Neuropathology Practice Questions

Q: A 70-year-old male patient presented with forgetfulness of keys and difficulty performing household chores. The patient has trouble driving and takes longer to recognize family members' faces. On examination, the Mini-Mental State Examination (MMSE) score was 20/30. Consciousness was intact, and no focal neurological deficits were present. A Bielschowsky stain revealed characteristic findings. Which anatomical region typically shows the earliest pathological changes in this condition?

Hippocampus. **Explanation:** The clinical presentation of progressive memory loss, executive dysfunction (difficulty with chores), and prosopagnosia (difficulty recognizing faces) in an elderly patient with an MMSE score of 20/30 is classic for **Alzheimer’s Disease (AD)**. The mention of **Bielschowsky silver stain** is a high-yield histological clue, as it is used to visualize **Senile (Neuritic) Plaques** and **Neurofibrillary Tangles (NFTs)**, the pathological hallmarks of AD. **Why Hippocampus is Correct:** In Alzheimer’s Disease, the neurodegenerative process follows a predictable anatomical progression. The earliest pathological changes (NFTs) typically begin in the **entorhinal cortex**, followed rapidly by the **hippocampus** (part of the medial temporal lobe). This explains why episodic memory impairment is the earliest clinical symptom. **Analysis of Incorrect Options:** * **A. Mammillary body:** Atrophy of the mammillary bodies is a hallmark of **Wernicke-Korsakoff Syndrome** (due to Thiamine deficiency), not AD. * **B. Thalamus:** While involved in various dementias and metabolic encephalopathies, it is not the primary or earliest site of pathology in AD. * **D. Caudate nucleus:** Atrophy of the caudate nucleus is the characteristic finding in **Huntington’s Disease**, leading to boxcar ventricles on imaging. **NEET-PG High-Yield Pearls:** * **Pathology:** Extracellular Amyloid-beta plaques and Intracellular Tau protein (hyperphosphorylated) tangles. * **Genetics:** Early-onset AD is associated with APP (Chr 21), Presenilin 1 (Chr 14), and Presenilin 2 (Chr 1). Late-onset is associated with **ApoE4**. * **Neurochemistry:** Significant decrease in **Acetylcholine** levels due to degeneration of the **Nucleus Basalis of Meynert**. * **Imaging:** MRI shows bilateral hippocampal atrophy and compensatory ventriculomegaly.

Q: All of the following are true about hemangioblastoma except?

Premalignant condition. **Explanation:** Hemangioblastoma is a benign, WHO Grade I vascular tumor. The correct answer is **D** because hemangioblastomas are **not premalignant**; they do not undergo malignant transformation or metastasize. They are slow-growing, benign lesions, though they can cause significant morbidity due to their location and associated edema. **Analysis of Options:** * **Option A:** It is indeed the **most common primary intra-axial tumor** of the adult posterior fossa (cerebellum). While metastases are the most common overall, among primary tumors, hemangioblastoma leads in this demographic. * **Option B:** Radiologically, the classic presentation (60% of cases) is a **sharply demarcated cyst with a highly enhancing mural nodule** that abuts the pial surface. They can also present as purely solid masses (30%). * **Option C:** These tumors are known for ectopic hormone production. They can secrete **erythropoietin**, leading to secondary **polycythemia** in approximately 10–20% of patients. **High-Yield Clinical Pearls for NEET-PG:** * **Association:** 25% of cases are associated with **von Hippel-Lindau (VHL) syndrome** (Chromosome 3p). In VHL, tumors are often multiple and occur at a younger age. * **Location:** 80% occur in the cerebellum; other sites include the spinal cord and brainstem. * **Imaging Sign:** On angiography, they show a dense vascular blush. On MRI, look for "flow voids" within the mural nodule due to high vascularity. * **Tumor Markers:** They are typically **Inhibin-alpha positive**, which helps differentiate them from metastatic renal cell carcinoma (which is Inhibin-negative).

Q: Which is the earliest site affected by Alzheimer's disease?

