Term
| Childhood Absence epilepsy Rx |
|
Definition
ethosuximide
valproic acid |
|
|
Term
|
Definition
ABC
IV lorazepam
IV fosphenytoin / phenytoin |
|
|
Term
|
Definition
|
|
Term
bacterial meningitis organisms
neonate |
|
Definition
group B strep
listeria
E.coli |
|
|
Term
bacterial meningitis organisms
before 50 |
|
Definition
Pneumococci
neisseria
H-flu |
|
|
Term
bacterial meningitis organisms
after 50 |
|
Definition
pneumococcus
listeria
gram-negative |
|
|
Term
bacterial meningitis
treatment |
|
Definition
cephalosporin + vancomycin(resist pneumococcus)
ampicillin (listeria)
dexamethasone |
|
|
Term
viral meningitis
organisms |
|
Definition
enterobacteria (coxsackievirus, echovirus, poliovirus)
arborvirus, mumps, herpes |
|
|
Term
Bacterial meningitis
diagnosis |
|
Definition
LP
antigen study - not 100% specific
culture |
|
|
Term
viral meningitis
diagnosis |
|
Definition
| LP reverse transcription: possibility for arbovirus |
|
|
Term
|
Definition
PCR (100% specific, <75% sensitive)
LP (glucose)
mycobacterial and acid fast stain (not very sensitive) |
|
|
Term
fungal meningitis
diagnosis |
|
Definition
LP
antigen studies (cryptococcus)
india ink for cryptococcus |
|
|
Term
fungal meningitis
treatment |
|
Definition
amphotericin B
flucytosein (AIDS with cryptococcus)
fluconazole (AIDS maintenance) |
|
|
Term
viral encephalitis
diagnosis |
|
Definition
LP
antibody studies (arbovirus)
PCR studies (west nile) |
|
|
Term
herpes viral encephalitis
diagnosis |
|
Definition
LP - RBC in CSF
PCR (100% specific, ,100% sensitive)
imaging |
|
|
Term
herpes viral encephalitis
tx |
|
Definition
|
|
Term
neurocysticercosis
diagnosis |
|
Definition
| serum and CSF immunologic studies |
|
|
Term
|
Definition
albendazole
AED
shunt/resection (hydrocephalus) |
|
|
Term
bacterial brain abscess
organisms |
|
Definition
Streptococcus
S. aureus
enterobacter
anaerobics |
|
|
Term
|
Definition
imaging
cerebritis: non-enhancing on MRI/CI
abscess: ring enhancement mass with surrounding edema |
|
|
Term
|
Definition
biopsy, resection/aspiration
cephalosporin, penicillin, metronidazole (anaerobics) |
|
|
Term
herpes varicella zoster virus
tx |
|
Definition
|
|
Term
|
Definition
encephalopathy
opportunistic infection
vascuolar myelopathy
peripheral neuropathy |
|
|
Term
| HIV opportunistic infection |
|
Definition
toxoplasma
cryptococcus
EBV - CNS lymphoma
JB - progressive, multifocal leukoencephalopathy |
|
|
Term
| HIV nervous sys treatment |
|
Definition
single lesion: biopsy
multiple lesion: toxoplasma titers
negative titers: biopsy
positive titer: empirical abx
PML and CNS lymphoa: HAART |
|
|
Term
| progressive multifocal leukoencaphopathy pathology |
|
Definition
oligodendrocytes with enlarged nuclei,
viral inclusions,
giant bizzare astrocytes |
|
|
Term
syphilis
neurologic syndrome |
|
Definition
aseptic meningitis
meningovascular syphili
general paresis
tabes dorsalis |
|
|
Term
syphilis
neurologic syndrome
diagnossis |
|
Definition
non specific serologic test (RPR, VDRL)
specific antibodies (FTA, MHA-TP)
if positive FTA -> LP |
|
|
Term
syphilis
neurologic syndrome
tx |
|
Definition
|
|
Term
| juvenile myoclonic epilepsy |
|
Definition
generalized tonic-clonic seizures
history of myoclonic jerks in the mornings |
|
|
Term
|
Definition
seizures with hemi-facial twitching, drooling
predominately at night
idiopathic localization-related epilepsy |
|
|
Term
