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94 Cards in this Set

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parts of the substantia nigra

pars reticularis --> outer column
pars compacta --> inner column

substantia nigra is located in the midbrain dorsal to cerebral peduncles and anterior to red nucleus

caudate and putamen
lenticular nucleus
globus pallidus and putamen

major inputs to striatum

1. all areas of the cortex --> excitatory/glutamate neurons
2. intralaminar nucleus of the thalamus
3. SNc --> dopaminergic neurons enhance direct and indirect pathways via D1 and D2 receptors respectively.
4. locus cereleus and raphe nucleus
major outputs of basal ganglia in general
from GPi and SNr to:

1. VA and VL thalamus --> primary motor cortex
2. pedunculopontine nucleus
projections of GPi
1. VA and VL thalamus
2. pedunculopointine nucleus
3. centromedian thalamic nucleus
projections of the SNr
1. VA and VL thalamus
2. pedunculopointine nucleus
3. mediodorsal thalamic nucleus and superior coliculi
sensorimotor division of striatum
starts in all areas of cortex to putamen then back to premotor, primary and summplementary motor cortex

mediates movement
associative division of striatum
starts in cortex of all lobes, goes to dorsal caudate nucleus then back to the prefrontal cortex

mediates cognition
limbic division of striatum
starts in hippocampus, amygdala, cigulate cortex, temporal and orbitofrontal cortex

goes to the ventral striatum then out to anterior cigulated cortex and medial orbitofrontal cortex

mediates emotion and motivation
functional divisions of the striatum
sensorimotor --> mediates motor functions
associative --> mediates cognition
limbic --> mediates emotion and motivation
default output of GPi and SNr
inhibitory GABA neurons to VL and VA thalamus
action of corticostriatal neurons
excitatory glutamatergic neurons to striatum

inputs to GPi and SNr
direct pathway --> from striatum --> GPi/SNr
indirect pathway --> from striatum -->GPe --> STN --> GPi/SNr
direct pathway
corticostriatal glutamate neurons stimulate striatum
inhibitory GABA neurons in striatum inhibit GPi/SNr (which contains GABAergic neurons)
inhibited GPi/SNr disinhibits VA/VL thalamus (which contains glutamatergic projections)

net effect is thalamocortical stimulation enhanced by SNr via D1
indirect pathway
corticostriatal glutamate neurons stimulate striatum
GABA neurons in striatum inhibit GPe
inhibited GABA neurons of GPe disnhibits STN
increased glutamate from STN stimulate GPi/SNr leading to inhibition of VA/VL thalamus

net effect is thalamocortical inhibition enhanced by SNr via D2
palatal myoclonus


serotonergic centers of the brain

dorsal raphe nucleus, hippocampus, cerebellum, spinal cord

norepinephrine centers in the brain

locus cereleus
lateral tegmentum

movement disorder tauopathies
Four-repeat tau
movement disorder synucleinopathies
polyQ versus non-polyQ
most trinucleotide repeat disorders are CAG repeats

CAG in coding regions --> glutamine (Q) --> polyglutamine or polyQ disorder
CAG in non coding regions --> non-polyQ disorders
polyQ movement disorders
dentatorubral-pallidoluysian atrophy (DRPLA)
several of the spinocerebellar ataxias
prevalence of parkinsonian syndromes
PD --> 80%

non-motor symptoms of PD
autonomic --> bloating/constipation, frequency, urgency, impotence, orthostatic hypotension
cognitive --> decreased memory, attention and executive functions; dementia in 30%
psych --> anxiety, depression
sleep --> almost all have sleep disturbances --> REM behavior disorder, RLS, vivid dreams and nightmares, ""sleep attacks""
PD red flags -->
cognitive impairment within first year --> dementias
symetrical presentation of prominent bulbar signs --> MSA or PSP
early gait disorder with falls --> PSP or vascular disease
wheelchair-bound by 5 years --> PSP or MSA
early autonomic dysfunction --> MSA
severe sleep apnea or onvoluntary sighing --> MSA
apraxia, alien limb or cortical sensory loss --> CBD
PD red flags --> cognitive impairment within first year

PD red flags --> symetrical presentation of prominent bulbar signs

PD red flags --> early gait disorder with falls
PSP or vascular disease

PD red flags --> wheelchair-bound by 5 years

PD red flags --> early autonomic dysfunction

PD red flags --> severe sleep apnea or onvoluntary sighing

PD red flags --> apraxia, alien limb or cortical sensory loss

DaTscan (PET)

iodine 123 radioisotope with high affinity for presynaptic DAT transporters which are decreased in the striatum in PD

used to differentiate PD from essential tremor
Lewy body
intracytoplasmic eosinophilic inclusions with halo in pigmented neurons, including SN
stain positive for alpha-synuclein and ubiquitin
Lewy neurites
tiny projections growing out of alpha-synuclein positive neurons and inclusions

