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90 Cards in this Set

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Gerstmann Syndrome
Deficits in calculation, right-left confusion, finger agnosia, and agraphia
Deficit in all four areas implicate the dominant parietal lobe
Lewy Body Disease
Possibly accounts for up to 30% of cases dx as Alzheimer's
Relatively rapid development of dementia
Is accompanied by ild extrapyramidal faetures such as: masked face, bradykinesia, resting tremor, and gait impairment
Can also present with depression, delusions, and visual hallucinations
Definitive diagnosis confirmed with abundant and unusual location of Lewy bodies, which are distributed diffusely throughout the cerebral cortex
Characteristic features of basal ganglia (i.e. extrapyramidal) injury involve these involuntary movement disorders
Parkinsonism (combination of resting tremor, rigidity, bradykinesia, and postural abnormalities
Athetosis - slow continuous writhing movements of fingers, hands, face, and throat
chorea - intermittent jerking of limbs and trunk
hemiballismus - intermittent flinging of the arm and leg on one side of the body (PACH)
West syndrome
aka infantile spasms
clusters of myoclonic seizures
cease between 2-4 yrs old, but give rize to other seizure types in 25-60% of cases
Lennox-Gastaut
Mixed seizure disorder - includes atypical absence, atonic, myoclonic
Age of onset 208
EEG has background slowing and slow spike and wave
Seizures difficult to control and status is common
Benign Focal Epilepsy of Childhood
Most common type of partial seizure: centrotemporal spikes
Rolandic Epilepsy (nocturnal seizures) EEG has CT spikes, remission almost always complete by 15, automsomal dominance with incomplete penetrance
Childhood absence epilepsy
onset b/t 4 and 8
Easy to treat, spontaneous remission in 80%
Onset in adolescence more likely to experience generalized seizures and persist into adulthood
Juvenile Myoclonic Epilepsy
Onset late childhood, early adolescence
Myoclonic jerks of upper extremities, no loc, but 90-95% have generalized tonic-clonic seizurs
Responds to AED's but resolution is infrequent
generally inherited
IQ usually OK
Landau Kleffner
Acquired aphasis with convulsive disorder
Abnormal EEG required (usually over temporal areas)for dx, but no actual seizures required
Sudden or gradual onset of auditory agnosia, may involve total unresponsiveness to language or progressive deterioration of expressive speech
Usually associated with behavioral difficulties as well
Seizure type varies, but usually generalized motor seizures
earlier onset assoc with poorer prognosis
Lesions in cerebellum cause
Ataxia
Hypotonia
Ipsilateral malfunctioning
Dysdiadochokinesis (failure of rapid alternatinve movements)
Past pointing (inability to touch finger to nose)
Spastic hemiparesis
Arm on affected side held flexed and immobile against body, can be caused by lesions in cerebellum
Huntington's disease
Affects striatum/basal ganglia, especially caudate nucleus
Triad of sx: motor, cognitive, and behavioral
Dx relies on emergence of chorea
Most common CNS tumors in adults
glioblastoma, brain metastases (from neoplasms elsewhere)
30% infratentorial, 70% supratentorial
Most common CNS tumors in children
astrocytoma, medulloblastoma, ependymoma
70% posterior fossa, 30% supratentorial
Since many in posterior fossa, tend to cause hydrocephalus through compression/obstruction of fourth ventricle
Types of tumors
Glioma - arise from glial cells
Meningiomas - arise from achnoid villa
Pituitary adenoma - cause endocrine disturbance, bitemporal visual field defect by compressing optic chiasm
Schwannoma - most common on CN VIII
Lymphoma
Common antiepileptic drugs
Carbamazepine, vaklproate, phenobarbital, phenytoin

