Neuropathology — MCQs

Neuropathology Indian Medical PG Practice Questions and MCQs

Question 281: Confirmatory diagnosis of rabies on postmortem:

A. Negri bodies in saliva
B. Anti-rabies antibodies in blood
C. Negri bodies in brain (Correct Answer)
D. Negri bodies in corneal scrapings

Explanation: ***Negri bodies in brain*** - The presence of **Negri bodies** (eosinophilic intracytoplasmic inclusions) found upon histological examination of brain tissue (specifically **Purkinje cells of the cerebellum** and **pyramidal cells of the hippocampus**) is the **pathognomonic microscopic finding** for rabies [1]. - This is considered the **gold standard for postmortem confirmation** because the rabies virus primarily targets and replicates in neuronal tissue, leading to these characteristic inclusions. *Negri bodies in saliva* - While rabies virus can be present in saliva, the presence of **Negri bodies** themselves in saliva is **not a diagnostic criterion**. - **Viral isolation** or **PCR** from saliva might detect the virus, but Negri bodies are cellular inclusions, not free virus particles. *Anti-rabies antibodies in blood* - The presence of **anti-rabies antibodies** in blood typically indicates **prior exposure or vaccination**, not necessarily an active, fatal infection as required for a postmortem diagnosis. - In unvaccinated individuals with clinical rabies, antibodies may only appear in the very late stages, if at all, due to the rapid progression of the disease and the virus's evasion of the immune system in the CNS. *Negri bodies in corneal scrapings* - While rabies virus antigens can sometimes be detected in **corneal impressions or scrapings** during life using **fluorescent antibody tests**, Negri bodies are not typically found or used for diagnosis in these samples [1]. - Corneal testing is primarily a **pre-mortem diagnostic aid** for antigen detection, not for visualizing Negri bodies. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1279-1280.

Question 282: Diffuse axonal injury is characterized by lesion at:

A. Basal ganglia
B. Corpus callosum
C. White matter
D. Junction of gray and white matter (Correct Answer)

Explanation: ***Junction of gray and white matter*** - Diffuse axonal injury (DAI) is characteristically located at the **gray-white matter junction** (interface between cortex and subcortical white matter) [1] - The differential density and movement between gray and white matter during **rotational acceleration-deceleration injuries** creates maximum shearing forces at this junction [1] - This is the **classic and most characteristic location** of DAI lesions, particularly in the **parasagittal white matter** of cerebral hemispheres - Other common sites include corpus callosum and dorsolateral brainstem, but the gray-white junction is the hallmark location *White matter* - While DAI does involve damage to white matter tracts and axons, simply stating "white matter" is too non-specific [1] - Many other conditions affect white matter (demyelination, ischemia, etc.) - The **distinguishing feature of DAI** is its predilection for the gray-white interface, not white matter in general *Corpus callosum* - The corpus callosum is indeed a common site for **visible macroscopic DAI lesions** and hemorrhages [1] - However, this is just one specific location rather than the characteristic pattern - DAI is more broadly characterized by lesions at gray-white junctions throughout the brain *Basal ganglia* - The basal ganglia are deep gray matter structures not typically involved in classic DAI - These structures may be affected by other traumatic injuries like **contusions** or **deep hemorrhages** - DAI predominantly affects the interface zones and white matter tracts, not deep gray matter nuclei **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1262-1264.

Question 283: The CSF in a patient with multiple sclerosis will typically exhibit

A. Elevated immunoglobulin content, including oligoclonal bands (Correct Answer)
B. Mildly elevated WBC count (less than 50 cells/μL)
C. Normal glucose levels in CSF
D. Mildly elevated total protein content

Explanation: ***Elevated immunoglobulin content, including oligoclonal bands*** - The presence of **oligoclonal bands** in the CSF, which are absent in the serum, is a hallmark of **intrathecal immunoglobulin production** and is found in over 90% of MS patients. - This indicates a **clonal expansion of plasma cells** within the central nervous system, characteristic of the inflammatory demyelination in MS [1]. *Mildly elevated WBC count (less than 50 cells/μL)* - While there can be a **mild elevation in CSF white blood cell count** in MS, it typically ranges from **5-20 cells/μL**, and rarely exceeds 50 cells/μL. - A WBC count greater than 50 cells/μL should prompt investigation for **alternative diagnoses** such as infection or other inflammatory conditions [1]. *Normal glucose levels in CSF* - **CSF glucose levels are typically normal** in patients with multiple sclerosis, as MS is a demyelinating disease and does not primarily affect glucose metabolism or transport. - **Decreased CSF glucose** would be more suggestive of bacterial meningitis, fungal meningitis, or carcinomatosis. *Mildly elevated total protein content* - **Total protein in CSF may be normal or mildly elevated** in MS, usually not exceeding **100 mg/dL** [1]. - Significantly elevated protein levels would point towards other conditions like **guillain-barre syndrome**, chronic meningitis, or spinal cord compression. *Elevated Kappa light chains and oligoclonal bands in CSF* - While **oligoclonal bands** are characteristic of MS, the statement specifically emphasizing **elevated Kappa light chains** in conjunction with oligoclonal bands is less precise as the primary diagnostic finding. - **Kappa free light chains** can be elevated in MS, reflecting intrathecal antibody synthesis, and their presence can complement oligoclonal bands in diagnosis, but the **oligoclonal bands themselves are the more established and direct marker** of B-cell activity within the CNS. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1286-1288.

