Neuropathology Practice Questions

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.

Q: Lumbar puncture was done in a patient with raised intracranial tension. The patient died suddenly on the table. The cause of the death is most likely to be

Tentorial herniation. ***Tentorial herniation*** - In a patient with **raised intracranial pressure (ICP)**, performing a lumbar puncture can cause a rapid drop in spinal CSF pressure, creating a pressure gradient that forces the **brainstem** through the **tentorial notch**. [1] - This sudden compression of the brainstem, specifically the **medulla oblongata**, can lead to **respiratory and circulatory center dysfunction**, resulting in rapid death. [1] *Rupture of an aneurysm* - While a ruptured aneurysm can cause sudden death, it typically presents with a sudden, severe headache (**thunderclap headache**) and neurological deficits, not directly as a result of a lumbar puncture in this context. [2] - Aneurysm rupture is a spontaneous event and not directly induced by a change in CSF pressure during a lumbar puncture. *Middle cerebral artery hemorrhage* - A **middle cerebral artery (MCA) hemorrhage** would present with focal neurological deficits such as hemiparesis, aphasia, or neglect, depending on the affected hemisphere. - It is a type of stroke and not a direct complication of a lumbar puncture in a patient with raised ICP. *Loss of CSF* - While **CSF leakage** is a potential complication of a lumbar puncture, it typically leads to a **post-lumbar puncture headache** (low-pressure headache) due to reduced intracranial pressure. - It does not directly cause sudden death on the table, as the primary risk in raised ICP is herniation, not the mere loss of CSF volume.

Q: The characteristic feature of Lhermitte-Duclos disease is:

Thickened cerebellar folia. ***Thickened cerebellar folia*** - **Lhermitte-Duclos disease**, also known as **dysplastic gangliocytoma of the cerebellum**, is characterized by a **hamartomatous overgrowth** of the granular layer of the cerebellum. - This overgrowth leads to **thickened, abnormal cerebellar folia** that can exert mass effect on surrounding structures. *Atrophic cerebellar folia* - **Cerebellar atrophy** involves the loss of cerebellar tissue, resulting in **thinned or shrunken folia**, which is the opposite of Lhermitte-Duclos disease. - Atrophy is typically seen in neurodegenerative conditions, chronic alcohol abuse, or certain genetic disorders. *Septum pellucidum agenesis* - **Septum pellucidum agenesis** is the absence of the septum pellucidum, a thin membrane separating the lateral ventricles, and is associated with conditions like **septo-optic dysplasia** or certain chromosome abnormalities. - This is a structural anomaly of the forebrain, **unrelated to cerebellar pathology** like Lhermitte-Duclos disease. *Vermian hypoplasia* - **Vermian hypoplasia** refers to the **underdevelopment of the cerebellar vermis**, a central part of the cerebellum, often seen in conditions like **Dandy-Walker malformation** or Joubert syndrome. - While it affects cerebellar development, it is primarily a **volume reduction** rather than a specific dysplastic overgrowth of the folia.

Q: Which part of brain shows consistent changes in heat stroke

Purkinje cells. ***Purkinje cells*** - **Purkinje cells** in the cerebellum are particularly vulnerable to **hyperthermia** due to their high metabolic rate and sensitivity to oxidative stress. - Consistent changes, including **necrosis** and **apoptosis**, are often observed in these cells during heat stroke. *Hippocampus* - While the **hippocampus** can be affected in severe heat stroke, showing neuronal damage, it is **not as consistently or uniquely vulnerable** as Purkinje cells. - Damage to the hippocampus is often associated with more generalized **hypoxic-ischemic injury** seen in severe cases. *Midbrain* - The **midbrain** is part of the brainstem and primarily responsible for functions like motor control, vision, and hearing, and typically shows **less consistent or specific damage** in heat stroke compared to Purkinje cells. - Injury to the midbrain during heat stroke is usually a consequence of **widespread cerebral edema** or global anoxia, rather than a primary effect of hyperthermia. *Cerebral cortex* - Damage to the **cerebral cortex** in heat stroke can occur, leading to cognitive dysfunction and seizures, but it is **not as consistently affected** across all cases as Purkinje cells. - Cortical damage is more often linked to severe and prolonged hyperthermia or secondary complications like **cerebral edema**.

