Neuropathology Practice Questions

Q: All are congenital myopathies except

Polymyositis. ***Polymyositis*** - This is an **acquired inflammatory myopathy** characterized by **autoimmune muscle inflammation** and weakness, typically presenting in adulthood [1]. - It is not a genetic or congenital condition but rather an **immune-mediated disorder** [1]. *Nemaline myopathy* - This is a **congenital myopathy** characterized by the presence of **rod-like inclusions (nemaline bodies)** in muscle fibers. - Symptoms often begin in infancy or childhood, including **muscle weakness** and **feeding difficulties**. *Central Core myopathy* - This is a **congenital myopathy** associated with mutations in the **RYR1 gene**, leading to abnormalities in muscle fibers characterized by **central cores**. - It is often linked to **malignant hyperthermia susceptibility** and presents with early-onset **proximal muscle weakness**. *Centronuclear myopathy* - This is a **congenital myopathy** where muscle fibers have an **abnormal central placement of nuclei** that normally reside at the periphery. - It typically presents at birth or in early childhood with **muscle weakness** and **hypotonia**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1240-1242.

Q: The most characteristic feature of Primary central nervous system lymphoma (PCNSL) is -

It is seen in immunosuppressed patients. ***It is seen in immunosuppressed patients*** - **Primary central nervous system lymphoma (PCNSL)** is **strong associated with immunosuppression**, particularly in patients with **HIV/AIDS** [1] or those on immunosuppressive therapy post-transplant [1]. - The compromised immune surveillance allows for uncontrolled B-cell proliferation, which is the characteristic origin of PCNSL [1]. - This is the **most defining clinical association** that distinguishes PCNSL epidemiologically [3]. *It is usually solitary* - While PCNSL can present as a solitary lesion, particularly in immunocompetent individuals, this is not the most characteristic feature. - In immunocompromised patients (especially HIV-associated cases), **multifocal lesions** are common [1]. *It is most commonly formed of T cells* - This is **incorrect**. The vast majority of PCNSL cases (>90%) are of **B-cell origin** [1]. - Specifically, they are typically **diffuse large B-cell lymphomas (DLBCL)** [1]. - T-cell primary CNS lymphomas are extremely rare. *Multifocal lesions are seen* - While multifocal lesions are frequently seen in PCNSL, especially in immunocompromised patients, this is not the **most characteristic** distinguishing feature [1]. - The **strong association with immunosuppression** is the key epidemiological and clinical characteristic that defines PCNSL [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1315-1316. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 262-263. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 711-712.

Q: Multiple sclerosis is characterized by all EXCEPT -

Oligodendrocytes. ***Oligodendrocytes*** - Multiple sclerosis is characterized by the **loss or destruction** of oligodendrocytes, not by their presence [1]. - The mere presence of oligodendrocytes is normal in CNS tissue and does not characterize MS. - What characterizes MS is **oligodendrocyte damage** leading to demyelination, but oligodendrocytes themselves as a cell type are not a characteristic feature of the disease [1]. - This makes "Oligodendrocytes" alone the correct answer to this EXCEPT question. *Demyelination* - **Demyelination** is the hallmark pathological feature of multiple sclerosis [2], [3]. - Progressive destruction of myelin sheaths disrupts nerve impulse conduction. - This is a defining characteristic of MS pathology. *Grey-tan plaques in the white matter* - Characteristic **plaques** (sclerotic lesions) in CNS white matter are pathognomonic for MS [2]. - These lesions appear **grey-tan** on gross examination at autopsy [2]. - Represent areas of chronic demyelination, inflammation, and gliosis [3]. - The term "multiple sclerosis" literally refers to these multiple sclerotic plaques [2]. *Increased protein concentration in CSF* - CSF analysis in MS typically shows **increased protein**, particularly **immunoglobulins (IgG)**. - **Oligoclonal bands** on CSF electrophoresis are found in ~95% of MS patients. - Reflects intrathecal immune activation and inflammation within the CNS. - This is a characteristic laboratory finding in MS. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1255-1256. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1286-1287. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 713-715.

Q: All of the following are prion associated diseases except?

