Pediatric Neuroradiology — MCQs

10 questions

A child presented to the casualty with seizures. On examination multiple oval hypo-pigmented macules were noted on the trunk, along with sub-normal IQ. Probable diagnosis of the child is:

What is the most common cerebellar tumor in children?

Symptomatic neonatal CNS involvement is most commonly seen in which group of congenital intrauterine infections?

Which of the following is the most common neurocutaneous syndrome?

True statement regarding brain tumors in children is:

A 10 year old child presented with headache, vomiting, gait instability and diplopia. On examination he had papilledema and gait ataxia. The most probable diagnosis is –

All of the following are neural tube defects except:

Which of the following is not a neuronal migration disorder

Parameningeal Rhabdomyosarcoma is best diagnosed by:

Q10

Shape of extradural hematoma on NCCT is?

Pediatric Neuroradiology Indian Medical PG Practice Questions and MCQs

Question 1: A child presented to the casualty with seizures. On examination multiple oval hypo-pigmented macules were noted on the trunk, along with sub-normal IQ. Probable diagnosis of the child is:

A. Tuberous Sclerosis (Correct Answer)
B. Neurofibromatosis
C. Incontinentia Pigmenti
D. Sturge-Weber syndrome

Explanation: ***Tuberous Sclerosis*** - The combination of **seizures**, **hypopigmented macules** (ash-leaf spots), and **subnormal IQ** (intellectual disability) is highly characteristic of tuberous sclerosis. - Tuberous sclerosis is an **autosomal dominant neurocutaneous disorder** causing benign tumor growth in various organs, including the brain, skin, and kidneys. *Neurofibromatosis* - Characterized by **café-au-lait spots**, **neurofibromas**, and **Lisch nodules** (iris hamartomas), not hypopigmented macules. - While seizures can occur, intellectual disability is less common than in tuberous sclerosis, and the distinctive skin lesions differ. *Incontinentia Pigmenti* - Typically presents with **swirling hyperpigmented lesions** (marbled or whorled appearance) that evolve through different stages (vesicular, verrucous, hyperpigmented), not hypopigmented macules. - While it can be associated with seizures and intellectual disability, the skin findings are distinctly different. *Sturge-Weber syndrome* - Characterized by a **port-wine stain** (facial cutaneous angioma) typically involving the ophthalmic division of the trigeminal nerve, ipsilateral leptomeningeal angioma, and ocular abnormalities like glaucoma. - Seizures are common due to brain involvement, but **hypopigmented macules** and subnormal IQ in this configuration are not primary features.

Question 2: What is the most common cerebellar tumor in children?

A. Ependymoma
B. Medulloblastoma (Correct Answer)
C. PNET
D. Astrocytoma

Explanation: ***Medulloblastoma*** - **Medulloblastoma** is the most common **malignant** cerebellar tumor in children, accounting for about 20% of all childhood brain tumors [2]. - In the context of this question, medulloblastoma is considered the "most common cerebellar tumor" as it is the most frequently encountered **malignant** tumor requiring aggressive treatment. - These tumors arise from neuroectodermal cells in the cerebellum and are typically **highly aggressive**, often spreading through the cerebrospinal fluid (CSF) pathways [1], [2]. - Peak incidence is between 5-9 years of age, with a male predominance [1]. *Astrocytoma* - **Cerebellar pilocytic astrocytomas** are actually the most common **benign** cerebellar tumor in children and represent a significant portion of all cerebellar tumors [1]. - However, in competitive exam contexts, when asking about "most common cerebellar tumor," the question typically refers to **malignant tumors**, where medulloblastoma takes precedence. - **Pilocytic astrocytomas** are usually low-grade (WHO Grade I) and have an excellent prognosis, often presenting as cystic lesions with a mural nodule. *Ependymoma* - **Ependymomas** are the third most common posterior fossa tumor in children (after medulloblastoma and pilocytic astrocytoma). - They typically arise from the ependymal lining of the **fourth ventricle**, making them cerebellar-adjacent rather than primarily cerebellar tumors [3], [4]. - They account for about 10% of pediatric brain tumors and have an intermediate prognosis. *PNET* - **PNET (Primitive Neuroectodermal Tumor)** is a historical term that has largely been replaced by more specific classifications in the current WHO CNS tumor classification. - Medulloblastoma was previously classified as a type of PNET, but is now recognized as a distinct entity. - The term PNET is now rarely used in modern neuropathology practice, having been superseded by molecular and genetic classification systems. **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. 1314-1315. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 726-727. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1312-1313.

