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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
Q

Huntington’s

A

Motor impersistence + chorea (vs Wilson’s)

AD, CAG repeat
-can’t test ASx minors until they can consent

26
Q

Sydenham’s disease

A

Autoimmune, 1-8 months after infection group A strep or only Sx
-BL chorea , emotional lability
Tx: DA antagonist - antipsychotic

27
Q

Neuro acanthocytosis

A

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
Q

Dentatorubral-pallidoluysian atrophy

A

AD neurodegenerative, Asian pts
Trinucleotide repeat CAG - atrophin-1 gene chr 12p - choreoathetosis, dementia, ataxia like Huntington
Start in 40s

29
Q

Hemiballismus

A

Tx anti DA therapy

30
Q

Tardive dyskinesia

A

Tx: clonazepam, tetrabenazine

31
Q

Primary generalized dystonia

A

Generalized: At least two body parts involved
AD inherited dystonia - torsin A gene mutation
Sx in childhood, then progress
Not DA responsive

32
Q

Meige’s syndrome

A

Blepharospasm
Oromandibular dystonia

33
Q

Segawa Dopa-responsive dystonia

A

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
Q

Lance Adams syndrome

A

Months or years after hypoxia

35
Q

Palatal tremor/myoclonus

A

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
Q

Paroxysmal kinesigenic dyskinesia

A

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
Q

Episodic Ataxia type I
Episodic ataxia type II
Type iii
Type Iv

A

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
Q

Stiff person

A

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
Q

Hyperrekplexia

A

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
Q

Cerebellum anatomy

A

Purkinje cells - GABA - inhibitory

Cerebellar efferents - superior cerebellar peduncle
Afferents - inferior CP, middle CP, superior CP, inferior olivary nucleus

Spinocerebellar - afferents = mossy fibers

41
Q

Acquired cerebellar ataxia

A
  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
Q

Friedreich’s ataxia

A

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
Q

Ataxia telangiectasia

A

AR mutation ATM gene - DNA repair problem

Can’t move eyes without head thrust
Cancer, immunocomp (hypogammaglobulinemia)
-high AFP

44
Q

Spinocerebellar ataxias

A

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
Q

Fragile X syndrome

A

Trinucleotide repeat expansion CGG on FMR1 gene
X linked, affects males more
Dysautonomia, ataxia, parkinsonism

Hyper intensities cerebellum

FHx intellectual disability (vs MSA)

46
Q

Cerebrotendinous xanthomatosis

A

AR
Ataxia, Psych Sx, Parkinsonism, tendon deposition fat

Dx: cholestanol not cholesterol bc normal

47
Q

Fahr disease / striopallidodentate calcinosis

A

BL calcification BG and cerebellum
Idiopathic or genetic
Any parathyroidism hyper or hypo

48
Q

Nonketotic hyperglycemia

A

T1 hyper intensity unilateral striatum
Tx: DA R antagonist if persistent Sx

49
Q

DAT scan

A

pre-synaptic DA (does not measure putamen b/c post-synaptic)
-loss of density and intensity in striatum, asymmetrical corresponding with contralateral Sx

50
Q

PET

A

fluorodopa - reduced uptake in putamen

51
Q

neurodegerneration with brain iron accumulation

A

-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
Q

Kernicterus

A

BL BG / globus pallidus hyperintensity on T2

53
Q

no loss of olfaction early

A

MSA, PSP

54
Q

C9orf72 disease

A

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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