Monotherapy best for cognition and mood
Meningitis
Infectious disease involving inflammation of the meningeal membranes surrounding the brain
Viral meningitis
Sequelae unclear, most common type
Bacterial meningitis
More commonly fatal
Hearing loss very common
About 20% have lasting effects
About 40% have acute effects like seizures, hemiplegia
Neurofibromatosis
AKA Von Recklinghausen disease
Caused by mutation on chromosome 17
Higher incidence of LD, behavioral impairment, and brain tumors
Automosomal dominant
Visual-spatial disorder almost always present
Have hyperintensities on MRI, only found in kids
Proteus syndrome
AKA Elephant man disease (not neurofibromatosis)
Fragile X
Males: leads to mental retardation
Females: borderline to average IQ, verbal a strength, social anxiety
XYY
Language and reading problems
Ulrich Turner Syndrome
Bad spatial, bad executive function
Weight of LBW
<2500 g (normal birth weight is above 2500 g
Weight of VLBW
<1500 g
Weight of ELBW
<1000 g
Most common medical disorder among lbw infantsq
Hyaline membrane diseaser, also known as RDS
HMD/RDS
result of immature lung development accompanied by surfactant deficiency
surfactant
substance that coats alveoli of lungs, thereby preventing their collapse
complications secondary to HMD/RDS
patent ductus arteriosus - a blood vessel that joins aorta and pulmonary artery doesn't close, leading to pulmonary edema

extrapulmonary extravasation of air

bronchopulmonary dysplasia
PVH/IVH in premature infants
susceptible to this - bleeding into subependymal germinal matrix
the lower the birthweight,
the worse off cognitively
Cerebral palsy
nonprogressive motor disorder of early onset
CP is antepartum in what percent of cases
70-80
Predominant manifestations associated with CP
movement disorders that may be spastic ataxic, or athetoid
Half children with CP are _____ intelligence, one-fourth are _____ ______
normal,
severely retarded
periventricular leukomalacia
principal ischemic lesion of the preterm infant - refers to bilateral white matter necrosis adjacent to lateral ventricles, very sensitive to decreases in cerebral perfusion, this region is traversed by descending fibers from motor cortex
five subtypes of CP
parasagittal cerebral injury (leads to spastic quadriplegia)
periventricular laukomalacia
focal/multifocal ischemic brain necrosis
principal pathogenetic mechanism underlyng neuropathology associated with intrapartum hyposia ischemia
impaired CBF, most likely to occur as consequence of interruption in placental blood flow
FAS
maternal exposure, facial anomalies, growth retardation, CNS abnormalities
ARND
maternal exposure, CNS abnormalities, Cognitive abnormalities
excitotoxicity
death of neurons caused by oberstimulation of excitatory, mainly glutamate, neurotransmitter receptors. a cascade of biochemical/histopathologic events triggered by hpoxic ischemia
Turner Syndrome
only one X chromosome
sexual infantilism
webbed neck
short stature
primary ameenorrhea
most are NOT MR
visual spatial deficit is most hallmark cognitive symptom
great heterogeneity in presentation
Phenylketonuria (PKU)
Genetic mutation that inactivates the enzyme reponsible for converting or hydroxylating phenylalanine into tyrosine

