Neuropathology — MCQs

Neuropathology Indian Medical PG Practice Questions and MCQs

Question 311: Which brain tumor has the worst prognosis in children?

A. Brainstem glioma (Correct Answer)
B. Craniopharyngioma
C. Cerebellar astrocytoma
D. Pineal body tumor

Explanation: ***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 312: All of the following statements about cerebellar astrocytomas in paediatric age group are true, EXCEPT:

A. These tumors have a good prognosis
B. These tumours are more common in females (Correct Answer)
C. These are usually Low grade tumors
D. These are more commonly seen in the 1st and 2nd decades

Explanation: ***These tumours are more common in females*** - Cerebellar astrocytomas, particularly **pilocytic astrocytomas (PA)**, show a slight male predominance [1], or equal sex distribution, rather than being more common in females. - This statement is the exception to the generally true characteristics of cerebellar astrocytomas in children. *These tumors have a good prognosis* - **Pilocytic astrocytomas (PAs)**, which constitute the majority of pediatric cerebellar astrocytomas, are generally **low-grade (WHO grade I)** tumors. - They have an **excellent prognosis** following gross total resection, with high 5-year survival rates. *These are usually Low grade tumors* - The vast majority of cerebellar astrocytomas in children are **pilocytic astrocytomas**, which are classified as **WHO grade I** [2]. - This low-grade nature contributes significantly to their favorable prognosis compared to higher-grade astrocytomas. *These are more commonly seen in the 1st and 2nd decades* - Pediatric cerebellar astrocytomas primarily affect **children and young adults**, with the peak incidence observed in the **first two decades of life** [1], [2]. - They are among the most common brain tumors in this age group, typically presenting between the ages of 5 and 15 years. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.

Question 313: Which of the following most likely causes a communicating (nonobstructive) hydrocephalus?

A. Tuberculous meningitis
B. Stenosis of the duct of Sylvius
C. Blockage of the arachnoid granulations (Correct Answer)
D. Ependymoma of the fourth ventricle

Explanation: ***Blockage of the arachnoid granulations*** - **Communicating hydrocephalus** occurs when CSF flow from the ventricles is unobstructed, but its **reabsorption** into the venous system is impaired [1] - **Arachnoid granulations** (pacchionian bodies) are responsible for reabsorbing CSF into the dural venous sinuses [1] - Blockage (e.g., due to **subarachnoid hemorrhage**, chronic **meningitis**, or venous thrombosis) prevents proper reabsorption, leading to CSF accumulation [2] - This is the **direct pathophysiologic mechanism** of communicating hydrocephalus *Tuberculous meningitis* - This can cause **communicating hydrocephalus** through inflammation and fibrosis of the **basilar meninges**, which obstructs the arachnoid granulations and impairs CSF reabsorption - It is a clinically important cause, especially in endemic regions - However, it works through the mechanism of arachnoid granulation blockage, making it an indirect cause - Less commonly, it can cause obstructive hydrocephalus if inflammatory exudates block the basal cisterns [2] *Stenosis of the duct of Sylvius* - Also known as **aqueductal stenosis**, this is a classic cause of **non-communicating (obstructive) hydrocephalus** - It blocks the flow of CSF from the third to the fourth ventricle, leading to dilation of the lateral and third ventricles - CSF cannot communicate between the ventricular system and subarachnoid space *Ependymoma of the fourth ventricle* - An **ependymoma** in this location causes **non-communicating (obstructive) hydrocephalus** [2] - The tumor physically blocks the outflow of CSF from the fourth ventricle through the foramina of Luschka and Magendie into the subarachnoid space - Ependymomas are the most common posterior fossa tumor in children **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1256-1257. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 703-704.

Question 314: Xanthochromia of CSF is seen in all except:

A. Bloody tap (Correct Answer)
B. Increased proteins
C. Carotene
D. Subarachnoid hemorrhage

Explanation: ***Bloody tap*** - A bloody tap refers to the presence of blood from a **traumatic lumbar puncture**, which introduces fresh blood into the CSF. - This fresh blood would **not have had sufficient time to lyse** and release hemoglobin or oxyhemoglobin to cause xanthochromia. - Xanthochromia typically develops **2-12 hours after hemorrhage** as RBCs lyse and hemoglobin breaks down into bilirubin. *Subarachnoid hemorrhage* - **Subarachnoid hemorrhage (SAH)** is the **classic cause of xanthochromia**. - Following SAH, RBCs in the CSF undergo lysis, releasing **oxyhemoglobin** (peaks at 12-36 hours) and later **bilirubin** (peaks at 2-4 days). - Xanthochromia is detectable by **spectrophotometry** and helps distinguish SAH from traumatic tap. *Increased proteins* - **Elevated protein levels** (>150 mg/dL) in the CSF can cause yellowish discoloration. [1] - Seen in conditions like **Guillain-Barré syndrome** (albuminocytologic dissociation), **Froin's syndrome**, or tumors causing CSF obstruction. - The yellow color is due to protein concentration and binding properties. *Carotene* - **Severe carotenemia** (hypercarotenemia) from excessive intake of carotene-rich foods can rarely cause xanthochromia. - Carotene is a **lipophilic pigment** that can cross into CSF and impart a yellow color. - This is an uncommon but documented cause. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1275-1276.

