Neuropathology Practice Questions

Q: Homer-Wright rosette is seen in -

Neuroblastoma. ***Neuroblastoma*** - **Homer rosettes** are characteristic histopathological findings associated with neuroblastoma, indicating differentiation [1]. - This type of cancer typically occurs in **children**, primarily arising from the **adrenal glands** or sympathetic nervous system [1]. *Hepatoma* - Primarily affects the **liver** and is characterized by the presence of **hepatocytes** rather than Homer rosettes. - Typically associated with underlying **chronic liver disease** such as cirrhosis or hepatitis B/C. *Ependymoma* - Consists of cells arising from **ependymal cells** lining the ventricles of the brain and spinal cord, mainly involves **perivascular pseudorosettes**, not Homer rosettes. - Often presents with symptoms related to **increased intracranial pressure** due to obstruction. *Nephroblastoma* - Commonly known as **Wilms tumor**, it typically presents with a **palpable abdominal mass** in children and does not feature Homer rosettes. - Characterized by a triphasic histological pattern (blastemal, epithelial, and stromal components) rather than neuroblastic differentiation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-485.

Q: What is the characteristic feature of neuropraxia?

No structural damage to the nerve. ***No structural damage to the nerve*** - **Neuropraxia** is the mildest form of nerve injury, characterized by a **temporary block in nerve conduction** without structural damage to the axon or surrounding connective tissues. - This typically results in **temporary sensory and/or motor deficits** that fully resolve within weeks to months. *Damage to the endoneurium* - Damage to the **endoneurium** would indicate a more severe injury, such as **axonotmesis**, where the axon is damaged but the connective tissue sheaths are preserved. - This level of injury suggests that wallerian degeneration would occur distal to the lesion, leading to **slower and incomplete recovery**. *Damage to the epineurium* - Damage to the **epineurium**, along with the endoneurium and perineurium, signifies **neurotmesis**, the most severe nerve injury. - This involves a **complete transection of the nerve**, requiring surgical intervention for any chance of functional recovery. *Damage to the axon* - Damage to the **axon** itself, often alongside preserved connective tissues, is characteristic of **axonotmesis**. - While recovery is possible through axonal regeneration, it is **slower and less complete** than in neuropraxia.

Q: What is the primary process involved in Wallerian degeneration?

Nerve degeneration. ***Nerve degeneration*** - **Wallerian degeneration** specifically refers to the process of **axon degeneration** that occurs distal to the site of injury when a nerve fiber is severed [2]. - This process involves the breakdown of the **axon** and its myelin sheath, leading to loss of function [1]. *Muscle degeneration* - While prolonged nerve degeneration can lead to muscle **atrophy** due to denervation, **muscle degeneration itself** is not the primary process of Wallerian degeneration. - Wallerian degeneration focuses on the **nerve itself**, not the target tissue. *Nerve regeneration* - **Nerve regeneration** is the process where damaged nerves attempt to regrow and re-establish connections [2]. - This is a subsequent, and not always successful, event that can occur *after* Wallerian degeneration has cleared the debris [1]. *Muscle regeneration* - **Muscle regeneration** refers to the repair and regrowth of damaged muscle tissue, typically involving satellite cells. - It is unrelated to Wallerian degeneration, which is a process affecting the **nerve**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 697-698. [2] 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. 109-110.

Q: What is the first cellular response observed after a sharp nerve cut?

Chromatolysis. ***Chromatolysis*** - **Chromatolysis** is the dissolution of the Nissl bodies (rough endoplasmic reticulum) in the neuron cell body following axonal injury, which is the **first observable cellular response** [1]. - This process is a preparatory step for neuronal regeneration, indicating the cell's attempt to repair the damaged axon [1]. *Polymorphic arrangement* - This term is not typically used to describe an immediate cellular response to a nerve cut; it might refer to diverse cell shapes or arrangements in different contexts but is not a recognized initial post-injury event. - The neuron's immediate response involves changes within the cell body, not a re-arrangement of its cellular structure with other cells. *Increased protein synthesis* - While increased protein synthesis does occur during neuronal repair and regeneration, it is a consequence of chromatolysis and part of a later, more sustained response, not the very first visible cellular change [1]. - **Chromatolysis precedes** and facilitates the subsequent increase in protein synthesis necessary for axonal regrowth [1]. *Macrophage activation* - **Macrophage activation** is a crucial part of the inflammatory response and debris clearance following nerve injury, but it is not the *first cellular response* of the neuron itself [2]. - Macrophages migrate to the site of injury hours to days after the initial insult, whereas chromatolysis begins within the neuron's cell body much earlier [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1254-1256. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 697-698.

Q: Which of the following is a common deformity associated with Chiari Malformation Type II?

Meningomyelocele. ***Meningomyelocele*** - **Chiari Malformation Type II** is almost always associated with a **meningomyelocele**, a severe form of spina bifida where the spinal cord and nerves protrude through an opening in the spine. - This neural tube defect is considered a hallmark of Chiari Malformation Type II and contributes to many of the associated neurological symptoms. *Syringomyelia* - While **syringomyelia** (a fluid-filled cyst within the spinal cord) can be a **complication** of Chiari Malformation Type I or Type II, it is not a direct anatomical deformity of the malformation itself but rather a secondary effect from altered CSF flow [1]. - It develops in a significant portion of patients but is not the primary associated birth defect defining Chiari Type II. *Hydrocephalus* - **Hydrocephalus** (excessive accumulation of cerebrospinal fluid in the brain) is a very **common complication** of Chiari Malformation Type II due to the obstruction of CSF flow at the level of the hindbrain [1]. - However, like syringomyelia, it is a secondary condition resulting from the primary anatomical malformation (downward displacement of cerebellar tonsils and brainstem) and the associated meningomyelocele, not the core deformity itself. *None of the options* - This option is incorrect because **meningomyelocele** is a direct and defining deformity strongly associated with Chiari Malformation Type II. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 718-719.

