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73 Cards in this Set

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Osteochondroma
Benign tumor containing both bone and cartilage, and usually occuring near the end of a long bone (it is a developmental defect). The most common benign bone tumor. Each tumor is covered by a cartilaginous cap. Develops osteocartilagenous exostoses (spurs or bony outgrowths from the bone) at metaphyses. Peak 10-20 years old. Increased risk of developing chondrosarcoma, a malignant cartilage cancer.
Enchondroma
Tumor consisting of cartilaginous tissue, especially arising where cartilage does not normally exist; the cartilage grows within and inside bone. Increased risk of developing chondrosarcoma, a malignant cartilage cancer.
Chondroblastoma
Tumor of cartilage producing cells, rare, peak: 10-20 years old; occurs in femur, tibia, humerus epiphyses.
Chondrosarcoma
Sarcoma containing cartilage, malignant, occurs in spine and pelvic bones. Slower growing than osteosarcoma
Benign tumor: Osteoma
Usually multiple, painless. Occurs primarly in skull and facial bones, forming projections from those bones.
Benign tumor: Osteoid Osteoma
Small, painful. Occurs especially in the extremities of teens and young adults.
Benign tumor: Osteoblastoma
Larger, milder pain - achy, dull. Occurs primarily in the spone.
Malignant bone tumors: Osteosarcoma
Occurs in any bone. Painful and progressively enlarging mass. 1st symptom is a pathologic fracture (fracture with no trauma). Is both lytic and blastic. May form a Codman's triangle - a triangular, right angled mass representing tumor growth that has been broken through the cortex and lifted the periosteum, yielding reactive bone formation under the periosteum in that area. Bimodal peaks: most common in those <20 years old and the elderly.
Malignant bone tumors: Ewing's sarcoma
Peak 10-15 years old. Tumor arises in the medullary cavity and invads the cortex and periosteum, producing a soft tissue mass. + Homer-Wright rosettes on histology. Usually occurs in the diaphyses of long bones, especially the femur, and the flat bones of the pelvis. Painful, surrounding area is tender and swollen; also there is frequently fever, anemia, leukocytosis. Radiologically: destructive lytic lesion. The tumor causes a periosteal reaction that produces layer upon layer of reactive bone: "onion skin"
Muscular dystrophies all yield....
all yield elevated creatine kinase.
Duchenne muscular dystrophy
due to lack of dystrophin. Dystrophin is necessary for normal skeletal muscle function; without it, skeletal muscle atrophies and dies. pectoral muscle weakening occurs first. Onset 2-3 years old. Later: pelvic girdle weakness. Wheelchair bound by 12 years old. Delayed motor milestones, cognitive impairment. +Gower's sign, hypertrophied calves. Deaht usually occurs in the 20s, often due to pneumonia.
Becker's
Initially not as severe as Duchenne's, but has the same outcome which onloy is delayed as compared to Duchenne's. Due to decreased amount of dystrophin. Onset @ 5-15 years old and ambulatory beyond age 15.
Limb Girdle
AR, proximal hip an dshoulder girdle weakness, very heterogenous.
Facioscapulohumeral
AD, onset in late childhood/adolescence; face and should girlde weakness +/- cardiac defects, mental retardation.
Myotonic
AD, onset at any age - patients are born with a long face, produces inability to voluntarily relax muscles after forceful contraction, with primarily distal weakness, also causes temporal and masseter muscle wasting, ptosis, hypersomnolence, formation of cataracts, premature frontal balding, atrophy of gonads with infertility, MR, hyperglycemia, cardiac arrythmias.
Brain tumors
Patients with brain tumors frequently present with headaches, particularly postional headaches. Focal neurologic deficits of a body part may also result, depending upon the location of the tumor. New onset seizures are not common.
Neural Tube Derived Brain tumors: Gliomas
Formed from tissue that has arisen from the neural tube. All affect males more than females, except for oligodenodroblastomas which affect males and females equally.
Neural Tube Derived Brain tumors: Gliomas - Fibrillary astrocytoma
Represent 80% of all adult primary brain tumors. Usually in the cerebral hemispheres. Comprised of neopalstic astrocytes. 3 Types of fibrillary astrocytomas.
