Neuropathology — MCQs

Neuropathology Indian Medical PG Practice Questions and MCQs

Question 301: Porencephaly refers to -

A. Neural tube defects
B. Fetal alcohol syndrome
C. Dandy-Walker syndrome
D. Vascular lesions due to degenerative vessel disease and head injury (Correct Answer)

Explanation: ***Vascular lesions due to degenerative vessel disease and head injury*** - **Porencephaly** refers to cysts or cavities within the brain parenchyma, which may communicate with the ventricular system or subarachnoid space. - Porencephaly can be **congenital** (developmental) or **acquired** (destructive). This option describes **acquired porencephaly**, which results from destructive processes such as **ischemic or hemorrhagic strokes**, **perinatal hypoxic-ischemic injury**, and **traumatic brain injury** [1]. - These vascular insults and trauma cause tissue necrosis and subsequent cyst formation, which is the hallmark of porencephalic cavities. *Neural tube defects* - These are **congenital malformations** resulting from incomplete closure of the neural tube during early embryonic development (weeks 3-4). - Examples include **spina bifida** and **anencephaly**, which represent failures of neural tube closure rather than destructive cystic lesions in formed brain tissue. *Fetal alcohol syndrome* - This condition results from **maternal alcohol consumption** during pregnancy and causes a spectrum of physical, neurodevelopmental, and behavioral abnormalities. - While it may cause brain structural abnormalities (microcephaly, corpus callosum abnormalities), it does not typically manifest as focal **porencephalic cysts**. *Dandy-Walker syndrome* - This is a **congenital posterior fossa malformation** characterized by hypoplasia/agenesis of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlarged posterior fossa. - It represents a developmental anomaly of the cerebellum, not a destructive cystic lesion of the cerebral hemispheres. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1260-1261.

Question 302: False regarding Alzheimer's disease (AD) is:

A. Number of neurofibrillary tangles is associated with the severity of dementia
B. Number of senile (neuritic) plaques correlates (increases) with age
C. Presence of tau protein suggest neurodegeneration
D. Extracellular inclusion (lesion) can occur in the absence of intracellular inclusions to make pathological diagnosis of AD (Correct Answer)

Explanation: ***Extracellular inclusion (lesion) can occur in the absence of intracellular inclusions to make pathological diagnosis of AD*** - A definitive pathological diagnosis of **Alzheimer's disease** requires both the presence of **extracellular amyloid plaques** and **intracellular neurofibrillary tangles** [1]. - Neither inclusion type alone is sufficient for the diagnosis, as amyloid plaques can be found in non-demented elderly individuals [1]. *Number of neurofibrillary tangles is associated with the severity of dementia* - The **density and distribution of neurofibrillary tangles** (NFTs) directly correlate with the severity of cognitive impairment and **dementia** in AD [1]. - Tangles are composed of hyperphosphorylated **tau protein** and disrupt neuronal function, leading to neurodegeneration [2]. *Number of senile (neuritic) plaques correlates (increases) with age* - The accumulation of **senile (neuritic) plaques**, composed primarily of **beta-amyloid protein**, generally increases with age, even in cognitively normal individuals [1]. - While plaques are a hallmark of AD, their mere presence is not always diagnostic of clinical dementia [1]. *Presence of tau protein suggest neurodegeneration* - The presence of **hyperphosphorylated tau protein**, especially when forming **neurofibrillary tangles**, is a strong indicator of **neurodegeneration** [2]. - **Tauopathy** is a key pathological feature in AD and other neurodegenerative diseases [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1294. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722.

Question 303: Which part of brain shows consistent changes in heat stroke

A. Hippocampus
B. Purkinje cells (Correct Answer)
C. Midbrain
D. Cerebral cortex

Explanation: ***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**.

Question 304: Most common site of subependymal astrocytoma is:

A. Trigone of lateral ventricle
B. Foramen of Munro (Correct Answer)
C. Temporal horn of lateral ventricle
D. 4th ventricle

Explanation: ***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.

Question 305: Which of the following is characterized by denervation atrophy of the muscles?

A. Werdnig-Hoffman disease (Correct Answer)
B. Carnitine palmityl transferase deficiency
C. McArdle disease
D. Pompe disease

Explanation: ***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.

Question 306: Negri bodies are not found in which part of CNS?

