Neuropathology Practice Questions

Q: A 65-year-old man presents with progressive bradykinesia, resting tremor, and cogwheel rigidity. He later develops visual hallucinations and fluctuating cognition. He dies 8 years after symptom onset. Autopsy shows depigmentation of substantia nigra, and microscopy reveals eosinophilic cytoplasmic inclusions in neurons that are immunoreactive for alpha-synuclein. These inclusions are also found in the cortex. What is the most likely diagnosis?

Dementia with Lewy bodies. ***Dementia with Lewy bodies*** - Characterized by the triad of **parkinsonism**, **fluctuating cognition**, and **visual hallucinations**, with cognitive decline occurring within one year of motor symptoms. - Histopathology shows **alpha-synuclein** positive eosinophilic inclusions called **Lewy bodies** in both the **substantia nigra** and the **cerebral cortex**. *Parkinson disease with dementia* - Clinical diagnosis requires that **motor symptoms** (tremor, rigidity, bradykinesia) precede **dementia** by at least one year. - While it also features **Lewy bodies**, they are initially localized to the **brainstem** and only reach the cortex in the very late stages of the disease. *Progressive supranuclear palsy* - Presents with **vertical gaze palsy**, postural instability, and frequent falls early in the disease course. - Pathologically characterized by **tau protein** aggregates forming **globose neurofibrillary tangles**, rather than alpha-synuclein inclusions. *Corticobasal degeneration* - Features progressive **asymmetric parkinsonism**, limb apraxia, and the **alien limb phenomenon**. - Histology reveals **tau-positive** neuronal and glial inclusions, specifically **astrocytic plaques** and thread-like lesions in the cortex and basal ganglia. *Multiple system atrophy* - Characterized by a combination of parkinsonism, **autonomic dysfunction** (orthostatic hypotension), and **cerebellar ataxia**. - Histopathology shows **alpha-synuclein** inclusions, but these are uniquely found within **oligodendrocytes** as **glial cytoplasmic inclusions**.

Q: A 28-year-old woman presents with vision loss in her right eye and numbness in her left leg that resolved spontaneously over 2 weeks. MRI shows multiple periventricular white matter lesions perpendicular to the lateral ventricles (Dawson fingers). Lumbar puncture reveals oligoclonal bands and elevated IgG index. Which pathologic finding would be expected on brain biopsy?

Perivenular demyelination with relative axonal preservation. ***Perivenular demyelination with relative axonal preservation*** - This finding is the hallmark of **Multiple Sclerosis (MS)**, where autoimmune attacks target **oligodendrocytes**, causing plaques in the **white matter**. - The preservation of **axons** (relative to the loss of myelin) is a key diagnostic feature, especially in the presence of **Dawson fingers** and **oligoclonal bands**. *Concentric layers of demyelination and remyelination (onion bulbs)* - This is characteristic of **Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)** or **Charcot-Marie-Tooth disease**, involving the **peripheral nervous system**. - It represents repeated cycles of **segmental demyelination** and Schwann cell proliferation, rather than **CNS white matter** plaques. *Spongiform vacuolation of grey matter* - This finding is typical of **Prion diseases**, such as **Creutzfeldt-Jakob Disease (CJD)**, which present with rapidly progressive dementia. - It involves the formation of **small vacuoles** within the neuropil and neurons, unlike the **inflammatory demyelination** seen in MS. *Lewy bodies in substantia nigra neurons* - These are **alpha-synuclein** inclusions pathognomonic for **Parkinson's disease**, leading to motor symptoms like **bradykinesia** and resting tremors. - This pathology affects **deep grey matter** nuclei rather than the **periventricular white matter** lesions seen in this patient. *Neurofibrillary tangles and neuritic plaques* - These are the classic pathologic markers for **Alzheimer's Disease**, involving **hyperphosphorylated tau** and **amyloid-beta** deposition. - They primarily affect the **cerebral cortex** and **hippocampus**, resulting in cognitive decline rather than **focal neurological deficits** like vision loss.

Q: A 72-year-old man with a history of chronic hypertension presents with sudden onset right hemiparesis and aphasia. CT scan shows a hyperdense lesion in the left basal ganglia with surrounding edema and mass effect. He dies 48 hours later. Autopsy reveals a cavity filled with blood clot and hemosiderin-laden macrophages. Which vessels were most likely affected?

