Term
| In acute neuronal injury, describe the morphology of the "dead red" neuron. |
|
Definition
| pyknotic, loss of nucleoli, and eosinophilic |
|
|
Term
| What sort of inclusions are present in neurons damaged by RNA viruses? |
|
Definition
|
|
Term
| In neurosyphilis, how does the microglia compensate for damage? |
|
Definition
| formation of elongated nuclei called rod cells |
|
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Term
|
Definition
| macrophages that are swollen from having phagocytosed debris |
|
|
Term
| White matter is primarily affected by what type of cerebral edema? |
|
Definition
|
|
Term
| In interstitial edema, what happens to the white matter? |
|
Definition
| The periventricular white matter volume is reduced as CSF moves across the ependymal cell border |
|
|
Term
| In subfalcine herniation, which artery is compromised? |
|
Definition
| the branches of the anterior cerebral artery |
|
|
Term
| What is Kernohan's notch due to? |
|
Definition
| When the extent of herniation is large enough that the contralateral cerebral peduncle may be compressed, resulting in hemiparesis ipsilateral to the side of the herniation |
|
|
Term
| When there is central transtentorial herniation, what often happens in the midbrain and pons? |
|
Definition
| Hemorrhagic lesions called Duret hemorrhages form as there is distortion and tearing of penetrating veins and arteries supplying the upper brainstem |
|
|
Term
| What symptoms are commonly present in the late stages of Alzheimer's Disease? |
|
Definition
| mutism, incontinence, and inability to walk |
|
|
Term
| Upon gross pathological examination of the brain, what two structures have notably atrophied? |
|
Definition
| the cortex and the hippocampus |
|
|
Term
| What are Hirano bodies and in which situation are they usually found? |
|
Definition
| intracellular actin aggregates/ Alzheimer's disease |
|
|
Term
| In Pick disease, what is traditional spared from disease? |
|
Definition
| posterior 2/3 of the superior temporal gyrus |
|
|
Term
| What histologic feature is typically found in the affected regions of progressive supranuclear palsy? |
|
Definition
| globose neurofibrillary tangles |
|
|
Term
| In progressive supranuclear palsy, how is movement disorder typically characterized? |
|
Definition
| truncal rigidity, vertical gaze palsy, nuchal dystonia, abnormal speech |
|
|
Term
| In corticobasal degeneration, what is chracteristic of the neurons? |
|
Definition
| they have a "ballooned" appearance |
|
|
Term
| Exrapyramidal rigidity, asymmetric motor disturbances (jerking movements of limbs), and sensory cortical dysfunction are features found in what disease? |
|
Definition
| corticobasal degeneration |
|
|
Term
| frontotemporal dementia + Parkinson's typically involves what gene mutation? |
|
Definition
|
|
Term
| What is Biswanger Disease? |
|
Definition
| subcortical matter infarcts leading to dementia |
|
|
Term
| What drug features parkinonism like effects as an adverse effect? |
|
Definition
|
|
Term
| Multiple system atrophy is characterized by the presence of what? |
|
Definition
| glial cytoplasmic inclusions |
|
|
Term
| Autonomic dysfunction in Multiple System Atrophy is also known as what? |
|
Definition
|
|
Term
| What three areas are affected by Friedrich Ataxia? |
|
Definition
| superior cerebellar vermis (Purkinje cells), spinal cord (Clarke column), dentate nucleus (CN nuclei VIII, X, and XII) |
|
|
Term
| Bulbospinal Atrophy (Kennedy Syndrome) is linked with what gene defect? |
|
Definition
| Mutation leading to expansion of CAG/polyglutamine repeat in the androgen receptor |
|
|
Term
| 5-10% of familial cases of ALS relate to what mutation? |
|
Definition
| copper-zinc superoxide dismutase gene on Chromosome 21 |
|
|
Term
| Besides occipital encephalocele in Meckel-Gruber syndrome, what other conditions do we commonly find? |
|
Definition
| polydactylyl, polycystic kidneys, hepatic fibrosis with bile duct proliferation |
|
|
Term
| Polymicrogyria is typically associated with what 3 primary pathologies? |
|
Definition
| intrauterine infection (CMV), intrauterine hypoperfusion,and metabolic disorders (Zellwegger syndrome) |
|
|
Term
| In Type I lissencephaly, how many cortical layers are there? |
|
Definition
|
|
Term
| XLIS gene mutation encoding doublecortin leads to what pathology in women? |
|
Definition
|
|
Term
| ARX gene mutation in males produces what two primary signs? |
|
Definition
| type I lissencephaly and ambiguous genitalia |
|
|
Term
| In type III, what 3 organ-system functions are compromised, primariliy? |
|
Definition
| cerebellar, ocular, and muscular |
|
|
Term
| What is the etiology of Type II lissencephaly? |
|
Definition
| defect in O-mannosylation |
|
|
Term
