Notes 3b Flashcards
Progressive muscular atrophy
features
spectrum of ALS: only LMN
focal asymmetric weakness hat later involves more proximal regions and the extremities: atrophy, hyporeflexia, and fasciculations
Spinal cord blood supply?
one anterior spinal artery and 2 posterior spinal arteries
Anterior spinal artery supplies? what tracts are in the territory?
Ant ⅔ of the spinal cord
corticospinal and spinothalamic tracts
Posterior spinal arteries supplies? what tracts are in the territory?
posterior ⅓ of the spinal cord
dorsal column
Largest radicular artery?
Artery of adamkiewicz
Why do mets from the pelvic region commonly migrate to the CNS?
epidural venous plexus is a valveless system
West Nile Virus
features
MRI
meningitis, encephalitis
presents with flaccid weakness, areflexia, proximal and asymmetric
MRI can show cauda equina, spinal cord, leptomeningeal enhancement
Adrenoleukodystrophy
inheritance, mutation
caused by
features
MRI
Tx
X linked, ABCD1 gene on X chromosome q28 (ATP-binding cassette transporter)
impaired VLCFA causing peroxisome dysfunction
paraparesis, sensory neuropathy, bulbar sx, hypogonadism and cognitive impairment
T2 hyperintensities in WM SPARING U FIBERS affecting predominantly the parieto-occipital regions and posterior corpus callosum (may show a rim of enhancement unlike other leukodystrophy)
steroids, or Lorenzos oil (4:1 glyceryl trioleate-glyceryl trierucate)
Central spinal cord lesions (syringomyelia)
presents with
“cape like” sensory loss of pain and temp in the spine
In the spine intramedullary tumors are more likely to be primary/mets?
primary if intramedullary
Most common spinal intramedullary tumor in adults? children
ependymoma
ependymoma and astrocytoma
Most common nonmetastatic extramedullary tumors
neurofibromas, schwannoma, meningiomas
Difference between intra and extramedullary
ADEM (acute disseminated encephalomyelitis)
features
caused by
tx
inflammation of brain and spine at the same time
monophasic disease caused by immune response to a virus
IV steroids
When ADEM afefcts with spine it presents with
transverse myelitis (>3 segments affected)
Copper myelopathy causes a pattern similar to ?
B12
Kennedy’s disease (X-linked spinobulbar muscular atrophy)
features
mutation
presents in males, 40s: proximal weakness with LMN features, gynecomastia, hypogonadism, DM
expansion of CAG repeat in the androgen receptor on X chromosome
BUZZWORDS: survival motor neuron 1 gene mutation
spinal muscular atrophy
BUZZWORDS: motor neuron diseases with only lower motor neuron involvement
Progressive muscular atrophy, spinal muscular atrophy, benign focal amyotrophy
BUZZWORDS: post-gastric bypass neurologic syndrome
copper def
BUZZWORDS: Devic’s Disease- Aquaporin-4
NMO
BUZZWORDS: nitrous oxide toxicity, assoc with B12 depletion
Anesthesia paresthetica
BUZZWORDS: ipsilateral loss of motor and sensation to vibration and proprioception below the level of the lesion, with contralateral loss of sensation to pain and temp
Brown-Sequard Syndrome
BUZZWORDS: tabes dorsalis is assoc with what infection
neurosyphilis
BUZZWORDS: MRI finding seen in patients on chronic steroids?
epidural lipomatosis
BUZZWORDS: Gene in adrenoleukodystrophy, X-linked
ABCD1 gene on chromosome Xq28
Conversion rate of MCI to dementia is
10-15%/ year
routine dementia screening labs
B12, CBC, CMP, TSH, depression screening
further testing includes HIV, VDRL, LP, heavy metals
genes involved in inherited dementia
Presenilin-2 on chromosome 1 leads to early onset familial AD
Presenilin-1 on chromosome 14 accounts for nearly 70-80% of early onset aggressive familial cases of AD
APP (amyloid precursor protein) on chromosome 21
Which mutation is assoc with 3x risk of Alzheimers
Apo-E4 on chromosome 19
Earliest signs of Alzheimers
recent memory loss of events
FDG-PET in Alzheimers
parietotemporal hypometabolism
FDG-PET in FTD?
