Neuropathology Practice Questions

Q: Varrocay bodies are present in which of the following conditions?

None. **Explanation:** **Verocay bodies** are a classic histopathological hallmark of **Schwannomas** (Neurilemmomas) [1]. They consist of two compact rows of well-aligned, palisading nuclei separated by an intervening acellular zone of eosinophilic fibrillar processes [1]. These structures are found within **Antoni A** (hypercellular) areas of the tumor [1]. **Why the correct answer is "None":** The question lists Periapical granuloma and Multiple myeloma, neither of which feature Verocay bodies. * **Option A (Periapical granuloma):** This is a mass of chronically inflamed granulation tissue at the apex of a non-vital tooth. Histologically, it shows fibroblasts, capillaries, and inflammatory cells (lymphocytes, plasma cells). It does not exhibit neural palisading. * **Option B (Multiple myeloma):** This is a plasma cell dyscrasia [2]. The characteristic histological finding is a dense infiltration of **malignant plasma cells** (with "clock-face" nuclei and perinuclear halos) [2]. Other associated findings include **Russell bodies** (intracytoplasmic) and **Dutcher bodies** (intranuclear) immunoglobulin inclusions, but not Verocay bodies. **High-Yield Clinical Pearls for NEET-PG:** * **Schwannoma:** Usually a solitary, encapsulated tumor. It is S-100 positive [1]. * **Antoni A vs. Antoni B:** Antoni A is dense/cellular with Verocay bodies; Antoni B is loose/myxoid [1]. * **Acoustic Neuroma:** A Schwannoma of the CN VIII (vestibulocochlear nerve), often associated with Neurofibromatosis Type 2 (NF2) if bilateral. * **Differential Diagnosis:** Do not confuse Verocay bodies with **Psammoma bodies** (Meningioma) or **Negri bodies** (Rabies). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, p. 1250. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 617-618.

Q: Dawson disease is also known as which of the following?

Subacute sclerosing panencephalitis (SSPE). **Explanation:** **Subacute sclerosing panencephalitis (SSPE)**, also known as **Dawson disease**, is a rare, progressive, and fatal neurodegenerative disease caused by a persistent infection with a mutant strain of the **Measles virus**. It typically occurs years after an initial measles infection, usually in children who were infected before the age of two. The term "Dawson disease" refers to James Dawson, who first described the characteristic **Cowdry type A intranuclear inclusion bodies** found in the neurons and glial cells. **Analysis of Options:** * **Option A (Correct):** SSPE is characterized by widespread inflammation (panencephalitis) and demyelination. Pathologically, it shows viral inclusions, microglial nodules, and perivascular cuffing. * **Option B (Incorrect):** ADEM is an immune-mediated demyelinating disease that typically follows a viral infection or vaccination (monophasic), but it is not caused by persistent viral replication. * **Option C (Incorrect):** NMO (Devic disease) is an autoimmune inflammatory disorder targeting **Aquaporin-4 (AQP4)** channels, primarily affecting the optic nerves and spinal cord [1]. * **Option D (Incorrect):** Paralysis agitans is the historical name for Parkinson’s disease, a degenerative disorder of the dopaminergic system in the substantia nigra. **High-Yield Clinical Pearls for NEET-PG:** * **EEG Finding:** Periodic, high-voltage slow-wave complexes (Radermecker complexes). * **CSF Finding:** Significantly elevated titers of anti-measles antibodies (**Oligoclonal bands** are present). * **Pathology:** Cowdry type A eosinophilic intranuclear inclusions (Dawson bodies). * **Clinical Stages:** Progresses from behavioral changes to myoclonic jerks, followed by dementia and eventually a vegetative state. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1287-1288.

Q: Which of the following is not a histological feature of adamantinomatous craniopharyngioma?

Papillae lined by well-differentiated squamous epithelium. **Explanation:** Craniopharyngiomas are benign tumors arising from remnants of **Rathke’s pouch**. They exist in two distinct clinico-pathological variants: **Adamantinomatous** (common in children) and **Papillary** (common in adults). **Why Option D is the correct answer:** Option D describes the hallmark of the **Papillary Craniopharyngioma**, not the adamantinomatous type. Papillary variants lack the complex architecture of the adamantinomatous type and are characterized by solid sheets and **papillae** lined by well-differentiated, mature squamous epithelium. They notably lack "wet keratin," calcification, and stellate reticulum. **Analysis of Incorrect Options (Features of Adamantinomatous type):** * **Option A:** This variant typically shows peripheral palisading of squamous cells surrounding a loose, mesh-like center known as **stellate (spongy) reticulum**, mimicking the enamel organ of a developing tooth. * **Option B:** A pathognomonic feature is the presence of **"wet keratin"**—large, nodules of compact, lamellar keratin that often undergo secondary changes. * **Option C:** The "wet keratin" frequently undergoes **dystrophic calcification** (visible on CT scans). When keratin cells degenerate, they release **cholesterol crystals**, giving the cyst fluid a characteristic "machinery oil" appearance. **NEET-PG High-Yield Pearls:** * **Bimodal Distribution:** Peaks at 5–15 years (Adamantinomatous) and 45–60 years (Papillary). * **Imaging:** Adamantinomatous types are typically cystic and **calcified** (90%); Papillary types are usually solid/mixed and rarely calcified. * **Molecular Marker:** Adamantinomatous craniopharyngiomas are associated with **CTNNB1 (β-catenin)** mutations, while Papillary types often harbor **BRAF V600E** mutations. * **Location:** Most common suprasellar tumor in children, often causing bitemporal hemianopia and endocrine dysfunction.

