Neuropathology — MCQs

Neuropathology Indian Medical PG Practice Questions and MCQs

Question 291: Most common optic nerve tumor in children causing blindness:

A. Astrocytoma
B. Craniopharyngioma
C. Meningioma
D. Glioma (Correct Answer)

Explanation: ***Glioma*** - **Optic pathway gliomas** (also called optic nerve gliomas) are the **most common primary tumors of the optic nerve in children**, accounting for approximately 66% of all optic nerve tumors in the pediatric population. - These are typically **low-grade pilocytic astrocytomas (WHO Grade I)** and are strongly associated with **neurofibromatosis type 1 (NF1)** in 15-30% of cases [1]. - They cause **progressive visual loss** leading to **blindness**, and may present with **proptosis**, strabismus, and optic disc swelling. - The term "glioma" is the **standard clinical nomenclature** used for these tumors in pediatric ophthalmology and neuro-oncology. *Astrocytoma* - While optic nerve gliomas are histologically **pilocytic astrocytomas** [1], the accepted clinical term for this entity is **"optic nerve glioma"** or **"optic pathway glioma"**, not simply "astrocytoma." - Using "astrocytoma" alone is too generic and could refer to various brain astrocytomas (diffuse, anaplastic, glioblastoma) rather than the specific entity of optic nerve tumors [1]. - In clinical practice and literature, these are consistently referred to as **optic gliomas**. *Craniopharyngioma* - This is a **suprasellar tumor** arising from Rathke's pouch remnants, causing **bitemporal hemianopsia** due to **chiasmal compression**. - It does **not originate from the optic nerve** itself—it compresses the optic chiasm from above. - While common in children, it is a chiasmal/suprasellar lesion, not an optic nerve tumor. *Meningioma* - **Optic nerve sheath meningiomas** are rare in children and primarily affect **adults (peak in 40s)**, especially women. - These arise from meningothelial cells of the optic nerve sheath and cause **progressive painless visual loss** and **optic disc edema**. - In children, meningiomas represent **<2% of CNS tumors**, making them an uncommon cause of pediatric optic nerve pathology. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-726.

Question 292: Demyelination is seen in:

A. Leukodystrophy
B. Human leukoencephalopathy
C. Multiple sclerosis (Correct Answer)
D. AIDS

Explanation: ***Multiple sclerosis*** - **Multiple sclerosis (MS)** is the prototypical **autoimmune demyelinating disease** of the central nervous system (CNS) [1]. - Characterized by **inflammation** and **active demyelination** with formation of **plaques** (lesions) in the brain and spinal cord where the myelin sheath is destroyed [2]. - Results in impaired nerve signal transmission leading to varied neurological symptoms. - MS is the **classic example of a primary demyelinating disorder**. *Leukodystrophy* - **Leukodystrophies** are a heterogeneous group of inherited disorders affecting myelin in the CNS white matter. - While traditionally considered **dysmyelinating disorders** (abnormal myelin formation), many leukodystrophies also involve **secondary demyelination** (e.g., adrenoleukodystrophy, metachromatic leukodystrophy, Krabbe disease) [4]. - The distinction is that the primary defect is in myelin metabolism or formation, whereas in MS the primary process is inflammatory demyelination of previously normal myelin. - Not the best answer as demyelination is secondary rather than the primary pathological process. *Human leukoencephalopathy* - This is an overly broad, non-specific term referring to any disease affecting the **white matter of the brain**. - Can encompass demyelinating conditions, dysmyelinating disorders, and other white matter abnormalities. - Not a specific diagnosis and too vague to be the best answer. *AIDS* - **AIDS (Acquired Immunodeficiency Syndrome)** itself does not directly cause demyelination. - However, AIDS patients can develop **opportunistic infections** that cause demyelination, particularly **progressive multifocal leukoencephalopathy (PML)** caused by JC virus reactivation [3]. - Other neurological complications include **HIV-associated neurocognitive disorder (HAND)**, toxoplasmosis, and CNS lymphomas. - Demyelination in AIDS is due to secondary opportunistic infections, not a direct feature of HIV/AIDS. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1286-1287. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 714-715. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1280-1281. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1304-1305.

Question 293: All are congenital myopathies except

A. Polymyositis (Correct Answer)
B. Central Core myopathy
C. Centronuclear myopathy
D. Nemaline myopathy

Explanation: ***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.

Question 294: The most characteristic feature of Primary central nervous system lymphoma (PCNSL) is -

A. It is usually solitary
B. It is most commonly formed of T cells
C. It is seen in immunosuppressed patients (Correct Answer)
D. Multifocal lesions are seen

Explanation: ***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.

Question 295: Multiple sclerosis is characterized by all EXCEPT -

A. Demyelination
B. Grey-tan plaques in the white matter
C. Increased protein concentration in CSF
D. Oligodendrocytes (Correct Answer)

Explanation: ***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.

Question 296: All of the following are prion associated diseases except?

A. Alzheimer's disease (Correct Answer)
B. Kuru
C. Scrapie
D. Creutzfeldt-Jakob disease

Explanation: ***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.

Question 297: Which is the most common type of primary CNS lymphoma in AIDS Positive patients?

A. Lymphoplasmacytic Lymphoma
B. Immunoblastic Large cell lymphoma (Correct Answer)
C. Centroblastic large cell lymphoma
D. Anaplastic B-Cell Lymphoma

Explanation: ***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.

Question 298: Most common supratentorial tumor in children

A. Astrocytoma
B. Oligodendroglioma
C. Meningioma
D. Craniopharyngioma (Correct Answer)

Explanation: ***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.

Question 299: The site of occurrence of Subependymal Giant Cell Astrocytoma is

A. Medulla Oblongata
B. Cerebellum
C. Pineal gland
D. Foramen of Monro (Correct Answer)

Explanation: ***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.

Question 300: Which of the following is the most frequent primary malignant tumor of the CNS?

A. Glioblastoma multiforme (Correct Answer)
B. Oligodendroglioma
C. Medulloblastoma
D. Meningioma

Explanation: ***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.

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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