Dementia and Lewy Body Quiz
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Questions and Answers

What is the primary protein involved in the formation of Lewy bodies associated with dementia with Lewy bodies (DLB)?

  • Tau
  • Beta-amyloid
  • Prion protein
  • Alpha-synuclein (correct)

Which of the following diseases is NOT classified as a Lewy body dementia?

  • Parkinson's disease dementia
  • Dementia with Lewy bodies
  • Creutzfeldt-Jakob disease (correct)
  • Vascular dementia

Which of the following statements accurately describes Creutzfeldt-Jakob disease (CJD)?

  • It exclusively affects younger individuals.
  • It is primarily caused by alpha-synuclein deposits.
  • It results from the benign form of a prion protein changing to an insoluble form. (correct)
  • It is the same as Alzheimer's disease.

Which pathological feature is prominently observed in Creutzfeldt-Jakob disease (CJD)?

<p>Vacuolation and neuronal loss (B)</p> Signup and view all the answers

What is the human equivalent of 'mad cow disease' as per the content provided?

<p>Variant Creutzfeldt-Jakob disease (vCJD) (D)</p> Signup and view all the answers

What histological feature differentiates grade II diffuse astrocytomas from grade III tumors?

<p>Ki67 proliferation index (A)</p> Signup and view all the answers

Which statistic accurately describes the occurrence of malignant transformation in choroid plexus papilloma?

<p>10-30% become histologically malignant (D)</p> Signup and view all the answers

What is the primary component of the cyst wall in a choroid plexus cyst?

<p>Cuboidal to columnar epithelium (A)</p> Signup and view all the answers

What is a defining feature of choroid plexus papillomas compared to normal choroid plexus?

<p>Crowded epithelial cells (B)</p> Signup and view all the answers

What type of tumor is most commonly found within the central nervous system?

<p>Glial tumors (D)</p> Signup and view all the answers

Which is NOT a subtype of glial tumors?

<p>Neuroblastoma (A)</p> Signup and view all the answers

How are gangliogliomas typically classified in terms of cellular composition?

<p>Mixed ganglion and glial cells (B)</p> Signup and view all the answers

What histological parameter is NOT considered significant for the WHO grading of diffuse astrocytic tumors?

<p>Presence of necrosis (C)</p> Signup and view all the answers

Which of the following characteristics is definitive for the diagnosis of ependymoma?

<p>Columnar cells arranged around a central lumen (B)</p> Signup and view all the answers

What demographic is most commonly affected by diffuse astrocytoma?

<p>Adults in their mid 30s (A)</p> Signup and view all the answers

Which imaging characteristic often indicates progression of a diffuse astrocytoma to a higher grade?

<p>Presence of contrast enhancement (A)</p> Signup and view all the answers

What is the typical histological feature observed in ependymomas?

<p>Presence of salt and pepper chromatin (B)</p> Signup and view all the answers

Which statement is true regarding the prognosis of spinal ependymomas compared to infratentorial ependymomas?

<p>Spinal ependymomas have better excision rates and prognosis (D)</p> Signup and view all the answers

What is the gross appearance of diffuse astrocytoma?

<p>Ill-defined neoplasm with blurring of gray-white junction (A)</p> Signup and view all the answers

What is the median age for diagnosis of astroblastoma?

<p>11 years (D)</p> Signup and view all the answers

Which of the following features is more prevalent in ependymoma than in glioma?

<p>Presence of ependymal rosettes (C)</p> Signup and view all the answers

Which of the following features is characteristic of the gross description of astroblastoma?

<p>Peripheral cerebral hemispheric masses (C)</p> Signup and view all the answers

What type of cell differentiation is exhibited by ependymomas?

<p>Glial and epithelial differentiation (C)</p> Signup and view all the answers

How is medulloblastoma commonly classified in terms of its grade?

<p>Grade IV (B)</p> Signup and view all the answers

What is a key microscopic feature of classic medulloblastoma?

