Neuropathology Practice Questions

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.

Q: Suprasellar cystic mass in children is –

Craniopharyngioma. ***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.

Q: Which part of spinal cord is involved in poliomyelitis?

Anterior horn. ***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.

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

Huntington's disease. ***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.

Q: Which of the following is associated with bilateral eighth nerve neuromas?

Neurofibromatosis II. ***Neurofibromatosis II*** - **Bilateral vestibular schwannomas** (eighth nerve neuromas) are the hallmark diagnostic criterion for Neurofibromatosis type 2 (NF2) [1]. - NF2 is an **autosomal dominant** disorder caused by a mutation in the **NF2 gene** on chromosome 22. *Tuberous sclerosis* - This condition is characterized by **hamartomas** in multiple organs, including the brain, skin, kidneys, and heart. - It does not typically involve the formation of **eighth nerve neuromas**. *Neurofibromatosis I* - NF1 is primarily associated with **café-au-lait spots**, **cutaneous neurofibromas**, and **Lisch nodules**. - While NF1 can lead to various neurological tumors, **bilateral eighth nerve neuromas** are not a characteristic feature; unilateral vestibular schwannomas can rarely occur but are much more common in NF2 [1]. *All of the options* - Only **Neurofibromatosis II** is specifically associated with bilateral eighth nerve neuromas as a defining feature. - Tuberous Sclerosis and Neurofibromatosis I do not typically present with this finding.

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

Syringomyelia. ***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.

Q: Which brain tumor has the worst prognosis in children?

Brainstem glioma. ***Brainstem glioma*** - **Diffuse intrinsic pontine gliomas (DIPG)** are particularly aggressive, typically unresponsive to conventional therapies, and have a median survival of less than one year. - Their critical location in the **brainstem** makes surgical resection extremely difficult and often impossible without causing severe neurological deficits [2]. *Craniopharyngioma* - While they can be challenging to treat due to their proximity to vital structures like the **optic chiasm** and **hypothalamus**, they are generally benign and have a good prognosis with complete surgical resection. - They tend to recur if not completely removed, but recurrence is often amenable to further treatment. *Cerebellar astrocytoma* - These are often **low-grade (pilocytic astrocytomas)** and have an excellent prognosis, especially if surgical gross total resection is achieved [2]. - They are usually cystic and well-demarcated, making them more amenable to curative surgical removal. *Pineal body tumor* - The prognosis of pineal region tumors varies greatly depending on the **histology** (e.g., germinoma, pineoblastoma, pineocytoma) [1]. - While some are aggressive (like pineoblastomas), a significant portion, such as germinomas, are highly sensitive to **radiation therapy** and have a relatively good prognosis [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1140-1141. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.

Question 1

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 2

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 3

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 4

Negri bodies are not found in which part of CNS?

Accepted Answer

White matter

Answer

Basal ganglia

Answer

Purkinje cells

Answer

Hippocampus

Question 5

Suprasellar cystic mass in children is –

Accepted Answer

Craniopharyngioma

Answer

Medulloblastoma

Answer

Secondaries

Answer

Meningioma

Question 6

Which part of spinal cord is involved in poliomyelitis?

Accepted Answer

Anterior horn

Answer

Posterior column

Answer

Lateral column

Answer

Posterior horn

Question 7

Loss of striatal fibres in caudate nucleus is associated with?

Accepted Answer

Huntington's disease

Answer

Hemiballismus

Answer

Charcot-Marie-Tooth disease

Answer

Parkinson's disease

Question 8

Which of the following is associated with bilateral eighth nerve neuromas?

Accepted Answer

Neurofibromatosis II

Answer

Tuberous sclerosis

Answer

Neurofibromatosis I

Answer

All of the options

Question 9

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

Accepted Answer

Syringomyelia

Answer

Subacute combined degeneration (SACD) of cord

Answer

Brown Sequard syndrome

Answer

Tabes dorsalis

Question 10

Which brain tumor has the worst prognosis in children?

Accepted Answer

Brainstem glioma

Answer

Craniopharyngioma

Answer

Cerebellar astrocytoma

Answer

Pineal body tumor

Neuropathology — MCQs

Neuropathology — MCQs

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