Question 3: Symptomatic neonatal CNS involvement is most commonly seen in which group of congenital intrauterine infections?

A. Rubella and toxoplasmosis
B. CMV and syphilis
C. Rubella and HSV
D. CMV and toxoplasmosis (Correct Answer)

Explanation: ***CMV and toxoplasmosis*** - Both **cytomegalovirus (CMV)** and **Toxoplasma gondii** are well-known causes of congenital infections that frequently lead to significant and symptomatic central nervous system (CNS) involvement in neonates. - Congenital CMV can cause **microcephaly**, **periventricular calcifications**, **hearing loss**, and developmental delay, while congenital toxoplasmosis can result in **hydrocephalus**, **intracranial calcifications**, **chorioretinitis**, and seizures. *Rubella and toxoplasmosis* - While **toxoplasmosis** causes significant CNS involvement, **congenital rubella syndrome** typically presents with cataracts, heart defects (e.g., patent ductus arteriosus), and hearing loss, with CNS involvement being less consistently severe or frequently symptomatic in the immediate neonatal period compared to CMV or toxoplasmosis. - Although rubella can cause **encephalitis** or **meningoencephalitis**, these are not as common or consistently severe as the direct destructive CNS lesions seen with CMV or toxoplasmosis. *CMV and syphilis* - **CMV** is a major cause of neonatal CNS symptoms. However, **congenital syphilis** primarily affects bones, skin, and mucous membranes (e.g., "snuffles"), with CNS involvement typically presenting as **meningitis**, **hydrocephalus**, or neurodevelopmental delays, but often not as overtly symptomatic in the immediate neonatal period as CMV or toxoplasmosis. - While syphilis can cause neurosyphilis, the spectrum and severity of immediate symptomatic CNS involvement are distinct from the widespread calcifications and structural abnormalities seen with CMV or toxoplasmosis. *Rubella and HSV* - **Rubella** primarily causes classic congenital defects in eyes, ears, and heart, with CNS effects being less common and severe. - **Congenital herpes simplex virus (HSV)** infection, while causing severe CNS disease (e.g., encephalitis) when disseminated, is relatively rare overall compared to CMV and toxoplasmosis, and often presents with skin, eye, and mouth lesions first.

Question 4: Which of the following is the most common neurocutaneous syndrome?

A. Sturge Weber syndrome
B. Neurofibromatosis (Correct Answer)
C. Von Hippel Lindau disease
D. Tuberous sclerosis

Explanation: ***Neurofibromatosis*** - **Neurofibromatosis type 1 (NF1)**, also known as von Recklinghausen disease, is the most common of all neurocutaneous syndromes, affecting about 1 in 3,000 live births. - It is characterized by **café-au-lait spots**, **axillary/groin freckling**, **neurofibromas**, **Lisch nodules**, and optic pathway gliomas. *Sturge Weber syndrome* - This syndrome is characterized by a **port-wine stain (facial angioma)**, leptomeningeal angioma, and glaucoma; it is less common than neurofibromatosis. - It is a **sporadic disorder** and does not show an inherited pattern like neurofibromatosis. *Von Hippel Lindau disease* - This is a rare, **autosomal dominant** inherited disorder characterized by various tumors such as **hemangioblastomas** in the cerebellum, retina, and spinal cord, as well as renal cysts and pheochromocytomas. - Its prevalence is significantly lower than neurofibromatosis. *Tuberous sclerosis* - This is an **autosomal dominant** disorder marked by the growth of **benign tumors** in the brain, heart, kidneys, skin, and other organs, such as **facial angiofibromas** and **epilepsy**. - While significant, its incidence is approximately 1 in 6,000 to 1 in 10,000 live births, making it less common than neurofibromatosis.

