Neuropathology Practice Questions

Q: CNS tumor seen in Von Hippel Lindau syndrome is:

Cerebellar hemangioblastoma. ***Cerebellar hemangioblastoma*** - **Cerebellar hemangioblastomas** are characteristic CNS tumors associated with **Von Hippel-Lindau syndrome** due to mutations in the VHL gene [1]. - Patients typically present with symptoms related to raised **intracranial pressure** or focal neurological deficits depending on the tumor's size and location. *Meningioma* - Meningiomas are **benign tumors** arising from the meninges and are generally not a primary feature of **Von Hippel-Lindau syndrome** [1]. - They are more commonly associated with **Neurofibromatosis type 2** if linked to a genetic syndrome [1]. *Glioma* - Gliomas, which include astrocytomas, oligodendrogliomas, and ependymomas, are a diverse group of brain tumors that are not typically or specifically linked to **Von Hippel-Lindau syndrome** [1]. - While central nervous system tumors, they lack the specific association seen with **hemangioblastomas** in VHL. *CNS lymphoma* - **CNS lymphoma** is primarily associated with **immunocompromised states**, such as HIV/AIDS, or occurs as a primary brain tumor in older adults [1]. - It has no known direct association with **Von Hippel-Lindau syndrome** [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-728.

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: Radiation-induced necrosis can be diagnosed by:

Biopsy. ***Biopsy*** - A **biopsy** is the definitive diagnostic method for radiation-induced necrosis, allowing for histological examination of tissue to confirm necrosis and rule out residual or recurrent tumor. [1], [2] - It provides a direct view of cellular changes, identifying **necrosis, atypical cells**, and ruling out **malignancy**. *MRI* - While **MRI** can show structural changes indicative of necrosis (e.g., mass effect, edema), it often cannot definitively differentiate between **radiation necrosis** and **tumor recurrence.** [2] - It often shows **T1 hypointensity** and **T2 hyperintensity**, but these findings are not specific. *CT* - **CT scans** are useful for detecting gross changes like **mass effect** and **edema** but have limited sensitivity for distinguishing necrosis from tumor recurrence. - It may show **low-density lesions** but lacks the resolution and specificity for precise diagnosis. *PET* - **PET scans** measure metabolic activity and can help distinguish between **tumor recurrence** (high uptake) and **radiation necrosis** (low uptake) in some cases. - However, false positives can occur, as some inflammatory processes in necrosis can also show increased uptake, making it **less definitive** than a biopsy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1307-1308. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 340-341.

Q: Histopathological feature of HIV encephalitis is/are-

Microglial nodules. ***Microglial nodules*** - **Microglial nodules** are a hallmark histopathological feature of **HIV encephalitis**, representing clusters of activated microglia and macrophages in the brain parenchyma [2]. - These nodules often contain **multinucleated giant cells**, which are believed to be formed by the fusion of HIV-infected macrophages and are pathognomonic for HIV encephalitis [1]. *Lewy body* - **Lewy bodies** are abnormal aggregates of protein (primarily **alpha-synuclein**) that develop inside nerve cells, primarily associated with **Parkinson's disease** and **Lewy body dementia**. - They are not characteristic of HIV encephalitis or other viral infections of the brain. *Fibrillary plaque* - **Fibrillary plaques**, specifically **amyloid plaques**, are extracellular deposits of aggregated **beta-amyloid protein** found in the brains of individuals with **Alzheimer's disease**. - These are a key feature of neurodegenerative conditions but are not seen in HIV encephalitis. *Negri body* - **Negri bodies** are eosinophilic, sharply demarcated neuronal cytoplasmic inclusions found in the pyramidal cells of the hippocampus and Purkinje cells of the cerebellum in individuals with **rabies**. - They are specific to rabies infection and are not associated with HIV encephalitis. **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] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1255-1256.

Question 1

CNS tumor seen in Von Hippel Lindau syndrome is:

Accepted Answer

Cerebellar hemangioblastoma

Answer

Meningioma

Answer

Glioma

Answer

CNS lymphoma

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

Radiation-induced necrosis can be diagnosed by:

Accepted Answer

Biopsy

Answer

MRI

Answer

CT

Answer

PET

Question 9

A 20-year-old woman complains of weakness that is worse in the afternoon, worse during prolonged activity, and improved by rest. When fatigued, the patient is unable to hold her head up or chew her food. She often notes diplopia when driving home from work. On physical examination, she has no loss of reflexes, sensation, or coordination. Which of the following is the likely pathogenesis of this disease?

Accepted Answer

Autoantibodies directed against the postsynaptic acetylcholine receptor causing neuromuscular transmission failure

Answer

Destruction of anterior horn cells by virus

Answer

Demyelinating disease

Answer

Progressive muscular atrophy caused by spinal degeneration

Question 10

Histopathological feature of HIV encephalitis is/are-

Accepted Answer

Microglial nodules

Answer

Lewy body

Answer

Fibrillary plaque

Answer

Negri body

Neuropathology — MCQs

Neuropathology — MCQs

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