Neuropathology Practice Questions

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: 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.

Q: Which of the following is diagnostic of rabies?

Negri bodies. ***Negri bodies*** - **Negri bodies** are eosinophilic, sharply outlined, inclusion bodies found in the cytoplasm of certain nerve cells, particularly in the **hippocampus** and **Purkinje cells** of the cerebellum, in cases of rabies infection [1]. - Their presence in brain tissue is **pathognomonic** for rabies [1]. *Guarneri bodies* - **Guarneri bodies** are acidophilic cytoplasmic inclusion bodies found in cells infected with **variola (smallpox) virus** or **vaccinia virus**. - They are characteristic of poxvirus infections, not rabies. *Cowdry A body* - **Cowdry A bodies** are intranuclear inclusion bodies seen in cells infected with certain viruses like **herpes simplex virus (HSV)**, **varicella-zoster virus (VZV)**, and **cytomegalovirus (CMV)**. - They are not associated with rabies. *Bollinger bodies* - **Bollinger bodies** are eosinophilic cytoplasmic inclusion bodies primarily found in epithelial cells infected with **fowlpox virus**, a type of avian poxvirus. - They are not relevant to human rabies diagnosis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1279-1280.

Q: Which of the following is NOT a typical pathologic feature of the brain in AIDS?

Temporal lobe infarction. ***Temporal lobe infarction*** - While infarction can occur due to various causes, **temporal lobe infarction** is not a typical or primary neuropathologic feature directly associated with HIV infection itself. - HIV-related neurological complications more commonly involve widespread inflammation, opportunistic infections, or direct neurotoxic effects, rather than focal ischemic events in the temporal lobe [1]. *Perivascular giant cell invasion* - This is a **hallmark feature of HIV encephalitis (HIVE)**, characterized by the presence of multinucleated giant cells derived from macrophages/microglia, often surrounding blood vessels [1]. - These cells are infected with HIV and contribute to the inflammatory and destructive processes in the brain, representing one of the classic pathologic findings [1]. *Microglial nodules* - **Microglial nodules** are aggregations of activated microglia and macrophages, often found within the brain parenchyma in HIV-infected individuals. - They represent a **typical inflammatory response** to HIV infection and are a characteristic feature of HIV encephalitis along with multinucleated giant cells and white matter changes. *Vasculitis* - HIV infection can occasionally cause CNS vasculitis, leading to inflammation of blood vessels in the brain. - While recognized as a potential complication, it is relatively uncommon compared to the classic triad of microglial nodules, multinucleated giant cells, and white matter pathology that define typical HIV neuropathology. **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.

Q: All of the following hereditary conditions predispose to central nervous system tumors, except.

Xeroderma pigmentosum. ***Xeroderma pigmentosum*** - This condition primarily predisposes individuals to **skin cancers** due to defects in DNA repair mechanisms, rather than to central nervous system tumors. - **Neurological complications** can occur, but they are not associated with an increased risk of CNS tumors. *Neurofibromatosis 1 & 2* - Both types are well known for a strong association with **central nervous system tumors** [2][3], particularly **gliomas** and **meningiomas**. - Neurofibromatosis type 1 is especially linked with **optic pathway gliomas**, observable in pediatric patients. *Von-Hippel-Lindau syndrome* - This syndrome is associated with **hemangioblastomas** in the CNS [2][4], particularly in the **cerebellum** and **spinal cord** [4]. - Also predisposes to other tumors, including **renal cell carcinoma** and pancreatic neuroendocrine tumors. *Tuberous sclerosis* - Patients often develop **subependymal giant cell astrocytomas** and other brain tumors due to this condition [1][2]. - Additionally, it can lead to the formation of **hamartomas** in various organs, including the brain [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1318-1319. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-725. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 726-727.

Question 1

In which of the following conditions are neurons predominantly affected?

Accepted Answer

Spinocerebellar ataxia

Answer

Supranuclear palsy

Answer

Multisystem Atrophy

Answer

Corticobasal degeneration

Question 2

Complex granular corpuscles are produced by?

Accepted Answer

Microglia

Answer

Oligodendrocytes

Answer

Astrocytes

Answer

Vascular endothelium

Question 3

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 4

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

Accepted Answer

Brain

Answer

Liver

Answer

Lung

Answer

Placenta

Question 5

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

Accepted Answer

Brain

Answer

Spleen

Answer

Lymph node

Answer

Skull

Question 6

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 7

Rosenthal fibres are seen in which of the following tumours?

Accepted Answer

Pilocytic astrocytoma

Answer

Glioblastoma

Answer

Medulloblastoma

Answer

Ependymoma

Question 8

Which of the following is diagnostic of rabies?

Accepted Answer

Negri bodies

Answer

Guarneri bodies

Answer

Cowdry A body

Answer

Bollinger bodies

Question 9

Which of the following is NOT a typical pathologic feature of the brain in AIDS?

Accepted Answer

Temporal lobe infarction

Answer

Microglial nodules

Answer

Perivascular giant cell invasion

Answer

Vasculitis

Question 10

All of the following hereditary conditions predispose to central nervous system tumors, except.

Accepted Answer

Xeroderma pigmentosum

Answer

Neurofibromatosis 1 & 2

Answer

Von-Hippel-Lindau syndrome

Answer

Tuberous sclerosis

Neuropathology — MCQs

Neuropathology — MCQs

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