Neuropathology Practice Questions

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

Neuronal loss with spongiform change. ***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.

Q: 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?

Neurofibrillary tangles. ***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.

Q: In which of the following conditions are neurons predominantly affected?

Spinocerebellar ataxia. ***Spinocerebellar ataxia*** - Spinocerebellar ataxias are a group of **neurodegenerative disorders** characterized by progressive dysfunction and loss of **neurons** in the cerebellum, brainstem, and spinal cord [1]. - The primary pathology involves **neuronal degeneration** leading to ataxia, dysarthria, and ocular motor abnormalities [1]. *Supranuclear palsy* - This condition is characterized by the degeneration of structures above the ocular motor nuclei, affecting movements of the eyes, neck, and trunk . - While it involves neuronal loss, it primarily affects specific brain regions involved in motor control, rather than a broad, predominant neuronal destruction across multiple systems like SCAs. *Corticobasal degeneration* - This neurodegenerative disorder is characterized by asymmetric motor dysfunction, often affecting one limb with rigidity and dystonia. - It involves the accumulation of **tau protein** in neurons and glial cells in the cerebral cortex and basal ganglia, leading to widespread cellular damage, but not exclusively neuronal death. *Multisystem Atrophy* - Multisystem atrophy is a **synucleinopathy** that involves degeneration of neurons and glial cells in multiple parts of the central nervous system, including the cerebellum, brainstem, basal ganglia, and spinal cord. - It is often characterized by **glial cytoplasmic inclusions** leading to prominent autonomic dysfunction, parkinsonism, and cerebellar ataxia.

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

Lewy bodies. ***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.

Q: Complex granular corpuscles are produced by?

Microglia. ***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.

Q: Absence of lymph nodes is characteristic of which type of cancer?

Brain. ***Brain*** - The brain lacks conventional **lymphatic drainage** vessels and **lymph nodes**, making metastasis to regional lymph nodes impossible [1]. - Due to the **blood-brain barrier** and unique immunological environment, brain tumors typically spread via cerebrospinal fluid or direct invasion rather than lymphatic routes [1]. *Liver* - The **liver** has an extensive lymphatic system with numerous **intrahepatic** and **extrahepatic lymph nodes** that can be involved in metastatic spread. - Hepatic malignancies, both primary and secondary, frequently metastasize to regional lymph nodes, such as the **hilar** and **periportal nodes**. *Lung* - The **lungs** are richly supplied with lymphatic vessels and contain a vast network of **bronchopulmonary**, **hilar**, and **mediastinal lymph nodes** [2]. - Lung cancers commonly metastasize to these regional lymph nodes, which is a critical factor in **staging** and treatment planning. *Placenta* - The **placenta** is a highly vascular organ but lacks an intrinsic lymphatic system and associated lymph nodes. - While it can be affected by gestational trophoblastic disease (a type of cancer), its metastatic spread occurs predominantly via **hematogenous routes**, not lymphatic. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1314-1315. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, pp. 724-725.

Q: Durck's granuloma is primarily associated with which of the following?

Brain. ***Brain*** - **Durck's granulomas** are microglial nodules specifically found in the **brain tissue** of patients with **typhoid fever**. [1] - They are a characteristic neuropathological finding associated with **S. typhi** infection. [1] *Spleen* - The spleen is a common organ affected by bacterial infections like **typhoid fever**, leading to **splenomegaly** and sometimes splenic abscesses. - However, **Durck's granulomas** are not found in the spleen; they are specific to brain lesions in typhoid. *Lymph node* - **Lymph nodes** can show reactive changes and hyperplasia in many systemic infections, including typhoid fever. - While lymphadenopathy might be present, **Durck's granulomas** are not a feature of typhoid infection in lymph nodes. *Skull* - The **skull** is the bony protection for the brain and is not directly involved in the formation of **Durck's granulomas**. - These granulomas are microscopic lesions within the brain parenchyma, not within the skull itself. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1281-1282.

Q: In which clinicopathologic category of amyloidosis is the deposition of protein 'Aβ (amyloid beta)' primarily observed?

