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</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)</p> Signup and view all the answers

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

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

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

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

    Which is NOT a subtype of glial tumors?

    <p>Neuroblastoma</p> Signup and view all the answers

    How are gangliogliomas typically classified in terms of cellular composition?

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

    What demographic is most commonly affected by diffuse astrocytoma?

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

    What is the typical histological feature observed in ependymomas?

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

    What is the median age for diagnosis of astroblastoma?

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

    What type of cell differentiation is exhibited by ependymomas?

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

    How is medulloblastoma commonly classified in terms of its grade?

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

    What is a key microscopic feature of classic medulloblastoma?

    <p>Homer Wright rosettes</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.</p> Signup and view all the answers

    In which locations does medulloblastoma most frequently occur?

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

    What describes the histological arrangement of cells in medulloblastoma?

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

    What is the most common primary brain tumor type?

    <p>Meningioma</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.</p> Signup and view all the answers

    What characterizes the MRI appearance of a meningioma?

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

    What is the primary characteristic of WHO grade III meningiomas?

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

    What histological feature is common in meningiomas?

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

    Which bone change could be caused by meningiomas?

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

    What is the prognosis typically associated with meningeal carcinomatosis?

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

    What is the most common cause of congenital hydrocephalus?

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

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

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

    What neurological feature is typical in Herpes simplex encephalitis?

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

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

    <p>Cytomegalovirus</p> Signup and view all the answers

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

    <p>Hearing loss</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.</p> Signup and view all the answers

    What diagnostic method is most definitive for Herpes simplex encephalitis?

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

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

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

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

    <p>Analgesics</p> Signup and view all the answers

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

    <p>Necrotizing and hemorrhagic encephalitis</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.</p> Signup and view all the answers

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

    <p>Encephalocele</p> Signup and view all the answers

    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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    Related Documents

    Arnold-Chiari Malformation PDF

    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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