hypo-metabolism in frontal and anterior temporal regions
FDG-PET in Huntington’s
caudate hypometabolism
FDG-PET in Lewy Body Dementia
hypometabolism in occipital regions
earliest feature of Alzheimer’s (pathologically)
Amyloid deposition is first, then plaques and tangles
What are Hirano bodies
eosinophilic rod like inclusions in the hippocampal pyramidal neurons seen in Alzheimers
Where is loss of cholinergic neurons in Alzheimers
Nucleus basalis of meynert
CSF findings in Alzheimers
reduced beta amyloid and increased total and phosphorylated tau
BUZZWORDS: NT in locus ceruleus
NE
BUZZWORDS: NT in median and dorsal raphe nucleus
serotonin
BUZZWORDS: NT in nucleus accumbens, ventral tegmental area
Dopamine
BUZZWORDS: NT in substantia nigra pars compacta
gaba
Triad in Lewy Body Dementia
Best neuroleptic?
parkinsonism
fluctuating cognitive impairment
visual hallucinations
Best neuroleptic is clozapine (hella side effects) and quetiapine
Familial FTD
chromosome assoc
types?
17
- Behavioral variant (most common): personality change, abulia, apathy, poor hygiene, hyperphagia
- Progressive confluent aphasia: anomia, word-finding difficulty, impaired object naming and effortful speech with preserved comprehension (similar to Brocca)
- Semantic dementia (progressive fluent aphasia): normal fluency but impaired comprehension, anomia, and semantic paraphrasias.
bilateral globus pallidus interna lesions (and sometimes bilateral ACA Infarcts) can cause?
akinetic mutism: preserved awareness with opened eyes but remains immobile, mute and does not respond to commands.
FTD is also assoc with what syndrome?
Kluver-Bucy Syndrome (bilateral lesions of the temporal lobe)
- hyperorality, hypermetamorphosis , blunted emotional effects, hyper sexuality, and visual agnosia
MOA of TGA
thought to be due to temporary ischemia of the medial temporal lobes/hippocampus
What are lewy bodies?
cytoplasmic inclusions with anti-ubiquitin and anti-alpha-synuclein immunohistochemistry
pathology for FTD?
- present of silver-staining, spherical aggregations of tau protein in neurons
Psychogenic amnesia: characteristic findings?
loss of autobiographical memory, sometimes with preserved ability for new learning
MOA of memantine
NMDA antagonist
MOA of donepezil
Acetylcholinesterase inhibitor
MOA of rivastigmine
combined acetylcholinesterase and butyrylcholinesterase antagonist
MOA of galantamine
- acetylcholinesterase inhibitor and nicotine modulator
What are NF tangles?
intraneuronal collection of hyperphosphorylated tau protein
Prion protein gene (PRNP) coding for PrP is located on chrom?
20
In NPH, what procedure predicts a better chance of improvement from a shunt?
Large volume tap (30-50cc) with improvement in sx
Topographagnosis?
inability to navigate in familiar places, read maps, draw floor maps of familiar places
Misoplegia?
severe hatred of a limb, rare form of agnosia seen in hemiparetic or hemiplegic patients after strokes
Balint’s syndome: location and triad
BL parieto-occipital lobes
- Optic ataxia (deficit of reaching for objects under visual guidance)
- Oculomotor apraxia (gaze apraxia, inability to voluntarily move the eyes to a new point of visual fixation despite normal EOM)
- Simultagnosia (inability to visually perceive more than one object at a time)
Anton’s Syndrome: location and sx
cortical blindness and visual agnosia from bilateral lesions to medial occipital lobes.
Denial of visual loss and confabulation (patient is blind but completely unaware)
Charles Bonnet Syndrome: cause and sx
vivid hallucinations that occur in people with severe visual impairment due to a variety of reasons, most commonly ophthalmic (macular degen)
Patients are aware they are hallucinating.
Alexia without agraphia: location and sx
Due to a lesion in the dominant PCA affecting the medial and inferior occipito-temporal region and splenium of the corpus callosum
alexia: loss of reading comprehension
writing and language comprehension are normal.
Patient will also have contralateral homonymous hemianopsia.
Pure word deafness (verbal auditory agnosia): location and sx
Lesion in the bilateral middle portion of the superior temporal gyri, disrupting connection between Wernicke’s and Heschl’s gyrus
- impaired auditory comprehension though hearing is intact (normal audiogram)
- normal comprehension of written language (distinguishes it from Wernicke)~
Aphemia: location and sx
Lesion in dominant frontal operculum
pure word mutism or verbal apraxia:
inability to speak fluently, impaired repetitions and intact auditory comprehension (retained ability to write and comprehend written language)
Foix-Chavany-Marie Syndrome: location and sx
AKA anterior operculum syndrome, lesions to bilateral anterior operculums (frequently in setting of multiple infarcts)
- severe dysarthria
- bilateral voluntary paralysis of the lower CN with preserved involuntary and emotional innervation.
