Neuropathology Practice Questions

Q: Cowden syndrome is associated with which of the following findings?

Dysplastic gangliocytoma. **Explanation:** **Cowden Syndrome** (Multiple Hamartoma Syndrome) is an autosomal dominant disorder caused by a mutation in the **PTEN gene** on chromosome 10q23. PTEN is a tumor suppressor gene that inhibits the PI3K/AKT/mTOR pathway. **Why the correct answer is right:** The pathognomonic CNS manifestation of Cowden syndrome is **Lhermitte-Duclos Disease (LDD)**, also known as **Dysplastic Gangliocytoma of the cerebellum**. It is a rare, slowly progressing tumor characterized by the replacement of the internal granular layer of the cerebellum with large, dysplastic ganglion cells. On MRI, it classically presents as a "tiger-striped" cerebellar mass. **Why the incorrect options are wrong:** * **Medulloblastoma:** While associated with familial syndromes like **Gorlin syndrome** (PTCH1 mutation) [1] and **Turcot syndrome** (APC mutation), it is not a feature of Cowden syndrome. * **Astrocytoma:** These are common primary brain tumors but are more typically associated with **Neurofibromatosis type 1 (NF1)** and **Li-Fraumeni syndrome** (TP53). * **PNET:** These aggressive embryonal tumors are not specifically linked to the PTEN mutation or Cowden syndrome. **High-Yield Clinical Pearls for NEET-PG:** * **PTEN Hamartoma Tumor Syndrome (PHTS):** Includes Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome. * **Associated Malignancies:** Patients have a significantly increased risk of **Breast cancer** (most common), **Follicular Thyroid cancer**, and **Endometrial cancer**. * **Mucocutaneous findings:** Trichilemmomas (benign hair follicle tumors) and oral papillomas are hallmark diagnostic features. * **Lhermitte-Duclos Disease** is now considered a Grade I WHO tumor and is often the first clinical sign leading to a Cowden syndrome diagnosis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 306-307.

Question 1

In hypoxic damage to the brain, which of the following structures is most vulnerable?

Accepted Answer

Purkinje layer of cerebellum

Answer

Claustrum

Answer

Mamillary body

Answer

Layer of corpus callosum

Question 2

Verocay bodies are seen in which of the following tumors?

Accepted Answer

Schwannoma

Answer

Meningioma

Answer

Glioma

Answer

Medulloblastoma

Question 3

Which of the following is NOT a sequela of cellular events in atherosclerotic infarction?

Accepted Answer

Astrocytes

Answer

Neutrophilic infiltration

Answer

Ingress of macrophages

Answer

Intense eosinophilia

Question 4

Which of the following can cause spinal cord compression?

Accepted Answer

Cysticercosis

Answer

Ankylostomiasis

Answer

Echinococcus Granulosus

Answer

Visceral larva migrans

Question 5

Dumbbell tumors are characteristic of which of the following neoplasms?

Accepted Answer

Neurofibroma

Answer

Meningioma

Answer

Ependymoma

Answer

Thymoma

Question 6

Negri bodies are characteristically seen in which of the following anatomical locations?

Accepted Answer

Hippocampus

Answer

Hypothalamus

Answer

Medulla

Answer

Midbrain

Question 7

Cowden syndrome is associated with which of the following findings?

Accepted Answer

Dysplastic gangliocytoma

Answer

Medulloblastoma

Answer

Astrocytoma

Answer

Primitive neuroectodermal tumor (PNET)

Question 8

Which type of glial cell is commonly found to be malfunctioning in Alzheimer's disease?

Accepted Answer

Microglia

Answer

Radial glia

Answer

Dendrites

Answer

Oligodendrocytes

Question 9

Shearing damage is seen in which of the following organs?

Accepted Answer

Brain

Answer

Heart

Answer

Liver

Answer

Spinal cord

Question 10

Neurofibrillary tangles and amyloid plaques are characteristic features of which condition?

Accepted Answer

Alzheimer's disease

Answer

Creutzfeldt-Jakob disease (CJD)

Answer

Hepatic encephalopathy

Answer

Parkinsonism

Neuropathology — MCQs

Neuropathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?