| mesial temporal sclerosis |
|
Definition
symptomatic localization-related epilepsy
fibrile szs history
atrophy of hippocampus |
|
|
Term
|
Definition
AED
surgery
vagus nerve stimulation
ketogenic diet |
|
|
Term
| communicating hydrocephalus |
|
Definition
external hydrocephalus
normal pressure hydrocephalus
pseudotumor cerebri |
|
|
Term
| tx for pseudotumor cerebri |
|
Definition
medical - acetazolamide
CSF shunt
optic nerve sheath fenestration |
|
|
Term
| congenital causes of hydrocephalus |
|
Definition
myelomeningocele/Chiari II
encephalocele
dandy walker
primary aqueductal stenosis
X-linked |
|
|
Term
|
Definition
one of the general presentations of hydrocephalus
paralysis of upgaze |
|
|
Term
| ex cavcu dilatation of ventricles |
|
Definition
one ddx of hydrocephalus
encephalomalacia (softening brain) after injury or insult |
|
|
Term
| susceptible areas of global hypoxia |
|
Definition
cortical layers 3 and 5
hippocampus: pyramidal neurons
cerebellum: purkinje cells, dendate nuclei
basal ganglia
inferior olivary nucleus |
|
|
Term
| susceptible area of CO poisoning |
|
Definition
globus pallidus
internal capsule
hippocampus
cortex |
|
|
Term
|
Definition
cherry red brain
globus pallidus hemorrhage -> cavity |
|
|
Term
| clinical features of thiamine deficiency |
|
Definition
acute: wernicke's encephalopathy
chronic: Korsakoff syndrome
Beriberi - congestive cardiomyopathy, peripheral polyneuropathy |
|
|
Term
| Wernicke's encephalopathy |
|
Definition
acute sign of thiamine deficiency
confusion
ophthalmoplegia
ataxia |
|
|
Term
|
Definition
chronic signs of thiamine deficiency
antegrade amnesia
confabulation
other cognitive functions spared |
|
|
Term
| susceptible areas of thiamine deficiency |
|
Definition
mammalian bodies
regions border 3rd and 4th ventricles and cerebral aquaduct
- medial thalamic nuclei
- floor of 3rd ventricle
- periaqueductal region |
|
|
Term
|
Definition
pernicious anemia
inadequate intake: vegan |
|
|
Term
Lhermitte's phenomenon
diseases |
|
Definition
|
|
Term
|
Definition
cofactor
- methylmalonic CoA -> succinyl CoA
- homocystein -> methionine -> SAM |
|
|
Term
| pathology of B12 deficiency |
|
Definition
spinal cord (vacuolation)
posterior columns, (cortiospinal, spinocerebellar tracts)
anterior column is spared |
|
|
Term
| susceptible areas of b12 deficiency |
|
Definition
cervical cord
thoracic cord |
|
|
Term
| clinical features of hepatic encephalopathy |
|
Definition
|
|
Term
hepatic encephalopathy
pathogenesis |
|
Definition
hyperammonemia
increase production of glutamine within astrocytes
increase benzodiazepine-like substance |
|
|
Term
hepatic encephalopathy
pathology |
|
Definition
| Alzheimer's type II cells |
|
|
Term
hepatic encephalopathy
susceptible area |
|
Definition
cortex
basal ganglion
thalamus, hypothalamus, subthalamus
dendate nuclei of cerebellum |
|
|
Term
| management of hepatic encephalopathy |
|
Definition
reduce protein intake
decrease ammonia level
lactulose: acid and laxative |
|
|
Term
central pontine myelinolysis
cinical features
pathology |
|
Definition
severely ill patients, esp one with hyponatremia corrected rapidly
limb weakness progress days to weeks
in basis pontis, demyelination |
|
|
Term
| location of REM on neurons |
|
Definition
| tegmentum of pons and midbrain |
|
|
Term
| location of hypnogenic neurons |
|
Definition
|
|
Term
| projections of REM on neurons |