Question 315: Which of the following statements about astrocytoma is false?

A. Low grade tumors are more common in children
B. These are mostly infratentorial in children
C. Pilocytic astrocytoma is most common childhood brain tumor
D. They are more common in males than females (Correct Answer)

Explanation: ***They are more common in males than females*** - This statement is **false** because, while specific types of astrocytomas might show slight sex predominance, astrocytomas as a whole do not consistently show a significantly higher incidence in males compared to females. [1] - The incidence of astrocytomas is generally considered to be **roughly equal** between sexes or with only minor, inconsistent differences depending on the specific subtype and age group. *Low grade tumors are more common in children* - This statement is **true** because pediatric gliomas, including astrocytomas, are predominantly **low-grade (WHO grade I or II)**, accounting for about half of all central nervous system tumors in children. [2] - **Pilocytic astrocytoma (WHO grade I)** is a common example, known for its slow growth and often favorable prognosis. [2] *These are mostly infratentorial in children* - This statement is **true** as astrocytomas in children frequently occur in the **infratentorial region**, particularly the cerebellum, brainstem, and optic pathways. [1], [2] - This contrasts with adults, where supratentorial locations are more common; **cerebellar astrocytoma** is a classic example in children. [1] *Pilocytic astrocytoma is most common childhood brain tumor* - This statement is **true** as **pilocytic astrocytoma (WHO grade I)** is indeed the most common type of pediatric primary brain tumor, accounting for a significant proportion of all childhood CNS neoplasms. [2] - It often presents as a **cystic lesion with a mural nodule** and has a relatively good prognosis compared to higher-grade astrocytomas. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.

Question 316: Negri bodies are seen in

A. Herpes encephalitis
B. Prion disease
C. Rabies (Correct Answer)
D. Measles

Explanation: ***Rabies*** - **Negri bodies** are pathognomonic, **eosinophilic intracytoplasmic inclusions** found in the neurons of individuals infected with the rabies virus [1]. - They are most often observed in the **hippocampus** (Ammon's horn) and **Purkinje cells** of the cerebellum [1]. *Herpes encephalitis* - Characterized by **Cowdry type A intranuclear inclusions**, not Negri bodies, which are found in infected neurons and glial cells. - It primarily causes **necrotizing encephalitis** in the temporal and frontal lobes. *Prion disease* - Defined by the accumulation of **abnormal prion proteins** (PrPsc), leading to spongiform degeneration of brain tissue. - It does not involve viral inclusions like Negri bodies, but rather **vacuolation** and neuronal loss. *Measles* - Associated with **Warthin-Finkeldey giant cells** (syncytia) and intranuclear and intracytoplasmic inclusions in lymphoid tissues and respiratory epithelium. - It does not produce Negri bodies; neurological complications typically present as **subacute sclerosing panencephalitis** much later with different histological findings. **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 317: Which part of the basal ganglia is primarily involved in Parkinsonism?

A. Subthalamic nucleus
B. Substantia nigra (Correct Answer)
C. Caudate nucleus
D. Globus pallidus

Explanation: **Substantia nigra** - **Parkinsonism** is predominantly caused by the degeneration of **dopaminergic neurons** in the **substantia nigra pars compacta (SNpc)** [1], [2]. - The loss of these neurons leads to a reduction in **dopamine** in the striatum, disrupting the basal ganglia's motor control circuits [1]. *Subthalamic nucleus* - The **subthalamic nucleus (STN)** is part of the indirect pathway of the basal ganglia and becomes **hyperactive** in Parkinson's disease due to dopamine deficiency. - While involved in the pathology, its primary role is not the initial site of degeneration in Parkinsonism. *Caudate nucleus* - The **caudate nucleus** is primarily involved in **cognitive functions** and receives input from cortical association areas. - While part of the striatum and affected by dopamine depletion, its primary involvement is not the defining characteristic of Parkinsonism's motor symptoms. *Globus pallidus* - The **globus pallidus** (both internal and external segments) is a major output nucleus of the basal ganglia, regulating thalamic and brainstem activity [1]. - Its activity is altered in Parkinson's disease, but it's not the primary site of neurodegeneration leading to the condition [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 723-724. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1297-1298.