Senile cerebral. ***Senile cerebral*** - **Amyloid-beta (Aβ)** deposition is the hallmark of **Alzheimer's disease** and is found in the brains of individuals with **senile cerebral amyloidosis**. - Aβ plaques accumulate primarily in the **cerebral cortex** and **vessel walls**, contributing to neurodegeneration. *Familial mediterranean fever* - This is an **autoinflammatory disorder** associated with deposition of **AA amyloid** (amyloid A protein). - It leads to **systemic amyloidosis**, affecting organs like the kidneys, rather than cerebral Aβ deposition. *Hemodialysis associated* - This type of amyloidosis is caused by the deposition of **β2-microglobulin** due to its inadequate clearance in patients undergoing long-term hemodialysis. - It primarily affects the **musculoskeletal system**, including joints and tendons, not the brain. *Systemic senile* - **Systemic senile amyloidosis** is characterized by the deposition of **transthyretin (TTR)**, primarily affecting the heart and other organs. - While it occurs in older individuals (senile), it does not involve Aβ deposition in the brain.

Q: Rosenthal fibres are seen in which of the following tumours?

Pilocytic astrocytoma. ***Pilocytic astrocytoma*** - **Rosenthal fibres** are highly characteristic histological findings in pilocytic astrocytomas, appearing as thick, elongated, corkscrew-shaped eosinophilic aggregates. - These are typically **well-circumscribed** tumors, most commonly found in the cerebellum of children and young adults. *Glioblastoma* - Characterized by **necrosis with pseudopalisading cells** and **microvascular proliferation**, not Rosenthal fibres. - It is a highly aggressive, grade IV astrocytoma with a poor prognosis. *Ependymoma (characterized by perivascular rosettes)* - Histologically, ependymomas are known for forming **perivascular pseudorosettes**, where tumor cells arrange around blood vessels. - These tumors arise from ependymal cells lining the ventricles and spinal cord, and do not contain Rosenthal fibres. *Medulloblastoma* - Medulloblastomas are highly malignant embryonic tumors typically found in the **cerebellum**, characterized by **small, round blue cells** and often forming Homer Wright rosettes. - They are not associated with Rosenthal fibres.

Question 1

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

Accepted Answer

Neuronal loss with spongiform change

Answer

Neuronal loss with gliosis

Answer

Spongiform change in brain

Answer

Prion protein accumulation

Question 2

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?

Accepted Answer

Neurofibrillary tangles

Answer

Central chromatolysis

Answer

Lewy bodies

Answer

Gliosis of the caudate nucleus

Question 3

In which of the following conditions are neurons predominantly affected?

Accepted Answer

Spinocerebellar ataxia

Answer

Supranuclear palsy

Answer

Multisystem Atrophy

Answer

Corticobasal degeneration

Question 4

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

Accepted Answer

Lewy bodies

Answer

Neurofibrillary tangles

Answer

Senile (neuritic) plaques

Answer

Amyloid Angiopathy

Question 5

Complex granular corpuscles are produced by?

Accepted Answer

Microglia

Answer

Oligodendrocytes

Answer

Astrocytes

Answer

Vascular endothelium

Question 6

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

Accepted Answer

CNS lesions

Answer

Lymph nodes

Answer

Kaposi's sarcoma

Answer

Opportunistic infection

Question 7

Absence of lymph nodes is characteristic of which type of cancer?

Accepted Answer

Brain

Answer

Liver

Answer

Lung

Answer

Placenta

Question 8

Durck's granuloma is primarily associated with which of the following?

Accepted Answer

Brain

Answer

Spleen

Answer

Lymph node

Answer

Skull

Question 9

In which clinicopathologic category of amyloidosis is the deposition of protein 'Aβ (amyloid beta)' primarily observed?

Accepted Answer

Senile cerebral

Answer

Hemodialysis associated

Answer

Familial mediterranean fever

Answer

Systemic senile

Question 10

Rosenthal fibres are seen in which of the following tumours?

Accepted Answer

Pilocytic astrocytoma

Answer

Glioblastoma

Answer

Medulloblastoma

Answer

Ependymoma

Neuropathology — MCQs

Neuropathology — MCQs

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