|
Definition
thalamus
reticular formation of pons and midbrain
reticulospinal tract
lateral geniculate and occipital cortex
brainstem neclus |
|
|
Term
|
Definition
|
|
Term
hypocretin
production and function |
|
Definition
produced in hypothalamus
stimulate REM-off nucleus -> inhibit REM sleep
promote wakefulness, feeding, motor activity |
|
|
Term
|
Definition
|
|
Term
| intrinsic clock is controlled by |
|
Definition
suprachiasmatic nucleus of hypothalamus
SNC - pineal circuit |
|
|
Term
| obstructive sleep apnea Treatment |
|
Definition
|
|
Term
| narcolepsy clinical features |
|
Definition
excessive daytime somnolence
cataplexy
sleep paralysis
hypnagogic/hypnopompic hallucination |
|
|
Term
|
Definition
| insufficient hypocretin neurons |
|
|
Term
|
Definition
somnolence: modafinil, armodafinil, Ritalin, gamma-hydroxybutyrate
cataplexy: TCA, gamma-hydrooxybutyrate |
|
|
Term
| delayed sleep phase syndrome |
|
Definition
| one of circadian rhythm disorders |
|
|
Term
|
Definition
delayed sleep onset syndrome
advanced sleep phase syndrome - mutation in casein kinase I
jet lag
shift work |
|
|
Term
| management of REM sleep bahavior disorder |
|
Definition
safety
associated neurologic diseases
clonazepan (low dose) |
|
|
Term
| restless leg syndrome associated conditions |
|
Definition
|
|
Term
restless leg syndrome and periodic leg movement
medication |
|
Definition
| levodopa, dopamine agonist, clonazepan, gabapentin |
|
|
Term
DDI with halogenated hydrocarbon anesthetic
- catecholamines
- beta blocker, CCB
- opioids, CNS depressants
- nondepolarizing NMJ blockers
- succinylcholine |
|
Definition
- increase arrhythmogenicity
- increase CV depression
- prolong ventilator depression
- prolong muscle paralysis
- increase malignant hyperthermia risk |
|
|
Term
|
Definition
halogenated hydrocarbon anesthetics
less risky
tachycardia, vasodilation, CO stable |
|
|
Term
| malignant hyperthermia treatment |
|
Definition
| dantrolene O2 reduce temperature |
|
|
Term
Malignant hyperthermia
FEVER |
|
Definition
fever
encephalopathy
vital unstable
elevated enzymes
rigidity of muscles |
|
|
Term
|
Definition
IV benzodiazepines
rapid induction/sedation
antegrade amnesia
no analgesia, muscle relaxation
conscious sedation: with fentanyl |
|
|
Term
Thiobarbiturates
methohexital |
|
Definition
Utra short acting IV barbiturates
fast induction
decrease ICP, CBF, CMR
risk: CV depression, laryngospasm |
|
|
Term
|
Definition
|
|
Term
|
Definition
fentanyl/subfentanyl
induction or maintenance |
|
|
Term
| management of diffuse acute encephalopathy |
|
Definition
1. ABC
2. Oxygen (100%), glucose (1 AMP d50), Naloxone
3. Pupils and dolls' eye (transtentorial herniation)
3. motor symmetry (decorticate / decerebrate posturing)
4. other electrolytes, renal, hepatic, temperature
5. everything else |
|
|
Term
Alzheimer's disease
Pathology |
|
Definition
temporal-parietal atrophy, hippocampus
neuritis plaque - Abeta-protein
Neurofibrillary tangles - tau |
|
|
Term
alzheimer's disease
pathophysiology |
|
Definition
abnormal amyloid processing: Abeta-protein and alpha-secretases
tau protein -> toxic
affect cholinergic projections |
|
|
Term
alzheimer's disease
management |
|
Definition
donepezil: cholinesterase inhibitors (6 months)
memantine: glutamate receptor antagonist
vitamine E
manage other aspects |
|
|
Term
dementia with lewy bodies (DLB)
sxs |
|
Definition