Q: Which of the following is considered a good prognostic factor for neuroblastoma?

Trk-A expression present. ***Trk-A expression present*** - **High TrkA (NTRK1) expression** is a well-established **favorable prognostic marker** in neuroblastoma - Associated with younger age, lower stage disease, and better differentiation - Tumors with high TrkA expression tend to be less aggressive and may undergo **spontaneous regression** [1] - Reflects activation of neurotrophin signaling pathways that promote differentiation rather than proliferation *Presence of 1p deletion* - **1p deletion** is a **poor prognostic factor** in neuroblastoma - Associated with aggressive disease and worse outcomes - The **absence** (not presence) of 1p deletion would be favorable *MYCN amplification* - **MYCN amplification** is the **strongest adverse prognostic factor** in neuroblastoma [1] - Found in approximately 20-25% of cases - Associated with rapid tumor progression, advanced stage, and poor survival - Patients with MYCN amplification are classified as high-risk regardless of other factors *Age greater than 18 months at diagnosis* - **Age > 18 months** is a **poor prognostic factor** - Younger children (especially < 12-18 months) have significantly better outcomes - Age is a key component of neuroblastoma risk stratification systems - Older children tend to present with more advanced disease and have worse response to therapy **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-487.

Q: Most common CNS tumor associated with NF1

Optic glioma. ***Optic glioma*** - **Optic gliomas** (specifically **pilocytic astrocytomas**) are the most common CNS tumor found in association with **Neurofibromatosis type 1 (NF1)** [1]. - These tumors typically affect the **optic nerve** and can cause vision impairment. *Optic nerve schwannoma* - **Schwannomas** are tumors arising from Schwann cells, and while they can affect cranial nerves, an **optic nerve schwannoma** is very rare and not characteristic of NF1. - The most common schwannoma associated with neurofibromatosis is a **vestibular schwannoma** (acoustic neuroma) in NF2, not NF1 [2]. *Astrocytoma* - While optic gliomas are a type of astrocytoma, simply stating "astrocytoma" is too broad; the specific location (optic nerve) and type (pilocytic) are key in NF1 [1]. - Other types of astrocytomas (e.g., glioblastoma) are not typically associated with NF1 as the *most common* CNS tumor. *Bilateral acoustic neuroma* - **Bilateral acoustic neuromas** (vestibular schwannomas) are the hallmark CNS tumor of **Neurofibromatosis type 2 (NF2)**, not NF1 [2]. - This symptom strongly points to NF2, a distinct genetic disorder from NF1 [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 727-728.

Question 1

Homer-Wright rosette is seen in -

Accepted Answer

Neuroblastoma

Answer

Nephroblastoma

Answer

Hepatoma

Answer

Ependymoma

Question 2

What is the characteristic feature of neuropraxia?

Accepted Answer

No structural damage to the nerve

Answer

Damage to the endoneurium

Answer

Damage to the epineurium

Answer

Damage to the axon

Question 3

What is the primary process involved in Wallerian degeneration?

Accepted Answer

Nerve degeneration

Answer

Muscle degeneration

Answer

Nerve regeneration

Answer

Muscle regeneration

Question 4

What is the first cellular response observed after a sharp nerve cut?

Accepted Answer

Chromatolysis

Answer

Polymorphic arrangement

Answer

Increased protein synthesis

Answer

Macrophage activation

Question 5

Which of the following statements about peripheral nerve injury is false?

Accepted Answer

Neuropraxia is irreversible

Answer

Wallerian degeneration starts in axonotmesis

Answer

Neurotmesis is the most severe form of injury

Answer

Epineurium is intact in axonotmesis

Question 6

What is a watershed infarct in the brain?

Accepted Answer

Occurs in the border zones between major arteries

Answer

Occurs in the areas supplied by only one artery

Answer

Occurs in the terminal portion of main arteries

Answer

Occurs only in areas with complete arterial occlusion

Question 7

Which of the following is a common deformity associated with Chiari Malformation Type II?

Accepted Answer

Meningomyelocele

Answer

Syringomyelia

Answer

Hydrocephalus

Answer

None of the options

Question 8

Which of the following is considered a good prognostic factor for neuroblastoma?

Accepted Answer

Trk-A expression present

Answer

MYCN amplification

Answer

Age greater than 18 months at diagnosis

Answer

Presence of 1p deletion

Question 9

What is the most common cerebellar tumor in children?

Accepted Answer

Medulloblastoma

Answer

Ependymoma

Answer

PNET

Answer

Astrocytoma

Question 10

Most common CNS tumor associated with NF1

Accepted Answer

Optic glioma

Answer

Astrocytoma

Answer

Bilateral acoustic neuroma

Answer

Optic nerve schwannoma

Neuropathology — MCQs

Neuropathology — MCQs

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