| What is subcortical band heterotopia, and in which individuals is it commonly found? |
|
Definition
| masses of gray matter between the ventricular surface and the underlying cortex/ commonly found in epileptic individuals |
|
|
Term
| What two conditions are commonly associated with holoprosencephaly? |
|
Definition
| trisomy 13 and maternal diabetes |
|
|
Term
| Defect in what protein synthesized by the notochord and neural plate can lead to holoprosencephaly? |
|
Definition
|
|
Term
| An Arnold Chiari Malformation (Chiari Type II) leads to the displacement of what into the upper cervical canal? |
|
Definition
| The superior cerebellar vermis |
|
|
Term
| What other condition is almost always present in a Arnold Chiari malformation? |
|
Definition
|
|
Term
| In a Dandy Walker Malformation, what structure is typically absent? |
|
Definition
| the cereballar vermis as the 4th ventricle becomes dilated |
|
|
Term
| The maternal use of what during pregnancy may be associated with a dandy walker malformation? |
|
Definition
|
|
Term
| What type of cerebral hemorrhage is most common in the subependymal/intraventricular region? When it does occur? |
|
Definition
| germinal matrix hemorrhage/ between 5 and 35 hours after birth |
|
|
Term
| Cerebral hemisphere hemorrhage indicates what? |
|
Definition
| mechanical trauma (child abuse) |
|
|
Term
| What type of white matter infarct develops through the entire matter? |
|
Definition
| perinatal telencephalic encephalopathy |
|
|
Term
| What are the symptoms commonly found in periventricular leukomalacia/infarct? |
|
Definition
| lethargy/inactivity, hyperactivity, weak cry/oor suck, INCOMPLETE MORO REFLEX |
|
|
Term
| Where is ulegyria typically located? |
|
Definition
| in the arterial border zones |
|
|
Term
| What is stratus marmoratus? |
|
Definition
| abnormally myelinated scars in the basal ganglia/thalamus |
|
|
Term
| Survivors of kernicterus often develop what conditions? |
|
Definition
| choreoathetosis, ataxia, mental deficiency |
|
|
Term
| Where are the common sites of cerebral thrombosis? |
|
Definition
| carotid bifurcation, origin of the MCA, ends of the basilar artery |
|
|
Term
| In an acute infarct, what is the appearance of the cerebral tissue? |
|
Definition
| soft and edematous, and the corticomedullary junction becomes indistinct |
|
|
Term
| In a subacute infarct, what cells are predominantly found in the gelatinous, friable tissue? |
|
Definition
| macrophages,reactive astrocytes |
|
|
Term
| What fact distinguishes cavernous angiomas from capillary telangiectasias? |
|
Definition
| In telangiectasias, there is intervening brain tissue, which is not there in cavernous angiomas |
|
|
Term
| Patients with CADASIL often present how, clinically? |
|
Definition
|
|
Term
| Histological examination of CADASIL reveals what about the white matter and the arteries? |
|
Definition
| there is concentric thickening of the media and adventitia of white matter and leptomeningeal arteries |
|
|
Term
| What is the term used to refer to abnormal positions, shapes, or positions due to mechanical forces acting on the cells? |
|
Definition
|
|
Term
| When does anencephaly typically develop and how it can be detected? |
|
Definition
| day 28 of gestation/ screen maternal blood for elevated α-fetoprotein |
|
|
Term
| Describe the appearance of the area cerebrovasculosa? |
|
Definition
| flattened remnant of disorganized brain tissue with admixed ependyma, chroid plexus, and meningothelial cells |
|
|
Term
| What happens to the calvarium in anencephaly? |
|
Definition
| it is hypoplastic or absent, and the base of the skull is thickened |
|
|
Term
| When is a saccular aneurysm most likely to occur? |
|
Definition
| in the 5th decade of life |
|
|
Term
| What are the two most common predisposing factors to saccular aneurysms? |
|
Definition
|
|
Term
| Name the struture- flattened remnant of disorganized brain tissue with admixed ependyma, choroid plexus, and meningothelial cells |
|
Definition
|
|
Term
| Myelomeningocele typically occurs where and is associated with what other defect? |
|
Definition
| lumbosacral region/ Chiari II malformation |
|
|
Term
| What's the etiology of the Type II Lissencephaly? |
|
Definition
| defect in O-mannosylation--> cerebellar and ocular abnormalities/ congenital muscular dystrophy |
|
|
Term
| What is the etiology of holoprosencephaly? |
|
Definition
| mutation in the Sonic Hedgehog gene |
|
|
Term
| Holoprosencephaly is associated with what two conditions? |
|
Definition
| maternal diabetes and trisomy 13 |
|
|
Term
| What is a risk factor associated with chiari II formation? |
|
Definition
| maternal vitamin A deficiency |
|
|
Term
What disease has these characteristics? What drug must not be used during pregnancy?