core:
- parkinsonia features early in course
- recurrent visual hallucination early in course
- marked fluctuations in cognitive or awareness
Suggestive:
- REM sleep behavior disorder
- ext sensitive to antipsychotic med
- low dopamine transporter uptake in basal ganglia |
|
|
Term
|
Definition
lewy body - alpha syneclein and ubiquitin
2/3 AD features |
|
|
Term
|
Definition
cholinesterace inhibitor (may be beneficial)
dopaminergic agents - parkinsonism
other symptomatic management |
|
|
Term
|
Definition
in DLB and parkinson's disease
neuronal inclusion with eosinophilic core surrounded by pale halo
alpha-synuclein and ubiqitin |
|
|
Term
|
Definition
in Frontotemporal dementia
neuronal inclusion, basophilic, tau with 3-repeat isoform |
|
|
Term
|
Definition
progressive behavior disturbance progressive language disturbance logopenic/phonologic
- progressive non-fluent aphasia
- semantic dementia
variant hereditary: 10%, with parkinsonian features |
|
|
Term
|
Definition
pick's disease - tau
tau and ubiquitin
TDP 43 |
|
|
Term
|
Definition
|
|
Term
|
Definition
clinical
CT, MRI - FT atrophy
PET |
|
|
Term
| vascular dementia management |
|
Definition
primary and secondary prevention of stroke
cholinersterase inhibitors
symptom management |
|
|
Term
Creutzfeldt-Jakob Disease
clinical features |
|
Definition
rapid progression
ataxia, dysarthria
myoclonic jerks
behavioral disturbance/psychosis |
|
|
Term
Creutzfeldt-Jakob Disease
pathology |
|
Definition
spongiform disease
activation of astrocytes
little inflammatory response |
|
|
Term
Creutzfeldt-Jakob Disease
pathophysiology and transmission |
|
Definition
Prion
Hereditary
iatragenic / brain eating
sporadic
ingestion: bovine spongiform encephalopathy |
|
|
Term
|
Definition
postural/kinetic tremor
hereditary (dominant)
exacerbated by stress
improved by EtOH |
|
|
Term
|
Definition
drug induced: lithium, valproate, caffeine
Withdrawal: EtOH, benzodiazepines
hyperthyroidism
neurologic disease: MS, wilson's disease
heavy metal poisoning
cushing's |
|
|
Term
|
Definition
used only if needed
beta blocker
primidone, topiramate
DBS, VIM thalamotomy |
|
|
Term
parkinson's disease
clinical features |
|
Definition
rest tremor
bradykinesia: mask face, soft, expressionless voice
rigidity
festination
micrographia
gait: en bloc turning
dementia, hallucination, depression, pain, sleep disorders |
|
|
Term
parkinson's disease
pathology |
|
Definition
substantial nigra and locus coerulus atrophy
lewy bodies and lewy neuritis |
|
|
Term
parkinson's disease
pathophysiology |
|
Definition
basal ganglia circuitry: dopamine -> stimulate thalamus and cortex
- too little dopamine -> inactive thalamus cortex
protein accumulation: lewy body (alpha synuclein, ubiqitin)
mutated gene for glucocerebrosidase
mitrodronia dysfunction: MPTP -> by MOA B -> MPP -> damage DA neurons |
|
|
Term
|
Definition
L-DOP -> DA by DA decarboxylase
SE (due to PA in peripheral)
- compete with large aa for absorption
- early: nausea, vomit (may need to take with food)
- late: motor manifestation (dyskinesia, motor fluctuation, hallucination
- neurotoxicity |
|
|
Term
COMT inhibitors
MOA and SE |
|
Definition
inhibit L-DOPA metabolism in gut
inhiti DA metabolism in synapses
Tolcapone -> significant hepatotoxicity |
|
|
Term
|
Definition
inhibit MPTP -> MPP -> reduce oxidative stress
inhibit metabolism of DA in neurons
delay start of L-DOPA |
