Neuropathology Practice Questions

Q: Xanthochromia of CSF is seen in all except:

Bloody tap. ***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.

Q: What is not seen in tuberous sclerosis?

Ependymoma. ***Ependymoma*** - **Ependymomas** are primary central nervous system tumors that arise from **ependymal cells**, typically found in the ventricles and spinal cord. - While they are brain tumors, they are **not characteristic lesions** associated with **Tuberous Sclerosis Complex (TSC)**. *Giant Cell Astrocytoma* - **Subependymal giant cell astrocytomas (SEGAs)** are a classic feature of **Tuberous Sclerosis Complex (TSC)**, often growing within the ventricles. - They can cause **hydrocephalus** and are a significant source of morbidity in TSC patients. *White matter migration lines* - **Radial glial migration lines**, or **cerebral white matter lesions**, are due to abnormalities in neuronal migration during development and are a common finding in **Tuberous Sclerosis Complex (TSC)**. - These are typically seen as **linear hypdensities** on neuroimaging. *Sub-ependymal nodules* - **Subependymal nodules (SENs)** are small, benign lesions located just beneath the ependyma of the lateral ventricles in **Tuberous Sclerosis Complex (TSC)**. - They are a diagnostic hallmark of TSC and can sometimes develop into **Subependymal Giant Cell Astrocytomas (SEGAs)**.

Q: Which of the following statements about astrocytoma is false?

They are more common in males than females. ***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.

Q: Negri bodies are seen in

Rabies. ***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.

Q: Which part of the basal ganglia is primarily involved in Parkinsonism?

Substantia nigra. **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.

Q: The most common tumor of the cerebellopontine angle is -

Acoustic neuroma. ***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.

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

Central transtentorial herniation. ***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.

Q: Which of the following is a pathognomonic feature of Alzheimer's disease?

Plaques and tangles. ***Plaques and tangles*** - The presence of **amyloid plaques** (extracellular deposits of beta-amyloid protein) and **neurofibrillary tangles** (intracellular aggregates of hyperphosphorylated tau protein) are the defining neuropathological hallmarks of Alzheimer's disease [1]. These are considered **pathognomonic** features upon post-mortem examination. - While other neurological conditions can have some tauopathy or amyloid deposition, the specific combination, distribution, and extensive nature of these two pathologies are unique to Alzheimer's [1]. [3] *Presence of Lewy bodies* - **Lewy bodies** are abnormal aggregates of alpha-synuclein protein that are characteristic of **Parkinson's disease** and **Lewy body dementia** [3]. - Their presence indicates a distinct neurodegenerative process, separate from Alzheimer's disease. *Presence of Pick bodies* - **Pick bodies** are cytoplasmic inclusions composed primarily of tau protein, but they are characteristic of **Pick's disease**, a type of frontotemporal dementia [2]. - Pick's disease has a different clinical presentation and neuropathological profile than Alzheimer's disease [2]. *Red neuronal degeneration (general feature)* - **Red neuronal degeneration** refers to the morphological changes seen in neurons undergoing acute irreversible ischemic injury, such as during a **stroke**. - It is a general feature of acute neuronal death and is not specific to, nor a pathognomonic feature of, Alzheimer's disease. **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] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1294-1295. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722.

Question 1

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

Accepted Answer

Blockage of the arachnoid granulations

Answer

Tuberculous meningitis

Answer

Stenosis of the duct of Sylvius

Answer

Ependymoma of the fourth ventricle

Question 2

Xanthochromia of CSF is seen in all except:

Accepted Answer

Bloody tap

Answer

Increased proteins

Answer

Carotene

Answer

Subarachnoid hemorrhage

Question 3

What is not seen in tuberous sclerosis?

Accepted Answer

Ependymoma

Answer

Giant Cell Astrocytoma

Answer

White matter migration lines

Answer

Sub-ependymal nodules

Question 4

Which of the following statements about astrocytoma is false?

Accepted Answer

They are more common in males than females

Answer

Low grade tumors are more common in children

Answer

These are mostly infratentorial in children

Answer

Pilocytic astrocytoma is most common childhood brain tumor

Question 5

Negri bodies are seen in

Accepted Answer

Rabies

Answer

Herpes encephalitis

Answer

Prion disease

Answer

Measles

Question 6

Which part of the basal ganglia is primarily involved in Parkinsonism?

Accepted Answer

Substantia nigra

Answer

Subthalamic nucleus

Answer

Caudate nucleus

Answer

Globus pallidus

Question 7

The most common tumor of the cerebellopontine angle is -

Accepted Answer

Acoustic neuroma

Answer

None of the options

Answer

Meningioma

Answer

Neurofibroma

Question 8

Porencephaly is due to -

Accepted Answer

Cerebral infarction

Answer

Trauma

Answer

Dandy-Walker syndrome

Answer

Fetal alcohol syndrome

Question 9

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

Accepted Answer

Central transtentorial herniation

Answer

Transforaminal herniation

Answer

Upward cerebellar herniation

Answer

Unilateral transtentorial herniation

Question 10

Which of the following is a pathognomonic feature of Alzheimer's disease?

Accepted Answer

Plaques and tangles

Answer

Presence of Lewy bodies

Answer

Presence of Pick bodies

Answer

Red neuronal degeneration

Neuropathology — MCQs

Neuropathology — MCQs

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