•Cystic dilatation of the 4th ventricle
•Abnormalities of the cerebellar vermis ranging from hypoplasia to aplasia
•Enlargement of the posterior fossa
•Elevation of the tentorium & transverse sinus
•Hydrocephalus
|
|
Definition
| Dandy Walker formation/ isotretinoin use during pregnancy |
|
|
Term
| What are some of the common pathological findings in periventricular leukomalacia? |
|
Definition
| sharp, circumscribed, yellowish or chalky lesions adjacent to lateral ventricles/ coagulative necrosis followed by macrophage infiltration and caviation/ swollen and mineralized axons |
|
|
Term
| In perinatal telencephalic leukoencephalopathy, what happens to the cells in the white matter? |
|
Definition
| The astrocytes become hypertrophic and the glial nuclei become pyknotic |
|
|
Term
| What is status marmoratus? |
|
Definition
| Abnormally myelinated scars in basal ganglia/thalamus |
|
|
Term
|
Definition
| When the depths of sulci become reduces, and the gyri become gliotic and thinned out due to ischemic injury |
|
|
Term
| In an acute infarct, what does the tissue feel like? |
|
Definition
edematous/ filled with red neurons and neutrophils
|
|
|
Term
| What are the three populations of neurons most susceptible to diffuse hypoxic/ischemic encephalopathy? |
|
Definition
| CA1 region of hippocampus (Sommer sector); Purkinje cells of cerebellum; Pyramidal neurons in neocortex (layers III and V) |
|
|
Term
| Hemorrhagic infarctions are typically associated with what kind of events? |
|
Definition
| embolic events- hemorrhage presumed to be secondary to reperfusion of the damaged vessels and tissue/ either through collaterals or directly after dissolution of intravascular occlusive material |
|
|
Term
| The icelandic type of familial cerebral amyloid angiopathy is due to what genetic defect? |
|
Definition
|
|
Term
| What is the Dutch type of familial cerebral amyloid angiopathy likely to due? |
|
Definition
| mutation in amyloid precursor protein |
|
|
Term
| What distinguishes a AVM from a capillary hemangioma? |
|
Definition
| the former's vascular channels are separated by gliotic brain/ the latter's aren't |
|
|
Term
| What are the two most common sites of extrapontine myelinolysis? |
|
Definition
| cerebellum and lateral geniculate (best identified with diffusion-weighted imaging studies) |
|
|
Term
| Where are the Gaucher cells commonly found in the CNS? |
|
Definition
| in the perivascular spaces |
|
|
Term
| What's the appearance of the Gaucher cell on histopathological examination? |
|
Definition
| like wrinkled tissue paper |
|
|
Term
| What unique histologic feature is found in mucopolysaccharidoses? |
|
Definition
| membrane-bound collections of lipid lamellae- zebra bodies |
|
|
Term
| What is the predominant end-game for patients with Batten disease or neuronal ceroid lipofuscinosis? |
|
Definition
| neurologic dysfunction + blindness |
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