|
|
Term
|
Definition
Ach in basal ganglion circuitry -> oppose DA efect
best effect for patients with tremor |
|
|
Term
| surgical management of parkinson's disease |
|
Definition
Thalamotomy and pallidotomy
Deep brain stimulation of MGP and STN |
|
|
Term
|
Definition
levodopa
levodopa with carbidopa (Sinemet)
dopamine agonist
COMT inhibitors
MAO-B inhibitors
antimuscarinic agents
surgical management |
|
|
Term
|
Definition
DLB
progressive supranuclear palsy
corticobasal degeneration
mulitple system atrophy (OPCA) |
|
|
Term
progressive supranuclear palsy
clinical features |
|
Definition
supranuclear gaze palsy
trunical rigidity early in course
frontal lobe features
dysarthria, dysphagia |
|
|
Term
progressive supranuclear palsy
pathology and sites |
|
Definition
neurofibrillary tangles
tau deposits
substantia nigra, locus coeruleus, globus pallidus, substantial nucleus, pontine tegmentum |
|
|
Term
corticobasal degeneration
sxs |
|
Definition
clumsinss, stiffness or jerking of one arm -> ipsilateral limb -> contralateral body
dysarthria, dystonia, dysphagia
frontal lobe features - dementia |
|
|
Term
Corticobasal degeneration
pathology |
|
Definition
frontal and parietal lobes atrophy
swollen neurons (similar to pick's disease)
Tau inclusions |
|
|
Term
multiple system atrophy
clinical features |
|
Definition
combination of parkinsonism, cerebellar dysfunction, automatic insufficiency
REM sleep behavior disorders (2/3)
peripheral polyneuropathy |
|
|
Term
multiple system atropy
pathology |
|
Definition
glial inclusions
atrophy of cerebellum, mid cerebellar peduncles, pons, substantia nigra, locus coerulus |
|
|
Term
Friedreich's ataxia
clinical features |
|
Definition
autosomal recessive
affect posterior column
affect spinocerebellar tract (ataxic gait, speech)
bilateral babinski signs: upper motor neurons |
|
|
Term
Friedreich's ataxia
pathophysiology |
|
Definition
GAA expansion
(normal: make iron available for mitochondria processes)
free radicals -> oxidative stress |
|
|
Term
Friedreich's ataxia
pathology |
|
Definition
atrophy in spinal cord and dorsal root
degeneration of posterior column, pyramidal and spinocerebellar tracts |
|
|
Term
ataxia telangiectasia
clinical features |
|
Definition
1st decade of life
ataxia: ataxic gait, choreathetosis, oculomotor apraxia
telangiectasia: after ataxia, earlobe, eyelid
immunodeficiency: malignancy |
|
|
Term
ataxia telangiectasia
Pathophysiology |
|
Definition
multiple mutations - impair damage surveillance
immunodeficiency |
|
|
Term
ataxia telangiectasia
pathology |
|
Definition
atrophy in
- cerebrellar cortex, inferior olive
- posterior column
- anterior horn
- lateral column |
|
|
Term
ataxia telangiectasia
diagnosis |
|
Definition
clinical
genetic testing not available (>300 mutation) |
|
|
Term
spinocerebellar ataxias
clinical features |
|
Definition
ataxia in gait, limb and speech
seizures, dementia, parkinsonism
autosomal dominant |
|
|
Term
spinocerebellum ataxias
pathology |
|
Definition
atrophy in cerebellum, pontine nuclei, inferior olivary nuclei
abnormal protein aggregates |
|
|
Term
spinocerebellum ataxias
pathophysiology |
|
Definition
Expanded CAG in gene
CAG expansion in non-coding region
conventional mutation
ion channel dysfunction |
|
|
Term
spinocerebellum ataxias
diagnosis |
|
Definition
clinical, radiologic (rule out other causes)
