Neuropathology — MCQs

Neuropathology Indian Medical PG Practice Questions and MCQs

Question 351: In which condition is retinal astrocytoma commonly seen?

A. Tuberous sclerosis (Correct Answer)
B. Sturge weber syndrome
C. Von Hippel-Lindau syndrome
D. Neurofibromatosis

Explanation: ***Tuberous sclerosis*** - **Retinal astrocytoma**, also known as **retinal astrocytic hamartoma**, is a common ocular manifestation of **tuberous sclerosis complex (TSC)** [1]. - These lesions can appear as **mulberry-like nodules** or flat, whitish patches on the retina. - Found in approximately **40-50% of patients** with tuberous sclerosis. *Sturge weber syndrome* - Characterized by a **port-wine stain (facial nevus flammeus)**, **leptomeningeal angioma**, and **glaucoma**. - Ocular manifestations include **choroidal hemangiomas** and increased episcleral venous pressure. - Does not typically involve **retinal astrocytomas**. *Von Hippel-Lindau syndrome* - Associated with **retinal capillary hemangioblastomas** (vascular tumors) and various tumors in other organs like the kidneys, pancreas, and brain. - This condition involves **vascular tumors** in the retina, not astrocytic tumors. - Important differential as it also presents with retinal lesions. *Neurofibromatosis* - A neurocutaneous syndrome characterized by **café-au-lait spots**, **neurofibromas**, and **Lisch nodules** (iris hamartomas). - Ocular manifestations include **optic nerve gliomas** and iris hamartomas, but **not retinal astrocytomas**. - NF-1 is the most common neurocutaneous syndrome but has different retinal pathology. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1318-1319.

Question 352: Perivascular lymphocytes & microglial nodules are seen in -

A. HIV encephalitis (Correct Answer)
B. CMV meningitis
C. Bacterial meningitis
D. Multiple sclerosis

Explanation: ***HIV encephalitis*** - **Perivascular lymphocytes** and **microglial nodules** are the characteristic histopathological hallmarks of **HIV encephalitis (HIV-associated dementia complex)** [1][2]. - Microglial nodules are formed by activated microglia and macrophages, often accompanied by **multinucleated giant cells** (the classic triad) [2]. - These features reflect chronic CNS inflammation and neuronal damage caused by HIV infection. *CMV meningitis* - Cytomegalovirus (CMV) infection in immunocompromised patients causes meningoencephalitis with characteristic **intranuclear ("owl's eye") inclusion bodies** and necrotizing inflammation. - The histological pattern differs from the microglial nodules and perivascular lymphocytes seen in HIV encephalitis. *Bacterial meningitis* - Characterized by prominent **neutrophilic infiltrate** in the subarachnoid space, fibrinopurulent exudate, and potential vasculitis. - Acute bacterial meningitis does not show the lymphocytic and microglial nodular pattern characteristic of viral encephalitis. *Multiple sclerosis* - An autoimmune demyelinating disease with **perivenular demyelinating plaques** containing lymphocytes and macrophages. - While perivascular inflammation occurs, **microglial nodules** are not a characteristic feature; instead, MS shows demyelination with reactive gliosis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, p. 1278. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 711-712.

Question 353: Which condition is characterized by spongiform degeneration of the cerebral cortex?

A. Creutzfeldt-Jakob disease (Correct Answer)
B. Subacute sclerosing panencephalitis
C. Fatal familial insomnia
D. Cerebral toxoplasmosis