Q: What is the probable diagnosis for a cyst in a child that is located at and associated with vertebral defects?

Neuroenteric cyst. **Explanation:** The correct answer is **Neuroenteric cyst**. **1. Why Neuroenteric Cyst is correct:** A neuroenteric cyst (also known as a gastrocytoma of the spinal cord) is a rare congenital lesion resulting from the failure of the **notochordal canal** to separate from the **endoderm** (primitive foregut) during the third week of embryogenesis. This persistent connection results in a cyst lined by mucin-secreting alimentary or respiratory epithelium. Characteristically, these cysts are associated with **vertebral anomalies** (such as hemivertebrae, butterfly vertebrae, or spina bifida) because the abnormal connection interferes with the normal development of the vertebral bodies. **2. Why other options are incorrect:** * **Myelocele:** While associated with vertebral defects (spina bifida aperta), a myelocele is a form of neural tube defect where the spinal cord is exposed to the surface. It is not a "cyst" lined by epithelium but rather a failure of neural tube closure. * **Bronchogenic cyst:** These are also foregut derivatives but are typically located in the mediastinum. While they share similar histology, they are not classically associated with vertebral defects. * **Neuroblastoma:** This is a solid malignant tumor derived from neural crest cells (adrenal medulla or sympathetic chain). It is not a cystic lesion and does not typically cause congenital vertebral body malformations. **3. High-Yield Pearls for NEET-PG:** * **Location:** Most commonly found in the **cervical or upper thoracic** spine, usually intradural-extramedullary. * **Histology:** Lined by simple or pseudostratified columnar/cuboidal epithelium (often with goblet cells). * **Triad:** Think of Neuroenteric cyst when you see: **Intraspinal cyst + Vertebral defect + Foregut-derived lining.** * **Imaging:** Often presents as a well-circumscribed cyst on MRI; look for associated "split cord" or vertebral anomalies.

Question 1

Varrocay bodies are present in which of the following conditions?

Accepted Answer

None

Answer

Periapical granuloma

Answer

Multiple myeloma

Answer

Both

Question 2

A 60-year-old woman has a history of being treated for endometrial cancer with surgery and radiation 5 years ago. She now presents with a large necrotic tumor that follows the course of the sciatic nerve. What is the most likely diagnosis?

Accepted Answer

Malignant peripheral nerve sheath tumor

Answer

Solitary neurofibroma

Answer

Neurofibromatosis type 2

Answer

Schwannoma

Question 3

Physical examination of a 14-year-old boy demonstrates six coffee-colored skin macules up to 3 cm in diameter. No other skin lesions are noted, but a small mass lesion is felt in the subcutaneous tissues below two of the macules. These masses are most likely closely associated with which of the following structures?

Accepted Answer

Peripheral nerves

Answer

Arrector pili muscles

Answer

Hair follicles

Answer

Sebaceous glands

Question 4

Dawson disease is also known as which of the following?

Accepted Answer

Subacute sclerosing panencephalitis (SSPE)

Answer

Acute disseminated encephalomyelitis (ADEM)

Answer

Neuromyelitis optica (NMO)

Answer

Paralysis agitans (Parkinson's disease)

Question 5

The most common site of Berry aneurysm is:

Accepted Answer

Junction of anterior communicating artery with anterior cerebral artery

Answer

Junction of posterior communicating artery with internal carotid artery

Answer

Bifurcation of middle cerebral artery

Answer

Vertebral artery

Question 6

Which of the following is not a histological feature of adamantinomatous craniopharyngioma?

Accepted Answer

Papillae lined by well-differentiated squamous epithelium

Answer

Nests and cords of squamous epithelium embedded in spongy reticulum

Answer

Compact, lamellar keratin formation ("wet keratin")

Answer

Dystrophic calcification and cholesterol crystals

Question 7

Which of the following is NOT a pathological finding seen in Alzheimer's disease?

Accepted Answer

Lewy body

Answer

Cortical atrophy

Answer

Neurofibrillary tangles

Answer

Neuritic plaques

Question 8

What is the probable diagnosis for a cyst in a child that is located at and associated with vertebral defects?

Accepted Answer

Neuroenteric cyst

Answer

Myelocele

Answer

Bronchogenic cyst

Answer

Neuroblastoma

Question 9

a-synuclein, a lipid-binding protein, is a major component of what?

Accepted Answer

Lewy bodies

Answer

Amyloid

Answer

Tau protein

Answer

Neurofibrillary tangles

Question 10

Neurofibrillary tangles are a deposition of which of the following substances?

Accepted Answer

Tau protein

Answer

Amyloid-beta peptide

Answer

Lewy bodies

Answer

Hirano bodies

Neuropathology — MCQs

Neuropathology — MCQs

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