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Neurology

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Answer
Majority of malignant gliomas are:   grade IV tumors (GBM or gliosarcomas)  
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Gliomas include:   astro; oligo; ependymoma; glial cells: support  
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Grade 1 Glioma =   Pilocytic Astrocytomas  
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Grade 2 Glioma =   Astrocytomas, Oligodendrogliomas  
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Grade 3 Glioma =   Anaplastic Astrocytomas, Anaplastic Oligodendrogliomas  
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Grade 4 Glioma =   GBM; Gliosarcoma  
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Diffuse astrocytomas include:   Low-grade infiltrative Astro (WHO II); anaplastic astro (WHO III); Glioblastoma (WHO IV)  
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Circumscribed astrocytomas include:   Pilocytic Astro (WHO grade I); Pleomorphic xanthoastrocytoma (PXA); Subependymal giant cell Astro (SEGA)  
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Well-differentiated Astro:   10-15% of Astros; 35 yo; 5-10 yr surviv; cerebral hemi / cortex; slow growing; often undergo malig progression to AA or GBM  
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High-grade gliomas (III/IV):   invade via white matter tracts, cross via corpus callosum;  
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Primary characteristic of a grade IV glioma =   necrosis with vascular proliferation  
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Anaplastic astro   High-Grade; 1/3 of Astros; 45 yo; 3 yr surviv; usu in cerebral white matter; relatively fast-growing; Frequent progression to GBM  
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GBM   50% of Astros; >60 yo; rare <30; 1 yr surviv; usu in cerebral hemi white matter; spreads rapidly & diffusely; Ring of tissue around necrotic core; Highly vascular  
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Oligodendroglioma   Usu presents at low-grade (II) stage; young / middle-aged; grade II >10 yr surviv; Fried-egg cells  
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Most common primary brain neoplasm =   GBM  
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Untreated GBMs: growth   double in size in 14 days  
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Brain tumor: genl clin presentation   HA; seizures; Cognitive dysfn; Focal neuro deficits; N/V; Sx endocrine dysfn; Visual sx; Sx from plateau waves (ICP changes)  
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Headache in brain tumor   20% of pts present w/HA (more w/ post fossa tumor); usu d/t inc ICP; Progressive increase in HA freq & severity; Classic HA on waking or HA that wakens one from sleep  
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Seizure in brain tumor   35% of pts present with this; often the sx that precedes dx; focal or secondarily generalized  
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Probably most common problem in pts w/brain tumors =   cognitive dysfn  
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Cognitive dysfn in brain tumor:   Frontal personality; Memory problem; Depression  
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Cognitive dysfn: Left hemispheric tumors:   language dysfunction  
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Cognitive dysfn: Right hemispheric tumors:   problems with visual perception & scanning  
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Brain tumor: Focal neurologic deficits   Hemiplegia; Hemiparesis; Ataxia; Nystagmus; Can mimic stroke  
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N/V more common in:   post fossa tumors  
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Endocrine sx:   hypothyroid; dec libido  
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Visual symptoms include:   Contralateral flashing lights; Visual field loss; Diplopia  
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Symptoms from plateau waves   Transitory episodes of altered consciousness & visual disturbances  
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Brain tumor: eval & dx   H&P; CT +/- MRI; EEG; LP; PET  
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Factors of better prognosis:   Young; High KPS; Lower pathological grade  
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Less significant prognostic predictors:   Long duration of sx; Absence of mental status changes; Cerebellar location; Small preop tumor size; Completeness of surgical resection  
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BCNU (chemo) adverse effects   fatigue, low blood counts, pulmo fibrosis  
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Tx for high grade gliomas   Primary tx = surg resection, RTx, CTx; most sig prognostic factors: extent of surg resection, age, & performance status  
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First treatment modality for high grade glioma   Surgery  
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Goals of surgery:   Confirm patho dx; improve sx rapidly; reduce number of ca cells requiring tx (removes hypoxic core of the tumor; relatively resistant to radiation & inaccessible to CTx)  
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Std tx for WHO III &IV gliomas:   Radiation tx (role of RT in WHO II gliomas is controversial)  
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RTx Modalities:   Whole brain (ltd use now in gliomas); Focal RTx: Conventional high-dose (59.4 Gy x 42 days; 2 cm border around tumor area) or IMRT; Stereotactic (gamma-knife)  
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RTx injury   Acute (fever, neuro def) or delayed encephalopathy (edema, MRI enhance); tx w/c’steroids; focal cerebral necrosis (mos-yrs): tx w/steroids & hyperbaric  