epidemiology of PD
90-95% idiopathic with 15% having first-degree relatives with PD
5-10% familial with specific mutations, various modes of inheritance, phenotypes and penetrance
manifestations of bradykinesia
slow small-amplitude movements, progressively worse with repetition

in limbs --> decreased arm-swing, micrographia, lack of dexterity
speech --> hypophonia
swallowing --> dysphagia
gait --> shortened stride length
face --> hypomimia
posture --> stooping or leaning
most essential sign for diagnosis of parkinsonism

characteristics of parkinsonian tremor
most noted at rest
increased with mental concentration during tasks
characteristics of parkinsonian gait
postural instability and gait disturbance (if early in the course --> PSP, MSA)
narrow-based, short stride length, reduced arm swing, slow intiation, turning en-bloc
PSP and MSA have impaired tandem gait and wider-based gait
criteria for PD diagnosis
1. bradykinesia AND resting tremor, rigidity or postural instability
2. unilateral onset, excellent response to levodopa, levodopa-induced dyskinesia

if early prominent postural instability, dysautonomia, dementia, gaze palsy, neuroleptics or poor response to levodopa --> consider parkinsonian syndromes
classic PSP presentation
usually starts with postural instability with falls
visual and speech difficulties
decreased vertical sacades with square-wave jerks
no significant dysautonomia
midbrain atrophy
humingbird sign
eyelid apraxia
PSP-parkinsonian variant
treat with botox
variants of PSP
PSP Richardson syndrome (classic)
PSP-pure akinesia with gait freezing
signs of dysautonomia in MSA
urinary urgency
orthostatic hypotension
erectile dysfunction
parkinsonism in MSA
parkinsonian features usually become prominent after onset of dysautonomia
usually symetrical parkinsonism
gait may be parkinsonian (narrow) or cerebellar (wide-based)
stimulus-induced myoclonus is more common than resting tremor
putamen hyperintense external rim and hypodensity
pontine atrophy in sagital view
hot bun cross sign
MSA response to levodopa
30% of MSA responds to levodopa and get dyskinesias

presentation of corticobasal syndrome
asymetric progressive ideomotor apraxia staring in the hand and progressing to leg
associated with rigidity, myoclonus and dystonia
alien limb phenomenon --> involuntary grasping, purposeless movements and levitation of affected arm

overlaps with AD, PSP and FTD
MSA pathology
neuronal loss and gliosis in striatum, SN, LC, inferior olive, pontine nuclei, intermediolateral cell column, onuf nucleus

depletion of cholinergic neuron in PPN and laterodorsal tegmental nucleus

alpha synuclein inclussions are in glia cells throughout
HD genetic testing
CAG repeat expansion > 37 is pathogenic
discouraged in asymptomatic patients
genetic counseling before and after testing are recommended
HD pathology
neuronal loss and gliosis in striatum seen with GFAP stain
mostly affects GABAergic medium spiny neurons of striatum predominately in indirect pathway
it also affects other areas of the cortex and brainstem nuclei
types of tremor
rest tremor --> at rest
postural tremor --> when arms are held up
kinetic tremor --> during movement, specially towards the end of movement
intention tremor --> also a kinetic tremor but implies cerebellar dysfunction
rubral tremor --> at rest, worse with posture and even worse with movement and intention
differential diagnosis of rest tremor
vascular parkinsonism
SCA 2 and 3
essential and dystonic tremors can have rest component
essential tremor characteristics
bilateral symetrical postural and kinetic tremor of hands, forearms and/or head

differential diagnosis of postural tremor
enhanced physiologic tremor
essential tremor
dystonic tremor
metabolic derangements
PD may have a postural component
dystonic tremor
associated with the body part affected by dystonia or a different body part
usually jerky tremor, position-specific and sometimes task specific
rubral tremor lesion
midbrain tegmentum, specially superior cerebellar peduncle

physiologic myoclonic jerks
hypnic jerks
things to evaluate in myoclonus
progressive Vs. nonprogressive
at rest Vs. on action
spontaneous Vs. sensory stimulation
segmental Vs. generalized
myoclonus-dystonia presentation
myoclonus in the upper trunks and proximal arms associated with or without mild cervical dystonia or writter's cramp

myoclonus-dystonia genetics
autosomal dominant with complete paternal penetrance and 15% maternal penetrance
DYT11 --> chr 7; SGCE gene
DYT15 --> chr 18; unknown gene
orthostatic myoclonus etiology
Lance-Adams syndrome
posthypoxic myoclonus
subcortical myoclonus in survivors of cardiorespiratory arrest
specially following respiratory dysfunction and hypercapnia as in asthma
what is opsoclonus
chaotic myoclonic eye movements