Lenticulostriate branches of the middle cerebral artery. ***Lenticulostriate branches of the middle cerebral artery*** - These small penetrating arteries are the most common site of **hypertensive intracerebral hemorrhage**, specifically involving the **basal ganglia** (putamen) and internal capsule. - Chronic hypertension leads to **Charcot-Bouchard microaneurysms** and **lipohyalinosis**, making these vessels prone to rupture and subsequent hematoma formation. *Posterior cerebral artery perforating branches* - These branches typically supply the **thalamus** and midbrain; while a site for hypertensive bleeds, they do not primarily supply the **basal ganglia**. - Rupture here usually results in **pure sensory loss** or thalamic syndromes rather than the motor hemiparesis and aphasia described. *Superior cerebellar artery branches* - These vessels supply the **cerebellum** and midbrain; an injury here would present with **ataxia**, vertigo, and nausea. - They are not associated with **aphasia** or the specific CT finding of a left basal ganglia lesion. *Anterior choroidal artery branches* - This artery supplies the **optic tract**, choroid plexus, and parts of the internal capsule, but is a less frequent site for spontaneous hypertensive hemorrhage. - Obstruction or rupture would typically cause a clinical triad of **hemiplegia**, hemianesthesia, and **homonymous hemianopia**. *Anterior communicating artery branches* - This is the most common site for **saccular (berry) aneurysms**, which typically cause **subarachnoid hemorrhage** rather than focal intraparenchymal bleeding. - A bleed here usually presents with a sudden "**worst headache of life**" and blood localized within the cisterns on a CT scan.

Q: A 45-year-old woman presents with progressive weakness, ataxia, and dementia over 6 months. MRI shows diffuse cortical atrophy and T2 hyperintensities in the basal ganglia. EEG demonstrates periodic sharp wave complexes. CSF analysis shows elevated 14-3-3 protein and normal cell count. Brain biopsy reveals spongiform changes and no inflammation. What is the most likely underlying pathogenic mechanism?

Accumulation of misfolded prion protein (PrPSc). ***Accumulation of misfolded prion protein (PrPSc)*** - This patient presents with **Creutzfeldt-Jakob disease (CJD)**, characterized by rapid **progressive dementia**, ataxia, and **periodic sharp wave complexes** on EEG. - The pathogenesis involves the conversion of normal **PrPC** into the abnormal **PrPSc** isoform, leading to **spongiform degeneration** and the presence of **14-3-3 protein** in CSF. *Autoimmune antibodies against neuronal surface antigens* - This describes **Autoimmune Encephalitis** (e.g., Anti-NMDA receptor encephalitis), which typically reveals a **pleocytosis** on CSF analysis unlike this case. - While it causes rapid cognitive decline, it lacks the characteristic **spongiform changes** and specific EEG findings seen in CJD. *Progressive demyelination due to oligodendrocyte loss* - This mechanism is characteristic of **Progressive Multifocal Leukoencephalopathy (PML)**, usually associated with **JC virus** reactivation in immunocompromised patients. - MRI in PML shows focal white matter lesions rather than the **diffuse cortical atrophy** and basal ganglia involvement seen here. *Accumulation of hyperphosphorylated tau protein* - Tau pathology is central to **Alzheimer's disease** (neurofibrillary tangles) and **Frontotemporal dementia**, which typically progress over years rather than months. - These conditions do not present with **periodic sharp waves** on EEG or the specific CSF marker **14-3-3 protein**. *Deposition of beta-amyloid plaques* - Extracellular **amyloid-beta plaques** are the hallmark of chronic neurodegeneration in **Alzheimer's disease**. - This mechanism does not explain the **spongiform vacuolation** of the neuropil or the acute clinical deterioration observed in this patient.

