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Cheng-Ching > Movement > Flashcards

Movement Flashcards

1
Q

Direct pathway

A

Substantia nigra pars compacta D1 neurons activate striatum (caudate/putamen)
Cinnamon Candy Great in Taste: cortex ->caudate/putamin->globus pallidus internus ->thalamus
Only SNc, cortex, thalamus positive outputs

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2
Q

Indirect pathway

A

Substantia nigra pars compacta D2 projections inhibit striatum (caudate/putamen)
CCGeSGiT
Cinnamon Candy Great Excitement and Supergreat in Taste: cortex ->caudate/putamin->globus pallidus externa -> STN -> GPi/substantia nigra pars reticulata ->thalamus
Only STN, thalamus, cortex positive outputs

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3
Q

Basal ganglia neurotransmitter

A

Glutamate

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4
Q

Vascular Parkinson’s

A

Upper extremities usually spared

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5
Q

Non motor PD Sx

A

Shoulder pain bc less movement of joint
Constipation
REM sleep behavior not just in DLB

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6
Q

Alpha synucleinopthy vs tauopathy vs beta amyloid

A

Alpha syn - PD, LBD, MSA, AD

Tau - CBD, PSP, Pick’s

Beta amyloid - AD, CAA, vascular dementia

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7
Q

Pd + dementia criteria

A

PD present for 1+ yr before dementia

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8
Q

PD path
MSA path

A

Alpha synuclein deposition

Loss of DA in SNpc
Lewy bodies - neuronal intrachtoplastic inclusions

MSA - oligodendroglial inclusions

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9
Q

PD genes

A
  • LRRK2 mutation - AD, common cause familial PD
  • Parkin (PARK2 gene) - AR, juvenile onset
  • alpha-synuclein (PARK1 gene) - AD- young onset - synaptic vesicle trafficking problem
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10
Q

tolcapone

A

-Tall Al Capone gangster: tolcapone (a peripheral and central COMT inhibitor) increases the bioavailability of L-Dopa in periphery + CNS
-Hepatic gun: tolcapone can cause hepatic failure

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11
Q

entacapone

A

Al Capone gangster at entrance:
-a peripheral COMT inhibitor-increases the bioavailability of
levodopa

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12
Q

carbidopa

A

peripheral dopa-decarboxylase inhibitor;
it reduces conversion of levodopa into dopamine in the periphery while not inhibiting central conversion
-decreases nausea

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13
Q

trihexyphenidyl and benztropine

A

Tri-hex Benz car: (antimuscarinic agents used to treat parkinsonism)
-antimuscarinic agents
-Trembling getaway car- improve tremor and rigidity of
PD with no effect on bradykinesia

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14
Q

DBS

A

-reducing tremor + bradykinesia
- not levodopa-unresponsive gait freezing, falls, or other axial symptoms

STN, GPi
-Ventral intermediate (VIM) nucleus -tremor (PD or ET)

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15
Q

PSP

A

no ataxia, autonomic Sx vs MSA
-onset 70s, later vs PD, MSA
-symmetric parkinsonism
-downgaze palsy, square wave jerks
-midbrain atrophy-hummingbird
-retrocollis (vs MSA-antecollis)

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16
Q

MSA

A

alpha synucleinopathy
-glial cytoplasmic inclusions in oligodendrocytes
-antecollis neck
-bilateral rest tremor, ataxic-jerky myoclonic tremor
-mottled cold hands
-inspiratory sigh/gasp, stridor (laryngeal spasm weakness)
-not L dopa responsive (or minimal)
-start in 60s

MSA-A-/Shy-Drager - autonomic dysfxn; syncope
-urinary incontinence - Onuf’s nucleus, impotence, larygneal dystonia (fatal)

MSA-P - parkinsonism- rapidly progressive
*MSA-C-cerebellar ataxia *

-hyperintense putamen rim
-cross buns sign - loss pontocerebellar tract

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17
Q

DLB

A

cognitive impairment within 1 yr PD Sx
Core clinical features: fluctuating cognition
formed visual hallucinations,
REM sleep behavior disorder
1 cardinal feature of PD
-L Dopa not tolerated; very sensitive to neuroleptics
-can have autonomic dysfxn ~Msa

Washout MIBG scan, no cardiac uptake of isotope , horseshoe shaped gap - in PD and DLB, bc pre and post ganglionic autonomic failure
(normal in MSA bc preganglionic autonomic failure)