<p>Homer Wright rosettes (B)</p> Signup and view all the answers

Which statement about astroblastoma is true regarding its prevalence?

<p>It represents less than 3% of primary brain gliomas. (D)</p> Signup and view all the answers

In which locations does medulloblastoma most frequently occur?

<p>Cerebellar parenchyma (B)</p> Signup and view all the answers

Which imaging feature is characteristic of astroblastoma when viewed through radiology?

<p>Discrete supratentorial mass with cystic change (D)</p> Signup and view all the answers

What describes the histological arrangement of cells in medulloblastoma?

<p>Syncytial arrangement of densely packed cells (C)</p> Signup and view all the answers

What is the most common primary brain tumor type?

<p>Meningioma (D)</p> Signup and view all the answers

Which of the following is NOT true about meningiomas?

<p>They usually present with rapid-onset severe symptoms. (D)</p> Signup and view all the answers

What characterizes the MRI appearance of a meningioma?

<p>Strong and homogenous enhancement with contrast (B)</p> Signup and view all the answers

What is the primary characteristic of WHO grade III meningiomas?

<p>Malignant with metastatic potential. (B)</p> Signup and view all the answers

Which of the following describes metastatic brain tumors?

<p>Commonly arise from melanoma or carcinoma of various origins. (B)</p> Signup and view all the answers

What histological feature is common in meningiomas?

<p>Meningothelial whorls (C)</p> Signup and view all the answers

Which bone change could be caused by meningiomas?

<p>Reactive (hyperostotic) changes (A)</p> Signup and view all the answers

What is the prognosis typically associated with meningeal carcinomatosis?

<p>Poor prognosis is commonly observed. (C)</p> Signup and view all the answers

Which Chiari malformation type is primarily associated with lumbar spina bifida with a meningomyelocele?

<p>Chiari type II (A)</p> Signup and view all the answers

What is the most common cause of congenital hydrocephalus?

<p>Chiari type II malformation (B)</p> Signup and view all the answers

Which of the following is a treatment modality for symptomatic Chiari malformation?

<p>Surgical decompression (C)</p> Signup and view all the answers

What neurological feature is typical in Herpes simplex encephalitis?

<p>Focal neurological signs (D)</p> Signup and view all the answers

What is the most common pathogen causing congenital fetal infection leading to neurological damage?

<p>Cytomegalovirus (A)</p> Signup and view all the answers

Which clinical feature is NOT commonly associated with Chiari malformation symptoms?

<p>Hearing loss (D)</p> Signup and view all the answers

Which of the following statements about Dandy-Walker malformation is TRUE?

<p>The primary issue is enlargement of the posterior fossa. (B)</p> Signup and view all the answers

What diagnostic method is most definitive for Herpes simplex encephalitis?

<p>PCR detection of viral nucleic acid (A)</p> Signup and view all the answers

Which Chiari malformation is characterized by hypoplasia or aplasia of the cerebellum?

<p>Chiari type IV (B)</p> Signup and view all the answers

In symptomatic treatment of Chiari malformations, which medication is commonly used for alleviating pain?

<p>Analgesics (B)</p> Signup and view all the answers

What is a common clinical symptom of cytomegaloviral infections in immunocompromised individuals?

<p>Necrotizing and hemorrhagic encephalitis (A)</p> Signup and view all the answers

What key characteristic differentiates Toxoplasma gondii infections from other congenital infections?

<p>It forms bradyzoite cysts in brain tissue. (C)</p> Signup and view all the answers

Which of the following is a characteristic feature of Chiari type III malformation?

<p>Encephalocele (D)</p> Signup and view all the answers

Flashcards

Dementia with Lewy bodies (DLB)

A progressive neurological disease that causes dementia, characterized by Lewy bodies, clumps of abnormal alpha-synuclein protein, within neurons.

Lewy body dementias

A group of neurological disorders that include Dementia with Lewy bodies and Parkinson's disease dementia, characterized by Lewy bodies, clumps of alpha-synuclein protein, within neurons.

