CNS + Eyes Flashcards
(287 cards)
What is medulloblastoma?
Medulloblastoma is a malignant brain tumor that occurs predominantly in children and exclusively in the cerebellum.
What markers are nearly always expressed in medulloblastoma?
Neuronal and glial markers are nearly always expressed in medulloblastoma.
What is the prognosis for untreated patients with medulloblastoma?
The prognosis for untreated patients is dismal; however, medulloblastoma is exquisitely radiosensitive.
What are primitive neuroectodermal tumors (PNETs), and how are they related to medulloblastoma?
Tumors of similar histologic type and a poor degree of differentiation found elsewhere in the nervous system are called primitive neuroectodermal tumors (PNETs), which are related to medulloblastoma.
Describe the morphology of medulloblastoma.
Medulloblastomas are extremely cellular, with sheets of anaplastic (“small blue”) cells. Individual tumor cells are small, with little cytoplasm and hyperchromatic nuclei.
Focal neuronal differentiation may be seen in the form of the Homer Wright or neuroblastic rosette.
What are some clinical features associated with medulloblastoma?
Tumors with MYC amplifications are associated with poor outcomes, while those linked with mutations in genes of the WNT signaling pathway have a more favorable course.
Many tumors also have mutations that activate the sonic hedgehog (shh) pathway, which plays a critical role in tumorigenesis.
T167 What is cerebral edema?
Cerebral edema is the accumulation of excess fluid within the brain parenchyma, resulting in widened gyri, narrowing of sulci, and compression of ventricles. It can be focal or diffuse.
What are the two types of cerebral edema that often occur together, particularly after injury?
The two types of cerebral edema are vasogenic edema and cytotoxic edema.
Describe vasogenic edema
Vasogenic edema occurs when the integrity of the normal blood-brain barrier (BBB) is disrupted, allowing fluid to shift from the vascular compartment into the extracellular spaces of the brain.
It can be either generalized or localized and is often associated with focal lesions such as primary metastatic tumors or abscesses.
Describe cytotoxic edema
Cytotoxic edema involves an increase in the intracellular fluid, secondary to neuronal and glial cell membrane injury, such as that following a generalized hypoxic-ischemic insult or exposure to certain toxins.
What is interstitial edema?
Interstitial edema results from increased intracerebral influx of cerebrospinal fluid (CSF) through the ependymal lining, leading to fluid accumulation in the periventricular white matter. It is typically a complication of hydrocephalus.
How does the brain appear macroscopically in generalized cerebral edema?
In generalized edema, the gyri are flattened, the sulci are narrowed, and the ventricular cavities are compressed, giving the brain a softer appearance that seems to “overfill” the cranial vault.
What is intracranial pressure (ICP)?
Intracranial pressure is the pressure exerted inside the skull, which can increase due to various factors and may result in or from brain injury.
What are some causes of increased intracranial pressure?
Some causes of increased intracranial pressure include:
a. Rise in pressure of the cerebrospinal fluid (CSF), such as in increased CSF volume or meningitis.
b. Tumors.
c. Hydrocephalus.
d. The added mass of epidural, subdural, and intracranial hematomas.
e. Cerebral edema, which can develop around large contusions, from diffuse vascular injury, or as a result of hypoxic-ischemic encephalopathy (HIE).
How can increases in intracranial pressure damage the brain?
Increases in intracranial pressure can damage the brain by:
Decreasing perfusion, which can lead to the collapse of brain capillaries and result in global ischemia.
Displacing tissue across dural barriers inside the skull or through openings in the skull, known as herniation.
What is herniation?
Herniation is the displacement of brain tissue between the dural barriers or out of the skull, occurring when the volume inside the skull increases beyond the limit permitted by compression of veins and displacement of cerebrospinal fluid (CSF) due to increased intracranial pressure.
What are the main types of herniation?
Subfalcine, Transtentorial (uncinate) herniation, Tonsillar herniation, Retrograde transtentorial herniation., Herniation out of the skull, not through the foramen magnum.
Subfalcine (cingulate) herniation
Unilateral or asymmetric expansion of a cerebral hemisphere displaces the cingulate gyrus under the edge of the falx, potentially compressing branches of the anterior cerebral artery.
Transtentorial (uncinate) herniation
The medial aspect of the temporal lobe is compressed against the free margin of the tentorium,
leading to compression of the third cranial nerve (resulting in pupillary dilation and impairment of ocular movements), compression of the posterior cerebral artery (resulting in ischemic injury to the primary visual cortex), and potentially ipsilateral hemiparesis if the contralateral cerebral peduncle is compressed against the tentorium.
Tonsillar herniation
Displacement of the cerebellar tonsils through the foramen magnum, causing compression of vital respiratory and cardiac centers in the medulla and presenting a life-threatening situation.
What is hydrocephalus?
Hydrocephalus is the accumulation of excessive cerebrospinal fluid (CSF) within the ventricles of the brain.
What are the main reasons for hydrocephalus?
Impaired CSF flow.
Impaired CSF resorption.
Rarely, overproduction of CSF (e.g., tumor of the choroid plexus).
What are the two types of hydrocephalus?
- Non-communicating hydrocephalus: Caused by CSF flow obstruction, resulting in enlargement of a portion of the ventricles while the remainder doesn’t. Mainly due to obstruction of the foramen of Monro or compression of the cerebral aqueduct.
- Communicating hydrocephalus: Caused by impaired CSF reabsorption in the absence of CSF flow obstruction between the ventricles and the subarachnoid space, leading to enlargement of the entire ventricular system.
What is the difference in presentation between hydrocephalus developing in infancy and that developing after cranial suture closure?
Hydrocephalus developing in infancy before the closure of cranial sutures is associated with enlargement of the head. In contrast, hydrocephalus developing after fusion of the sutures is associated with expansion of the ventricles and increased intracranial pressure, without a change in head circumference.