3 types of fibrillary astrocytomas: Low grade astrocytoma
Remain static or progress very slowly for a number of years, then the patient enters a period of rapid deterioration
fibrillary astrocytomas: anaplastic astrocytoma
more aggressive
fibrillary astrocytomas: glioblastoma multiforme
extremely high grade: hightly anaplastic tumor cells with increased nuclear density line up along the edges fo necrotic regions --> pseudopalisading. Usually in both cerebral hemispheres, forming a butterfly shape. Mean survival time from diagnosis 8-10 months. Always fatal.
Neural Tube Derived Brain tumors: Gliomas - Pilocytic astrocytoma
Occurs in children and young adults. Usually occurs in the cerebellum. Usually cystic with cells that hav elong thing hair like processes. Rarely infiltrates neighboring tissues, and grows slowly.
Medulloblastoma
Most common childhood brain tumor. Occurs exclusively in the cerebellum, and occurs in the midline in children. Rapid growth of the tumor may block CSF flow, resulting in hydrocephalus. Extremely cellular, with sheets of anaplastic cells. Very pooly differentiated. Dissemination through the CSF is common. Highly malignant, and, if untreated, death will likely occur BUT it is exquisitely radiosensitive.
Oligodendrogliomas
More common in middle age. Calcifications are common. Occurs in cerebral whit ematter, has a less aggressive course. Frequently causes seizure. The oligodendroglioma histologically has cells with fried egg appearance. An oligodendroblastoma is a type of oligodendroglioma.
Ependymoma
Arises from the ependymal lining of the ventricular system. CSF dissemination is common. If occurs in childhood or teen years, typically occurs in the 4th bentricle. If it occurs later in life, the spinal cord is the most common site. The tumor cells may form "ependymal" rosettes or "perivascular pseudorosettes".
Neural Crest Derived Brain Tumors
Arise in tissues derived from the neural crest. Occur more frequentl in females, with the exception of neurofibromas.
Neural Crest Derived Brain Tumors: Meningioma
Predominantly a benign tumor of adults. Often associated with NF2 gene mutation. Comprised of encapsulated, well defined dural masses. Often cause damamge by compressing underlying brain tissue. Histologically, cells have a 'whirling pattern' and psamoma bodies.
Neural Crest Derived Brain Tumors: Schwannoma
Also known as neurilemmoma or acoustic neuroma. Benign. Most commonly associated with the vestibular branch of the 8th cranial nerve at the cerebellopontine angle. Well circumscribed and encapsulated. Axons are excluded from the tumor; byt they may be entrapped within it. Occurs within the confines of the dura, but is comprised of Schwann cells (myelin cells of peripheral nervous system). Associated with neurofibromatosis II (and, therefore, with the loss of the NF2 gene)
Neural Crest Derived Brain Tumors: Neurofibroma
Comprised of connective tissue and nervous tissue; may occur within the cranial cavity along a peripheral nerve. Not encapsulated, and is invested well with and within neighboring neural tissue. Benign, associated with NF1 gene loss and with neurofibromatosis I.
Ectoderm derived brain tumors
Formed form tissue that has developed from ectoderm; by convention, these tumors do not include those that arose from CNS.
Ectoderm derived brain tumors: Craniopharyngioma
Derived from vestigial remnants of Rathke's pouch. slow-growing, may encroach on the hypothalamus, ventricles, or optic chiasm. Usually occurs during childhood and adolescence. Typically cystic with calcifications, and are comprised of a mixture of squamous epithelial cells and connective tissue. The most common supratentorial tumor o children.
Ectoderm derived brain tumors: Pituitary adenoma
Excess production of anterior pituitary hormones is usually caused by a pituitary adenoma, these hormones include prolactin, ACTH, TSH, FSH, LH, or GH. Functional adenomas are usually comprised of one cell type, so only one hormone is produced in excess.
Prolactinoma
The most common functional pituitary tumor. Produces prolactin. Most are macroadenomas, may result in amenorrhea, galactorrhea, loss of libido, infertility, and visial distrubance.
Growht hormone adenoma
Second most common functional pituitary tumor. Produces GH causing acromegaly or gigantism.