genetic testing for specific SCA (not all 29 mutations) |
|
|
Term
Huntington's disease
clinical features |
|
Definition
personality change: first sign
chorea
global cognitive deficit |
|
|
Term
huntington's disease
patholoy |
|
Definition
atrophy of caudate and putamen***
intraneuronal inclusions - huntingtin and ubiquitin |
|
|
Term
huntington's disease
pathophysiology |
|
Definition
CAG repeat >40
capase mediated apoptosis
damage to medium spiny neurons (straitum) -> decrease inhibition of LGP -> increase cortical stimulation |
|
|
Term
| huntington's disease management |
|
Definition
tetrabenazine - chorea
- can cause/exacerbate depression |
|
|
Term
Wilson's disease
clinical |
|
Definition
autosomal recessive
hepatic and neurologic manifestation
tremor, rigidity, chorea, pyschiatic features
Kayer-Fleischer ring |
|
|
Term
wilson's disease
pathology |
|
Definition
brown and shrunken caudate and putamen
pigment-laden macrophages
alzheimer type II astrocytes |
|
|
Term
wilson's disease
pathogenesis |
|
Definition
Cu -> apo-ceruloplasmin -> hepatocytes
- Cu can't be excreted into bile
spill into blood |
|
|
Term
wilson's disease
diagnosis |
|
Definition
slit lamp for Kayer-Fleischer's ring
decrease ceruloplasmin (80% sensitive and positive)
increase 24 hour urinary Cu |
|
|
Term
wilson's disease
management |
|
Definition
decerase copper intake
decrease GI absorption - Zinc
increase urinary Cu - penicillamine, trientine
liver transplantation |
|
|
Term
|
Definition
torticollis
blepharospam
writer's cramp
onset: adult |
|
|
Term
|
Definition
Generalized dystonia
autosomal dominant
begins with 1 limb -> rest of body
mutation in torsin gene |
|
|
Term
|
Definition
generalized dystonia
autosomal dorminant
diurnal progression
med: L-DOPA |
|
|
Term
gilles de la tourette's
clinical features and med |
|
Definition
autosomal dorminant
Tics
phrasic
gradual improvement overtime
OCD in 50% ADHD in 50%
antipyshcotics, haloperidol pimiozide
tetrabenzine |
|
|
Term
|
Definition
Global development delay
MR, Autism, ADHD, impaired motor skills, craniofacial features
more in male
expanded trinucleotide repeats - proteins continuously made, can't reform synapses |
|
|
Term
|
Definition
global developmental delay
MR, obesity, insatiable hunger later
angelman syndrome: same gene, paternal vs maternal |
|
|
Term
|
Definition
any static encephalopathy can be a cause
non-progressive abnormality of motor control
hyptonia early, spastic later
hemiparesis, quadriparesis, paraparesis
choreathetosis, ataxia |
|
|
Term
amyotrophic lateral sclerosis ALS
clinical features |
|
Definition
focal weakness to arm and contiguous muscle
not in distribution of nerve roots/nerves
no sensory involvement
weak muscle of respiration |
|
|
Term
ALS
pathology and pathogenesis |
|
Definition
atrophy of anterior horn and motor neurons
Superoxide dismutase mutation - oxidative stress
protein aggregation - TDP-43 |
|
|
Term
|
Definition
Riluzole: anti-glutamate agent
- extends survival for 6 months |
|
|
Term
spinal muscular atrophy SMA
clinical features |
|
Definition
LMN pathology
Type 1, Type 2 and type 3
areflexia
postural/kinetic tremor common in types 2 and 3 |
|
|
Term
spinal muscular atrophy
pathogenesis |
|
Definition
| mutations in survival motor neurons 1 gene |
|
|
Term
primary lateral sclerosis
clinical features |
|
Definition
UMN pathology
onset after 50
slowly progressively spastic paraparesis |
|
|
