Pathology Exam 3
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lovedosage on April 16, 2012
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120 terms
Terms | Definitions |
|---|---|
 Cerebral Palsy | non-heredity, non-progressive lesion of cerebral cortex multifactorial- result of cerebral anoxia, hemorrhage, cortex damage |
| CP Neuromuscular deficits | posture, voluntary movement, speech, vision, hearing, perception, seizures, hydrocephalus, microcephalus, retardation |
| CP Muscle Tone | hypotonia- low tone hypertonia- high tone mixutre- fluctuations graded mild, moderate or severe |
| CP Spasticity | Monoplegia, diplegia, hemiplegia, quadriplegia Ataxia, Dyskinesia (choreoathetiosis) |
| CP Incidence | 2.5 to 4.2 per 1000 births |
| CP Pathogenesis | Neuropathic lesions leading to subependymal hemorrhage, malformation of CNS Hypoxia leading to systemic degeration, brain edema, acidosis |
| CP Clinical Manifestations | change in muscle tone, abnormal posture, abnormal reflex, delayed motor development & performance, abnormal head circumference, MS problems, joint restriction, contracture, decrease muscle mass & power & endurance |
| CP Diagnosis | neurological exam, EEG, hip radiograph, CBC, UA, CT dx usually takes time |
| CP Treatment | Multidisciplinary Rx- baclofen, diazepam, anti seizure, carnitine Neurosurgery- motor point block, botox, dorsal rhizotomy Orthopedic surgery- femoral de-rotation, osteotomy, orthosis |
| CP Prognosis | Mild- resolution with maturity Moderate- limited mobility Severe- poor prognosis Ambulation prognosis based on motor milestones |
| Signs of Hypotonia | wide based sitting head lag decreased reaching & kicking |
| Spina Bifida Definition | Common congenital defects of neural tube closure incomplete fusion of posterior vertebral arch |
| Most common location for spina bifida | lumbosacral region |
| Spina Bifida Occulta | bone defect with spinal cord, meninges & spinal fluid in tact dimple in skin & patch of hair, port wine nevi |
| Meningocele | protruding CSF sac containing only fluid & meninges only spinal cord/cauda equina in tact neurological deficits are rare |
| Myelomeningocele | most severe form protrusion of meninges & spinal cord spine is open- sac contains CSF, meninges & spinal cord |
| Spina Bifida Incidence | most severe form is most frequent apx 1 in 1000 more children have spina bifida than MD, MS & CF combined |
| Spina Bifida Etiolgoy | Genetic predisposition Nurtient deficiency (folic acid & vitamin B) Radiation, environment, alcohol, viral, epileptic rx |
| Spina Bifida Pathogenesis | posterior portion of neural tube fails to close at apx 20-23 days post conception- degree of dysfunction related to anatomical location |
| Spina Bifida Clinical Manifestations | Motor dysfunction- uneven distributation Muscle imbalance Scoliosis MS deformities |
| Myelomeningocele 0-2 years S & Sx | truncal hypotonia, delayed reflexes, 90% have normal intelligence & hydrocephalus, obesity, risk of seizures |
| Myelomeningocele Childhood & Adolescence | kyphoscoliosis, decrease ambulatory status, strabismus, vasomotor insufficiency |
| Spina Bifida Characteristics | flaccid or spastic paralysis bowel & bladder incontinence sensory disturbances MS deformities Latex allergy |
| Hydrocephalus | enlarged ventricles of the brain hindbrain is displaced through foramen magnum increasing pressure & splitting of neural tube |
| Arnold-Chiari Malformation | Brainstem, 4th ventricle & part of cerebellum pulled through foramen magnum increasing pressure on cranial nerves |
| Ventriculoperitoneal Shunt | drainage of CSF from ventricles to extra cranial compartment |
| Risk Factors for Pressure Ulcers | ammonia from urine friction & pressure from casts bony prominence vascular problems asymmetrical weight bearing |
| S2-S4 Spinal Level | Control of bowel & bladder function |
| Spina Bifida Prenatal Diagnosis | ultrasound, serum alpha-fetoprotein (AFP) test, amniocentesis |
| Spina Bifida surgery | skin closure- within 24-48 hours after birth common- hip varus, spinal fusion, tendon transfer & muscle release |
| Spina Bifida Prognosis | early aggressive care increases prognosis dependent on neurological deficit most deaths before age 4, 85% into adulthood |
| Tethered Cord Syndrome S & Sx | scoliosis, spasticity, asymmetrical posture, abnormal gait, decrease coordiation, back pain |
| Contraindications for bracing | severe spinal deformity decreased UE strength moderate obesity plantar flexion contracture > 15-20 degrees hip flexion contracture > 35 degrees |
| 3 Forms of Congential Hip Dysplasia | 1. Unstable- positioned normal, easily displaced 2. Subluxation & Incomplete dislocation- femoral head partially displaced 3. Complete dislocation- femoral head outside acetabulum |
| DDH Incidence | 1 in 1000 85% female increase risk with breech, large neonates, twins, idiopathic scoliosis, myelomeningocele, arthrogryposis & CP |