Question 284: Which of the following is the MOST accurate statement regarding medulloblastoma?

A. Occurs in young age group
B. Radiosensitive tumor
C. Spreads through CSF (Correct Answer)
D. It is a supratentorial tumor

Explanation: ***Spreads through CSF*** - Medulloblastoma is well-known for its propensity to spread via the **cerebrospinal fluid (CSF)**, leading to **leptomeningeal dissemination** throughout the neuroaxis [1], [2]. This characteristic spread pattern significantly impacts treatment planning and prognosis. - Due to this CSF spread, imaging of the **entire craniospinal axis** is crucial during diagnosis and staging to detect metastatic disease. *Occurs in young age group* - While medulloblastoma primarily affects **children and young adults**, particularly those under 10 years old, this statement is not the *most accurate* as it doesn't highlight a unique or pathognomonic feature like CSF seeding. - It is one of the most common **malignant brain tumors in childhood** [2], but the age prevalence alone does not define its key biological behavior. *Radiosensitive tumor* - Medulloblastoma is indeed a **highly radiosensitive tumor**, responding well to radiation therapy, which is a cornerstone of its treatment [1]. - However, its radiosensitivity, while important for treatment, is not as singularly defining of its biology or clinical behavior as its distinctive CSF dissemination. *Surgical Rx not done* - This statement is incorrect; **surgical resection** is the **initial and primary treatment** for medulloblastoma, aiming for maximal safe tumor removal [1]. - The extent of surgical resection is a significant prognostic factor, often followed by adjuvant chemotherapy and radiotherapy. *It is a supratentorial tumor* - This statement is incorrect; medulloblastoma arises in the **cerebellum** [1], which is located in the **posterior cranial fossa**, making it an **infratentorial tumor**. - Its typical location in the cerebellar vermis explains common presenting symptoms such as **hydrocephalus** and **ataxia**. **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] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-727.

Question 285: Which of the following is the most common tumor of the brain?

A. Cerebral metastasis (Correct Answer)
B. Meningioma
C. Glioma
D. Pituitary adenoma

Explanation: ***Cerebral metastasis*** - **Cerebral metastases** are the most common type of brain tumor overall, significantly outnumbering all primary brain tumors combined [1]. - They originate from cancers elsewhere in the body (most commonly **lung, breast, melanoma, renal, and colorectal carcinomas**) and spread to the brain via the bloodstream [1]. - Metastases typically occur at the **gray-white matter junction** and are often **multiple**. *Meningioma* - **Meningiomas** are the most common *primary* **benign** brain tumors, originating from arachnoid cap cells of the meninges [2]. - They account for ~30% of primary intracranial tumors but are still less frequent than metastatic brain tumors overall. - More common in **middle-aged females** and typically slow-growing [2]. *Glioma* - **Gliomas** are common *primary* **malignant** brain tumors, arising from glial cells (astrocytes, oligodendrocytes, or ependymal cells) [3]. - **Glioblastoma** is the most common primary malignant brain tumor in adults, but all gliomas combined are still less common than metastatic brain lesions overall. *Pituitary adenoma* - **Pituitary adenomas** are common benign tumors of the sellar region, accounting for ~10-15% of intracranial tumors. - While frequent among primary tumors, they are less common than metastatic brain lesions overall. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1317-1318. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1316-1317. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726.

Question 286: All of the following are true about Status marmoratus:

A. Associated with asphyxia
B. Present in basal ganglia
C. Have a marbled appearance
D. All of the options (Correct Answer)

Explanation: ***All of the options*** - This is the correct answer because **all three statements accurately describe Status marmoratus**. - Status marmoratus is a well-defined neuropathological finding with characteristic features that include association with asphyxia, localization to basal ganglia, and marbled gross appearance [1]. **Key Features of Status Marmoratus:** *Associated with asphyxia* - Status marmoratus is a classic sequela of **perinatal hypoxic-ischemic injury** (birth asphyxia) [1]. - Results from severe and prolonged oxygen deprivation during the perinatal period [1]. - The hypoxic injury leads to selective neuronal loss in vulnerable brain regions. *Present in basal ganglia* - The characteristic lesions are located in the **basal ganglia** (especially putamen and caudate) and **thalamus** [2]. - These deep gray matter structures are particularly vulnerable to hypoxic-ischemic injury [2]. - This distribution explains the extrapyramidal movement disorders (choreoathetosis, dystonia) seen clinically [1]. *Have a marbled appearance* - The term "marmoratus" means **marbled** in Latin, describing the gross pathological appearance. - The marbling results from **abnormal myelination** in damaged areas mixed with **gliosis** and loss of neurons. - On sectioning, there is a distinctive pattern of white (myelinated fibers) streaking through gray matter, creating a marble-like appearance. - Microscopically shows neuronal loss, gliosis, and status dysmyelinatus. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1260-1261. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1266-1268.