Explanation: ***Creutzfeldt-Jakob disease*** - This is a **prion disease** characterized by rapid cognitive decline, myoclonus, and distinctive EEG changes, with **spongiform degeneration of the cerebral cortex** as the hallmark neuropathological feature [1]. - The spongiform changes are due to intracellular vacuoles within neurons and astrocytes, giving the brain tissue a **spongy appearance** [2]. - CJD shows **widespread cortical involvement**, making it the classic answer for cortical spongiform degeneration [2]. *Subacute sclerosing panencephalitis* - This condition is a rare, **chronic, progressive encephalitis** caused by persistent measles virus infection. - It is characterized by widespread **demyelination, gliosis, and intranuclear inclusion bodies**, but not spongiform degeneration. *Fatal familial insomnia* - This is another **prion disease** that also exhibits spongiform degeneration, but the key difference is **anatomical distribution** [2]. - FFI primarily affects the **thalamus** (a subcortical structure) and causes severe insomnia, dysautonomia, and motor signs [2]. - While spongiform changes occur in FFI, they are most prominent in the **thalamus rather than the cerebral cortex**, making CJD the better answer for cortical spongiform degeneration [2]. *Cerebral toxoplasmosis* - This is an **opportunistic infection of the brain** caused by **_Toxoplasma gondii_**, primarily seen in immunocompromised individuals. - It typically results in the formation of **abscesses or ring-enhancing lesions**, rather than spongiform degeneration. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 712-713. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1284-1286.

Question 354: What is the histological appearance of the brain in Creutzfeldt-Jakob disease?

A. Neuronal loss with gliosis
B. Neuronal loss with spongiform change (Correct Answer)
C. Spongiform change in brain
D. Prion protein accumulation

Explanation: ***Neuronal loss with spongiform change*** - This is the **most complete and accurate** description of CJD histology under light microscopy - The classic histological **triad** of CJD includes: (1) **spongiform vacuolation** (numerous small vacuoles in neuropil and neuronal cytoplasm), (2) **neuronal loss**, and (3) **astrocytic gliosis** [1] - This option captures the two most characteristic microscopic features that together define the disease - Spongiform change refers to the sponge-like appearance due to vacuoles, while neuronal loss with gliosis represents the degenerative process [1] *Spongiform change in brain* - While spongiform change is indeed the **hallmark feature** of CJD, this option is incomplete [1] - Describing only spongiform change without mentioning neuronal loss misses a critical component of the histological picture - In pathology, the most complete accurate description is preferred when available *Neuronal loss with gliosis* - These features are present in CJD but are **non-specific** findings - Neuronal loss and gliosis occur in many neurodegenerative diseases (Alzheimer's, Parkinson's, etc.) - Without mentioning spongiform change, this description lacks the defining characteristic of prion diseases [1] *Prion protein accumulation* - PrPSc accumulation is the **underlying cause** of the pathology, not a description of routine histological appearance - Prion proteins require **special immunohistochemical staining** (anti-PrP antibodies) for visualization [1] - Under standard H&E staining, prion deposits are not the primary visible feature; the **spongiform degeneration** is what pathologists observe [1] **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1284-1286.

Question 355: Which of the following tumors is not derived from the meninges?

A. Meningioma
B. Hemangiopericytoma
C. Schwannoma
D. Hemangioblastoma (Correct Answer)

Explanation: ***Hemangioblastoma*** - This tumor is derived from **vascular endothelial cells and stromal cells**, not meningeal cells [1] - Typically found in the **cerebellum** and strongly associated with **von Hippel-Lindau disease** [1] - Has **no meningeal origin** and represents a distinct vascular neoplasm *Meningioma* - Derived from **arachnoidal cap cells** of the meninges [2] - Most common **benign primary intracranial tumor** arising from meningeal coverings [2] - Clearly of **meningeal origin** [3] *Schwannoma* - Originates from **Schwann cells** of peripheral nerve sheaths (neural crest origin) [4] - While not meningeal in origin, it commonly occurs **intracranially** affecting cranial nerves (especially CN VIII) [2] - Though also not meningeal, **hemangioblastoma is the better answer** as it's purely parenchymal/vascular, whereas schwannomas can have anatomic association with meninges [4] *Hemangiopericytoma* - Now classified as **solitary fibrous tumor/hemangiopericytoma** (WHO classification) - Arises from **meningeal pericytes** around blood vessels in the meninges - Despite mesenchymal origin, it is considered part of the **meningeal tumor spectrum** and has meningeal associations **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 726-727. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 727-728. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1316-1317. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1248-1249.

