XXIII - The Nervous System Flashcards
These are infiltrative tumors that form gelatinous, gray masses, and may show cysts, focal hemorrhage, and calcification. On microscopic examination, the tumor is composed of sheets of regular cells with spherical nuclei containing finely granular chromatin (similar to normal oligodendrocytes) surrounded by a clear halo of cytoplasm.
Oligodendrogliomas(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 883
Blood vessel involved in epidural hematoma.
Middle meningeal artery(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870
In the immunosuprressed individual, the most common pattern of involvement in viral meningitis is:
Subacute encephalitis(TOPNOTCH)
Morphology: a neovascular membrane is positioned between the retinal pigment epithelium and Bruchs membrane
Age related macular degeneration (TOPNOTCH)
Flexner Wintersteine Rosettes
Retinoblastoma (TOPNOTCH)
This type of keratopathy develops in patients who are exposed chronically to high levels of ultraviolet light
Actinic band keratopathy (TOPNOTCH)
A 45 year old actor begins to experience hand tremors, and eventually develops a shuffling gait, a stooped posture, and diminished facial expressions. His mental faculties are intact. What is the expected histologic change in his brain? (A) loss of pigmented catecholaminergic neurons in his midbrain (B) loss of neurons in the striatum (C) foci of hemorrhage and necrosis in the mamillary bodies (D) multiple Lewy bodies in his cerebral cortex
loss of pigmented catecholaminergic neurons in his midbrain (pallor of substantia nigra in Parkinsonism) (B - Huntington disease; C - Wernicke encephalopathy; D - Lewy body dementia/disease) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893-894
Patient X who had a moderate traumatic injury presented with lethargy. 12 hours later it he progressed rapidly to having dilated pupils and impairment of ocular movements. This is most likely due to:
Transtentorial (uncinate) herniation/CN III compression (TOPNOTCH)
Grotton lesions (scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees) are seen in what disease entity?
Dermatomyositis (TOPNOTCH)
Morphology: histologic appearance similar to anaplastic astrocytoma with the additional feature of necrosis and vascular or endothelial cell proliferation
Glioblastoma multiforme(TOPNOTCH)
Subfalcine(cingulate) herniation leads to compression of what blood vessel?
Anterior cerebral artery(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1255
A 26 year old backpacker presents with seizures. On imaging, there are four 1-2 cm cysts in his cerebral cortex. He undergoes surgery, where two were removed. Histopathology showed an ovoid structure with a tegument, underlying smooth muscle, three oval suckers, all surrounded by an intense inflammmatory infiltrate of plasma cells, lymphocytes, and eosinophils. The surrounding brain tissue is gliotic. The patient acquired this infection by ingesting (A) raw pork (B) raw beef (C) ova from cat litter (D) raw freshwater crabs
raw pork (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880
This lesion caused by JC virus, consist of patches of irregular, ill-defined destruction of the white matter that enlarge. Each lesion is an area of demyelination, in the center of which are scattered lipid-laden macrophages and a reduced number of axons.
Progressive Multifocal Leukoencephalopathy (PML) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877
Macular star
Malignant hypertension (TOPNOTCH)
A form of neurosyphilis, resulting from damage to the sensory nerves in the dorsal roots producing impaired joint position sense and resultant ataxia, loss of pain sensation, leading to skin and “Charcot joints”. There is loss of both axons and myelin in the dorsal roots, with pallor and atrophy in the dorsal columns of the spinal cord.
Tabes dorsalis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 875
What is the most common cause of dementia in the elderly?
Alzheimer Disease(TOPNOTCH)
A rapidly progressive encephalitis, cerebral malaria, is the complication with the highest mortality caused by what etiologic agent?
Plasmodium falciparum(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 1280
Pathologic hallmarks of Alzheimer disease
Plaques and tangles(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1288
Neurofibrillary tangles are bundles of filaments in the cytoplasm of the neurons that displace or encircle the nucleus. What is the major component of this structure?
Tau proteins(TOPNOTCH)
In patients with peripheral neuropathy, what is the predominant histologic findings?
Axonal neuropathy (TOPNOTCH)
Gross morphology: gyri are flattened, intervening sulci are narrowed, and the ventricular cavities are compressed
Generalized cerebral edema(TOPNOTCH)
It is the most common cause of dementia in the elderly, characterized by presence of plaques and neurofibrillary tangles, composed of tau proteins.