Corticotroph tumor
Usually a microadenoma. Produces excess ACTH, which causes adrenal hypersecretion of cortisol. Causes Cushing's idsease, a form of Cushing's syndrome.
Mesoderm Dervied Brain Tumors
Form form tissue derived from mesodern (and so do not represent neurologic tissue)
Mesoderm Dervied Brain Tumors: Lymphoma
Always aggressive with poor chemotherapeutic response. Affects the elderly and AIDS patients. Also more common in the immunosuppressed (in this case, all are B cell neoplasms and contain EBV). considered an AIDS-defining cancer if it occurs in the brain of an HIV+ patient.
Mesoderm Dervied Brain Tumors: Lipoma
Delicately encapsulated, usually small. comprised of mature adipose tissue, benign, but life threatening if in brain since it represents an intracranial mass.
Mesoderm Dervied Brain Tumors: Hemangioblastoma
Occurs in association with the capillaries of the cerebellum and retina. Associated with von Hippel-Lindaue disease. Benign, but life threatening since it is a brain mass.
Alzheimer's Disease
Causes dementia (progressive loss of cognition regardless of state of attention). #1 most prominent symptom: memory loss. Starts with insidious diminution of intellectual ability, accompanied with changes in modd and behavior. Later, significant disorientation and memory loss occur; end stage disease is additionally marked by incontinence, muteness, loss of learned motor skills including walking. Symptomatic course lasts approximately 10 years; death is usually via pneumonia. Risk increases with age; highest risk group: those >80 years old. The most common CNS degenerative disorder and the most common cause of dementia. Diffuse significiant cortical atrophy --> the brain looks shrunken: small with widening cerebral sulci and with enlarged ventricles due to atrophy of tissue. A marked decreased in neurons is appreciated in the nucleus basalis of Meynert. The E4allele of the apolipoprotein E (ApoE) gene on chromosome 19, if present, increases the risk for Alzheimer's and decreases the age of onset - but is NOT necessary for its development; ApoE serves to bind amyloid beta protein in plaques.
Alzheimer's and plaques
Alzheimer's disease and amyloid beta protein forms beta-pleated sheets, binds Congo red stain, relatively resistant to degradation, directly neurotoxic, stimulates protectant cells in the brain, causes inflammatory and oxidative damage resulting in death of neurons, and is a product of processed amyloid precursor protein. The gene for APP is on chromosome 21. See Senile plaques = neuritic plaques = dilated, tortuous neuritic processes containing amyloid beta protein in spheres. Neurofibrillary tangles = paired helical filaments of tau protein + amyloid beta protein in neuronal cytoplasm that replace or encircle the nucleus of neurons. +Hirano bodies = eosinophilic rods in processes of hippocampus neurons. Amyloid angiopathy = vascular wall deposition of amyloid beta protein. Primarily affects ACh - transmitting neurons. Pharmacologic management includes cholinesterase inhibitors such as donepezil, galantamin, or rivastigmine +/- an NMDA receptor antagonist.
Pick's Disease
A type of frontotemporal dementia. Female > Male. Atrophy of frontal and temporal lobes, leading initially to change in personality and language problems. Then dementia occurs. Progresses quickly, reaching advanced stage in 2-3 years.
Pick bodies
pink inclusion bodies comprised of tau protein in large ballooned cells.
Parkinson's disease
>50 years old, and risk increases with age. Loss of substantia nigra and, as a result, locus ceruleus. Associated with dopamine reduction due to loss of substantia nigra. Slowly progressive. Key features: bradykinesia, muscle rigidity, resint tremor, postural instability/gait abnormalities including festinating gait, fluctuating hallucinations, may develop dementia. First symptom: loss of diminished sense of smell. Most cases are idiopathic, other causes include: Von Economo's encephalitis, repeated trauma, MPTP. Pharmacologic management often includes an anticholinergic such as benztropine, biperiden, procyclidine, or trihexyphenidyl.
Parkinson's Disease and lewy bodies
Intracytoplasmic, eosinophilic inclusions of alpha-synuclein, found in the substantia nigra and the nucleus basalis of Meynert. They first form in the olfactory bulb and the dorsal motor nucleus of the vagus; from there, the pathology then expands into the substantia nigra. Eventually, it will extend into the cerebral cortex.
Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
Male>female, onset usually between 50-70 years old. Degeneration of upper and lowe rmotor neurons. 90-95% are sporadic, the others are familial; a very small amount are due to a genetic defect in copper-zinc superoxide dismutase.
Starts with asymmetric weakness in hands with propensity for dropping objects, along with cramping of the arms and legs. Eventually, fasciculations ocurr. Preservation of extra ocular movement, sensation, and bowel and bladder function. Death within 5 years. DOC: riluzole
ALS Features both lower and upper motor neuron signs;
Lower: symmetrical muscle atrophy and weakness, fasciculations, hyporeflexia.
Upper: hyperreflexia, spasticity, and babinski sign, loss of fine motor dexterity.
Huntington's Disease
Autosomal dominant. Progressive degeneration and atrophy of the caudate nucleus, putamen, and frontal cortex. Symptoms begin in the 30s or 40s; symptomatic course is typically 15 years. Whole body choreoathetosis, then hypertonicity, fecal and urinary incontinence, anorexia, weight loss, depression, and eventually severe dementia and deaht. Involves mutation of the HD gene which is located on chromosome 4. The mutation results in a trinucleotide repeat of CAG. Affects cholinergic and GABA-ergic neurons, primarily medium spiny striatal neurons.
Friedreich's Ataxia
Autosomal recessive. Involves chromosome 9 with a trinucleotide repeat of GAA, leading to a deficiency in fratazin. Affects males and females equally. Lower prevalence among Asians and blacks. Symptoms begin around 11 years of age. Most patients become wheel-chair bound within 5 years of disease onset. Ataxia, dysrthria, decreased DTRs, Babinski, sensory an proprioceptive loss, pes cavus, progressive kyphoscoliosis, type i diabetes, cardiomyopathy, and cardiac arrhythmias. Degeneration of posterior columns, corticospinal tract, spinocerebellar tract, cerebellum, brainstem cranial nerve nuclei VIII, X, and XII, pls degeneration of large sensory peripheral neurons, and dorsal root ganglia.
Other causes and types of dementia: Neurosyphilis
occurs in tertiary syphilis
Other causes and types of dementia: prion disease
Creutzfeld-Jacob, Kuru
Other causes and types of dementia: multi-infarct dementia
second most common form of dementia; due to long-standing hypertension, marked by step-wise decreases in cognitive function resulting from multiple small infarcts.
Other causes and types of dementia: chronic alcoholism
Causes thiamin deficiency, which can lead to dementia.
Other causes and types of dementia: Binswanger's disease
associated with long-standing hypertension, characterized by multiple lacunar infarcts and progressive demyelination of subcortical area, marked by progressively worsening confusion with concomitant mood disorder.
Other motor neuron diseases: progressive bulbar palsy
brainstem and cranial nerve degeneration, resulting in diarrhea, respiratory difficulties, and difficulty with swallowing.
Other motor neuron diseases: Werdnig-Hoffman Syndrome
Autosomal recessive. Also known asn infantile spinal muscular atrophy. manifests in infancy. Onset: birth - 4 months; deaht usually by age 3. Involves destruction of the anterior horn cells of the spinal cord; atrophic muscles develop, resulting from denervation atrophy.
Demyelinating diseases: Multiple Sclerosis
Autoimmune demyeliating disorder. Associated with HLA-DR2. Onset 20-40 yeras old. See plaques in the white matter, especially around ventricles and the optic nerve, but can occur anywhere in the CNS presence of abundant lipid-laden macrophages. There is relative preservation of the axons but not of the overlying oligodenodroctyes. Plaques are seen on imaging with MRI. See oligocloncal bands --> increased CSF immunoglobulins identified on electrophoresis. Northern European ancestry and being raised in temperate climate increase the risk. See fatigue, diplopia, visual loss, vertigo, weakness of muscles +/- paresis, retrobulbar pain, sensory distrubances, detrusor hyperreflexia, babinski sign, increased DTRs, Lhermitte's sign. Marked by distinct separation of lesions in time and space. #1 form is relapsing, remitting MS.
Charcot's triad for multiple sclerosis
nystagmus, intention tremor, scanning speech.