Creutzfeldt-Jakob disease (CJD)

A rare and fatal brain disorder caused by prion proteins taking on an abnormal configuration, forming plaques in the brain and leading to cell death.

Variant Creutzfeldt-Jakob disease (vCJD)

A type of Creutzfeldt-Jakob disease linked to the variant form of the human prion protein.

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Spongiosis

Characteristic microscopic features of Creutzfeldt-Jakob disease (CJD) involving small cavities or holes in the brain tissue.

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Arnold-Chiari Malformation

A spectrum of congenital hindbrain abnormalities affecting the structural relationships between the brainstem, cerebellum, upper cervical cord and bony cranial base.

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Chiari type II malformation

The most common cause of congenital hydrocephalus. Cerebellar tonsils extend far down into the spinal canal, accompanied by an elongated fourth ventricle and brainstem. There is a lumbar spina bifida with a meningomyelocele.

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Chiari type I malformation

Elongation of one or both cerebellar tonsils, which protrude through the foramen magnum of the spinal canal and may become sclerotic; there may be associated syringomyelia or syringobulbia.

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Chiari type III malformation

Further herniation of the cerebellum below the foramen magnum forming an encephalocele, in addition to spina bifida, syringomyelia and hydrocephalus.

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Chiari type IV malformation

Hypoplasia or aplasia of the cerebellum.

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Dandy-Walker malformation

A congenital brain malformation involving the cerebellum. The essential features are: enlargement of the posterior fossa, cystic dilatation of the fourth ventricle and agenesis of the vermis.

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Herpes simplex encephalitis

A severe necrotizing viral infection preferentially involving the medial temporal and frontal lobes of the brain.

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Cytomegalovirus (CMV)

The largest of the human herpes viruses and may cause a variety of clinical manifestations ranging from asymptomatic to fatal. Severe, often fatal central (CNS) and peripheral nervous system infections are usually seen in patients with compromised immune systems or in maternal fetal infections in utero.

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Toxoplasmosis

A prevalent obligate intracellular parasite. Ability to form chronic and nonimmunogenic bradyzoite cysts, which typically form in the brain and muscle cells of infected mammals, including humans. In the majority of immunocompetent individuals, primary or chronic (latent) infections are asymptomatic, however certain populations are at higher risk for severe disease, including congenitally infected fetuses, newborns and immunodeficient patients, particularly those with T-cell immunity deficiency, such as hematologic malignancies, bone marrow and solid organ transplant and acquired immune deficiency syndrome (AIDS).

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Cerebellum

The part of the brain that controls coordination, balance, and movement. Damage to this region can result in problems with walking, talking, and other movements.

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Foramen magnum

The opening at the base of the skull through which the spinal cord passes.

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Syringomyelia

A cyst filled with fluid that develops in the spinal cord.

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Hydrocephalus

A build-up of fluid in empty spaces in the brain.

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Spina bifida

A birth defect in which the spinal cord does not close properly.

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Encephalocele

A protrusion of the meninges through a defect in the skull, usually at the base of the skull.

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Arteriovenous malformation (AVM)

A condition where there is an abnormal connection between the arteries and veins in the brain.

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Amyloid Plaques

A buildup of amyloid plaques in the brain, characterized by a hyaline eosinophilic core with radiating fibrils and a surrounding halo.

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Choroid Plexus Cyst

A small cyst containing cerebrospinal fluid (CSF) located in the choroid plexus.

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Choroid Plexus Papilloma

A rare, usually slow-growing tumor found in the ventricular system. It can obstruct CSF flow and cause hydrocephalus.

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Glial Tumors

The most common type of brain tumor, encompassing various subtypes like astrocytoma, ependymoma, glioblastoma, and oligodendroglioma.

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WHO Grading System for Astrocytomas

A histological grading system for diffuse astrocytomas based on four parameters: nuclear atypia, mitosis, microvascular proliferation, and necrosis.

