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90 Cards in this Set
- Front
- Back
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Gerstmann Syndrome
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Deficits in calculation, right-left confusion, finger agnosia, and agraphia
Deficit in all four areas implicate the dominant parietal lobe |
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Lewy Body Disease
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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 |
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Characteristic features of basal ganglia (i.e. extrapyramidal) injury involve these involuntary movement disorders
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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) |
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West syndrome
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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 |
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Lennox-Gastaut
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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 |
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Benign Focal Epilepsy of Childhood
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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 |
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Childhood absence epilepsy
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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 |
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Juvenile Myoclonic Epilepsy
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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 |
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Landau Kleffner
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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 |
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Lesions in cerebellum cause
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Ataxia
Hypotonia Ipsilateral malfunctioning Dysdiadochokinesis (failure of rapid alternatinve movements) Past pointing (inability to touch finger to nose) |
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Spastic hemiparesis
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Arm on affected side held flexed and immobile against body, can be caused by lesions in cerebellum
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Huntington's disease
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Affects striatum/basal ganglia, especially caudate nucleus
Triad of sx: motor, cognitive, and behavioral Dx relies on emergence of chorea |
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Most common CNS tumors in adults
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glioblastoma, brain metastases (from neoplasms elsewhere)
30% infratentorial, 70% supratentorial |
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Most common CNS tumors in children
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astrocytoma, medulloblastoma, ependymoma
70% posterior fossa, 30% supratentorial Since many in posterior fossa, tend to cause hydrocephalus through compression/obstruction of fourth ventricle |
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Types of tumors
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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 |
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Common antiepileptic drugs
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Carbamazepine, vaklproate, phenobarbital, phenytoin
Monotherapy best for cognition and mood |
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Meningitis
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Infectious disease involving inflammation of the meningeal membranes surrounding the brain
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Viral meningitis
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Sequelae unclear, most common type
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Bacterial meningitis
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More commonly fatal
Hearing loss very common About 20% have lasting effects About 40% have acute effects like seizures, hemiplegia |
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Neurofibromatosis
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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 |
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Proteus syndrome
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AKA Elephant man disease (not neurofibromatosis)
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Fragile X
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Males: leads to mental retardation
Females: borderline to average IQ, verbal a strength, social anxiety |
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XYY
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Language and reading problems
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Ulrich Turner Syndrome
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Bad spatial, bad executive function
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Weight of LBW
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<2500 g (normal birth weight is above 2500 g
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Weight of VLBW
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<1500 g
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Weight of ELBW
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<1000 g
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Most common medical disorder among lbw infantsq
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Hyaline membrane diseaser, also known as RDS
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HMD/RDS
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result of immature lung development accompanied by surfactant deficiency
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surfactant
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substance that coats alveoli of lungs, thereby preventing their collapse
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complications secondary to HMD/RDS
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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 |
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PVH/IVH in premature infants
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susceptible to this - bleeding into subependymal germinal matrix
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the lower the birthweight,
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the worse off cognitively
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Cerebral palsy
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nonprogressive motor disorder of early onset
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CP is antepartum in what percent of cases
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70-80
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Predominant manifestations associated with CP
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movement disorders that may be spastic ataxic, or athetoid
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Half children with CP are _____ intelligence, one-fourth are _____ ______
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normal,
severely retarded |
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periventricular leukomalacia
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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
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five subtypes of CP
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parasagittal cerebral injury (leads to spastic quadriplegia)
periventricular laukomalacia focal/multifocal ischemic brain necrosis |
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principal pathogenetic mechanism underlyng neuropathology associated with intrapartum hyposia ischemia
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impaired CBF, most likely to occur as consequence of interruption in placental blood flow
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FAS
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maternal exposure, facial anomalies, growth retardation, CNS abnormalities
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ARND
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maternal exposure, CNS abnormalities, Cognitive abnormalities
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excitotoxicity
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death of neurons caused by oberstimulation of excitatory, mainly glutamate, neurotransmitter receptors. a cascade of biochemical/histopathologic events triggered by hpoxic ischemia
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Turner Syndrome
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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 |
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Phenylketonuria (PKU)
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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 |
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Specific impairments in early treated PKU
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executive dysfunction
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Acute Lymphoblastic Leukemia (ALL)
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Most common malignancy of childhood
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Highly vulnerable to MTX-CRT combinations
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Females, moreso than males
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Sickle Cell Disease
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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 |
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Diabetes
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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 |
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Hypoglycemia unawareness
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Complete absence of physilogical warning symptoms of forthcoming hypoglycemic episode and don't take corrective measures
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Cognitive effects of long-term hyperglycemia
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attention, visual-spatial processing, eye-hand coordination and verbal skills appear particularly vulnerable
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End Stage Renal Disease
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Children with chronic renal failure experience high rate of intellectual impairment
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HIV
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cognitive functioning, language development, and emotional and behavioral difficulties often observed.