opsoclonus-myoclonus ataxia syndrome in children
opsoclonus with multifocal myoclonus and truncal/appendicular ataxia
usually paraneoplastic in setting of neuroblastoma or ganglioneuroblastoma
opsoclonus-myoclonus ataxia syndrome in adults
usually postinfectious or autoimmune; less commonly paraneoplastic from anti-Hu or anti-Ri
associated autoimmune antibodies include anti-Gad, anti-NMDA, anti-gliadin, antiendomysial
autoantibodies bind cerebellar Purkinje and granular cells
progressive myoclonic encephalopathies
Unverritch disease (Baltic myoclonus)
Ramsay-Hunt syndrome (mediterranean myoclonus)
Sialidosis (cherry-red spot myoclonus)
Neuronal ceroid lipofuscinosis
Myoclonic epilepsy with ragged red fibers (MERRF)
Loafer body disease
Gaucher disease type 3
Krabbe disease
pure myoclonic encephalopathies
Infantile myoclonic encephalopathy (Ohtahara syndrome)
Severe myoclonic epilepsy of infancy (Dravet syndrome)
Myoclonic astatic epilepsy (Doose syndrome)
West syndrome
Lennox-Gastaut syndrome
Ladau-Kleffner syndrome
Rasmussen syndrome
non-progressive myoclonic epilepsies
Benign rolandic epilepsy (Benign epilepsy of childhood with centrotemporal spikes (BECTS)
Myoclonic absence seizures
autosomal dominant presents mostly with hypertonia in infants (stiff baby)
tonic spasms and nocturnal myoclonus during first year of life
non-habituating startle responses to brief stimulation during wakefulness is hallmark
drugs that improve cortical myoclonus
drugs that worsen cortical myoclonus
cerebellar signs
oculomotor disturbances
speech deficits
disturbances in limb movements
deficits of posture and gait
deficits of cognitive operations
subtle autonomic signs
afferent ataxia Vs. cerebellar ataxia
afferent ataxia relies heavily on visual guidance (Romberg test)
afferent ataxia has no dysarthria and no oculomotor deficits
Romberg test
closing the eyes causes ataxia of balance
indicates afferent/proprioceptive ataxia
proprioception, vestibular and visual functions are required to maintain balance
autosomal dominant ataxias nonmenclature
Spinocerebellar Ataxias --> SCA

SCAs that have CAG repeat expansion
SCAs 1, 2, 3, 6, 7 and 17

Only SCA with macular degeneration

epidemiology of autosomal recessive ataxias
Friedreich Ataxia accounts for at least 1/3
20% of cases have an identified molecular defect in over 30 genes
50% remain without a diagnosis
genetics of Friedreich ataxia
autosomal recessive GAA repeat expansion in an intron of the frataxin (FXN) gene leading to decreased frataxin
frataxin is a mitochondrial protein involved in assembly of iron-sulfur clusters
presentation of Friedriech ataxia
progressive gait and limb ataxia prior to age 30
absent lower extremity DTRs and plantar extensor responses
dysarthria, oculomotor abnormalities
loss of proprioception and axonal sensory neuropathy
other features include --> diabetes, cardiopathy, scoliosis and pes cavus
cerebellar atrophy is minimal to none
x-linked ataxia
Fragile X tremor-ataxia syndrome

Fragile X tremor-ataxia syndrome
affects men over 50 years old
CGG repeat expansion in FMR1 gene on chr X
characterized by ataxia and action tremor
60% have parkinsonism
MRI --> FLAIR hyperintensities in middle cerebral peduncle and periventricular
diagnostic criteria for Tourette syndrome
motor and vocal tics of onset below age 21 with a duration of at least 1 year

Tourette syndrome presentation
motor and vocal tics
premonitory sensation
may be suppressed by volition to a variable extent
non-goal directed
worsens with stress and anxiety
usually present between 5-8 years of age
dopa-responsive dystonia
DYT5, Segawa's disease
more common in females, onset in early childhood
duirnl fluctuations, worse in the afternoons and evenings, may be asymptomatic earlier in the day
dystonia with parkinsonian features
responds to levodopa
HD presentation
gait instability
neuropsych features --> depression, psychosis, cognitive and executive dysfunction, personality changes
primary cervical dystonia
begins in adulthood with abnormal head posture, neck pain with or without blepharospasm
treat maily with Botox but also anticholinergics, benzodiazepines, baclofen
stereotypies Vs. complex tic
sterotypies don't have an premonitory urge with relief after executing tic

stiff person syndrome
begins in 4th-5th decades
increased axial tone, exagerated lumbar lordosis, stiff abdomen
worsened by startle response and anxiety
anti-GAD antibodies or anti-amphiphysin antibodies
treat with benzos and baclofen

autosomal recessive mutation of ATM gene leads to impaired DNA repair
presents in childhood with neuropathy, ataxia, opthalmoparesis, telengiectasias in conjunctiva
prone to infections due to hypogamaglobulinemia"