Alzheimer's disease. ***Alzheimer's disease*** - Alzheimer's disease is primarily characterized by the accumulation of **amyloid-beta plaques** and **neurofibrillary tangles** composed of hyperphosphorylated tau protein, not by prion proteins [4]. - While it shares some features of protein **misfolding and aggregation** seen in prion diseases, its pathogenic mechanism is distinct and does not involve infectious prions [1]. *Kuru* - Kuru is a transmissible spongiform encephalopathy (TSE) caused by infectious **prion proteins**, historically linked to **ritualistic cannibalism** in New Guinea [2]. - It is one of the classic examples of a **human prion disease**, primarily affecting the central nervous system. *Creutzfeldt-Jakob disease (CJD)* - CJD is a progressive, fatal **neurodegenerative disorder** caused by **prion proteins** that can be sporadic, genetic, or acquired [3]. - It is characterized by rapidly progressive dementia and spongiform changes in the brain [3]. *Scrapie* - Scrapie is a **prion disease** that affects **sheep and goats**, causing neurological symptoms and ultimately death. - It is considered the **prototypical prion disease**, and its study provided early insights into the nature of prions. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, p. 1284. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1284-1286. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 712-713. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1294.

Q: Which is the most common type of primary CNS lymphoma in AIDS Positive patients?

Immunoblastic Large cell lymphoma. ***Immunoblastic Large cell lymphoma*** - This is the **most common histopathological subtype** of primary central nervous system lymphoma (PCNSL) in patients with **AIDS**. [1] - Its prevalence is linked to the profound **immunosuppression** seen in AIDS, which facilitates uncontrolled B-cell proliferation, often driven by **Epstein-Barr virus (EBV)**. [1] *Lymphoplasmacytic Lymphoma* - This subtype is a **low-grade B-cell lymphoma** typically presenting in older adults and is not the most common form of PCNSL in AIDS. - It is often associated with Waldenström macroglobulinemia, which involves the production of **monoclonal IgM paraprotein**. *Anaplastic B- Cell Lymphoma* - While it is a **high-grade lymphoma**, it is a far less common subtype of PCNSL compared to immunoblastic large cell lymphoma in AIDS patients. - Anaplastic lymphomas typically show a **pleomorphic morphology** with bizarre-looking cells. *Centroblastic large cell lymphoma* - This is a subtype of **diffuse large B-cell lymphoma (DLBCL)**, which is the most common non-Hodgkin lymphoma overall, but specifically, the **immunoblastic variant** is more characteristic of PCNSL in AIDS. - While centroblastic morphology occurs in some AIDS-related lymphomas, **immunoblastic morphology** is more prevalent in primary CNS involvement. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 261-263.

Q: Most common supratentorial tumor in children

Craniopharyngioma. ***Craniopharyngioma*** - **Craniopharyngiomas** are the **most common supratentorial tumors in children**, accounting for approximately 5-10% of all pediatric intracranial tumors. - They arise from **Rathke's pouch remnants** near the pituitary gland and hypothalamus in the **sellar/suprasellar region**. - Present with **visual disturbances, endocrine dysfunction, and increased intracranial pressure**. - Bimodal age distribution with peaks at **5-14 years** and 50-74 years. *Astrocytoma* - While **astrocytomas** are the **most common primary CNS tumors in children overall**, they are predominantly located in the **posterior fossa (infratentorial)**, particularly as **cerebellar astrocytomas** [1], [2]. - In the **supratentorial compartment specifically**, astrocytomas are less common than craniopharyngiomas in the pediatric population. - They arise from **astrocytes** and range from low-grade (pilocytic) to high-grade (glioblastoma) [1]. *Oligodendroglioma* - **Oligodendrogliomas** are **rare in children** and more prevalent in adults (peak 40-50 years). - They originate from **oligodendrocytes** and are typically **slow-growing tumors**. - Account for only 1-2% of pediatric brain tumors. *Meningioma* - **Meningiomas** are **very rare in children** (less than 2% of pediatric brain tumors) and are primarily tumors of adulthood. - They arise from **arachnoid cap cells** of the meninges and show increasing incidence with age. - When they occur in children, they are often associated with **neurofibromatosis type 2** [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.

