Neuropathology Practice Questions

Q: Neurodegeneration with iron accumulation in basal ganglia is typically seen in which condition?

Hallervorden-Spatz disease. **Explanation:** **1. Why Hallervorden-Spatz Disease is Correct:** Hallervorden-Spatz disease, now more commonly known as **Pantothenate Kinase-Associated Neurodegeneration (PKAN)**, is a rare autosomal recessive neurodegenerative disorder caused by mutations in the *PANK2* gene. This defect leads to an abnormal accumulation of **iron** in the brain, specifically within the **globus pallidus** and **substantia nigra pars reticulata**. On T2-weighted MRI, this iron deposition creates a pathognomonic sign known as the **"Eye of the Tiger" sign** (a central area of hyperintensity surrounded by a rim of hypointensity). **2. Why the Other Options are Incorrect:** * **Hemochromatosis:** This is a systemic iron overload disorder. While iron accumulates in the liver, heart, and endocrine organs, it typically **spares the brain** due to the blood-brain barrier. * **Wilson’s Disease:** This is a disorder of **copper** metabolism, not iron. While it involves the basal ganglia (specifically the putamen), the primary pathology is copper toxicity leading to gliosis and cavitation. * **Cystic Fibrosis:** This is a multi-system disorder affecting chloride channels (CFTR gene), primarily impacting the lungs and pancreas. It has no association with iron accumulation in the basal ganglia. **3. NEET-PG High-Yield Pearls:** * **MRI Finding:** Look for the "Eye of the Tiger" sign in clinical vignettes. * **Genetics:** Autosomal Recessive; *PANK2* gene mutation on Chromosome 20p. * **Histology:** Presence of **Spheroid bodies** (swollen axonal terminals) and iron-containing pigment (neuromelanin and lipofuscin). * **Clinical Presentation:** Extrapyramidal symptoms like dystonia, parkinsonism, and choreoathetosis, usually beginning in childhood.

Q: What is the most common primary brain tumor in adults?

Astrocytoma. **Explanation:** **Astrocytoma** is the correct answer because it represents the most common group of primary intracranial neoplasms in adults [1]. Specifically, **Glioblastoma (GBM)**, which is a Grade IV astrocytoma, is the most frequent and aggressive primary malignant brain tumor in the adult population [1], [2]. Astrocytomas originate from astrocytes, the star-shaped glial cells that support neuronal function. **Analysis of Options:** * **B. Oligodendroglioma:** These are less common than astrocytomas. They typically present in middle-aged adults (30–50 years) and are characterized histologically by a "fried-egg" appearance and "chicken-wire" vascularity. * **C. Ependymoma:** While these can occur in adults (often in the spinal cord), they are much more common in children, typically arising in the fourth ventricle. * **D. Medulloblastoma:** This is a highly malignant Grade IV tumor, but it is primarily a **pediatric tumor** (the most common malignant brain tumor in children), occurring in the cerebellum [1]. It is rare in adults. **High-Yield Clinical Pearls for NEET-PG:** * **Most common brain tumor in adults (overall):** Metastatic tumors (Lung > Breast > Melanoma). * **Most common primary brain tumor in adults:** Astrocytoma (specifically Glioblastoma) [1]. * **Most common benign brain tumor in adults:** Meningioma (often associated with Psammoma bodies). * **Glioblastoma Marker:** GFAP (Glial Fibrillary Acidic Protein) positive. * **Radiology:** Glioblastoma typically shows a "butterfly glioma" pattern crossing the corpus callosum with ring enhancement on MRI [2]. **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. 1308-1310.

Q: Which of the following conditions is associated with brain tumors?

