Pathology 1 - Block 3 - Part 6

davidwurbel7's version from 2016-04-04 00:01


Question Answer
Transient monocular blindness occurs from an emboli of the central retinal artery of one eyeAmaurosis Fugax
This is the earliest sign of cerebral ischemiaRed Neurons
Reduction or complete cessation of blood flow to a localized area of the brain due to arterial occlusion or low perfusionFocal Cerebral Ischemia
Due to mural thrombi from heart, atrial fibrillation. Typically produces hemorrhagic (red) type of infarctEmbolism Occlusion
Associated with atherosclerosis and plaque rupture. Commonly seen at the carotid bifurcation, origin of middle cerebral artery, and either ends of basilar artery. Typically produces a pale infarctThrombotic Occlusion
Affects the deep penetrating arteries and arterioles that supply the basal ganglia. Associated with hypertension and hyaline arteriolosclerosis. Leads to single, or multiple small cavity infarctsLacunar Infarct
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Question Answer
Rupture of small intraparenchymal vessels results in a hemorrhage within the brainIntracerebral Hemorrhage
Microaneurysm in the penetrating branches of the middle cerebral artery. These measure less than 300 um in diameter. Pressure-induced rupture causes bleeding in the putamen, thalamus, adjacent white matter, pons, and cerebellum. Associated with chronic hypertensionCharcot-Bouchard Microaneurysms
Common sites: putamen, thalamus. extravasation of blood with compression of adjacent parenchyma. Hemosiderin laden macrophages appearIntracerebral Hemorrhage
Associated with Alzheimer disease. Leptomeningeal and cerebral cortical arterioles and capillaries. Dense and uniform deposits of amyloid seenCerebral Amyloid Angiopathy
Rupture of sacular (berry) aneurysm. Bleed occur into the subarachnoid spaceSubarachnoid Hemorrhage
Seen near major arterial branch points in anterior circulation. Structural anomaly . There is absence smooth muscle and intimal elastic lamina. This anomaly is mostly seen to occur along branching pointsSacular (Berry) Aneurysm
Predisposing factors include linked to genetic factors, autosomal polycystic kidney disease (ADPKD), Ehlers-Danlos synd (type IV), Marfan syndrome, neurofibromatosis (NF-1) or predisposing factors such as smoking and hypertensionSubarachnoid Hemorrhage
Clinical Features - “worst headache of my life”. Sudden loss of consciousness may be preceded by a brief moment of excruciating headache. Headache is usually generalized, often with neck stiffness, and vomiting is common. Xanthochromic spinal fluid if spinal tap is doneSubarachnoid Hemorrhage


Question Answer
Deficiency of galactocerebroside β-galactosidase. Absence of enzyme activity leads to accumulation of galactosylsphingosine. Brain shows loss of myelin and oligodendrocytes. Presence of ‘globoid ‘ cells in brainKrabbe disease
Clinical Features include rapidly progressive, predominant motor signs such as weakness. normal at birth, develop irritability, spasticity around 3 to 6 months of age. Usually die in two or three yearsKrabbe disease
Autosomal recessive disorder. deficiency of lysosomal enzyme: arylsulfatase A. deficiency leads to accumulation of sulfatides. macrophages show ‘metachromasia’ when stained with Toluidine blueMetachromatic Leukodystrophy
Mutation in a member of the ATP-binding cassette transporter family of proteins: ABCD1. Inability to catabolize very long chain fatty acids, leading to these to be elevated in serum. Leading to progressive loss of myelin in CNS and peripheral nerves. Adrenal insufficiency associated with adrenal atrophyAdrenoleukodystrophy
Inflammation, demyelination, gliosis (scarring), and neuronal loss; the course can be relapsing-remitting or progressive. Autoimmune destruction of myelin. more in temperate than tropical. more in females. 20 to 40 years. Caucasians have higher risk. Associated with HLA-DR2. Immune destruction of myelin by TH1 and TH17 T-cellsMultiple Sclerosis