Q: Cork screw inclusion bodies in brain biopsy specimen are seen in:

Pilocytic astrocytoma. ***Pilocytic astrocytoma*** - **Rosenthal fibers**, which have a **corkscrew appearance**, are characteristic histological findings in pilocytic astrocytomas. - These are **eosinophilic, elongated, glial inclusions** often found in the processes of tumor cells. - Pilocytic astrocytomas are typically **benign (WHO Grade I)** tumors commonly found in children and young adults. *Medulloblastoma* - Characterized by **small, round blue cells** and a high mitotic index, often forming **Homer-Wright rosettes**. - **Rosenthal fibers** are not typically associated with medulloblastoma. *Ependymoma* - Characterized by **ependymal rosettes** (tumor cells arranged around a central lumen) and **perivascular pseudorosettes** (tumor cells radiating around blood vessels). - Lacks the presence of **corkscrew inclusion bodies (Rosenthal fibers)**. *Pinealoma* - This is a general term for tumors of the pineal gland, which can include pineocytomas and pineoblastomas. - While various histological features can be seen depending on the specific type, **Rosenthal fibers** are not a characteristic finding for these tumors. *Glioblastoma* - The most common malignant primary brain tumor in adults, characterized by **pseudopalisading necrosis** and **microvascular proliferation**. - Histologically shows pleomorphic cells, not the organized **Rosenthal fibers** seen in pilocytic astrocytoma.

Q: All are true about the brain tumour associated with the below mentioned histopathological findings except:

Association with NF-1. ***Association with NF-1*** - The histopathological findings described (likely referring to **meningioma** based on the other options) are not typically associated with **Neurofibromatosis type 1 (NF-1)** [1]. - **NF-1** is more commonly linked to **optic nerve gliomas**, neurofibromas, and other peripheral nerve sheath tumors. *Most frequent cytogenetic abnormality is deletion of 22q* - **Deletion of 22q** is the most common cytogenetic abnormality found in **meningiomas** [2]. - This deletion often involves the **NF2 gene**, which is a tumor suppressor gene [2]. *Intracranial calcification* - **Intracranial calcification** is a common finding in **meningiomas**, particularly in older lesions [2]. - These calcifications can be seen on imaging studies like CT scans. *Psammoma bodies* - **Psammoma bodies** are characteristic histological features of **meningiomas**, especially the meningothelial and transitional subtypes [1][2]. - They are concentric, lamellated calcified structures formed from degenerating cells. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 727-728. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1316-1317.

Q: The following microscopic appearance is that of a schwannoma which most commonly involves the cerebellopontine angle. What does the area marked with the arrow represent?

Antony B pattern. ***Antony B pattern-Verocay cells*** - The area marked with the arrow represents **Antony B pattern**, which is characterized by **hypocellularity** and a **myxoid matrix** [1]. - **Verocay bodies** are specific to schwannomas and are formed by palisading nuclei surrounding an anucleate zone, typically found within the **Antony A pattern** [1]. *Antony A pattern* - **Antony A pattern** is characterized by **dense cellularity** with palisading nuclei and often contains **Verocay bodies** [1]. - This pattern is typically more organized and compact compared to the area shown. *Antony C pattern-Verocay cells* - There is no recognized **Antony C pattern** in the histological classification of schwannomas. - The primary patterns described are **Antony A** and **Antony B**. *Antony D pattern-Verocay cells* - Similar to Antony C, there is no recognized **Antony D pattern** in the histological classification of schwannomas. - This option is a distractor based on an incorrect classification. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, p. 1250.

Q: The following cell is seen in:

Plasmacytoma. ***Plasmacytoma*** - The image likely depicts a **plasma cell**, characterized by an **eccentric nucleus**, **perinuclear halo** (Golgi zone), and **basophilic cytoplasm**. These cells proliferate in plasmacytoma [2]. - Plasmacytoma is a localized proliferation of **neoplastic plasma cells**, which can occur in bone or extramedullary sites. *Langerhans cell histiocytosis* - This condition involves the proliferation of **Langerhans cells**, which are dendritic cells with characteristic **Birbeck granules** (tennis-racket shaped inclusions) on electron microscopy [1]. - The cells in Langerhans cell histiocytosis have folded or grooved nuclei, distinct from the plasma cell morphology [1]. *Viral encephalitis* - Viral encephalitis is characterized by inflammation of the brain parenchyma, often with **lymphocytic infiltrates** and **microglial nodules**. - While plasma cells can be present in chronic inflammation, they are not the defining cell type, and the primary pathology involves neuronal damage and glial reactions. *Leukemia* - Leukemia involves the proliferation of **immature white blood cells** (blasts) in the bone marrow and peripheral blood. - The morphology of leukemic cells varies depending on the type (e.g., myeloid blasts, lymphoid blasts) but does not typically resemble mature plasma cells. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 630. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 609-610.