Question 5: True statement regarding brain tumors in children is:

A. Is the most common tumor in children
B. Hydrocephalus is rare
C. Papilledema is rare
D. Mostly is infratentorial (Correct Answer)

Explanation: ***Mostly is infratentorial*** - Brain tumors in children are **predominantly infratentorial**, meaning they occur in the cerebellum, brainstem, and fourth ventricle. - This anatomical distribution accounts for many common pediatric brain tumors like **medulloblastoma** and **pilocytic astrocytoma**. *Is the most common tumor in children* - **Leukemia** is the most common cancer in children, not brain tumors. - Brain tumors are the **second most common** childhood cancer, after leukemia. *Hydrocephalus is rare* - **Hydrocephalus is common** in pediatric brain tumors, especially those in the posterior fossa, due to obstruction of CSF flow. - Tumors located near the **ventricles** or in the posterior fossa frequently block CSF pathways, leading to hydrocephalus and increased intracranial pressure. *Papilledema is rare* - **Papilledema is common** in children with brain tumors, as it is a sign of increased intracranial pressure. - The elevated pressure within the cranium due to tumor growth or hydrocephalus can compress the **optic nerve sheath**, impairing axoplasmic flow and causing optic disc swelling.

Question 6: A 10 year old child presented with headache, vomiting, gait instability and diplopia. On examination he had papilledema and gait ataxia. The most probable diagnosis is –

A. Suprasellar tumour
B. Hydrocephalus
C. Brain stem tumour
D. Midline posterior fossa tumour (Correct Answer)

Explanation: ***Midline posterior fossa tumour*** - The combination of **headache, vomiting, papilledema (signs of increased intracranial pressure)**, **gait instability, and ataxia** strongly suggests a **midline posterior fossa tumor** in a child. These tumors often obstruct CSF flow, leading to hydrocephalus and cerebellar symptoms. - Common tumors in this location in children include **medulloblastoma** and **pilocytic astrocytoma**, which frequently present with these symptoms due to their proximity to the **fourth ventricle** and **cerebellum**. *Suprasellar tumour* - **Suprasellar tumors** typically present with **visual field deficits** (e.g., bitemporal hemianopia) due to compression of the optic chiasm, and/or **endocrine dysfunction** (e.g., growth delay, diabetes insipidus). - While they can cause hydrocephalus and increased intracranial pressure if large, the prominent **gait instability and ataxia** point away from a primary suprasellar lesion as the most likely cause. *Hydrocephalus* - **Hydrocephalus** itself explains the **increased intracranial pressure (headache, vomiting, papilledema)** and sometimes **gait instability (ataxia)**. - However, hydrocephalus is usually a *consequence* of an underlying obstruction, and in a child presenting acutely with cerebellar dysfunction, a **tumor blocking CSF flow in the posterior fossa** is the most probable underlying cause, not hydrocephalus as the primary diagnosis. *Brain stem tumour* - **Brain stem tumors** typically cause **cranial nerve deficits** (e.g., facial weakness, dysphagia), **long tract signs (hemiparesis)**, and often **multiple types of ataxia**, alongside signs of increased intracranial pressure if they obstruct CSF flow. - While gait instability and diplopia can occur, the overall picture of prominent **gait ataxia** and papilledema without other focal cranial nerve signs makes a primary midline posterior fossa tumor compressing the cerebellum and fourth ventricle more likely.

Question 7: All of the following are neural tube defects except:

A. Holoprosencephaly (Correct Answer)
B. Encephalocele
C. Myelomeningocele
D. Anencephaly

Explanation: ***Holoprosencephaly*** - This condition results from the **failure of the prosencephalon (forebrain)** to properly divide into two hemispheres, often leading to facial abnormalities. - It is considered a **forebrain malformation**, distinct from neural tube closure defects. *Encephalocele* - An encephalocele is a **neural tube defect** characterized by the protrusion of brain tissue and meninges through an opening in the skull. - It results from the **incomplete closure of the neural tube** during embryonic development. *Myelomeningocele* - Myelomeningocele is a severe form of **spina bifida**, a neural tube defect where the spinal cord and meninges protrude through an opening in the spine [2]. - This defect arises from the **failure of the neural tube to close completely** in the caudal region [1]. *Anencephaly* - Anencephaly is a lethal **neural tube defect** characterized by the absence of a major portion of the brain, skull, and scalp [3]. - It occurs due to the **failure of the cranial end of the neural tube to close** [3].