Labs raised IgG in CSF. Raised oligoclonal bandsMultiple Sclerosis
Clinical features include reduction in muscle power, spasticity. sensory: pins and needles, numbness, pain, vertigo, scanning speech. Eye Findings optic neuritis, visual blurring, internuclear opthalmoplegia (characterized by medial longitudinal fasciculus) and bladder dysfunctionMultiple Sclerosis
Rare progressive clinical syndrome characterized by cognitive decline, spasticity of limbs, and seizures. Seen months or years after early-age acute infection with measles. Often fatalSubacute Sclerosing Panencephalitis
Type of encephalitis caused by JC polyoma virus. Clinically characterized by demyelination. Seen exclusively in immunosuppressed patients. Clinical features include visual loss, weakness, high mortalityProgressive Multifocal Leukoencephalopathy
Acute disorder characterized by loss of myelin in basis pontis and portion of the pontine tegmentum. Myelin loss appears to be symmetrical. Usually follows 2 to 6 days after rapid correction of hyponatremia. Clinical features include rapidly evolving quadriplegia. “locked in” syndromeCentral Pontine Myelinolysis
β-secretase acts on APP with formation of insoluble product instead of enzyme α-secretase, leading to formation of a soluble product. cortical atrophy widening of sulci (frontal, temporal, parietal), compensatory ventricular enlargement (hydrocephalus ex vacuo). Neuritic plaques seen around a central amyloid core (stain positive for Congo Red). Neurofibrillary tangles containing hyperphosphorylated tau protein Alzheimer's Diease
Elongated, glassy, eosinophilic bodies found in hippocampal pyramidal cellsHirano Bodies
Hirano bodies is associated with this conditionAlzheimer's Disease
Composed of fine filaments, densely packed in the core composed of α-synucleinLewy Bodies
Expanded repeats of of trinucleotide repeats (CAG)Huntington Disease
Brain is small, atrophy of caudate nucleus. Secondary atrophy of globus pallidus and dilation of the lateral ventriclesHuntington Disease
Degenerative disease characterized by loss of upper and lower motor neuronsALS
ALS characterized by loss of these cellsBetz Cells
Mutated SOD1 is a misfolded protein therefore triggers a misfolded protein responseALS
Clinical Features include asymmetric weakness of hands. Spasticity of arms and legs. Involuntary contractions and fasciculation. Combined upper motor neuron and lower motor neuron deficits. No oculomotor deficitsALS
Expansion of unstable trinucleotide repeats (GAA)Friedreich Ataxia
Clinical Features include manifests in 1st decade, gait ataxia (staggering), nystagmus, dysarthria, pes cavus, hammer toes, hypertrophic cardiomyopathy. Kyphoscoliosis in childhoodFriedreich Ataxia


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Question Answer
Gross: poorly defined, and infiltrative areas of hemorrhage and necrosis may be seen. Micro: increased glial cellularity. Variable nuclear pleomorphism. Network of astrocytic processes. Stain positive for GFAPGlioblastoma Multiforme
Clinical features include headache, seizures, and focal neurologic deficitGlioblastoma Multiforme
Atypical, Mitosis, Endothelial, Necrosis (AMEN)Glioblastoma Multiforme
Childhood tumor. Seen in cerebellum, slow growing. Grade I/IV (indicates low-grade, benign). Morphology: often cystic, may be well circumscribed. On micro: bipolar cells, with hair-like processes, Rosenthal fibers, eosinophilic granular bodies. GFAP positivePilocytic Astrocytoma
Clinical features include raised ICT. Recurrence free intervals of more than 20 yearsPilocytic Astrocytoma
Low-grade glioma. Less infiltrative. Incidence: mostly adults (40 to 60 yrs). Mostly seen in cerebral hemispheres, with predilection for white matter. Morphology: well circumscribed. gelatinous with cystic spaces. calcification (important point). low proliferative indexOligodendroglioma