Alzheimer’s disease(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893
An autosomal dominant syndrome characterized by the development of hamartomas and benign neoplasms involving the brain and other tissues. The lesions are firm areas of the cortex that, in contrast to the softer adjacent cortex, have been likened to potatoes, hence the appellation “tubers.”
Tuberous Sclerosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901
The pathognomonic finding of this disease is a spongiform transformation of the cerebral cortex and deep gray matter structures (caudate, putamen); consists of a multifocal process that results in the uneven formation of small, empty, microscopic vacuoles of varying sizes within the neuropil and sometimes in the perikaryon of neurons.
Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880
Morphology: composed of bipolar cells with long, thin “hairlike” processes that are GFAP- Positive; Rosenthal fibers, eosinophilic granular bodies, and microcysts are often present.
Pilocytic Astrocytoma (TOPNOTCH)
These are round, faintly basophilic, PAS-positive, concentrically lamellated aggregates of polyglucosans that range between 5 and 50 micrometers, and are located wherever there are astrocytic end processes, especially in the subpial and perivascular zones, seen more frequently with advancing age.
Corpora amylacea (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
It is the accumulation of excess fluid within the brain parenchyma. The brain is softer than normal and often appears to “overfill” the cranial vault. In generalized edema the gyri are flattened, the intervening sulci are narrowed, and the ventricular cavities are compressed.
Cerebral Edema (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
It produces brain abscess, most ofen in the cerebral cortex and deep gray nuclei, central necrosis, petechial hemorrhages surrounded by acute and chronic inflammation, macrophage infiltration, and vascular proliferation; CT and MRI studies may show multiple ring enhancing lesions.
Toxoplasmosis of the CNS(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1280
Minute aneurysms in vessels that are less than 300 micrometers in diameter, associated with chronic hypertension.
Charcot-Bouchard microaneurysms (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868
This occurs when the integrity of the normal blood-brain barrier is disrupted. With increased vascular permeability, fluid shifts from the vascular compartment into the intercellular spaces of the brain.
Vasogenic edema (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
The abnormalities are predominantly in the cerebellum,with loss of Purkinje and granule cells; there is also degeneration of the dorsal columns, spinocerebellar tracts and anterior horn cells and a peripheral neuropathy
Ataxia Telangiectasia(TOPNOTCH)
Neurotic plaques are focal, spherical collection of dilated, tortuous, silver staining neuritic processes often around a central amyloid core, which may be surrounded by clear halo, and can be stained with Congo red. What is the most predominant component of this plaque core?
AB peptide(TOPNOTCH)
Syndrome caused by deletions in mitochondrial DNA, characterized by ophthalmoplegia, pigmentary degeneratation of the retina, and complete heart block
Kearns-Sayre syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1245
In the setting of global ischemiaThis is characterized by acute neuronal cell damage
Early change(TOPNOTCH)
An autoimmune demyelinating disorder characterized by distinct episodes of neurologic deficits, separated in time, attributable to white matter lesions that are separated in space. Affected areas show multiple, well-circumscribed, slightly depressed, glassy, gray-tan, irregularly shaped lesions, termed “plaques”.
Multiple Sclerosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 887
Gross morphology: the brain shows pronounced, frequently asymmetric, atrophy of the frontal and temporal lobes with conspicuous sparing of the posterior two thirds of the superior temporal gyrus
Pick Disease(TOPNOTCH)
After a vehicular crash, patient presented with quadriplegia and respiratory distress. Spinal cord injury was suspected. This can be due to damage to what level of vertebra?
Above C4(TOPNOTCH)
What is the most common intraocular malignancy in adults?
Metastasis to the uvea, typically to the choroid (TOPNOTCH)
This is an inflammatory disorder that involves multiple small to medium-sized parenchymal and subarachnoid vessels and is characterized by chronic inflammation, multinucleated giant cells and destruction of the vessel wall. Affected individuals manifest a diffuse encephalopathic clinical picture, often with cognitive dysfunction; improvement occurs with steroid and immunosuppressive treatment.