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Most frequent delayed effect of RTx   Diffuse cerebral injury; cortical dysfunction, progressive dementia, gait disturbance; WM destruction; MRI: lg ventricles, wide sulci, basal ganglia calcifn, hyperintense T2  
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Std of care: high grade gliomas   Resection; RTx & 42 days temozolomide; then (if stable dz), adjuvant CTx  
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Stupp study result:   temozolomide added to RTx improves survival  
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CTx MOA & AE   targets DNA replication; AE: Myelosuppression; N/V; Fatigue; Constipation or diarrhea; DI  
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CTx: passage across BB depends on:   Molecule Size; Lipid solubility; Ionization state  
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CTx DI:   Dex (closing BBB); phenobarbital (dec nitrosourea efficacy); anti-epileptics (affect CTx metabm)  
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Low grad glioma: Tx   Bx (confirm dx & r/o high grade); seizures only: defer tx til progresses; if progressive sx: resect +/- CTx (+ RTx only if refractory)  
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VEGF   higher the VEGF, worse the prognosis; anti-VEGF Ab’s effective in xenografts  
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Tumor blood vessels   dept on growth factor; leaky & fragile; less supported by pericytes; disorganized, twisted, variable pressure; inconsistent nutrient delivery to tumor tissue  
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Second leading COD in brain tumor pts:   thromboembolic complications  
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Meningioma   slow growing, benign; attached to dura mater; 13-26% of primary IC tumors; RF: prior radiation; often Asx; visual complications; DDx: neurofibromatosis; Firstline tx: Surgery  
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Ependymoma: worse prognosis:   <3 yo  
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Ependymomas within brain: locations   Infratentorial > supratentorial (2x)  
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Ependymoma   Usu slow growing; kids & YA; young adults; 4% all BT; from walls of ventricles or spinal canal (obstructive hydrocephalus); 90% are in brain, 10% in spinal cord (often adults)  
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Medulloblastoma   Malig, invasive embryonal cerebellar tumor; kids & YA; 70-80% 5-yr survival; Most arise in vermis (4th ventricle involvement)  
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Medulloblastoma: metastases   Metastasize via CSF pathways (Drop Mets); Rare mets to bone, lymph nodes, other extracranial sites  
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Medulloblastoma: Sig LT toxicities of tx:   Cognitive decline; Psychomotor / growth retardation; Hormonal deficits  
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Primary CNS lymphomas   Usu present deep in brain parenchyma; usu multifocal (diffuse lg cell B-cell lymphoma); Survival w/o tx <1 yr; Steroid tx & Methotrexate CTx  
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Primary CNS lymphomas: incidence increased in:   Immunodef pts; AIDS; Organ TP; older pt  
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Std of care: Grade I   Surgery +/- RT  
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Std of care: Grade II   Surgery; Observe; If progression: CTx  
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Std of care: Grade III   Surgery; RT with temozolomide; 12 cycles of temozolomide  
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Std of care: Grade IV   Surgery; RT with temozolomide; 52 weeks rotational CTx  
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HA worse in AM w/ focal neuro deficits   Brain Tumor (MC is glioma)  
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Majority of malignant gliomas are:   grade IV tumors (GBM or gliosarcomas)  
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Gliomas include:   Astro (Grade 1); oligo (2); ependymoma; glial cells  
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Grade 3 Glioma =   Anaplastic Astrocytomas, Anaplastic Oligodendrogliomas  
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Grade 4 Glioma =   GBM; Gliosarcoma  
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Diffuse astrocytomas include:   Low-grade infiltrative Astro (WHO II); anaplastic astro (WHO III); Glioblastoma (WHO IV)  
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Circumscribed astrocytomas include:   Pilocytic Astro (WHO grade I); Pleomorphic xanthoastrocytoma (PXA); Subependymal giant cell Astro (SEGA)  
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Well-differentiated Astro:   10-15% of Astros; 35 yo; 5-10 yr surviv; cerebral hemi / cortex; slow growing; often undergo malig progression to AA or GBM  
🗑
characteristic of high-grade gliomas (III/IV):   Invasion via white matter tracts, cross via corpus callosum  
🗑
Primary characteristic of a grade IV glioma =   necrosis with vascular proliferation  
🗑
Anaplastic astro   High-Grade; 1/3 of Astros; 45 yo; 3 yr surviv; usu in cerebral white matter; relatively fast-growing; Frequent progression to GBM  
🗑
GBM   usu in cerebral hemi white matter; spreads rapidly & diffusely; Ring of tissue around necrotic core; Highly vascular  
🗑
Oligodendroglioma   Usu presents at low-grade (II) stage; young / middle-aged; grade II >10 yr surviv; Fried-egg cells  
🗑
Most common primary brain neoplasm =   GBM  
🗑
Untreated GBMs: growth   double in size in 14 days  
🗑
Brain tumor: genl clinical presentation   HA; seizures; Cognitive dysfn; Focal neuro deficits; N/V; Sx endocrine dysfn; Visual sx; Sx from plateau waves (ICP changes)  
🗑
Headache in brain tumor   20% of pts present w/HA (more w/ post fossa tumor); usu d/t inc ICP; Progressive increase in HA freq & severity; Classic HA on waking or HA that wakens one from sleep  