| DDH Etiology | hormonally derived laxity of ligaments utero positioning spinal instability mechanical, physio & environmental factors |
| DDH Pathogenesis | Most dislocations occur during perinatal period breech position causes forced flexion & adduction secondary changes in soft tissue & bone |
| DDH Clinical Manifestations | asymmetrical ROM & gluteal fold leg length discrepancy bilateral dysplasia- duck waddle Unilateral dysplasia- tredelenberg flexion contractures |
| DDH Diagnosis | clinical examination radiograph > 6 weeks old ultrasound < 6 months old |
| DDH Treatment | position in flexion & abduction- 2 diapers or Pavlik harness surgical in older children- CRIF, ORIF, traction, osteotomy |
| DDH Prognosis | if dislocation correct in first few days- 100% reversible if uncorrected deformity will become permanent & secondary problems |
| Muscular Dystrophy | group of genetic diseases marked by progressive weakness & degeneration of skeletal muscle |
| MD Characteristics | symmetrical muscle wasting without neural deficits initial hypertrophy due to connective tissue & fat deposition replacement of muscle |
| Types of MD | Duchennes (pseudohypertrophic)- 50% Beckers (benign pseudohypertrophic) Facioscapulohumeral (Landouzy-Dejerine) Limb-girdle dystrophy |
| MD Incidence | 20-30 in 100,000 |
| MD Genetics | DMD & BMD are X-linked recessive caused by mutation in dystrophin gene, male dominant FSH is autosomal dominant, same sex equal LGD autosomal recessive, same sex equal |
| DMD Characteristics | low levels of dsytrophin difficult raising from floor, frequent falling waddling gait, walking on toes due to weakposterior tibialis, peroneals & hip abductors deterioration of ambulation shoulder girdle involvement in 3-5 years |
| dystrophin gene | found in skeletal muscle, cardiac muscle & brain tissue links sarcolemma to actin, muscle contraction causes necrosis |
| BMD Characteristics | normal dystrophin levels, but dystrophin is abnormal size same as DMD with later onset primary involvement of neck, trunk, pelvis & shoudler girdle muscle cramp, scoliosis, contracture |
| FSH Characteristics | begins with facial muscles & shoulder girdle expressionless face, inability to close eyes, sacpula wing contracture, deformity, hypertrophy are uncommon |
| LGD Characteristics | slow course, mild development starts in biceps & deltoids & pelvic muscles serum creatine kinase levels high |
| MD Treatment | no known treatment maintain function & movement strenuous exercises facilitates breakdown of muscle low rep, maximum exercise contraindicated maintain chest mobility & breathing |
| MD Prognosis | all forms are progressive prognosis varies with type earlier the dx & more rapid is poor prognosis |
| Dorsal Column DCML | synapse at brainstem level fine touch, vibration, proprioception, pressure |
| anterolateral spinothalamic ALS | syanpse at spinal cord- white commisure anterior- crude touch lateral- pain & temperature |
| Somatosensation | proprioception- sensory input from joints & muscles |
| Plans & Strategy | establish by cortex |
| Execution of task & modifications | established by brainstem & spinal cord |
| Cranial Nerves | peripheral nerves- sensory & motor to head & neck nuclei lie withing brainstem & midbrain |
| Cerebellum | coordination of skeletal muscle movements |
| Cerebeullar ataxia | incoordination of movement |
| dysmetria | under or over estimation of movement |
| dysiadochokinesis | inability to perform rapid alternating movements slow without rhythym or consistency |
| Scanning speech | ataxia of laryxn- word selection normal, but slow without melody, tone or rhythym |
| ataxia of the eye | nystagmus |
| Stupor & coma | hypo-arousal- state of unresponsiveness |
| Reticular Activating System | complex interaction between brainstem & cerebral cortex |
| Attnetion deficits | Frontal & Prefrontal damage or under development |
| Limbic System | false beliefs, paranoia, delusions |
| Hippocampus | Working memory- ability to hold short-term memory info Declarative memory- retention of experiences Procedural memory- learning of skills & habits |
| Expressie Aphasia | deficit in language output- garbled, inappropriate words |
| Repetitive Aphasia | inability to understand language |
| Dysarthria | disturbance in articulation |
| Anarthria | inability to produce speech |
| Alexia | inability to read |
| Agraphia | inability to write |
| Apraxia | acquired disorder of skills not a result of paresis, akinesia, ataxia, or sensory loss ideomotor most common |
| Agnosia | inability to recognize an object, no meaning attached to an object |
| Right Hemisphere | Spatial relationships, relates body to environment, touch, facial recognition, left sided neglect, loss of 3D |
| Left Hemisphere | interpretation & speech centers, language |
| Temproal Lobe | auditory sensation & perception |
| Limbic Area | emotions, pain, pleasure, anger, fear |