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Nuclear Atypia

The presence of abnormal nuclear shapes and increased staining intensity in tumor cells, indicating potential malignancy.

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Mitosis In Astrocytomas

The occurrence of cell division in a tumor, indicating a higher risk of aggressive growth.

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Microvascular Proliferation

The formation of new blood vessels within the tumor, which can nourish and accelerate its growth.

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What is Ependymoma?

A slowly growing tumor originating from ependymal cells, lining the ventricles or spinal canal, showing both glial and epithelial features.

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Why are Ependymomas more common in children?

Ependymomas are more common in children, and may cause blockage and fluid buildup in the brain (hydrocephalus).

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How does the location of an Ependymoma impact its prognosis?

Spinally located ependymomas are often seen in adults between 30-40 years old. These tumors have a better prognosis due to a higher success rate in full removal.

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What are the key microscopic features of Ependymomas?

Ependymomas often have uniform cells with round nuclei and very few mitoses, forming distinctive perivascular pseudorosettes and ependymal rosettes.

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What are ependymal rosettes?

These structures consist of columnar cells arranged around a central lumen, which may be round or oval, and lack a bright pink (hypereosinophilic) edge.

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What are perivascular pseudorosettes?

More common than ependymal rosettes, these structures are formed as ependymal cell processes extend around blood vessels, creating zones with fewer nuclei.

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What is Diffuse Astrocytoma?

This type of glioma is well differentiated, infiltrating and diffusely spread throughout the brain with astrocytic features.

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Who is most often affected by Diffuse Astrocytoma?

Most commonly affecting young adults in their mid-thirties, Diffuse Astrocytomas often present with slow-onset symptoms like seizures, personality changes, and neurological deficits.

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Astroblastoma

A rare brain tumor (< 3% of primary brain gliomas) characterized by compact glial cells arranged around blood vessels, forming perivascular pseudorosettes.

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Supratentorial Astroblastoma

A type of astroblastoma often seen in children and young adults, commonly located in the cerebral hemispheres, and shows a tendency to form cysts.

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Astroblastoma Histology

Microscopically, astroblastomas exhibit well-defined borders, pushing aside surrounding tissue, but can be infiltrative in higher grade tumors. Their distinctive feature is the presence of perivascular pseudorosettes, which resemble ependymomas but have thicker processes extending from the cell body toward the blood vessel.

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Medulloblastoma

The most common embryonal brain tumor in children, characterized by small, undifferentiated cells with high mitotic activity.

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Medulloblastoma Location

Medulloblastomas are typically found in the cerebellum and fourth ventricle, commonly causing symptoms of increased intracranial pressure like headaches, nausea, and vomiting.

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Medulloblastoma Grade

Medulloblastomas are classified as WHO grade IV, indicating their aggressive nature and high potential for spread.

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Medulloblastoma Imaging

Medulloblastomas appear as solid masses on CT and MRI scans, intensely enhancing with contrast.

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Medulloblastoma Histology

Medulloblastomas exhibit a classic histological pattern with densely packed small, blue, round cells, forming syncytial arrangements, and containing mitosis and apoptotic bodies. They are often recognized by the presence of Homer-Wright rosettes.

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Meningioma

The most common primary brain tumor, accounting for 20-30% of cases, originating from arachnoid cap cells.

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WHO Grade I Meningioma

Benign, slow-growing tumor of the arachnoid cap cells. Typically affects adults, especially women, and often appears solitary.

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Meningioma Radiological Feature

Shows a distinct 'dural tail' on MRI.

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Metastatic Brain Tumor

The most frequent brain tumor in community hospitals, usually originating from elsewhere in the body (e.g., lung, breast, melanoma).

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Meningioma Grades

Meningiomas can be categorized into different grades based on their potential for growth and spread.

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Meningeal Carcinomatosis

A widespread spread of cancer cells in the subarachnoid space, often associated with lung and breast cancer.

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Meningioma Bone Changes

May cause reactive bone changes, indicating a potential for interaction with adjacent bone structures.