Typically, it is described as a loss of voluntary control of palatal, glossal, pharyngeal, masticatory and oral muscles, but with preservation of reflex movements.
Amelodia/Affective motor aprosodia: location and sx
Lesion in non-dominant hemispheres analog to Broca’s area
inability of a person to properly convey or interpret emotional prosody.
Receptive aprosodia: location and sx
Lesion in nondominant analog to Wernicke’s area
inability to perceive and understand the emotional context of other’s speech
Pseudo-bulbar affect: location and sx
condition that’s characterized by episodes of sudden uncontrollable and inappropriate laughing or crying
bilateral lesions that disconnect the corticobulbar tracts from the brainstem CN nuclei, seen in ALS (most common) MS, TBI
Treatment option for pseudobulbar affect ?
Dextromethorphan/quinidine
Apraxia: location and sx
dominant parietal lobe
impaired ability to execute a previously known task, in the absence of motor weakness, incoordination or deficit in comprehension.
Ideomotor apraxia? location and sx
dominant parietal cortex: superior marginal/angular gyrus
use of a body part as an object during pantomime (using finger as a toothbrush when asked to pantomime brushing teeth vs pantomiming holding the toothbrush)
Ideational apraxia? location and sx
bifrontal or biparietal cortex
impairment in the sequence of motions needed to carry out a specific movement. (ex: ask to pour water in a glass and drink, the patient would drink from the glass before pouring water in)
Conduction apraxia?
impairment in imitation of movements
dissociation apraxia?
cannot execute a movement command but can imitate.
orbitofrontal lesions are assoc with changes in?
personality
medial frontal cortex lesions are associated with?
amotivation
lateral frontal lobe lesions are associated with ?
executive difficulties
Gerstmann’s Syndrome: location and tetrad
dominant inferior parietal lobe: angular gyrus
- finger agnosia
- R-L confusion
- dyscalculia
- dysgraphia (inabilty to write)
Gaze deviation from pontine lesions vs frontal eye field lesions?
Frontal eye field: overcome by oculocephalic maneuver
pontine lesions: not overcome by maneuver
Kluver Bucy Syndrome: location and sx
bilateral anterior temporal lobe lesions (usually following HSV encephalitis, bilateral temporal lobectomy or neurodegenerative disease such as FTD)
hyperorality, visual agnosia, hypersexuality, blunted emotional affect, and hypermetamorphosis (excessive attentiveness to visual stimuli with a tendency to touch every such stimulus)
Alien Limb Syndrome: location and sx?
lesion to the contralateral ACA, involving the corpus callosum or SMA
movement of a limb not under voluntary control
Can also be seen in corticobasal syndrome
BUZZWORDS: Chromosome for Presenilin 2 in Alzheimers?
1
BUZZWORDS: chromosome for Presenilin 1 in Alzheimers
14
BUZZWORDS: chromosome for Apo-E4 in Alzheimers
19
BUZZWORDS: chromosome for APP (amyloid precursor protein) in Alzheimers
21
BUZZWORDS assoc with Alzheimers
neuritic plaques
neurofibrillary tangles
amyloid plaques
amyloid angiopathy
granulovacuolar degeneration
Hirano bodies
BUZZWORDS assoc with Lewy Body Dementia
Fluctuating cognition, visual hallucinations, parkinsonism
BUZZWORDS: CAG repeat, Autosomal dom
Huntington’s Disease
BUZZWORDS: list the synucleinopathies
MSA
Parkinsons
Dementia with Lewy Bodies
neuroaxonal dystrophy
BUZZWORDS: List the tauopathies
AD
corticobasal ganglionic degeneration (CBGD)
PSP
FTD
BUZZWORDS: globose neurofibrillary tangles and tufted astrocytes
PSP
BUZZWORDS: spongiform encephalopathy
CJD
BUZZWORDS: pathways assoc with “Where?” (where is an object in space)
parieto-occipital pathways
BUZZWORDS: pathways assoc with “What?” (what is an object)
parieto-temporal pathways
BUZZWORDS: dressing apraxia
nondominant parietal
BUZZWORDS: hemisensory neglect
nondominant parietal
BUZZWORDS: expressive aphasia with intact repetition
Transcortical motor aphasia
MCA-ACA watershed infarct that disconnects the supplementary motor area from Broca
BUZZWORDS: receptive aphasia with intact repitition
Transcortical sensory aphasia
MCA-PCA watershed or thalamic infarct