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46 Cards in this Set

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- the most severe form of neural tube defect;
-1 in 500 births
-More common in female fetuses
-Risk increase with with folate (folic acid) deficiency
-babies dead at birth
Spina Bifida
-Absence or hypoplasia of one or more of the dorsal arches of the vertebrae;
-meningeal and spinal defects possible
-Spina bifida cystica may cause gait disturbance, urinary incontinence, male impotence
-Spina bifida occulta:
unfused vertebral arch
(intact meninges and spinal cord) is common; 20% of general population and is usually symptomatic
-fused coronal suture
-fused saggital suture
- fused metopic suture
Cerebral Edema
Increased water content of the brain parenchyma
Vasogenic Edema
Disruption in blood-brain barrier that allows escape of fluid from the vasculature into the interstitial space of the brain
Cytotoxic Edema
An increase in intracellular fluid secondary to cellular injury
Brain Herniation
The displacement of a part of the brain from one dural compartment to another
Transtentorial Herniation
a medial displacement of temporal lobe against the tentorium cerebelli; causes pupillary dilation,
impaired eye movements, and compression of posterior cerebral artery with ischemia to visual cortex
Subfalcine Herniation
a displacement of c. gyrus under falx cerebri caused by expansion of cerebral hemisphere; results in compression of anterior cerebral artery with ischemia to cortex and weakness/sensory abnormality of leg.
types of hernia's
ID the sutures of the crown of head
Tonsillar Herniation
displacement of the cerebellar tonsils through the foramen magnum that compresses the respiratory centers of the medulla oblongata
accumulation of excess cerebrospinal fluid in the ventricular system of the brain
Causes of Hydrocephalus
-Decreased resorption of CSF (most common)
-Overproduction of CSF
-Obstruction (within ventricles, foramina of Lushka & Magendie or subarachnoid space)
Communicating hydrocephalus
when full communication exists between the ventricles and subarachnoid space
Noncommunicating hydrocephalus
when CSF flow is obstructed within the ventricular system or in its outlets to the arachnoid space
Obstructive hydrocephalus
obstruction of the flow of CSF (intraventricular or extraventricular). Most hydrocephalus is obstructive
Vascular Diseases of the Brain
Generalized reduction in blood flow (hypoxic-ischemic encephalopathy)
Hemorrhage (parenchymal or subarachnoid)
Causes of Hypoxic-ischemic Encephalopathy
Cardiac dysrhythmias
Increased intracranial pressure
Modifiers of Hypoxic-ischemic Encephalopathy
Age (younger more tolerant)
Duration (short duration “recoverable”)
Temperature (hypothermia slows rate of injury)
Brain Infarction
Most common form (70-80%) of strokes (CVA)
Of brain infarctions, about ¾ are ABI (atherothrombotic brain infarction)
7th decade; males>females
Thrombosis of atherosclerotic vessel predisposes to infarction (hypertension, diabetes & smoking)
Emboli (from heart or elsewhere can be a cause)
Features of Brain Infarction
-Transient ischemic attacks precede infarct in 1/3; TIA predictive of impending infarct
-Infarcts occur most often in areas supplied by middle cerebral artery
-Deficits can include visual field abnormalities, aphasia, apraxia, agnosia, contralateral hemiparesis & hemidysesthesia
-Paradoxical infarct related to collateral circulation
Primary Brain Parenchymal Hemorrhage
-Hypertension most common underlying cause (50%); other causes are coagulation disorders, amyloid, neoplasms, aneurysms, AVM (arteriovenous malformation)
-Mid-late adult life; peak at age 60
-Sudden headache, vomiting, loss of consciousness, coma, pupillary fixation, herniation, apena & death
distribution of hypertensive hemorrhages
-65% basal ganglia thalmus
-15% Pons
-10% cerebellum
Types of Aneurysm
-medial defect (berry or saccular)
-charcot (hypertension)
Saccular (berry) Aneurysm
-Present at bifurcations in 1%; risk of rupture when 6-10 mm in size; women > men, <50; incidence higher in polycystic kidneys, coarctation of aorta, AVM
-If ruptured, produces subarachnoid and/or brain hemorrhage
-Abrupt headache, vomiting, loss of consciousness with 50% death within days; infarction, hydrocephalus, herniation
Common sites of saccular (berry) aneurysms in the circle of Willis
Arteriovenous Malformation
-Most common congenital vascular anomaly of the brain; usually located in the cerebral hemispheres
-Hemorrhage and seizure most likely in 1st decade
-10% also have saccular aneurysm
CNS Trauma
Epidural hematoma
Subdural hematoma
Traumatic parenchymal injury
Epidural Hematoma
-Most frequently associated with skull fracture & ruptured middle meningeal artery; blood between skull and dura
-Concussion, “ lucid interval” followed by progressive loss of consciousness
-Can produce transtentorial and tonsillar herniation if not promptly treated
Subdural Hematoma
-Most acute cases caused by disruption of bridging veins between dura & arachnoid mater; “velocity change injuries”; chronic with atrophy of brain
-Symptoms slower onset than epidural; compression of brain with possible herniation if not resorbed or treated; may be chronic (neomembrane)
Traumatic Parenchymal Injury
Diffuse axonal injury
Traumatic intracerebral hemorrhage
Generalized brain swelling
contusion pattern
hit in front of head
hit in back of head
hit in back side of head
-in the front
-in the front
-on the opposite side of head and front
Neurocutaneous Syndromes (gene associations)
Neurofibromatosis I (17)
Neurofibromatosis II (22)
Tuberous sclerosis (9 or 16)
von-Hippel-Lindau disease (3)
Sturge-Weber disease (unknown)
Neurofibromatosis (type I)
cafe au lait spots
Lisch nodules (tan or brwon spots on the iris
Infection of the CNS
Leptomeningitis (meningitis)
Brain abscess
Tuberculosis and toxoplasmosis
Encephalitis (mostly viral)
Spongiform encephalopathies
Leptomeningitis (Meningitis)
-Acute (purulent); bacterial
-Acute lymphocytic; viral
-Chronic; fungal, TB & neurosyphilis
Evaluation of CSF
Cell type
Brain Abscess
Most often bacterial
Hematogenous spread
Contiguous spread
Direct implantation (trauma)
Viral Encephalitis
-Most common type of encephalitis
-Most often generalized,localized (HSV) to temporal lobe
-Arbovirus, HSV, CMV, HIV, JC
Prions as Agents of Disease
-Only known infectious agents devoid of RNA and DNA
-Manifest as infectious, sporadic or genetic diseases
-Accumulation of abnormal folded form of normal prion protein
-Prion conformation associated with specific disease
Spongiform Encephalopathies
Creutzfeldt-Jacob (CJD); classic and new-variant
Gerstmann-Straussler syndrome
Fatal familial insomnia
CNS Neoplasms
-Primitive neuroepithelial neoplasm (medulloblastoma)
CNS Neoplasms II
-Neuronal neoplasms (ganglioglioma, gangliocytoma, dysembryoplastic neuroepithelial tumor)
-Primary CNS lymphoma
-Metastatic neoplasms
-Most common of primary brain tumors; TP53 mutation thought to play a role
-Pilocytic type are slow growing; mostly in children
-Fibrillary types are diffuse and can grow rapidly; “grade” determines behavior; any age; astrocytoma, anaplastic astrocytoma, glioblastoma multiforme
Metastatic Neoplasms to the Brain
-The brain is a common site of metastasis
-Approximately 33% of all intracranial neoplasms are metastatic
-Excluding leukemia and lymphoma, frequent primaries are carcinomas of lung and breast and melanoma