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Meningioma Microscopic Features

Microscopically, meningiomas show a distinctive, lobulated architecture with 'meningothelial whorls'.

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Study Notes

Central Nervous System

  • The central nervous system (CNS) is a complex network responsible for controlling body functions and coordinating responses to stimuli.
  • The CNS comprises the brain and spinal cord.

Central and Peripheral Nervous System

  • The peripheral nervous system (PNS) connects the CNS to the rest of the body, enabling communication and control.
  • The PNS has two main branches: the autonomic and somatic systems.

Brain Anatomy

  • The brain is divided into different regions with specific functions.
  • Key structures include the frontal, parietal, temporal, and occipital lobes, the brainstem, and cerebellum.

Lobes and Functions

  • Frontal lobe: Associated with consciousness and higher cognitive functions (planning, decision-making).
  • Parietal lobe: Processes movement and stimulus perception, integrating sensory information.
  • Temporal lobe: Important for language processing (speech recognition).
  • Occipital lobe: Responsible for vision.
  • Brainstem: Regulates basic bodily functions like breathing and heart rate.
  • Cerebellum: Controls motor coordination and balance.

Spinal Cord

  • The spinal cord is a long, cylindrical structure connecting the brain to the PNS.
  • It transmits signals between the brain and the rest of the body.
  • It also controls reflexes.

Autonomic Nervous System

  • This system regulates involuntary bodily functions (heart rate, digestion).
  • It has a sympathetic (fight-or-flight) and parasympathetic (rest-and-digest) branches.

Peripheral Nervous System

  • The PNS carries information to and from the CNS.
  • It includes nerves and ganglia.
  • Includes the somatic and autonomic nervous systems.

Nervous System Cells

  • Neurons: Functional units of the nervous system, transmitting signals via action potentials.
  • Neuroglia: Supporting cells in the nervous system like oligodendroglia and Schwann cells.

Meninges

  • The meninges are protective membranes surrounding the brain and spinal cord.
  • The layers include dura mater, arachnoid mater, and pia mater.
  • Cerebrospinal fluid (CSF) is in the subarachnoid space.

Cerebral Arterial Circle (Circle of Willis)

  • The cerebral arterial circle is a network of blood vessels supplying blood to the brain.

Arnold-Chiari Malformation

  • A group of congenital malformations affecting the hindbrain (cerebellum, brainstem, upper cervical cord) and skull base.
  • Classified into four types (I-IV) based on severity.
  • Downward displacement of brain structures, causing compression and symptoms.
  • Treatment options include medications, shunting, and surgery.

Dandy-Walker Malformation

  • A congenital malformation of the brain, specifically the cerebellum.
  • Features include posterior fossa enlargement and fourth ventricle dilation, vermis is typically absent.
  • Symptoms vary depending on severity.
  • Treatment focuses on managing hydrocephalus and related complications.

Neural Tube Defects

  • Result from failures in the early development of the neural tube.
  • Types include craniorachischisis, anencephaly, encephalocele, spina bifida occulta, closed spinal dysraphism, meningocele, and myelomeningocele.
  • Diagnosis and management are crucial during fetal development.

Acute Viral Infections-Herpes Simplex Encephalitis

  • A severe viral infection affecting the temporal and frontal lobes.
  • Caused by herpes simplex virus (HSV).
  • Diagnosed through cerebrospinal fluid (CSF) analysis and/or PCR testing.
  • Treatment involves immediate acyclovir administration.

Cytomegaloviral Infections (CMV)

  • CMV is a common viral infection in immunocompromised individuals and pregnant women.
  • It can affect the nervous system and may lead to severe neurological damage or death.
  • Diagnosis involves detecting CMV in CSF or tissue samples.

Necrotizing and Haemorrhagic Encephalitis

  • CNS inflammation with tissue damage, often due to infectious or autoimmune processes.
  • Various causes like CMV and herpes infections.
  • Diagnose through histological examination of affected tissue.