Question 356: An elderly patient dies with chronic dementia. At autopsy, the brain shows diffuse cortical atrophy with relative sparing of primary motor and sensory areas. Which of the following would most likely be a prominent feature on microscopic examination of her brain tissue?

A. Central chromatolysis
B. Lewy bodies
C. Gliosis of the caudate nucleus
D. Neurofibrillary tangles (Correct Answer)

Explanation: ***Neurofibrillary tangles*** - Commonly associated with **Alzheimer's disease**, which can present with **chronic dementia** and shows cortical atrophy upon autopsy [1][2]. - The presence of **neurofibrillary tangles** is a hallmark feature in Alzheimer's, with relative sparing of areas like primary motor and sensory cortices [1][3]. - The number of neurofibrillary tangles correlates better with the degree of dementia than does the number of neuritic plaques [1]. *Gliosis of the caudate nucleus* - More characteristic of **Huntington's disease**, often presenting with **atrophy of the striatum** rather than cortical atrophy. - While gliosis can be present, it's not a prominent feature associated with diffuse cortical atrophy typical of Alzheimer's. *Lewy bodies* - These are typically found in **Lewy body dementia**, which presents differently and often includes **visual hallucinations** and fluctuating cognition. - This finding does not align with the chronic dementia and cortical atrophy described in the question. *Central chromatolysis* - This refers to changes seen typically in **neuronal injury**, not specifically related to chronic dementia or cortical atrophy. - It indicates a cellular response to injury, rather than the **neurodegenerative changes** noted in Alzheimer's disease. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1293. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 720-721. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722.

Question 357: Which of the following is not a characteristic feature of Alzheimer's disease?

A. Neurofibrillary tangles
B. Senile (neuritic) plaques
C. Amyloid Angiopathy
D. Lewy bodies (Correct Answer)

Explanation: ***Correct Answer: Lewy bodies*** - **Lewy bodies** are abnormal aggregates of protein, primarily **alpha-synuclein**, found in the brains of individuals with **Parkinson's disease** and **Lewy body dementia**, but **not typically in Alzheimer's disease** [1]. - While there can be overlap in pathologies, the presence of Lewy bodies as a primary feature suggests a different neurodegenerative process than Alzheimer's. - This is the correct answer because it is **NOT a characteristic feature** of Alzheimer's disease. *Incorrect: Neurofibrillary tangles* - **Neurofibrillary tangles** are intracellular aggregates of hyperphosphorylated **tau protein** and are a **hallmark pathological feature** of Alzheimer's disease. - These tangles disrupt neuronal function and axonal transport, leading to neuronal death. - This IS a characteristic feature of Alzheimer's disease. *Incorrect: Senile (neuritic) plaques* - **Senile plaques**, also known as **neuritic plaques**, are extracellular deposits of **beta-amyloid protein** that are a **characteristic pathological feature** of Alzheimer's disease. - These plaques accumulate outside neurons, leading to inflammation and neuronal dysfunction. - This IS a characteristic feature of Alzheimer's disease. *Incorrect: Amyloid Angiopathy* - **Cerebral amyloid angiopathy** (CAA) is the deposition of **amyloid-beta protein** in the walls of small and medium-sized blood vessels in the central nervous system, particularly the cerebral cortex and leptomeninges. - It is present in **over 90% of Alzheimer's disease cases** and is a significant contributor to cognitive decline and hemorrhagic events in these patients. - This IS a characteristic feature of Alzheimer's disease. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1296-1298.

Question 358: Complex granular corpuscles are produced by?