🗑
Seizure in brain tumor   35% of pts present with this; often the sx that precedes dx; focal or secondarily generalized  
🗑
Probably most common problem in pts w/brain tumors =   cognitive dysfn (Frontal personality; Memory problem; Depression)  
🗑
Cognitive dysfn: Left hemispheric tumors:   language dysfunction  
🗑
Cognitive dysfn: Right hemispheric tumors:   problems with visual perception & scanning  
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Brain tumor: Focal neurologic deficits   Hemiplegia; Hemiparesis; Ataxia; Nystagmus; Can mimic stroke  
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Brain tumor: N/V more common in:   posterior fossa tumors  
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Endocrine sxs in brain tumor:   hypothyroid; dec libido  
🗑
Visual symptoms in brain tumor include:   Contralateral flashing lights; Visual field loss; Diplopia  
🗑
Low grade glioma: Tx   Bx (confirm dx & r/o high grade); seizures only: defer tx til progresses; if progressive sx: resect +/- CTx (+ RTx only if refractory)  
🗑
Second leading COD in brain tumor pts:   thromboembolic complications  
🗑
Ependymoma: worse prognosis:   <3 yo  
🗑
Ependymomas within brain: locations   Infratentorial > supratentorial (2:1)  
🗑
Ependymoma   Usu slow growing; kids & YA; young adults; 4% all BT; from walls of ventricles or spinal canal (obstructive hydrocephalus); 90% are in brain, 10% in spinal cord (often adults)  
🗑
Characteristics of Medulloblastoma   Malig, invasive embryonal cerebellar tumor; kids & YA; 70-80% 5-yr survival; Most arise in vermis (4th ventricle involvement)  
🗑
Medulloblastoma: metastases   Metastasize via CSF pathways (Drop Mets); Rare mets to bone, lymph nodes, other extracranial sites  
🗑
Characteristics of Primary CNS lymphomas   Usu present deep in brain parenchyma; usu multifocal (diffuse lg cell B-cell lymphoma); Survival w/o tx <1 yr; Steroid tx & Methotrexate CTx  
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most common spinal tumor:   ependymomas; 10% of spinal tumors are intramedullary  
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Brain tumor: frontal lobe: S/S:   cog decline; contralat grasp reflex; expressive aphasia  
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Brain tumor: temporal lobe: S/S:   sz, olfactory hallucination, depersonalization, vis field def, auditory illusions  
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Brain tumor: parietal lobe: S/S:   contralat sensory def; cortical sens loss (stereognosis); inattention  
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Brain tumor: occipital lobe: S/S:   crossed homonymous hemianopia / partial field defect; visual agnosia  
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Brain tumor: brain stem/cerebellar: S/S:   CN palsies, ataxia, incoordination, nystagmus, pyramidal & sensory deficit  
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Astrocytoma   Glial tumors: 40-50% of CNS Neoplasms  
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Astrocytoma: Grade IV   GBM (45-55 yo); necrosis/ hemorrhage, edema, ring enhancement  
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Meningioma   50-60 yo; may increase in PG; various grades (90% benign)  
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Brain neoplasm: mets   1/3 of all intracranial neoplasms; lung, breast, melanoma, colon, lymphoma, prostate  
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Brain neoplasm: most common site for kids (unlike adults)   posterior fossa (medulloblastoma)  
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brain tumors seldom__   metastasize outside the CNS  
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__ spread the soonest with metastatic brain tumors   lung and renal cancer cells  
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mets to the brain in men generally come from __   lung, colon, and renal cancers  
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mets to the brain in women generally come from __   breast, lung, and melanoma  
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a tumor in the supratentorial region may result in which pathologic disorder   epilepsy  
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which cancers metastasize to the brain the fastest?   lung and renal  
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in which area of the brain do most mets tumors arise?   cerebrum 80%  
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tethered cord   abnormally low conus medullaris  
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Central cord syndrome S/S   motor deficit in UE > LE; Varying degrees of sensory loss (pain/temp); most common of the incomplete spinal cord lesions (better prognosis)  
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Ligamentum flavum buckles into spinal cord => contusion to central regions of spinal cord =   Central cord syndrome  
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Anterior cord syndrome S/S   Paraplegia and dissociated sensory loss with loss of pain and temperature sensation; 2/2 infarction of the cord in the region supplied by the anterior spinal artery  
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Anterior cord syndrome: what fn is preserved:   Posterior column (position, vibration, deep pressure) preserved  
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Brown-Sequard syndrome: cause   Hemisection of the cord; From penetrating injuries; Rare  
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Brown-Sequard syndrome S/S   Ipsilateral motor loss w/loss of vibration, pressure, proprioception; contralateral loss of pinprick, pain, temperature sensations  
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Benign nonencapsulated tumor of nerve fibers =   Neurofibroma  
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Benign tumor of nerve sheath =   Schwannoma  
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