this lack of tyrosine should have adverse effects on neurochemical metabolism, in particularly on metabolism of catecholamines such as dopamine
Specific impairments in early treated PKU
executive dysfunction
Acute Lymphoblastic Leukemia (ALL)
Most common malignancy of childhood
Highly vulnerable to MTX-CRT combinations
Females, moreso than males
Sickle Cell Disease
sickling of red blood cells
vaso-occlusive episodes are common
can have devastating neuropsych deficits, especially HbSS, which is most common genotype. leads to clinical strokes
Diabetes
autoimmune disease
body lacks insulin, a hormone needed to metabolize glucose
glucose accumulates in urine and body starves
exceedingly high levels of blood sugar lead to unconsciousness, coma, nd ultimately death
Hypoglycemia unawareness
Complete absence of physilogical warning symptoms of forthcoming hypoglycemic episode and don't take corrective measures
Cognitive effects of long-term hyperglycemia
attention, visual-spatial processing, eye-hand coordination and verbal skills appear particularly vulnerable
End Stage Renal Disease
Children with chronic renal failure experience high rate of intellectual impairment
HIV
cognitive functioning, language development, and emotional and behavioral difficulties often observed.
Vertically transmitted HIV
appears more vulnerable to delays in mental and motor development and to CNS abnormalities due to perinatal exposure of virus on developing brain
Epilepsy etiology
50% occurs durig first five years of life, most are idiopathic (no known cause)
Two main classes of epilepsy
Partial - begins in focal area, often starts with aura
Primary Generalized - abrupt onset, no aura, usually a sudden LOC
Partial seizures - classification
Simple partial: no amnesia, no alteration of consciousness
Complex Partial (aka psychomotor): confused, automatisms like lip smacking, often amnesic
Primary Generalized seizures - classification
Convulsive: tonic/clonic, aka grand mal, or could just be tonic or clonic
Atonic: loss of muscle tone, fall
Myoclonic: symmetrical jerking, no LOC since usually lasts <1 sec
Absence: petit mal
West syndrome aka
infantile spasms
Lennox Gestaut
seizures difficult to control
Absence seizures
usually resolve spontaneously
Juvenile myoclonic epilepsy
common, resolution infrequent, genetic
Landau Kleffner
first is onset of auditory agnosia, which leads to aphasia. Seizures before, during, or after. Language deficit is primary.
Earlier onset in epilepsy is _______ (worse/better) prognosis
worse
Common seizure meds
Carbamazepine
Valproate
Phenobarbital
Phenytoin

Monotherapy is best for cognition and mood - consistent finding
Meningitis
infectious disease involving inflammation of the meningeal membranes surrounding the brain
viral meningitis
most common, sequelae unclear
bacterial meningitis
more commonly fatal
hearing loss very common
about 20% have lasting effectsd
about 40% have acute effects like seizures, hemiplegia
Grades of tumors and survival rate
Grade 1: benign, 5 years plus
Grade 2: semibenign, 3-5 years
Grade 3: relatively malignant, 2-3 years
Grade 4: highly malignant, less than 15 months
Glasgow Coma Scale severity
> equal to 13 mild
9-12 moderate
less than equal to 8 severe (in coma)
Glasgow Coma Scale items
Eye opening (on command to with pain, 1-4)
Verbal 1-6
Best motor response (commands to decerebrate posturing to no responsee with pain, 1-5)
Severity of TBI based on PTA duration (although difficult to pinpoint when "continuous registration" begins)
< 5 min very mild
5-60 mild
1-24 hours moderate
1-7 days severe
1-4 weeks very severe
> 4 wks extremely severe
MRI can visualize
axonal injuries and small extra-axial hematomas but expensive
CT can visualize
surgically significant lesions
Blood serum markers
S-100B marker of glial damage in TBI
NSE neuronal tissue damage marker
Leading cause of PHI
gunshot wounds
ischemia
absence of normal blood flow
edema
tissue swelling
PHI associated with
high rate of seizure (up to 80% in first 24 hours)
DAI
diffuse axonal injury, in CHI, microscopic lesions, often within deep white matter
hydrocephalus
blockage of flow of CSF
final path of many disorders
principal etiologies of hydrocephalus
neural tube defects (spina bifida)
Dandy Walker syndrome
aqueductal stenosis
intraventricular hemorrhage related to perinatal asphyxia
neuropsychological sequelae of hydrocephalus
motor deficits
VIQ>PIQ (cocktail party syndrome)
VM, spatial deficits
reading comprehension deficits
tumors most associated with seizures
oligodendrogliomas
meningiomas
meningiomas arise from
arachnoid vilus cells
grow slowly
C-shaped structure that follow curve of lateral ventricles
fornix
caudate nucleus
corpus callosum
septum pallucidum
thin membraine that separates two lateral ventricles in the midline
Kayser-Fleischer rings
brownish outer corneal deposits of copper, a sign of Wilson's disease
Wilson's disease
autosomal recessive
biliary copper excretion, cuses progressive degeneration of the liver and basal ganglia