Neuropathology Practice Questions

Q: A 54-year-old male presents with gradual onset of mild dementia, ataxic gait, and myoclonus. An MRI scan is normal, and cerebrospinal fluid analysis reveals no abnormalities. However, the patient's EEG is remarkable for recurrent bursts of high-voltage slow waves. Over the next 6 months, the patient's dementia rapidly worsens, accompanied by hypertonicity and profound dysarthria. The patient dies shortly thereafter. Which of the following is the most likely neuropathological finding on autopsy?

Diffuse spongiform change. ### **Explanation** The clinical presentation of **rapidly progressive dementia**, **ataxic gait**, and **myoclonus**, culminating in death within months, is classic for **Creutzfeldt-Jakob Disease (CJD)** [1]. **1. Why "Diffuse spongiform change" is correct:** CJD is a prion-mediated neurodegenerative disorder [1]. The hallmark neuropathological finding is **spongiform encephalopathy**, characterized by the formation of small, clear vacuoles within the neuropil of the gray matter (cortex and deep nuclei), leading to a "sponge-like" appearance. The EEG finding of **periodic synchronous bi- or triphasic sharp wave complexes (PSWCs)**—often described as recurrent bursts of high-voltage slow waves—is highly suggestive of CJD. **2. Why the other options are incorrect:** * **Cerebellar hyperplasia (A):** CJD typically causes cerebellar *atrophy* (leading to ataxia), not hyperplasia. Hyperplasia is not a feature of any common neurodegenerative dementia. * **Multiple lacunar infarcts (C):** This is the hallmark of **Vascular Dementia**. While it causes cognitive decline, it usually presents with a "step-ladder" progression and focal neurological deficits, rather than rapid myoclonus and specific EEG bursts. * **Negri bodies (D):** These are eosinophilic cytoplasmic inclusions found in pyramidal cells of the hippocampus and Purkinje cells of the cerebellum, pathognomonic for **Rabies**, not CJD. ### **Clinical Pearls for NEET-PG** * **Triad of CJD:** Rapidly progressive dementia + Myoclonus + Ataxia/Extrapyramidal signs. * **CSF Marker:** Look for the **14-3-3 protein** or **RT-QuIC** (Real-Time Quaking-Induced Conversion) assay. * **MRI Finding:** "Pulvinar sign" (thalamus) or "Hockey stick sign" (striatum) and cortical ribboning on DWI sequences. * **Etiology:** Accumulation of misfolded PrP^Sc (beta-pleated sheets) which are resistant to proteases.

Q: Regarding Progressive multifocal leucoencephalopathy, all are true, except?

Disease of grey matter. **Explanation:** Progressive Multifocal Leukoencephalopathy (PML) is a severe demyelinating disease of the Central Nervous System. The core pathophysiology involves the **selective destruction of oligodendrocytes**, the cells responsible for producing and maintaining the myelin sheath in the brain. * **Why Option D is the correct answer:** PML is strictly a **white matter disease**. Since myelin is the primary component of white matter, the destruction of oligodendrocytes leads to extensive demyelination. It does **not** primarily involve the neuronal cell bodies found in the grey matter. Therefore, stating it is a "disease of grey matter" is factually incorrect. * **Why Option A is wrong:** PML is caused by the **JC virus** (John Cunningham virus), a human polyomavirus. It is a high-yield fact that this virus remains latent in the kidneys and lymphoid tissue of healthy individuals and reactivates during profound immunosuppression. * **Why Option B & C are wrong:** These options correctly describe the pathology. PML presents as **diffuse, asymmetric involvement of the subcortical white matter**. On MRI, these appear as multiple, non-enhancing T2/FLAIR hyperintense lesions without mass effect. **High-Yield Clinical Pearls for NEET-PG:** * **Risk Factors:** Most commonly seen in **AIDS patients** (CD4 count <200 cells/µL) and patients on monoclonal antibodies like **Natalizumab** (used in Multiple Sclerosis). * **Histopathology:** Look for the "Triad": 1. Demyelination, 2. **Bizarre giant astrocytes**, and 3. **Intranuclear inclusion bodies** in oligodendrocytes (ground-glass appearance). * **Diagnosis:** PCR for JC virus DNA in the Cerebrospinal Fluid (CSF) is the gold standard for non-invasive diagnosis.

Q: The marked area in the image of a Schwannoma represents which of the following?