Toxoplasmosis

  • Infection by the parasite Toxoplasma gondii, often asymptomatic in healthy adults but detrimental in immunocompromised individuals.
  • Neurologic symptoms develop from dormant parasites reactivating in the CNS.
  • Diagnosis via serologic testing and/or tissue identification.

Rhinocerebral Mucormycosis

  • Fungal infection, primarily seen in diabetic or immunocompromised patients.
  • Presents with septate hyphae.
  • Diagnosed via tissue samples of the involved nasal passages.

Alzheimer's Disease

  • A chronic neurodegenerative disease affecting the brain.
  • Its main symptoms are memory loss, and cognitive decline.
  • The disease progresses gradually, and there is no cure.
  • Potential causes include genetic factors, inflammatory processes, and environmental exposures.

Dementia with Lewy Bodies

  • A type of dementia that involves the abnormal accumulation of a protein called alpha-synuclein.
  • This protein accumulation affects parts of the brain responsible for movement, thinking, sleep, mood and behaviour.
  • Diagnosis by observing symptoms, and brain scans.

Parkinson's Disease

  • A progressive neurodegenerative disease affecting the brain.
  • It's primarily characterized by movement issues (tremors, rigidity, slowed movements).
  • Dopamine levels in the brain are compromised due to degeneration in the substantia nigra in the midbrain area.
  • Several types of symptoms and complications.

Creutzfeldt-Jakob Disease

  • A rare and fatal prion disease that rapidly progresses.
  • Causes severe brain damage and neurological abnormalities.
  • Varied disease etiology and severity
  • Often caused by a prion protein that converts itself into a harmful conformation.
  • Has several different variants, all with differing causes.

Choroid Plexus Cyst

  • A benign cyst that develops in the choroid plexus in the brain.
  • Filled with cerebrospinal fluid (CSF).
  • Symptoms include signs of increased intracranial pressure.
  • Diagnosis via imaging like CT or MRI.

Choroid Plexus Papilloma

  • A rare type of benign tumor arising from the choroid plexus.
  • Can cause hydrocephalus.
  • More frequent in children.
  • Presents as a mass within the ventricular system or brain.

Glial Tumors

  • A large group of brain tumors derived from glial cells.
  • They vary in their behavior from benign to aggressive.
  • Classification based on the WHO Grading System, which classifies the tumors based on their histology and behavior

Histological Grading of Diffuse Astrocytic Tumors

  • Grading system used for diffuse astrocytic tumors, based on 2016 WHO classifications.
  • Grading assessed using four histological parameters.

Pilocytic Astrocytoma

  • A low-grade brain tumor of childhood, typically in the cerebellum.
  • Good prognosis, often easily removable via surgery.

Ependymoma

  • Tumor arising from ependymal cells, lining the brain ventricles or spinal canal.
  • More common in children.
  • Infiltrative but more common to be circumscribed in CNS.

Diffuse Astrocytoma

  • Well-differentiated brain tumor with astrocytic features and infiltrating growth pattern.

Glioblastoma Multiforme

  • A high-grade and aggressive tumor having both a malignant and infiltrative component.
  • Usually presents with acute focal symptoms.

Astroblastoma

  • A rare type of primitive brain tumor.
  • Characterized by the presence of perivascular pseudorosettes formed from GFAP+ cells.
  • In children and young adults

Medulloblastoma

  • A malignant brain tumor of childhood arising from the cerebellum.
  • It is rapidly growing tumor.

Meningioma

  • Benign tumor of the meninges, which are the protective membranes surrounding the brain and spinal cord.
  • Usually located near the skull base or dura mater.

Metastatic Tumors

  • Tumors that have spread to the brain from elsewhere in the body.
  • Often caused by cancers in other body organs and tissues like the lung, breast or kidney.

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Description

Test your knowledge on dementia with Lewy bodies and Creutzfeldt-Jakob disease (CJD) through this quiz. Explore the primary proteins and pathological features associated with these conditions to enhance your understanding of neurodegenerative diseases.

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