| Limbic Lobe | hyppocampus, amygdala, cingulate gyrus memory, anger, emotional stress |
| Frontal Lobe | highest levels of cognition & processing personality, judgement, insight |
| Plasticity | change in organization of connections among neurons |
| Diaschisis | neuronal shock- injury to nerve, disruption of neural pathway |
| Denervation Supersensitivity | loss of presynaptic function- increase sensitivity of postsynaptic neurons |
| Regnerative Synaptogenesis | sprouting of injured axons |
| Collateral Sprouting | neighboring axons sprout to connect with sites that were previously innervated by injured neuron |
| Dementia | degeneration of gray matter |
| Involuntary movements | degeneration of basal ganglia |
| Disruption of coordination | cerebellum & brainstem |
| Amyotrophic Lateral Sclerosis | aka Lou Gehrigs- degeneration & scarring of motor neurons affects upper motor neurons- corticospinal & corticobulbar affects lower motor neurons- anterior horn (motor) |
| ALS pathogenesis | accumulation of pigmented lipid (lipofuscin) affects giant pyramidal cells demyelination causing atrophy of muscles |
| ALS Etiology | Sporadic- most common form Familial- genetic dominance inheritance Guamanian- high incidence in Guam |
| ALS Clinical Manifestations | Positive babinski & Hoffmanns asymmetrical weakness fasciculations of muscle fibers |
| ALS S & Sx categories | 1. Pseudobulbar Palsy- dysarthria 2. Progressive bulbar palsy- weakness of swallowing, chewing & facial gestures 3. Primary lateral sclerosis- hyperactivity & spasticity 4. Muscluar Atrophy- waekness, wasting, fasciculations |
| ALS tx | unknown- symptomatic therapy |
| Alzheimers Disease | chronic progressive degeneration & formation of fibrous amyloid of the hippocampus, amygdala, cerebral cortex |
| Alzheimerss Clinical Manifestations | inability to learn to information personality changes apraxia, alexia, agraphia, aphasia, ataxia loss of learning & memory Broca's & Werneike's areas |
| Friedreichs Ataxia | spinocerebellar degeneration (spinal cord) dengeneration of dorsal root ganglia & corticospinal tracts loss of Purkinge cells in heart |
| Friedreichs ataxia | movement with ataxic gait motor restlessness- tremor dysarthrias & scanning speech, nystagmus |
| Huntingtons Disease | progressive hereditary disorder characterized by choreic movements- changes in cortex & cerebellum |
| Huntingtons Pathogenesis | reduction in GABA, ACTH disruption of smooth movement- basal ganglia & thalamus chorea, bradykinesia, hypertonicity, dysarthria, dysphagia, asphyxia, cachexia |
| Chorea | wide-based staggering, dance-like gait |
| Huntington Abnormal Eye Movements | saccades-both eyes same direction gaze fixation smooth pursuit- following object |
| Stroke | Sudden loss of neurologic function due to impaired blood flow to the brain, non-conclusice greater than 24 hours |
| Transient Ischemic Attack | focal non-conclusive episode of neurologic dysfunction that last less than 24 hours |
| Stroke Popluation | 3 males > 2 females african americans > caucasians |
| Unmodifiable Risk Factors | age, gender, race, heredity |
| Modifiable Risk Factors | smoking, HTN, diabetes, alochol, obesity, high serum lipids, A-fib, cardiac disease, hematologic disorders |
| Types of Stroke | Ischemic- lack of blood supply without bleeding ex:thrombus, emobolis, lacunar Hemorrhagic- bleeding resulting from rupture of vessel ex: subarachnoid, intercerebral, hypercoagulative, vasculitis |
| Ischemic Stroke Pathogenesis | severity determined by duration, severity & location impaired metabolism- increase Ca+, acidosis & free radical ischemic penumbra- neurons fxnlly silent but intact |
| Hemorrhagic Stroke Pathogenesis | long exposed HTN causes aneurysm, secondary bleeding due to alteration of wall permeability displace adjacent brain-> increasse pressure-> vasospasm-> cerebral infarction |
| Stroke Clinical Manifestations | sx occur gradually & worsen over time headache & seizure may present named according to arteries feeding that area |
| Middle Cerebral Artery Syndrome | hemiplegia, hemiesthesia, global aphasia, weakness, sensory disturbance, motor aphasia |
| Anterior Cerebral Artery Syndrome | uncommon due to compensation of collateral blood flow |
| Traumatic Brain Injury Etiology | Young males affected most common cause is MVA, assaults, falls, intoxication |
| TBI Pathogenesis | Epidural hematoma- blood clot forms in dura Subdural hematoma- tearing btw dura & brain Intracerebral hematoma- direct trauma to intracranial vessel Contusion- hemorrhagic transformation- injury, edema, ICP Diffuse axonal injury- corpus callosum direct vascular injury:vascular injury after closed head injury |
| TBI S & Sx | basilar skull fracture battle signs (blood over mastoid process) Racoon eyes palpation & observation for open, depressed skull fx |
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