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Vertically transmitted HIV
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appears more vulnerable to delays in mental and motor development and to CNS abnormalities due to perinatal exposure of virus on developing brain
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Epilepsy etiology
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50% occurs durig first five years of life, most are idiopathic (no known cause)
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Two main classes of epilepsy
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Partial - begins in focal area, often starts with aura
Primary Generalized - abrupt onset, no aura, usually a sudden LOC |
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Partial seizures - classification
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Simple partial: no amnesia, no alteration of consciousness
Complex Partial (aka psychomotor): confused, automatisms like lip smacking, often amnesic |
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Primary Generalized seizures - classification
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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 |
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West syndrome aka
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infantile spasms
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Lennox Gestaut
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seizures difficult to control
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Absence seizures
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usually resolve spontaneously
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Juvenile myoclonic epilepsy
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common, resolution infrequent, genetic
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Landau Kleffner
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first is onset of auditory agnosia, which leads to aphasia. Seizures before, during, or after. Language deficit is primary.
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Earlier onset in epilepsy is _______ (worse/better) prognosis
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worse
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Common seizure meds
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Carbamazepine
Valproate Phenobarbital Phenytoin Monotherapy is best for cognition and mood - consistent finding |
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Meningitis
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infectious disease involving inflammation of the meningeal membranes surrounding the brain
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viral meningitis
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most common, sequelae unclear
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bacterial meningitis
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more commonly fatal
hearing loss very common about 20% have lasting effectsd about 40% have acute effects like seizures, hemiplegia |
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Grades of tumors and survival rate
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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 |
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Glasgow Coma Scale severity
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> equal to 13 mild
9-12 moderate less than equal to 8 severe (in coma) |
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Glasgow Coma Scale items
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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) |
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Severity of TBI based on PTA duration (although difficult to pinpoint when "continuous registration" begins)
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< 5 min very mild
5-60 mild 1-24 hours moderate 1-7 days severe 1-4 weeks very severe > 4 wks extremely severe |
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MRI can visualize
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axonal injuries and small extra-axial hematomas but expensive
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CT can visualize
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surgically significant lesions
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Blood serum markers
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S-100B marker of glial damage in TBI
NSE neuronal tissue damage marker |
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Leading cause of PHI
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gunshot wounds
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ischemia
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absence of normal blood flow
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edema
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tissue swelling
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PHI associated with
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high rate of seizure (up to 80% in first 24 hours)
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DAI
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diffuse axonal injury, in CHI, microscopic lesions, often within deep white matter
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hydrocephalus
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blockage of flow of CSF
final path of many disorders |
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principal etiologies of hydrocephalus
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neural tube defects (spina bifida)
Dandy Walker syndrome aqueductal stenosis intraventricular hemorrhage related to perinatal asphyxia |
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neuropsychological sequelae of hydrocephalus
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motor deficits
VIQ>PIQ (cocktail party syndrome) VM, spatial deficits reading comprehension deficits |
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tumors most associated with seizures
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oligodendrogliomas
meningiomas |
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meningiomas arise from
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arachnoid vilus cells
grow slowly |
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C-shaped structure that follow curve of lateral ventricles
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fornix
caudate nucleus corpus callosum |
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septum pallucidum
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thin membraine that separates two lateral ventricles in the midline
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Kayser-Fleischer rings
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brownish outer corneal deposits of copper, a sign of Wilson's disease
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Wilson's disease
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autosomal recessive
biliary copper excretion, cuses progressive degeneration of the liver and basal ganglia |