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18
Q

CBD

A

tauopathy
-myoclonus, higher cortical sensory loss, and dystonic posture with absence of prominent falls
Apraxia, can’t follow command
Posturing= dystonia
waving arm around (alien limb) waving fingers around
Myoclonic jerks that looks tremulous
prominently asymmetric
-focal limb rigidity/dystonia,
-alien limb
agraphesthesia
astereognosis
-parkinsonian

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19
Q

drug induced parkinsonism

A

manganese toxicity (not Mg toxicity)
-welders, miners, chronic liver disease
-parkinsonism without tremor
-psych Sx
-cock walk: toe walking+elbow flexion
-hyperintensity in BG

-carbon monoxide

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20
Q

essential tremor

A

Tx: propranolol; primidone; topiramate; clonazepam
-4-8 Hz

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21
Q

Tourette’s syndrome

A

motor+ photic tic (throat clearing)

DA hyper-stimulation ventral striatum + limbic system

Tx: Anti-DA agents such as haloperidol, pimozide, and the atypical antipsychotics.
Clonidine, an α-2-adrenergic agonist, ADHD and other behavioral aspects of Tourette’s syndrome, and improves tics

secondary tourettism -ASD, static encephalopathy, neuroacanthocytosis, HD, medications, infection

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22
Q

complex vs simple tic

A

complex motor tic: coordinated sequenced movements that resemble normal movements such as truncal flexion or head shaking.

Simple phonic tics include sniffing, throat clearing, grunting, or coughing.

Complex phonic tics include verbalizations such as shouting obscenities (coprolalia), or repeating others (echolalia) or oneself (palilalia)

23
Q

Amantadine

A

Tx Peak dose dyskinesia

24
Q

Wilson’s disease

A

AR mutation copper transporter -ATP7B.
Tremor: wing beating arms
Grin with drooling

MRI - giant panda - T2 signal in caudate, putamen, midbrain with red nucleus spared

Dx: urinary excretion of copper high, ceruloplasm low in serum

Tx: zinc supplement (binds copper), low copper diet, d penicillamine/trientine dihydrochloride