Clinical features include seizures, slow growing, long post-operative survival time. Overall survival rate is good. On microscopy ‘chicken-wire’ capillary pattern, “fried egg” cellsOligodendroglioma
Slow growing glioma, composed of neoplastic ependymal cells. Typically originates from wall of cerebral ventricles. Chromosome 22q, which contains the neurofibromatosis 2 (NF2) gene. Micro: perivascular pseudo-rosettesEpendymoma
Clinical features depends on the location of the neoplasm. Headache, nausea, vomiting, or vertigo. Secondary to increased ICP from obstruction of CSF flow through the fourth ventricle. Well-differentiated, slow growing. 5 year survival rate: 45 to 50%Ependymoma
Embryonal malignant tumor, poorly differentiated (grade IV). Arise from granular cells (cerebellum). Incidence: 3 to 10 years. slightly more in male child. Morphology: midline of cerebellum. may occlude CSF flow. often well-circumscribed. Micro: sheets of tumor cells. Homer-Wright rosettes. seeding of the CSF is commonMedulloblastoma
Clinical features include raised ICT, epilepsy, focal neurologic deficit, grade IV, aggressive. Children less than 3 years with metastases have poor prognosis Medulloblastoma
Benign tumors (majority) arising from meningothelial cell of arachnoid. Commonly located along the falx, cortical convexity, and sphenoid bone. Association with NF-2 (Chromosome 22q contains the NF2)Meningioma
Rounded, encapsulated masses, has a dural base, compress underlying brain en plaque: tumor spreads thinly over dural surface. Micro - Psammoma bodies may be seenMeningioma
Clinical features include slow growing. Parasagittal aspect of brain convexity, dura over lateral convexity, wing of sphenoid, olfactory groove, sella turcica, foramen magnum. Seizures, visual changes, mental status changes, hearing loss, muscle weakness. Surgery is best optionMeningioma
Aggressive malignancy arising exclusively in the CNS often multifocal. extra-nodal non-Hodgkin Lymphoma. incidence is increasing, particularly in immunocompromised individuals. Epstein-Barr virus (EBV) frequently plays an important role in the pathogenesis of HIV-related PCNSL. Risk factors include congenital or acquired immunodeficienciesPrimary CNS Lymphoma (PCNSL)
Morphology: mostly multiple, and deeply situated. tend to be well defined, but as discrete as metastases. periventricular spread. Micro: diffuse large B cell lymphoma (DLBCL). B-cell markers are positive (CD20, CD19). EBV markers positive, in setting of immunosuppressionPrimary CNS Lymphoma (PCNSL)
Clinical features include focal neurologic deficits, visual disturbances, neuropsychiatric, seizures, raised ICT, B symptoms: fever, weight loss, night sweats. Poor response to treatmentPrimary CNS Lymphoma (PCNSL)
Diagnosis by CSF, (commonly shows elevated protein and malignant cells), tissue sampling, CBC, HIV testing and bone marrowPrimary CNS Lymphoma (PCNSL)
Hypothalamic suprasellar tumor. Derived from vestigial remnants of Rathke pouch. Slow growing benign tumors, most seen in 5 to15 age group and second age group 65 years and older. Children present with growth retardationCraniopharyngioma
Morphology: small 3 to 4 cm diameter encapsulated, solid, and cystic pattern. Encroach on optic chiasm/cranial nerves. Micro: in children: adamantinomatous (enamel, calcification). In adults: rarely calcify. Cysts show cholesterol rich thick brownish-yellow fluidCraniopharyngioma
Clinical features include symptoms result from compression of adjacent structures, especially the optic chiasm leading to bitemporal hemianopia. Diabetes insipidus. Risk of recurrence associated with larger tumorsCraniopharyngioma
Clinical feature leukocoria (white pupillary reflex), strabismus, ocular pain. tumor extension into brain involving optic nerveRetinoblastoma