Nucleus basalis of Meynert. **Nucleus Basalis of Meynert (NBM)** is the correct answer because it is the primary source of cholinergic innervation to the cerebral cortex. In Alzheimer’s Disease (AD), the earliest pathological changes involve the loss of cholinergic neurons in this region, leading to a profound deficiency of acetylcholine. This "Cholinergic Hypothesis" forms the basis for using acetylcholinesterase inhibitors (like Donepezil) in treatment [2]. While the **Entorhinal cortex** and **Hippocampus** are the earliest sites for neurofibrillary tangles (Tau protein), the NBM is classically cited as the earliest subcortical nucleus to undergo significant neuronal loss and biochemical changes. Histologically, the disease is also characterized by senile plaques and neurofibrillary tangles in the cerebral cortex [1]. **Analysis of Incorrect Options:** * **A. Amygdala:** While the amygdala is involved in AD (contributing to emotional and behavioral symptoms), it is affected later in the disease progression compared to the NBM and hippocampal formation. * **C. Red Nucleus:** Located in the midbrain, this nucleus is involved in motor coordination (rubrospinal tract). It is typically spared in Alzheimer’s disease. * **D. Posterior Commissure:** This is a white matter tract involved in the bilateral pupillary light reflex. It is not a primary site of pathology in AD. **NEET-PG High-Yield Pearls:** * **Earliest Neurotransmitter affected:** Acetylcholine (Decreased). * **Earliest Anatomical site (Cortex):** Entorhinal Cortex (Brodmann area 28). * **Microscopic Hallmarks:** Senile plaques (Extracellular Amyloid-beta) and Neurofibrillary tangles (Intracellular Hyperphosphorylated Tau) [1], [2]. * **Hirano Bodies:** Eosinophilic, rod-like inclusions found in the hippocampus of AD patients. * **Genetics:** Early-onset AD is associated with APP (Chr 21), PSEN1 (Chr 14), and PSEN2 (Chr 1). Late-onset is associated with APOE-ε4.

Question 1

A 20-year-old man presents with numerous pigmented patches and pedunculated skin tumors. Biopsy of a tumor reveals a benign neoplasm of Schwann cells. The patient has no family history of this condition. The patient is at increased risk of developing which of the following malignant neoplasms?

Accepted Answer

Neurofibrosarcoma

Answer

Ganglioneuroma

Answer

Glioblastoma multiforme

Answer

Serous cystadenocarcinoma

Question 2

A 70-year-old male patient presented with forgetfulness of keys and difficulty performing household chores. The patient has trouble driving and takes longer to recognize family members' faces. On examination, the Mini-Mental State Examination (MMSE) score was 20/30. Consciousness was intact, and no focal neurological deficits were present. A Bielschowsky stain revealed characteristic findings. Which anatomical region typically shows the earliest pathological changes in this condition?

Accepted Answer

Hippocampus

Answer

Mammillary body

Answer

Thalamus

Answer

Caudate nucleus

Question 3

Which of the following is NOT a disease phenotype of Prion diseases?

Accepted Answer

Leigh syndrome

Answer

Creutzfeldt-Jakob disease

Answer

Gerstmann-Straussler-Scheinker syndrome

Answer

Fatal familial insomnia

Question 4

An 80-year-old female presents with progressive loss of memory, difficulty in recalling names, dysphasia, and irritability. Along with neurofibrillary tangles, what other pathological finding will be found in the brain?

Accepted Answer

Beta-amyloid plaques

Answer

Ceramidase

Answer

Lewy bodies

Answer

Pick's bodies

Question 5

A 68-year-old obese woman (BMI = 34 kg/m2) suffers a stroke and expires. Histologic examination of the brain at autopsy reveals extensive arteriolar lipohyalinosis and numerous Charcot-Bouchard aneurysms. Which of the following best accounts for the pathogenesis of these autopsy findings?

Accepted Answer

Hypertension

Answer

Atherosclerosis

Answer

Autoimmunity

Answer

Diabetes

Question 6

All of the following are true about hemangioblastoma except?

Accepted Answer

Premalignant condition

Answer

Most common primary intra-axial tumor in the adult posterior fossa

Answer

Solid or cystic with a mural nodule

Answer

Polycythemia due to increased erythropoietin production

Question 7

Which of the following is NOT a characteristic feature of cerebral malaria?

Accepted Answer

Lesion is not related to the parasite

Answer

Perivascular demyelination

Answer

Durck granuloma

Answer

Proliferated glial cells arranged radially

Question 8

Transtentorial herniation causes all of the following except?

Accepted Answer

Posterior cerebral artery infarct

Answer

Neck stiffness

Answer

Contralateral hemiparesis

Answer

3rd Nerve palsy with contralateral pupillary dilatation

Question 9

Which is the earliest site affected by Alzheimer's disease?

Accepted Answer

Nucleus basalis of Meynert

Answer

Amygdala

Answer

Red nucleus

Answer

Posterior commissure

Question 10

Which of the following is NOT a feature of Alzheimer's disease?

Accepted Answer

Narrowing of ventricles

Answer

Hirano bodies

Answer

Amyloid deposits

Answer

Neuritic plaques

Neuropathology — MCQs

Neuropathology — MCQs

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