25
Huntington’s
Motor impersistence + chorea (vs Wilson’s) AD, CAG repeat -can’t test ASx minors until they can consent
26
Sydenham’s disease
Autoimmune, 1-8 months after infection group A strep or only Sx -BL chorea , emotional lability Tx: DA antagonist - antipsychotic
27
Neuro acanthocytosis
Chorea-acanthocytosis- **SEIZURE + areflexia* Dystonia, chorea in 30s, 40s , self mutilating, tongue protrusion dystonia - VPS13A disease -elevated CK and liver enzymes -acanthocytes on smear with little protrusions from cells, does not rule out if negative Mcleod’s syndrome - XK disease (kell protein blood group); X linked chorea-acanthocytosis - XK disease-middle aged men -liver problems, decreased reflexes Abetalipoproteinemia - low serum cholesterol , low Vitamin E
28
Dentatorubral-pallidoluysian atrophy
AD neurodegenerative, Asian pts Trinucleotide repeat CAG - atrophin-1 gene chr 12p - choreoathetosis, dementia, ataxia like Huntington Start in 40s
29
Hemiballismus
Tx anti DA therapy
30
Tardive dyskinesia
Tx: clonazepam, tetrabenazine
31
Primary generalized dystonia
Generalized: At least two body parts involved AD inherited dystonia - torsin A gene mutation Sx in childhood, then progress Not DA responsive
32
Meige’s syndrome
Blepharospasm Oromandibular dystonia
33
Segawa Dopa-responsive dystonia
More in females AD inheritance, GCH1 mutation (growth hormone and fat ppl on segways, dominant) Mild Parkinsonism Diurnal variation - worse in afternoon/ PM -responds to low dose L Dopa without dyskinesias
34
Lance Adams syndrome
Months or years after hypoxia
35
Palatal tremor/myoclonus
Clicks in ear If from lesion in brainstem - persist during sleep -> Guillian Mollaret triangle dentate cerebellum, inferior olive, red nuc -can see hypertrophy inferior olive if symptomatic lesion
36
Paroxysmal kinesigenic dyskinesia
Familial or sporadic Trigger: startle, sudden movement Lasts Sec to 5 min (vs paroxysmal nonkinesigenic dyskinesia - min to hrs; fewer episodes) Tx: ASMs, carbamazepine
37
Episodic Ataxia type I Episodic ataxia type II Type iii Type Iv
Type I: KCNA1 potassium channel gene mutation -facial twitching sec-min **Tx: ASM, carbamazepine** Type II: Brainstem Sx - nystagmus, dysarthria (no facial twitching), HA min-hrs -CACNA1A4 Ca channel mutation - also familial hemiplegic migraine **Tx: acetazolamide** Type III: AD, tinnitus, vertigo; myokymia btw attacks Type IV: ocular motion problem
38
Stiff person
Tx: benzos, baclofen Lumbar lordosis, increased tone paraspinal muscles -if surprise ->get spasm, exaggerated startle response Age 40s-50s onset Autoimmune or paraneoplastic If anti-GAD Ab - can see endocrine problems, insulin dependent DM
39
Hyperrekplexia
Familial - glycine R, transporter mutations Glycine: inhibitory neurotransmitter at spinal interneurons Abnormal spinal la inhibitory interneuron reciprocal inhibition Sx: exaggerated startle reaction Dx: rule out startle evoked seizures - Tx: benzos, valproate
40
Cerebellum anatomy
Purkinje cells - GABA - inhibitory *Cerebellar efferents - superior cerebellar peduncle* Afferents - inferior CP, middle CP, superior CP, inferior olivary nucleus Spinocerebellar - afferents = mossy fibers
41
Acquired cerebellar ataxia
1. Celiac disease - ataxia only can have no GI Sx - check celiac AB-anti-gliadin Ab 2. Hypothyroidism 3. Toxins: mercury, bismuth salicylate, toluene
42
Friedreich’s ataxia
AR trinucleotide repeat expansion - GAA in frataxin gene High arched feet, scoliosis Presents ~young adult Cardiac conduction abnormalities Cardiomyopathy tx - idebenone - Coenzyme Q10 analog
43
Ataxia telangiectasia
AR mutation ATM gene - DNA repair problem Can’t move eyes without head thrust Cancer, immunocomp (hypogammaglobulinemia) -high AFP
44
Spinocerebellar ataxias
Autosomal dominant starts age 30s-50s Mostly trinucleotide repeat expansions Sca1- ATXN1 CAG repeat - presents 30s-40s Sca2- ATXN2 CAG repeat Most common: SCA3 - ATXN3 gene-CAG repeat expansion -facial, tongue atrophy , fasiculations, L dopa responsive parkinsonism Sca7 - retinopathy, vision loss
45
Fragile X syndrome
Trinucleotide repeat expansion CGG on FMR1 gene X linked, affects males more Dysautonomia, ataxia, parkinsonism Hyper intensities cerebellum FHx intellectual disability (vs MSA)
46
Cerebrotendinous xanthomatosis
AR Ataxia, Psych Sx, Parkinsonism, tendon deposition fat Dx: **cholestanol** not cholesterol bc normal
47
Fahr disease / striopallidodentate calcinosis
BL calcification BG and cerebellum Idiopathic or genetic Any parathyroidism hyper or hypo
48
Nonketotic hyperglycemia
T1 hyper intensity unilateral striatum Tx: DA R antagonist if persistent Sx
49
DAT scan
pre-synaptic DA (does not measure putamen b/c post-synaptic) -loss of density and intensity in striatum, asymmetrical corresponding with contralateral Sx
50
PET
fluorodopa - reduced uptake in putamen
51
neurodegerneration with brain iron accumulation
-parkinsonism, dystonia, choreoathestosis, ataxia - PKAN - eye of tiger PLAN - PLA2G6 MPAN - BPAN - beta propeller protein associated neurodegeneration - WD repeat domain 45 gene X linked most female-WDR45 -hyperintense lesion on T1 in cerebral peduncles/substantia nigra midbrain = Halo sign -psychomotor regression then stable static encephalopathy as kid; then dystonia later -seizures Chorea- neuroferritinopathy , aceruloplasminemia FAHN Kufor-Rakub CoPAN
52
Kernicterus
BL BG / globus pallidus hyperintensity on T2
53
no loss of olfaction early
MSA, PSP
54
C9orf72 disease
AD, hexanucleotide repeat expansion - GGGGCC -most common cause familial and sporadic ALS and FTD -early behavioral problems, upper motor neuron signs, chorea, dystopia, myoclonus, tremor, PD (phenotype not related to size of expansion)

Cheng-Ching (17 decks)

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