Q: The site of occurrence of Subependymal Giant Cell Astrocytoma is

Foramen of Monro. ***Foramen of Monro*** - **Subependymal Giant Cell Astrocytoma (SEGA)** is predominantly found near the **foramen of Monro**, growing into the **lateral ventricles**. - Its location often leads to **hydrocephalus** due to obstruction of cerebrospinal fluid flow at this strategic point [1]. *Medulla Oblongata* - Tumors in the medulla oblongata are rare, and SEGA is not typically associated with this brainstem region. - Tumors here would likely present with lower cranial nerve palsies or brainstem dysfunction, distinct from SEGA symptoms. *Cerebellum* - The cerebellum is a common site for other pediatric brain tumors like **pilocytic astrocytoma** or **medulloblastoma** [2], [4]. - SEGA does not primarily occur in the cerebellum. *Pineal gland* - The pineal gland is the site for pineal region tumors, such as **pineoblastoma** or **germinoma** [3]. - SEGA is not found in the pineal region. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 703-704. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1140-1141. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1314-1315.

Q: Which of the following is the most frequent primary malignant tumor of the CNS?

Glioblastoma multiforme. ***Glioblastoma multiforme*** - **Glioblastoma multiforme (GBM)** is the most common and aggressive primary malignant brain tumor in adults [1]. - It is a **grade IV astrocytoma**, characterized by rapid growth, necrosis, and microvascular proliferation [1]. *Oligodendroglioma* - **Oligodendrogliomas** are primary glial tumors but are less common than GBM. - They typically have a more indolent course than GBM and are often characterized by **IDH mutations** and **1p/19q co-deletion**. *Medulloblastoma* - **Medulloblastoma** is the most common malignant brain tumor in children, but it is rare in adults [2]. - It arises in the **cerebellum** and is a type of embryonal tumor [2]. *Meningioma* - **Meningiomas** are the most common primary brain tumors overall, but they are typically **benign** and originate from the meninges. - While they can be symptomatic due to compression, they are not primarily malignant in the way GBM is. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1308-1310. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726.

Question 1

Most common optic nerve tumor in children causing blindness:

Accepted Answer

Glioma

Answer

Astrocytoma

Answer

Craniopharyngioma

Answer

Meningioma

Question 2

Demyelination is seen in:

Accepted Answer

Multiple sclerosis

Answer

Leukodystrophy

Answer

Human leukoencephalopathy

Answer

AIDS

Question 3

All are congenital myopathies except

Accepted Answer

Polymyositis

Answer

Central Core myopathy

Answer

Centronuclear myopathy

Answer

Nemaline myopathy

Question 4

The most characteristic feature of Primary central nervous system lymphoma (PCNSL) is -

Accepted Answer

It is seen in immunosuppressed patients

Answer

It is usually solitary

Answer

It is most commonly formed of T cells

Answer

Multifocal lesions are seen

Question 5

Multiple sclerosis is characterized by all EXCEPT -

Accepted Answer

Oligodendrocytes

Answer

Demyelination

Answer

Grey-tan plaques in the white matter

Answer

Increased protein concentration in CSF

Question 6

All of the following are prion associated diseases except?

Accepted Answer

Alzheimer's disease

Answer

Kuru

Answer

Scrapie

Answer

Creutzfeldt-Jakob disease

Question 7

Which is the most common type of primary CNS lymphoma in AIDS Positive patients?

Accepted Answer

Immunoblastic Large cell lymphoma

Answer

Lymphoplasmacytic Lymphoma

Answer

Centroblastic large cell lymphoma

Answer

Anaplastic B-Cell Lymphoma

Question 8

Most common supratentorial tumor in children

Accepted Answer

Craniopharyngioma

Answer

Astrocytoma

Answer

Oligodendroglioma

Answer

Meningioma

Question 9

The site of occurrence of Subependymal Giant Cell Astrocytoma is

Accepted Answer

Foramen of Monro

Answer

Medulla Oblongata

Answer

Cerebellum

Answer

Pineal gland

Question 10

Which of the following is the most frequent primary malignant tumor of the CNS?

Accepted Answer

Glioblastoma multiforme

Answer

Oligodendroglioma

Answer

Medulloblastoma

Answer

Meningioma

Neuropathology — MCQs

Neuropathology — MCQs

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