Neuropathology Practice Questions

Q: Confirmatory diagnosis of rabies on postmortem:

Negri bodies in brain. ***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.

Q: Diffuse axonal injury is characterized by lesion at:

Junction of gray and white matter. ***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.

Q: Which of the following is the most common tumor of the brain?

Cerebral metastasis. ***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.

Q: All of the following are true about Status marmoratus:

All of the options. ***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.

Q: Progressive multifocal leukoencephalopathy spares:

Spinal cord and optic nerve. ***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.

Q: Neurofibrillary tangles are associated with:

Alzheimer's disease. ***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.

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

Muscular dystrophy. ***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.

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.

Question 1

Confirmatory diagnosis of rabies on postmortem:

Accepted Answer

Negri bodies in brain

Answer

Negri bodies in saliva

Answer

Anti-rabies antibodies in blood

Answer

Negri bodies in corneal scrapings

Question 2

Diffuse axonal injury is characterized by lesion at:

Accepted Answer

Junction of gray and white matter

Answer

Basal ganglia

Answer

Corpus callosum

Answer

White matter

Question 3

The CSF in a patient with multiple sclerosis will typically exhibit

Accepted Answer

Elevated immunoglobulin content, including oligoclonal bands

Answer

Mildly elevated WBC count (less than 50 cells/μL)

Answer

Normal glucose levels in CSF

Answer

Mildly elevated total protein content

Question 4

Which of the following is the MOST accurate statement regarding medulloblastoma?

Accepted Answer

Spreads through CSF

Answer

Occurs in young age group

Answer

Radiosensitive tumor

Answer

It is a supratentorial tumor

Question 5

Which of the following is the most common tumor of the brain?

Accepted Answer

Cerebral metastasis

Answer

Meningioma

Answer

Glioma

Answer

Pituitary adenoma

Question 6

All of the following are true about Status marmoratus:

Accepted Answer

All of the options

Answer

Associated with asphyxia

Answer

Present in basal ganglia

Answer

Have a marbled appearance

Question 7

Progressive multifocal leukoencephalopathy spares:

Accepted Answer

Spinal cord and optic nerve

Answer

White matter of periventricular area

Answer

White matter of parietal lobe

Answer

White matter of cerebrum

Question 8

Neurofibrillary tangles are associated with:

Accepted Answer

Alzheimer's disease

Answer

Bipolar disorder

Answer

Schizophrenia

Answer

Multiple infarctions

Question 9

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

Accepted Answer

Muscular dystrophy

Answer

Denervation of muscle with reinnervation

Answer

Denervation atrophy

Answer

Mitochondrial myopathy

Question 10

CNS tumor seen in Von Hippel Lindau syndrome is:

Accepted Answer

Cerebellar hemangioblastoma

Answer

Meningioma

Answer

Glioma

Answer

CNS lymphoma

Neuropathology — MCQs

Neuropathology — MCQs

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