Treatment for MS
glatiramer, interferon beta, or natalizumab.
Debic's syndrome
Primarily occus in Asia and South America. Bilateral optic neuritis followed in days or weeks by transverse myelitis. Due to demyelinatio of the optic nerve and spinal cord. Self-limited, but sometimes heralds the onset of MS or occurs with SLE.
Guillain-Barre
Follows viral infection or immunization or campylobacter gastroenteritis. Ascending muscle weakness and paralysis +/- acsending sensory loss/change. Represents ascending demyelination of the peripheral nerous system. Albumino-cytologic dissociation occus, which is significantly increased protein concentration with only mild increase in cell count in CSF.
Adrenoleukodystrophy
X-linked inheritance. Deficiency in peroxisomal transporter enzyme, resulting in the inability to properly catabolize very long chain fatty acids. Yields extremely high levels of veyr long chain fatty acids in the serum, and the inability to use such fatty acids in lipid metabolism. Starts with ADHD, motor and sensory asymmetric neuropathy, spastic paraplegia, cortical blindness, later adrenal insufficiency. Early deaht, onset between 10-20 years of age. Poor myelin production PLUS demylination of CNS and peripheral nerves with axonal degeneration.
Schilder's disease
A variant of adrenoleukodystrophy. A rare demyelinating disease. Mimics MS. Radiologically, often shows 1-2 very large plaques of demylination in the cerebral hemispheres.
Metachromatic leukodystrophy
Autosomal recessive. Sphingolipidosis that becomes symptomatic in early childhood or adulthood. A deficiency in arylsulfatase A, leading to an accumulation of sulfatides. Leads to demyelination of CNS and peripheral nervous system. Forgetufll ness, poor job performance, progresses to personality changes, psychiatric problems, ataxia, motor problems, mask facies, strange psotures. Progresses to dementia, paralysis, and muteness. CSF contains high protein content.
Subacute combined degeneration
Due to vitamin B12 deficiency. Features demyelination and eventual destrcution of spinal cord posterior columns, and later destruction of the corticospinal tract. Distal paresthesias, then weak, unsteady gait +/- megaloblastic anemia; progresses to spastic weakness of legs and eventually, paraplegia late in the diseas. Eventually both ascending and descending tracts of axons int he spinal cord degenerate.
Progressive multifocal leukoencephalopathy
Caused by JC virus. Causes complete destruction of all oligodendrocytes. Occurs only in the context of severe immunosuppression. On MRI, shows global white matter atrophy. -CSF oligoclonal banding.
Alcohol-mediated CNS disorders: Alcohol withdrawal
may result in seizures and hallucinations
Alcohol-mediated CNS disorders: hepatic encephalopathy
pathology to brain secondary to systemic delivery of ammonia and other bowel toxins due to inefficient removal of substances by a failed liver, causes acute confusion, asterixis.
Alcohol-mediated CNS disorders: Wernicke's syndrome
Due to aucte severe B1 deficiency, such as can b einduced by giving IV glucose to an alcoholic without first giving IV thiamine. Features focal hemorrhage and necrosis of mammillary bodies. Causes ophthalmoplegia, confusion, and ataxia. Reversible with B1 administration.
Alcohol-mediated CNS disorders: Korsakoff's encephalopathy
Not reversible, anf ollows some cases of Wernicke's. Results from macrophage accumulation at hemorrhage sites that were created in ernicke's and subsequent development of open cysts in brain lined with hemosiderin-laden macrophages. Associated with severe psychosis or dementia that usually does nto respond adequately to medical management for psychosis or dementia.
Alcohol-mediated CNS disorders: Alcoholic cerebellar degeneration
The vermis atrophoies secondary to alcohol toxicity. Features ataxis of the trunk.
Alcohol-mediated CNS disorders: Alcoholic neuropathy
Due to toxic effects of alcohol, resulting in pathology, peripheral nervous system. First, vibration sense is lost. Then painful sensory distrubance occurs, sometimes accompained by motor problems.
Alcohol-mediated CNS disorders: Marchiafava-Bignami Disease
Due to ingestion of large quanitities of red wine. Features degeneration of the corpus callosum and resulting frontal lobe dementia.