Q: Most common site of subependymal astrocytoma is:

Foramen of Munro. ***Foramen of Munro*** - This is the most common site for **subependymal giant cell astrocytomas (SEGA)**. - Tumors at this location often cause **hydrocephalus** due to obstruction of **CSF flow** from the lateral ventricles to the third ventricle [1]. *Trigone of lateral ventricle* - While it's a part of the lateral ventricle, the **trigone** is not the most frequent site for SEGA. - Tumors here are less likely to cause early **hydrocephalus** compared to those at the **Foramen of Munro** [1]. *Temporal horn of lateral ventricle* - This region is an uncommon site for the primary development of SEGA. - Tumors in the **temporal horn** are less likely to obstruct **CSF flow** at the level of the Foramen of Munro [1]. *4th ventricle* - The **4th ventricle** is located in the **brainstem**, far from the typical occurrence of SEGA, which are usually found in the lateral ventricles. - Tumors in the 4th ventricle would primarily cause **posterior fossa symptoms** and different patterns of **hydrocephalus** [1]. **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.

Q: Which of the following is characterized by denervation atrophy of the muscles?

Werdnig-Hoffman disease. ***Werdnig-Hoffman disease*** - This is a severe form of **spinal muscular atrophy (SMA)**, characterized by the degeneration of **anterior horn cells** in the spinal cord [1]. - The loss of motor neurons leads to **denervation atrophy** of skeletal muscles, resulting in profound weakness and hypotonia [1], [2]. *Carnitine palmityl transferase deficiency* - This is a **fatty acid oxidation disorder** that primarily affects muscle energy metabolism. - It causes muscle pain, weakness, and **rhabdomyolysis** during sustained exercise, but not denervation atrophy. *McArdle disease* - Also known as **glycogen storage disease type V**, this condition is caused by a deficiency in **myophosphorylase**. - It results in exercise intolerance, muscle cramps, and myoglobinuria, but the muscle damage is metabolic, not from denervation. *Pompe disease* - This is a **lysosomal storage disorder** caused by a deficiency of **acid alpha-glucosidase (GAA)**. - It leads to the accumulation of glycogen in lysosomes, causing muscle weakness, cardiomyopathy, and respiratory failure, but the muscle pathology is due to lysosomal dysfunction, not denervation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1239-1240, 1247-1248. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 730-731.

Q: Mad Cow disease (Bovine Spongioform Encephalopathy) is similar in man to

Creutzfeldt Jakob Disease. ***Creutzfeldt Jakob Disease*** - **Creutzfeldt-Jakob Disease (CJD)** in humans is a **transmissible spongiform encephalopathy (TSE)**, caused by **prions**, which is pathophysiologically similar to Bovine Spongiform Encephalopathy (BSE) or "Mad Cow Disease" in cattle [2]. - The **variant CJD (vCJD)**, specifically linked to consuming beef products from BSE-infected cattle, shares similar pathological features, including widespread **spongiform changes** in the brain [1], [2]. *Huntington's chorea* - **Huntington's chorea** is an **autosomal dominant neurodegenerative disorder** caused by a genetic mutation leading to an expansion of CAG trinucleotide repeats, resulting in the aggregation of **huntingtin protein**. - Its pathology involves specific neuronal loss in the **striatum** and cortex, lacking the prion-induced spongiform changes seen in CJD. *Alzheimer's Disease* - **Alzheimer's Disease** is characterized by the accumulation of **amyloid plaques** (extracellular deposits of beta-amyloid protein) and **neurofibrillary tangles** (intracellular aggregates of hyperphosphorylated tau protein) [4]. - It is not a prion disease and does not involve the characteristic spongiform changes or transmission pathways associated with CJD [3]. *Pick's Disease* - **Pick's Disease** is a type of **frontotemporal dementia** distinguished by the presence of **Pick bodies**, which are intracellular aggregates of **hyperphosphorylated tau protein**. - Unlike CJD, it is not a prion disease and primarily affects the frontal and temporal lobes, causing specific types of dementia and behavioral changes without spongiform encephalopathy.