A. White matter (Correct Answer)
B. Basal ganglia
C. Purkinje cells
D. Hippocampus

Explanation: **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 307: Suprasellar cystic mass in children is –

A. Craniopharyngioma (Correct Answer)
B. Medulloblastoma
C. Secondaries
D. Meningioma

Explanation: ***Craniopharyngioma*** - **Craniopharyngiomas** are the most common suprasellar tumors in children and are frequently **cystic with calcifications**, making them a prime consideration for a suprasellar cystic mass. - They arise from remnants of Rathke's pouch and often present with symptoms related to **pituitary hormone deficiencies** and **visual field defects** due to compression of the optic chiasm. *Medulloblastoma* - **Medulloblastomas** are typically located in the **posterior fossa** (cerebellum) of children, not the suprasellar region. - They are usually **solid, highly malignant tumors** and do not characteristically present as cystic suprasellar masses. *Secondaries* - **Metastatic tumors (secondaries)** to the brain, particularly to the suprasellar region, are **rare in children** and typically present as solid masses, not primarily cystic. - When they do occur, they usually originate from primary cancers like leukemia or sarcoma, which would have other systemic manifestations. *Meningioma* - **Meningiomas** are tumors that arise from the **meninges**, most commonly in adults, and while they can occur near the sella, they are typically **solid, dural-based tumors** and are very rare in children. - They do not usually present as a primary cystic suprasellar mass.

Question 308: Which part of spinal cord is involved in poliomyelitis?

A. Posterior column
B. Lateral column
C. Anterior horn (Correct Answer)
D. Posterior horn

Explanation: ***Anterior horn*** - Poliomyelitis specifically targets and destroys the **motor neurons** located in the **anterior horns** of the spinal cord [1]. - Damage to these motor neurons leads to characteristic **flaccid paralysis** and muscle weakness [1]. *Posterior column* - The posterior column is primarily responsible for **fine touch**, **vibration**, and **proprioception**. - While it can be affected in some neurological conditions, it is **not the primary site of damage** in poliomyelitis. *Lateral column* - The lateral column contains descending motor tracts like the **corticospinal tract**, responsible for **voluntary movement**, and ascending sensory tracts such as those for **pain and temperature**. - Although motor deficits occur in poliomyelitis, the primary lesion is in the motor neuron cell bodies, not the descending tracts within the lateral column. *Posterior horn* - The posterior horn mainly processes **sensory information** entering the spinal cord. - It is **not directly involved** in the pathogenesis of poliomyelitis, which primarily affects motor function. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 66-67.

Question 309: Loss of striatal fibres in caudate nucleus is associated with?

A. Hemiballismus
B. Huntington's disease (Correct Answer)
C. Charcot-Marie-Tooth disease
D. Parkinson's disease

Explanation: ***Huntington's disease*** - This neurodegenerative disorder is pathologically characterized by **atrophy of the striatum**, particularly the **caudate nucleus** [1]. - The loss of striatal neurons, especially medium spiny neurons, leads to the characteristic **chorea** and cognitive decline [1]. *Hemiballismus* - Characterized by **unilateral, violent, flinging movements** of the limbs. - It is typically caused by a lesion in the **subthalamic nucleus**, not the caudate nucleus. *Charcot-Marie-Tooth disease* - A group of inherited disorders that affect the **peripheral nerves**, leading to muscle weakness and sensory loss. - This condition does not involve the degeneration of the striatal fibers in the caudate nucleus. *Parkinson's disease* - Primarily caused by the degeneration of **dopaminergic neurons** in the **substantia nigra pars compacta**. - While it affects the basal ganglia circuitry, its primary pathology is not the loss of striatal fibers in the caudate nucleus but rather a **dopamine deficiency**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1299-1300.

Question 310: Fluid-filled cavity at the center of the spinal cord is seen in:

A. Subacute combined degeneration (SACD) of cord
B. Brown Sequard syndrome
C. Tabes dorsalis
D. Syringomyelia (Correct Answer)

Explanation: ***Syringomyelia*** - **Syringomyelia** is characterized by the formation of a **syrinx**, a fluid-filled cavity or cyst, within the spinal cord, most commonly in the cervical region - This cavity expands over time, compressing and damaging nerve fibers from the inside out, leading to progressive neurological deficits - Classic presentation includes **dissociated sensory loss** (loss of pain and temperature sensation with preserved touch and proprioception) in a "cape-like" distribution *Subacute combined degeneration (SACD) of cord* - SACD is primarily caused by **Vitamin B12 deficiency** and involves demyelination of the **dorsal and lateral columns** of the spinal cord [1] - Does not present with a fluid-filled cavity but rather with neuronal degeneration and demyelination [1] - Clinical features include both sensory ataxia (dorsal column) and spastic paraparesis (lateral corticospinal tract) [2] *Brown Sequard syndrome* - Results from **hemitransection (damage to one side)** of the spinal cord, typically from trauma or mass lesions - Leads to ipsilateral motor paralysis and loss of proprioception, with contralateral loss of pain and temperature sensation - Involves damage to specific tracts rather than formation of a central cavity *Tabes dorsalis* - **Tabes dorsalis** is a late manifestation of **tertiary syphilis**, causing degeneration of the **dorsal columns** and dorsal nerve roots - Characterized by sensory ataxia, lightning pains, Argyll Robertson pupils, and Charcot joints - Does not involve a fluid-filled cavity but rather progressive demyelination **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 716-717. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1306-1307.

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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