection/aspiration, and complications of falls
PD: CT/MRI show (X) abnormalities and SPET/PET show (symmetric/asymmetric) (Y) abnormalities.
X = no
Asymmetric
Y = decrease dopamine levels
DaTscan used to detect:
Dopamine transporters
(DaT) in the brain
Risk Factors for PD:
- Age
- Genetics
- Environmental (toxic products, well water, repeated head injury)
Alien hand syndrome can occur in (X), which is one of the (Y) Syndromes.
X = Corticobasal degeneration Y = Parkinson Plus
Corticobasal degeneration has (symmetric/asymmetric) onset with (slow/rapid) clinical course and (X) changes on CT/MRI.
Asymmetric;
Rapid (5-10y)
X = contralateral atrophy
Corticobasal degeneration: PET may show (increased/decreased) contralateral fluorodopa uptake in (X) parts of brain.
Decrease
X = basal ganglia and association cortices
T/F: Parkinson Plus Syndromes respond to PD drugs.
False
T/F: Parkinson Plus Syndrome pathology involves Tau protein
True
Parkinson plus syndrome with gait disturbance and frequent falls:
Progressive Supranuclear Palsy (PSP)
T/F: Neuro manifestations of Wilson’s includes tremor.
True - wing-beating
Wilson’s: (high/low) ceruloplasmin, and (high/low) urinary copper levels.
Low; high
Slit lamp examination revealing Kayser-Fleischer rings is indicative of (X) disease.
X = Wilson’s
MRI in Wilson’s often shows abnormal (T1/T2) signaling in (X) part of brain.
T2
X = basal ganglia
Medical therapies for Wilson’s include:
- Reducing copper
in the diet - Rx with zinc, penicillamine, or trientene
- Liver transplant
Huntington’s: (X) inheritence with gene on chromosome (Y). What’s the trinucleotide repeat sequence?
X = AD Y = 4
CAG
Huntington’s: Imaging studies reveal …
Atrophy of caudate and putamen
Which meds can be used to treat Huntington’s chorea?
- Dopamine antagonists (haloperidol, risperidone, olanzapine)
- Benzos may help
- Tetrabenazine (suppresses chorea)
What’s ataxia?
Lack of coordination while performing voluntary movements (may appear as
clumsiness, inaccuracy, or instability)
Ataxia usually caused by damage to the (X).
X = cerebellum (inputs/outputs)
Rx for ataxia:
Treat underlying cause; supportive therapy
Rx for myoclonus
Removing the offending agent, and considering treatment
with an antiepileptic (valproate), or a benzodiazepine
Cornerstone of symptomatic
management of PD that replaces (X) (pre/post)-synaptically.
Carbidopa/levadopa
X = DA
Pre-synaptically
PD treatment: DA receptor (agonist/antagonist) that acts directly on (pre/post)-synaptic receptors.
Pramipexole
Agonst; post-synaptic
(X) PD drug is COMT, aka (Y), (stimulator/inhibitor) that increases amount of (Z) available
X = entocapone
Y = Catechol O-methyl transferase
Inhibitor
Z = levodopa
(X) PD drug is MOA-B (stimulator/inhibitor) that increases amount of (Y) available
X = Rasalagine
Inhibitor
Y = dopamine
Carbidopa is added to levodopa to:
decrease the
nausea
Levodopa side effects:
N/V, dyskinesias (late side effect)
(Levodopa/Pramipexole) does not compete with protein in food and can be taken at any time.
Pramipexole (DA agonist)
Pramipexole side effects:
Drowsiness (sleep attacks);
Others: HA, confusion, compulsions, hallucinations and paranoia
Entacapone should be used in combo with (X) to be effective.
X = levodopa
since it’s a COMT inhibitor
Side effects of Entacapone:
Diarrhea, abdominal pain, urine discoloration, liver function changes
Side effects of Rasaligine:
HA, nausea, HT arthralgia, dyskinesias, hallucinations
(X) drug was introduced as an antiviral agent and reported
useful in PD in 1969. What’s its MOA?
X = amantadine
DA reuptake blockade
activity, anticholinergic action and blockade of NMDA receptors
Side effects of amantadine:
Confusion, hallucinations
Anticholinergic drug occasionally (now rarely) used for the treatment of PD:
Trihexyphenidyl HCl
Writing or dancing movements that patients may experience after they have been on dopaminergic medications for 5+ years
Dyskinesias (considered “ON” behavior and may not troublesome to patient)