Q: Negri bodies are not found in which part of CNS?

White matter. **White matter** - **Negri bodies** are eosinophilic inclusion bodies found in the cytoplasm of neurons infected with rabies virus [1]. - They are typically concentrated in the **grey matter** of the brain, as this is where neuronal cell bodies are primarily located. *Basal ganglia* - The **basal ganglia** are part of the **grey matter** of the brain, containing numerous neuronal cell bodies. - As such, **Negri bodies** can be found in the neurons within the basal ganglia in rabies infection [1]. *Purkinje cells* - **Purkinje cells** are large, distinctive neurons found in the **cerebellar cortex**, which is a region of **grey matter**. - They are a common site for the detection of **Negri bodies** in rabies [1]. *Hippocampus* - The **hippocampus** is a major component of the brain's **grey matter**, containing a high density of neurons. - **Negri bodies** are frequently observed in the neurons of the hippocampus, especially the **pyramidal cells**, and this is considered a preferential site for their formation [1]. **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 1

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

Accepted Answer

Glioblastoma multiforme

Answer

Oligodendroglioma

Answer

Medulloblastoma

Answer

Meningioma

Question 2

Lumbar puncture was done in a patient with raised intracranial tension. The patient died suddenly on the table. The cause of the death is most likely to be

Accepted Answer

Tentorial herniation

Answer

Rupture of an aneurysm

Answer

Middle cerebral artery hemorrhage

Answer

Loss of CSF

Question 3

Porencephaly refers to -

Accepted Answer

Vascular lesions due to degenerative vessel disease and head injury

Answer

Neural tube defects

Answer

Fetal alcohol syndrome

Answer

Dandy-Walker syndrome

Question 4

The characteristic feature of Lhermitte-Duclos disease is:

Accepted Answer

Thickened cerebellar folia

Answer

Atrophic cerebellar folia

Answer

Septum pellucidum agenesis

Answer

Vermian hypoplasia

Question 5

False regarding Alzheimer's disease (AD) is:

Accepted Answer

Extracellular inclusion (lesion) can occur in the absence of intracellular inclusions to make pathological diagnosis of AD

Answer

Number of neurofibrillary tangles is associated with the severity of dementia

Answer

Number of senile (neuritic) plaques correlates (increases) with age

Answer

Presence of tau protein suggest neurodegeneration

Question 6

Which part of brain shows consistent changes in heat stroke

Accepted Answer

Purkinje cells

Answer

Hippocampus

Answer

Midbrain

Answer

Cerebral cortex

Question 7

Most common site of subependymal astrocytoma is:

Accepted Answer

Foramen of Munro

Answer

Trigone of lateral ventricle

Answer

Temporal horn of lateral ventricle

Answer

4th ventricle

Question 8

Which of the following is characterized by denervation atrophy of the muscles?

Accepted Answer

Werdnig-Hoffman disease

Answer

Carnitine palmityl transferase deficiency

Answer

McArdle disease

Answer

Pompe disease

Question 9

Mad Cow disease (Bovine Spongioform Encephalopathy) is similar in man to

Accepted Answer

Creutzfeldt Jakob Disease

Answer

Huntington's chorea

Answer

Alzheimer's Disease

Answer

Pick's Disease

Question 10

Negri bodies are not found in which part of CNS?

Accepted Answer

White matter

Answer

Basal ganglia

Answer

Purkinje cells

Answer

Hippocampus

Neuropathology — MCQs

Neuropathology — MCQs

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