Primary angiitis of the CNS (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868
Morphology: In later stages of this disease, all muscles eventually become almost totally replaced by fat and connective tissue
Duchenne Muscle Dystrophy (TOPNOTCH)
Morphology: the anterior roots of the spinal cord are thin; the precentral gyrus may be atrophic and demonstrates a reduction the number of anterior horn neurons throughout the length of the spinal cord with associated reactive gliosis. Remaining neurons often contain Bunina bdoies and PAS positive cytoplasmic inclusions.
Amyotorphic Lateral Sclerosis(TOPNOTCH)
A type of vascular malformation which are characterized by of ectatic venous channels.
Venous angiomas (varices) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868
What is the histologic pathology that is usually seen in Duchene muscle dystrophy and is rare in Becker Muscle Dystrophy?
Enlarged, rounded, hyaline fibers that have lost their normal cross striation, believed to be hypercontracted fibers (TOPNOTCH)
Most common cause of bacterial meningitis in adolescents and young adults.
Neisseria meningitidis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1272
Most serious complications of chronic TB meningitis
Arachnoid fibrosis(producing hydrocephalus) and Obliterative endarteritis(causing arterial occlusion and infarction)(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1274
Cytoid bodies
Hypertension (TOPNOTCH)
Morphology: Perifascicular Atrophy
Dermatomyositis (TOPNOTCH)
Characterized by “flapping” tremor, depressed levels of consciousness, which may lead to coma. Result of decreased hepatic function.
Hepatic encephalopathy (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 891
Cells which produce myelin in the CNS.
Oligodendrocytes (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
The spinal cord shows loss of axons and gliosis in the posterior columns, the distal portions of the corticospinal tracts, and the spinocerebellar tracts.
Friedreich Ataxia(TOPNOTCH)
Gross morphology: the brain is small and shows striking atrophy of the caudate nucleus and the putamen. The globus pallidus may be atrophied secondarily, and the lateral and third ventricles are dilated.
Huntington Disease(TOPNOTCH)
Presents with generalized symptoms of headache, malaise, mental confusion, and vomiting. The subarachnoid space contains a gelatinous or fibrinous exudate, most often at the base of the brain, obliterating the cisterns and encasing cranial nerves. There may be discrete white granules scattered over the leptomeninges. Obliterative endarteritis may be seen.
Tuberculous meningitis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874
This refers to dilation of the ventricular system with a compensatory increase in CSF volume secondary to a loss of brain parenchyma, as may occur after infarcts or with a degenerative disease.
Hydrocephalus ex vacuo (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862
Morphology: endoneurial arterioles show thickening, hyalinization, and intense PAS positivity in their walls and extensive reduplicaton of the basement membrane.
Peripheral Neuropathy in DM Type 2 (TOPNOTCH)
CSF findings in tuberculous meningitis.
Moderate pleiocytosis - predominantly monocytesprotein - elevatedglucose - moderately reduced/normal(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874
Caused by invasion of the brain by Treponema pallidum and manifests as insidious but progressive loss of mental and physical functions with mood alterations (including delusions of grandeur), terminating in severe dementia.
Paretic neurosyphilis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874
These are firm areas of the cortex that, in contrast to the softer adjacent cortex, have been likened to potatoes and are composed of haphazardly arranged neurons that lack the normal laminar organization of neocortex.
Cortical hamartomas or “tubers’ of Tuberous Sclerosis (TOPNOTCH)
A 19 year old male presents with fever and changes in mood and behavior. His neck is supple, and shows no lateralizing signs, or cranial nerve defects. Memory is impaired. A lumbar tap is performed with a slight lymphocytosis and protein elevation. Bacterial cultures were negative. On PCR, herpes simplex virus I was detected. THe histologic changes of necrotizing inflammation and Cowdry Type A bodies would most likely be found in (A) temporal lobes (B) occipital lobes (C) cerebellar vermis (D) parietal lobes
temporal lobes (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876
Pattern of necrosis seen in nonhemorrhagic infarcts of the brain.
Liquefaction necrosis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863
This is due to an increase in intracellular fluid secondary to neuronal, glial, or endothelial cell membrane injury, as might be encountered in an individual with a generalized hypoxic/ischemic insult or with exposure to some toxins.