Antoni A pattern with Verocay bodies. ***Antoni A pattern with Verocay bodies*** - **Antoni A pattern** is characterized by **densely packed spindle cells** arranged in **parallel fascicles** with elongated nuclei showing **nuclear palisading**. - **Verocay bodies** are pathognomonic structures formed by **parallel rows of nuclei** separated by **anuclear eosinophilic zones** containing cell processes, exclusively found in Antoni A areas. *Myxoid tissue* - Represents **loose connective tissue** with abundant **extracellular matrix** and **mucopolysaccharides**, not a specific pattern in schwannomas. - Would appear as **pale, gelatinous areas** without the organized cellular architecture seen in Antoni patterns. *Antoni B pattern with Verocay bodies* - **Antoni B pattern** consists of **loosely arranged cells** in a **myxoid background** with **microcystic spaces** and lacks organized structure. - **Verocay bodies are never present** in Antoni B areas; they are **exclusive to Antoni A pattern**. *Antoni C pattern* - **Antoni C pattern does not exist** in the classification of schwannoma histological patterns. - Only **Antoni A** (dense, organized) and **Antoni B** (loose, myxoid) patterns are recognized in schwannoma pathology.

Question 1

Which is the most common intracranial brain tumor in adults?

Accepted Answer

Metastasis

Answer

Astrocytoma

Answer

Meningioma

Answer

Oligodendroglioma

Question 2

Which of the following sites in the brain is a common location for ischemic damage, rabies virus infestation, and toxic damage resulting from alcohol?

Accepted Answer

Purkinje cells of cerebellum

Answer

Nucleus basalis of Meynert

Answer

Third, fifth, and sixth cortical layers of neurons in cerebral hemispheric grey matter

Answer

Ammon's horn of the hippocampal gyrus

Question 3

A 54-year-old male presents with gradual onset of mild dementia, ataxic gait, and myoclonus. An MRI scan is normal, and cerebrospinal fluid analysis reveals no abnormalities. However, the patient's EEG is remarkable for recurrent bursts of high-voltage slow waves. Over the next 6 months, the patient's dementia rapidly worsens, accompanied by hypertonicity and profound dysarthria. The patient dies shortly thereafter. Which of the following is the most likely neuropathological finding on autopsy?

Accepted Answer

Diffuse spongiform change

Answer

Cerebellar hyperplasia

Answer

Multiple lacunar infarcts

Answer

Negri bodies

Question 4

A 65-year-old woman with a history of breast cancer and a recent melanoma presents to the emergency room following a tonic-clonic seizure. Blood chemistry values are within normal limits. There is no history of drug or alcohol use. MRI of the brain shows bilateral cerebral edema and a cystic, frontal lobe lesion. Frozen section obtained from a CT-guided biopsy reveals a hemorrhagic nodule of neoplastic cells. Immunohistochemical stains for which of the following antigens would be most helpful in making your diagnosis definitive for melanoma?

Accepted Answer

HMB-45

Answer

Alpha-fetoprotein

Answer

Human chorionic gonadotropin

Answer

Neuron-specific enolase

Question 5

Regarding Progressive multifocal leucoencephalopathy, all are true, except?

Accepted Answer

Disease of grey matter

Answer

Caused by JC virus

Answer

Diffuse cortical matter involvement

Answer

Disease of white matter

Question 6

Which of the following statements is true about medulloblastoma?

Accepted Answer

It is a radiosensitive tumor.

Answer

It is predominantly seen in individuals over 50 years of age.

Answer

Surgery is the sole treatment modality.

Answer

It is typically found in the anterior cranial fossa.

Question 7

All of the following are true about Primary CNS Lymphoma except:

Accepted Answer

Glucocorticoids should be given before biopsy to reduce the risk of edema.

Answer

Most common type in immunocompetent patients is DLBCL.

Answer

Stereotactic biopsy is necessary for histologic diagnosis.

Answer

High-dose methotrexate is used for the treatment.

Question 8

What is the most common central nervous system (CNS) manifestation of HIV infection?

Accepted Answer

Encephalopathy

Answer

Vacuolar myelopathy

Answer

Acute meningitis

Answer

Dementia

Question 9

A 10-year-old boy presents with persistent headaches for 3 months. He has an ataxic gait and dysdiadochokinesia. A CT scan reveals a 4-cm cystic mass in the right cerebellar hemisphere with enlarged lateral ventricles. CSF analysis shows elevated protein and normal glucose. Intraoperative gross examination of the resected mass shows a cyst filled with gelatinous material, a thin wall, and a 1-cm mural nodule. Microscopic examination reveals cells positive for glial fibrillary acidic protein (GFAP) with long, hair-like processes. What is the most likely diagnosis?

Accepted Answer

Astrocytoma

Answer

Ependymoma

Answer

Hemangioblastoma

Answer

Medulloblastoma

Question 10

The marked area in the image of a Schwannoma represents which of the following?

Accepted Answer

Antoni A pattern with Verocay bodies

Answer

Myxoid tissue

Answer

Antoni B pattern with Verocay bodies

Answer

Antoni C pattern

Neuropathology — MCQs

Neuropathology — MCQs

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