Sturge-Weber syndrome. **Explanation:** The correct answer is **Sturge-Weber Syndrome (SWS)**. While SWS is a classic phakomatosis characterized by vascular malformations, it is unique among the listed options because it is **not** typically associated with the development of brain tumors (neoplasms). Instead, it is characterized by a **leptomeningeal angioma** (a vascular malformation), usually involving the pia mater of the occipital and parietal lobes. This leads to cortical atrophy and "tram-track" calcifications rather than neoplastic growth. **Analysis of Options:** * **Neurofibromatosis (Type 1 & 2):** Strongly associated with brain tumors [1]. NF1 is linked to Optic Nerve Gliomas and Astrocytomas; NF2 is famous for the "MISME" mnemonic (Multiple Inherited Schwannomas, Meningiomas, and Ependymomas). * **Tuberous Sclerosis (TSC):** Characterized by **Subependymal Giant Cell Astrocytomas (SEGA)** and cortical tubers (which are hamartomas, but SEGA is a distinct neoplasm) [1],[2]. * **Von Hippel-Lindau (VHL) Syndrome:** Highly associated with **Hemangioblastomas**, particularly in the cerebellum and brainstem [1]. **NEET-PG High-Yield Pearls:** * **Sturge-Weber Syndrome:** Look for the triad of Port-wine stain (Nevus flammeus in V1/V2 distribution), Leptomeningeal angioma, and Glaucoma. It is caused by a somatic mutation in the **GNAQ gene**. * **Radiology:** "Tram-track" calcifications on CT are a classic finding in SWS due to cortical mineralization. * **Rule of Thumb:** Most phakomatoses (NF1, NF2, TSC, VHL) are autosomal dominant and predispose to tumors; SWS is **sporadic** and primarily **vascular/malformative** in nature. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-725. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1318-1320.

Question 1

Neurodegeneration with iron accumulation in basal ganglia is typically seen in which condition?

Accepted Answer

Hallervorden-Spatz disease

Answer

Hemochromatosis

Answer

Wilson's disease

Answer

Cystic fibrosis

Question 2

What is the most common primary brain tumor in adults?

Accepted Answer

Astrocytoma

Answer

Oligodendroglioma

Answer

Ependymoma

Answer

Medulloblastoma

Question 3

Rupture of Berry aneurysm causes which type of hemorrhage?

Accepted Answer

Subarachnoid hemorrhage

Answer

Extradural hemorrhage

Answer

Subdural hemorrhage

Answer

Intracerebral hemorrhage

Question 4

Which of the following statements is FALSE regarding Krabbe's disease?

Accepted Answer

Hyperdensity on T2 MRI image

Answer

Extensive involvement of deep white matter

Answer

Involvement of cerebellar white matter

Answer

Basal ganglia calcification in CT scan

Question 5

What is the most common type of pathological change seen in Rabies?

Accepted Answer

Brain stem encephalitis

Answer

Meningitis

Answer

Cranial arteritis

Answer

Ventriculitis

Question 6

What is a watershed infarct in the brain?

Accepted Answer

Occurs in the terminal portion of main arteries

Answer

Occurs in the proximal portion of main arteries

Answer

Occurs in the central portion of main arteries

Answer

None of the above

Question 7

A 42-year-old immunosuppressed male presents with rapidly progressing neurologic symptoms including mental deterioration, visual loss, abnormal speech, and ataxia. Radiographic studies demonstrate multifocal lesions in the white matter without mass effect. A stereotactic brain biopsy reveals areas of demyelination. Which histologic feature is most likely to be present in this patient?

Accepted Answer

Viral particles within oligodendrocytes

Answer

Beta-A4 amyloid within blood vessels

Answer

Prion protein within extracellular plaques

Answer

Rosenthal fibers within astrocytes

Question 8

Mutation in which of the following chromosomes is associated with meningioma?

Accepted Answer

Chromosome 22

Answer

Chromosome 1

Answer

Chromosome 19

Answer

Chromosome 9q

Question 9

Which of the following conditions is associated with brain tumors?

Accepted Answer

Sturge-Weber syndrome

Answer

Tuberous sclerosis

Answer

Von Hippel-Lindau syndrome

Answer

Neurofibromatosis

Question 10

Enamel-like superstructure is seen in which CNS lesion?

Accepted Answer

Craniopharyngioma

Answer

Pituitary tumor

Answer

Astrocytoma

Answer

Glioma

Neuropathology — MCQs

Neuropathology — MCQs

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