Cytotoxic edema(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
Morphology: foci of hemorrhage and necrosis, particularly in the mammillary bodies but also adjacent to the ventricle, especially the third and fourth ventricles
Wernicke encephalopathy(TOPNOTCH)
Cells which line the ventricles, and are located in the region of the obliterated central canal of the spinal cord.
Ependymal cells (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
Antoni A and Antoni B patterns of growth are seen in what tumors of the CNS?
Schwannoma(TOPNOTCH)
Morphology: wide asymmetric distribution of axonal swellings that appear within hours of the injury and are best demonstrated with silver impregnation techniques or with immunoperoxidase stains for AB protein
Diffuse axonal injury(TOPNOTCH)
A 12 year old student presents with lethargy, fever, and headaches. These progress to irritability, anxiety, and confusion, with difficulty in swallowing. He refuses any drink being offered. A few days later, he slips into a coma, and dies. On history, he was bitten by a stray dog 4 months ago. At autopsy, where would the characteristic infected neurons with cytoplasmic inclusions most likely be found? (A) frontal and parietal cerebral cortex (B) hippocampus and cerebellum (C) globus pallidus and medulla (D) pineal gland and pituitary
hippocampus and cerebellum (TOPNOTCH) Robbins Pathologic Basis of Disease 8th ed
Morphology: pathognomonic finding is a spongiform transformation of the cerebral cortex
Creutzfeldt-Jakob Disease(CJD)(TOPNOTCH)
Morphology: diffuse loss of ganglion cells and thinning of the retinal nerve fiber layer, in advanced cases, the optic nerve is both cupped and atrophic
Glaucomatous optic nerve damage (TOPNOTCH)
Patients with this type of intracranial hemorrhage complains of having “the worst headache I’ve ever had”/ thunderclap headache.
Subarachnoid Hemorrhage (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 866
Lesion of what area of the brain appear to be the best correlate of the memory disturbance and confabulation seen in Korsakoff syndrome?
Medial dorsal nucleus of the thalamus(TOPNOTCH)
Morphology: characterized by diffuse granulomatous inflammation of the urea. Plasma cells are typically absent, but eosiophils may be identified in the infiltrate
Sympathetic ophthalmia (TOPNOTCH)
A syndrome of sudden, deep intracerebral hemorrhage that follows even minor head trauma by an interval of 1-2 weeks
Spat-apoplexie (Delayed post traumatic hemorrhage) (TOPNOTCH)
What is known to be a reliable histological marker of diabetes mellitus in the eye?
Thickening of the BM of the epithelium of the pars plicata of the ciliary body (TOPNOTCH)
Morphology: perivascular accumulation or lymphocytes and/or neutrophils, multiple foci of necrosis of gray and white matter, single-cell neuronal necrosis with phagocytosis of debris, microglial nodules
Viral Encephalitis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1275
These are bone marrow-derived cells that function as the phagocytes of the CNS.
Microglia (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861
The most common prion disease that manifest clinically as a rapidly progressive dementia.
Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1283
This general reaction of the motor unit occurs when there is dysfunction of the Schwann cell or damage to the myelin sheath; there is no primary abnormality of the axon
Segmental demyelination (TOPNOTCH)
Transtentoria(uncinate) herniation compress what structures?
CN III and Posterior Cerebral artery(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1255
Consists of low-lying cerebellar tonsils which extend through the foramen magnum at the base of the skull. This can lead to obstruction of CSF flow and compression of the medulla, resulting in symptoms of headache or cranial nerve deficits.
Arnold-Chiari malformation type I(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872
Herniation of the meninges through a defect in the vertebral column.
Meningocoele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872
What are the two most common primary tumors of the optic nerve?
Glioma and Meningioma (TOPNOTCH)
This is a clinical syndrome characterized by masked facies, stooped posture, slowness of voluntary movement, festinating gait rigidity, and a “pill-rolling” tremor. There are single or multiple, intracytoplasmic, eosinophilic, round to elongated inclusions that often have a dense core surrounded by a pale halo or “Lewy bodies”.
Parkinson disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 894
The fundamental abnormality in this disease is the accumulation of A-beta and tau in specific brain regions.
Alzheimers Disease(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1288
This is a diverticulum of malformed CNS tissue extending through a defect in the cranium. It most often involves the occipital region or the posterior fossa.
Encephalocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872