Question 8: Which of the following is not a neuronal migration disorder

A. Lissencephaly
B. Schizencephaly
C. Porencephaly
D. Anencephaly (Correct Answer)

Explanation: **Anencephaly** - **Anencephaly** is a **neural tube defect** characterized by the absence of a major portion of the brain, skull, and scalp, resulting from a failure of the upper part of the neural tube to close during early embryonic development. - It is a **developmental malformation** but not specifically a disorder of neuronal migration, which concerns the movement of neurons to their correct locations in the cerebral cortex. *Lissencephaly* - **Lissencephaly** (literally "smooth brain") is a rare brain formation disorder characterized by the absence of normal **gyri** (folds) and **sulci** (grooves) in the cerebral cortex. - This condition results from defective neuronal migration during fetal development, leading to a smooth brain surface. *Schizencephaly* - **Schizencephaly** is a rare congenital disorder characterized by abnormal slits or clefts in the cerebral hemispheres, extending from the pial surface to the ventricles. - These clefts are caused by abnormal neuronal migration and development during gestation. *Porencephaly* - **Porencephaly** is a rare neurological disorder characterized by cyst-like cavities within the cerebral hemisphere that are usually remnants of destructive lesions or malformations. - While it can be associated with neuronal migration disorders, it is more often a result of **focal destructive lesions**, such as stroke or infection, rather than a primary migration defect itself.

Question 9: Parameningeal Rhabdomyosarcoma is best diagnosed by:

A. MRI (Correct Answer)
B. CT Scan
C. SPECT
D. PET

Explanation: ***MRI*** - **Magnetic Resonance Imaging (MRI)** provides excellent soft tissue contrast, which is crucial for visualizing the extent of **parameningeal rhabdomyosarcoma** and its relationship to critical structures like the **meninges**, **brainstem**, and **cranial nerves**. - It is superior for detecting **intracranial extension**, **bone erosion**, and assessing response to treatment, making it the preferred imaging modality for diagnosis and staging. *CT Scan* - **Computed Tomography (CT) scans** are good for evaluating bone involvement and calcifications but offer less detailed soft tissue resolution compared to MRI. - While it can identify large masses, it may miss subtle extensions or involvement of the **meninges** that are readily seen on MRI. *SPECT* - **Single-Photon Emission Computed Tomography (SPECT)** is a nuclear medicine imaging technique primarily used to assess organ function and blood flow, often in cardiology or neurology for functional studies. - It provides limited anatomical detail for the precise localization and characterization of soft tissue tumors like **rhabdomyosarcoma**. *PET* - **Positron Emission Tomography (PET) scans** are excellent for detecting metabolically active tumors, assessing disease burden, and identifying distant metastases, especially when combined with CT (**PET/CT**). - However, while useful for staging and follow-up, it does not provide the high-resolution anatomical detail of the primary tumor's local extent and its relationship to adjacent structures as effectively as **MRI**.

Question 10: Shape of extradural hematoma on NCCT is?

A. Bean shaped
B. Crescent shaped
C. Lens shaped (Correct Answer)
D. Medially concave

Explanation: ***Lens shaped*** - An **extradural hematoma** (EDH) appears **biconvex** or **lens-shaped** because it is limited by the cranial sutures, where the dura mater is tightly attached to the inner table of the skull. - This characteristic shape helps differentiate it from other intracranial hemorrhages on **non-contrast CT (NCCT)** scans. *Bean shaped* - While descriptive, "bean-shaped" is not the standard or most accurate descriptor for an EDH, which is typically more organized and sharply delineated due to its confinement. - This term might be loosely applied to other lesions but lacks the precision needed for a confident diagnosis of EDH. *Crescent shaped* - A **crescent shape** is characteristic of a **subdural hematoma (SDH)**, which spreads more freely over the brain surface as it is located between the dura and arachnoid mater, not limited by sutures. - This shape indicates bleeding across suture lines, a key differentiator from EDH. *Medially concave* - This describes the typical appearance of a **subdural hematoma** (SDH), where the collection of blood conforms to the curvature of the brain surface and can extend across suture lines. - An EDH is typically **convex** towards the brain parenchyma because of the dura's adherence to the skull at the sutures.

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