Question 287: Progressive multifocal leukoencephalopathy spares:

A. Spinal cord and optic nerve (Correct Answer)
B. White matter of periventricular area
C. White matter of parietal lobe
D. White matter of cerebrum

Explanation: ***Spinal cord and optic nerve*** - Progressive multifocal leukoencephalopathy (PML) predominantly affects the **white matter** of the cerebral hemispheres [1]. - While PML primarily involves the brain, the **spinal cord and optic nerves** are **relatively spared** compared to cerebral white matter involvement, though rare cases of involvement have been reported. - This relative sparing distinguishes PML from other demyelinating conditions and represents the best answer among the given options. *White matter of periventricular area* - The **periventricular white matter** is a common and characteristic site for PML lesions, as the JC virus targets **oligodendrocytes** which are abundant in these areas [1]. - Lesions here lead to demyelination and neurological deficits directly related to the affected brain regions [1]. *White matter of parietal lobe* - The **parietal lobe white matter** is a frequent location for PML lesions, particularly in the subcortical regions, contributing to the diverse neurological symptoms observed in affected individuals. - These lesions often appear as non-enhancing, ill-defined areas on MRI, reflecting the demyelinating process. *White matter of cerebrum* - PML is fundamentally a disease of the **cerebral white matter**, causing widespread destruction of myelin sheaths in the subcortical regions [1]. - This widespread damage to the cerebral white matter leads to severe and progressive neurological dysfunction, while the **cortical gray matter is typically spared**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1280-1281.

Question 288: Neurofibrillary tangles are associated with:

A. Alzheimer's disease (Correct Answer)
B. Bipolar disorder
C. Schizophrenia
D. Multiple infarctions

Explanation: ***Alzheimer's disease*** - **Neurofibrillary tangles** are intracellular aggregates of hyperphosphorylated tau protein, a hallmark pathological feature of Alzheimer's disease [1]. - These tangles disrupt neuronal function and transport, leading to neuronal death and contributing to cognitive decline in **Alzheimer's pathology** [2]. *Bipolar disorder* - **Bipolar disorder** is a mood disorder characterized by shifts between manic and depressive episodes. - It is not primarily associated with macroscopic brain pathology like neurofibrillary tangles; rather, it involves complex neurochemical dysregulation and genetic factors. *Schizophrenia* - **Schizophrenia** is a chronic mental disorder characterized by psychosis, disorganized thought, and impaired social functioning. - While it involves structural and functional brain changes, **neurofibrillary tangles** are not characteristic pathological findings in schizophrenia. *Multiple infarctions* - **Multiple infarctions** refer to multiple small strokes that can lead to **vascular dementia** [1]. - The primary pathology is cerebrovascular damage and neuronal loss due to ischemia, not the presence of neurofibrillary tangles. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1294.

Question 289: Variation in size and shape of muscle fibers, combined with degenerative changes and intramysial fibrosis, is typical of

A. Denervation of muscle with reinnervation
B. Denervation atrophy
C. Muscular dystrophy (Correct Answer)
D. Mitochondrial myopathy

Explanation: ***Muscular dystrophy*** - The combination of **variation in muscle fiber size and shape**, **degenerative changes**, and **intramysial fibrosis** is a hallmark of muscular dystrophies [1]. - These conditions are characterized by progressive muscle weakness and degeneration, often with cycles of degeneration and attempted regeneration, leading to fibrosis [1]. *Denervation of muscle with reinnervation* - This typically leads to **fiber type grouping** as reinnervated fibers take on the characteristics of the reinnervating nerve. - While there may be some variation in fiber size, the prominent **degenerative changes** and **intramysial fibrosis** described are less characteristic of this process alone. *Denervation atrophy* - Primarily features widespread **atrophy of muscle fibers**, often angular in shape. - It lacks the prominent combination of **fiber size variation** and **significant degenerative changes with fibrosis**. *Mitochondrial myopathy* - Characterized by the presence of **ragged red fibers** on Gomori trichrome stain and often **lipid accumulation** due to mitochondrial abnormalities. - While there may be some fiber size variation, the description of widespread degenerative changes and intramysial fibrosis isn't the primary defining feature. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1244-1245.

Question 290: CNS tumor seen in Von Hippel Lindau syndrome is:

A. Meningioma
B. Glioma
C. CNS lymphoma
D. Cerebellar hemangioblastoma (Correct Answer)

Explanation: ***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.

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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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