Question 318: The most common tumor of the cerebellopontine angle is -

A. None of the options
B. Meningioma
C. Acoustic neuroma (Correct Answer)
D. Neurofibroma

Explanation: ***Acoustic neuroma*** - Acoustic neuromas, also known as **vestibular schwannomas**, are the most common tumors of the **cerebellopontine angle (CPA)**, accounting for **80-90%** of CPA tumors. [1] - They arise from the **Schwann cells** of the **vestibular portion of cranial nerve VIII** (vestibulocochlear nerve). [1] - Typical clinical presentation includes **progressive unilateral sensorineural hearing loss**, **tinnitus**, **vertigo**, and in advanced cases, involvement of adjacent cranial nerves (V and VII). *Meningioma* - Meningiomas are the **second most common** CPA tumor (10-15% of cases), originating from the **arachnoid cap cells**. [1] - They typically grow more slowly than acoustic neuromas and may present with different cranial nerve deficits depending on their exact location. - On imaging, they show characteristic **dural tail sign** and homogeneous enhancement. *Neurofibroma* - While neurofibromas can affect cranial nerves, they are **far less common** in the CPA than acoustic neuromas. [1] - They are often associated with **Neurofibromatosis type 1 (NF1)**, whereas bilateral acoustic neuromas are characteristic of **NF2**. [1] - Isolated CPA neurofibromas are rare. *None of the options* - This option is incorrect as **acoustic neuroma** is the well-established most common tumor of the cerebellopontine angle. [1] - This is a fundamental concept in neurology, neurosurgery, and pathology. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 727-728.

Question 319: Porencephaly is due to -

A. Trauma
B. Dandy-Walker syndrome
C. Cerebral infarction (Correct Answer)
D. Fetal alcohol syndrome

Explanation: ***Cerebral infarction*** - **Porencephaly** is a rare neurological disorder characterized by a **cyst or cavity within the cerebral hemisphere** that is usually communicating with the ventricular system. - It results from the **destruction of brain tissue due to a focal cerebral injury**, most commonly caused by **ischemic events** (e.g., cerebral infarction) or **hemorrhage** during fetal development or early infancy [1]. *Trauma* - While trauma can cause brain injury, **porencephaly** specifically refers to tissue destruction and cavity formation, often distinguished from the immediate and direct tissue damage seen in acute traumatic brain injuries [1]. - Trauma typically leads to **hematomas, contusions, or diffuse axonal injury**, rather than the characteristic fluid-filled cysts of porencephaly. *Dandy-Walker syndrome* - **Dandy-Walker syndrome** involves a congenital malformation of the cerebellum and the fluid-filled spaces around it, specifically characterized by **enlargement of the fourth ventricle** and **absence of the cerebellar vermis** [2]. - It is a **developmental brain anomaly**, not typically associated with focal destructive lesions like those seen in porencephaly. *Fetal alcohol syndrome* - **Fetal alcohol syndrome (FAS)** is a group of birth defects caused by maternal alcohol consumption during pregnancy, leading to characteristic facial features, growth deficits, and **central nervous system abnormalities** [3]. - While FAS can cause brain damage including **microcephaly** and **cortical malformations**, the characteristic cysts of porencephaly are not a primary feature. **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] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 718-719. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 717-718.

Question 320: Which type of shift in intracranial content is common in children with progressive hydrocephalus –

A. Central transtentorial herniation (Correct Answer)
B. Transforaminal herniation
C. Upward cerebellar herniation
D. Unilateral transtentorial herniation

Explanation: ***Central transtentorial herniation*** - In progressive hydrocephalus, the increase in overall intracranial pressure causes a **downward displacement of the diencephalon and midbrain** through the tentorial incisura [1]. - This type of herniation is often due to **diffuse supratentorial pressure increase**, which is characteristic of unresolved hydrocephalus. *Transforaminal herniation* - Also known as **tonsillar herniation**, this involves the displacement of the cerebellar tonsils through the **foramen magnum** [2]. - It is typically caused by **posterior fossa masses** or severe supratentorial pressure leading to global brain edema, but less specifically linked to the common progression of hydrocephalus. *Upward cerebellar herniation* - This occurs when a **mass in the posterior fossa** or increased pressure within the posterior fossa causes the cerebellum to herniate **upward** through the tentorial opening. - It is not a common herniation pattern for generalized hydrocephalus, which usually produces a **diffuse downward pressure**. *Unilateral transtentorial herniation* - Also called **uncal herniation**, this type involves the medial temporal lobe (uncus) being forced over the petrous ridge and through the tentorial incisura [1]. - It is primarily caused by **unilateral supratentorial mass lesions** (e.g., tumors, hematomas), rather than the diffuse pressure seen in hydrocephalus. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 698-700. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1257-1258.

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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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