Question 1

A 65-year-old man presents with progressive bradykinesia, resting tremor, and cogwheel rigidity. He later develops visual hallucinations and fluctuating cognition. He dies 8 years after symptom onset. Autopsy shows depigmentation of substantia nigra, and microscopy reveals eosinophilic cytoplasmic inclusions in neurons that are immunoreactive for alpha-synuclein. These inclusions are also found in the cortex. What is the most likely diagnosis?

Accepted Answer

Dementia with Lewy bodies

Answer

Parkinson disease with dementia

Answer

Progressive supranuclear palsy

Answer

Corticobasal degeneration

Answer

Multiple system atrophy

Question 2

A 28-year-old woman presents with vision loss in her right eye and numbness in her left leg that resolved spontaneously over 2 weeks. MRI shows multiple periventricular white matter lesions perpendicular to the lateral ventricles (Dawson fingers). Lumbar puncture reveals oligoclonal bands and elevated IgG index. Which pathologic finding would be expected on brain biopsy?

Accepted Answer

Perivenular demyelination with relative axonal preservation

Answer

Concentric layers of demyelination and remyelination (onion bulbs)

Answer

Spongiform vacuolation of grey matter

Answer

Lewy bodies in substantia nigra neurons

Answer

Neurofibrillary tangles and neuritic plaques

Question 3

A 72-year-old man with a history of chronic hypertension presents with sudden onset right hemiparesis and aphasia. CT scan shows a hyperdense lesion in the left basal ganglia with surrounding edema and mass effect. He dies 48 hours later. Autopsy reveals a cavity filled with blood clot and hemosiderin-laden macrophages. Which vessels were most likely affected?

Accepted Answer

Lenticulostriate branches of the middle cerebral artery

Answer

Posterior cerebral artery perforating branches

Answer

Superior cerebellar artery branches

Answer

Anterior choroidal artery branches

Answer

Anterior communicating artery branches

Question 4

A 45-year-old woman presents with progressive weakness, ataxia, and dementia over 6 months. MRI shows diffuse cortical atrophy and T2 hyperintensities in the basal ganglia. EEG demonstrates periodic sharp wave complexes. CSF analysis shows elevated 14-3-3 protein and normal cell count. Brain biopsy reveals spongiform changes and no inflammation. What is the most likely underlying pathogenic mechanism?

Accepted Answer

Accumulation of misfolded prion protein (PrPSc)

Answer

Autoimmune antibodies against neuronal surface antigens

Answer

Progressive demyelination due to oligodendrocyte loss

Answer

Accumulation of hyperphosphorylated tau protein

Answer

Deposition of beta-amyloid plaques

Question 5

The following histological biopsy image shows presence of:

Accepted Answer

Henderson-Peterson bodies

Answer

Pappenheimer bodies

Answer

Negri bodies

Answer

Neurofibrillary tangles

Answer

Cowdry bodies

Question 6

Cork screw inclusion bodies in brain biopsy specimen are seen in:

Accepted Answer

Pilocytic astrocytoma

Answer

Medulloblastoma

Answer

Ependymoma

Answer

Pinealoma

Answer

Glioblastoma

Question 7

A 5-year-old child presented with gait ataxia and tremors in left hand. The image shows:

Accepted Answer

Medulloblastoma, Pseudorosettes

Answer

Cerebellar astrocytoma, Pseudorosettes

Answer

Meningioma, Psammoma bodies

Answer

Ependymoma, Perivascular pseudorosettes

Answer

Pilocytic astrocytoma, Rosenthal fibers

Question 8

All are true about the brain tumour associated with the below mentioned histopathological findings except:

Accepted Answer

Association with NF-1

Answer

Most frequent cytogenetic abnormality is deletion of 22q

Answer

Intracranial calcification

Answer

Psammoma bodies

Answer

Most common extra-axial tumor in adults

Question 9

The following microscopic appearance is that of a schwannoma which most commonly involves the cerebellopontine angle. What does the area marked with the arrow represent?

Accepted Answer

Antony B pattern

Answer

Antony A pattern

Answer

Antony C pattern-Verocay cells

Answer

Antony D pattern-Verocay cells

Answer

Verocay bodies

Question 10

The following cell is seen in:

Accepted Answer

Plasmacytoma

Answer

Langerhans cell histiocytosis

Answer

Viral encephalitis

Answer

Leukemia

Answer

Multiple myeloma

Neuropathology — MCQs

Neuropathology — MCQs

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