A. Oligodendrocytes
B. Astrocytes
C. Microglia (Correct Answer)
D. Vascular endothelium

Explanation: ***Microglia*** - **Microglia** are the primary **immune cells** of the central nervous system (CNS) and play a key role in clearing cellular debris and pathogens. - In response to damage or infection, microglia become activated, proliferate, and can form **complex granular corpuscles** (also known as **gitter cells**), which are phagocytic cells filled with lipid-rich debris. *Oligodendrocytes* - **Oligodendrocytes** are responsible for producing **myelin** in the CNS, which insulates axons and facilitates rapid nerve impulse conduction. - They are not primarily involved in phagocytosis or the formation of granular corpuscles. *Astrocytes* - **Astrocytes** are star-shaped glial cells that provide structural and metabolic support to neurons, regulate the **blood-brain barrier**, and maintain the extracellular environment. - While they can be reactive in injury, they do not typically form phagocytic granular corpuscles like microglia. *Vascular endothelium* - **Vascular endothelial cells** form the inner lining of blood vessels, including those in the brain, and are crucial for regulating blood flow and forming the **blood-brain barrier**. - They are not considered a source of complex granular corpuscles, which are a product of intrinsic CNS immune response.

Question 359: Which of the following lesions shows the most specific anatomic changes in HIV infection?

A. Lymph nodes
B. CNS lesions (Correct Answer)
C. Kaposi's sarcoma
D. Opportunistic infection

Explanation: ***CNS lesions*** - **HIV encephalopathy** (or AIDS dementia complex) involves specific changes in the central nervous system, such as **microglial nodules** and **multinucleated giant cells**, which are highly characteristic of direct HIV infection of the brain [1]. - These neurological manifestations are often a defining feature of advanced HIV disease and represent severe organ-specific pathology directly attributable to the virus [1]. *Lymph nodes* - **Lymphadenopathy** is common in HIV infection, particularly in the early and mid-stages, reflecting generalized immune activation [2]. - While there are architectural changes like follicular hyperplasia followed by follicular involution, these findings are less specific than CNS changes as they can be seen in various other viral infections or inflammatory conditions [2]. *Kaposi's sarcoma* - Kaposi's sarcoma is an **HIV-associated malignancy** caused by Human Herpesvirus 8 (HHV-8), occurring more frequently in immunocompromised individuals, including those with HIV. - However, it is not a direct consequence of HIV-induced cellular changes but rather an opportunistic tumor that arises due to severe immunodeficiency, and can also be seen in immunosuppressed organ transplant patients or elderly without HIV. *Opportunistic infection* - **Opportunistic infections** are very common in HIV-infected individuals due to the weakened immune system, serving as a hallmark of AIDS [1]. - While indicative of HIV-induced immunodeficiency, the specific features of these infections (e.g., *Pneumocystis jirovecii* pneumonia, toxoplasmosis) are generally not unique to HIV but rather depend on the specific pathogen involved [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 711-712. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 555-556.

Question 360: Liquefactive necrosis as a primary event is most often seen in:

A. Kidneys
B. CNS (Correct Answer)
C. Spleen
D. Liver

Explanation: ***CNS*** - **Liquefactive necrosis** primarily occurs in the **central nervous system** (CNS) due to the high lipid content of brain tissue and the rapid breakdown of neurons. - It often results from **ischemia** or severe **infections**, leading to the formation of a fluid-filled cystic area [1]. *Spleen* - The spleen typically undergoes **caseous necrosis** in conditions like tuberculosis, not liquefactive necrosis. - In infarcts, it often exhibits **coagulative necrosis**, where tissue architecture remains relatively intact. *Liver* - The liver primarily exhibits **coagulative necrosis** or **steatotic changes** due to various insults such as alcohol or viral hepatitis. - **Hemorrhagic necrosis** may occur in cases of fulminant hepatic failure but is still not liquefactive. *Kidneys* - Kidneys most commonly show **coagulative necrosis** during acute tubular injury rather than liquefactive necrosis. - In renal infarcts, the tissue remains firm, and necrotic areas do not undergo liquefaction as seen in the CNS. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1268-1269.

Practice by Chapter

Cellular Pathology of the Nervous System

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Cerebrovascular Diseases

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Trauma to the Central Nervous System

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Infections of the Nervous System

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Demyelinating Diseases

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Neurodegenerative Diseases

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CNS Tumors

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Peripheral Nerve Disorders

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Neuromuscular Junction Diseases

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Congenital and Developmental Disorders

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