Pediatric Neuro-ophthalmology — MCQs
A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?
Inability to abduct left eye with LMN fascial palsy on same side. The lesion is in
All of the following can lead to increased intracranial pressure except -
Third nerve palsy causes all of the following except:
Opsoclonus-myoclonus syndrome is seen in -
Toxoplasma in children causes:
The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?
What type of diplopia is typically associated with sixth cranial nerve palsy?
A patient with ptosis has the upper 4 mm of cornea covered by the upper eyelid. What is the grade of ptosis?
In blowout fractures, which of the following is seen?
Pediatric Neuro-ophthalmology Indian Medical PG Practice Questions and MCQs
Question 1: A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?
- A. Immunohistochemical detection of chromogranin is not useful for diagnosis. (Correct Answer)
- B. 70-80% of tumors are associated with elevated production of catecholamines.
- C. Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.
- D. The most common site of tumor is adrenal medulla.
Explanation: ***Immunohistochemical detection of chromogranin is not useful for diagnosis.*** - This statement is **false** because **neuroblastoma** cells, which originate from neural crest cells, commonly express **chromogranin A** and C, along with other neuroendocrine markers like **synaptophysin** and **neuron-specific enolase (NSE)** [1]. - **Immunohistochemical staining** for chromogranin is thus a **useful diagnostic tool** to confirm the neuroendocrine differentiation of the tumor [1]. *The most common site of tumor is adrenal medulla.* - This statement is **true**. Approximately **50% of neuroblastomas** originate in the **adrenal glands**, specifically the adrenal medulla, because it is derived from neural crest cells, the precursor cells for neuroblastoma [1]. - Other common sites include the paraspinal ganglia, such as the posterior mediastinum, pelvis, and neck. *70-80% of tumors are associated with elevated production of catecholamines.* - This statement is **true**. Neuroblastoma cells often retain the ability to synthesize and secrete **catecholamines** (**epinephrine, norepinephrine, dopamine**) [1]. - Elevated levels of **vanillylmandelic acid (VMA)** and **homovanillic acid (HVA)**, which are the **breakdown products (metabolites) of catecholamines**, are detected in the urine of 70-80% of patients and serve as **important diagnostic and prognostic markers** [1]. *Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.* - This statement is **true**. **Deletion** or **rearrangement** of the **short arm of chromosome 1 (1p36)** is a common **cytogenetic abnormality** found in 25-35% of neuroblastomas. - This genetic alteration is often associated with **poor prognosis** and more aggressive disease behavior. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-487.
Question 2: Inability to abduct left eye with LMN fascial palsy on same side. The lesion is in
- A. Left pons (Correct Answer)
- B. Cerebellar lesions
- C. CP angle tumor
- D. Right pons
Explanation: ***Left pons*** - A lesion in the **left pons** can affect both the **left abducens nucleus** (leading to inability to abduct the left eye) and the **left facial nucleus or nerve fibers** (causing left lower motor neuron facial palsy). [2] - This specific combination of ipsilateral (same-sided) symptoms is characteristic of a brainstem lesion, particularly within the pons where these cranial nerve nuclei are in close proximity. [1] *Cerebellar lesions* - **Cerebellar lesions** primarily cause symptoms like **ataxia**, dysmetria, and nystagmia, but typically do not cause isolated cranial nerve palsies of the abducens and facial nerves. [1] - While coordinating movements, the cerebellum does not house the nuclei for direct eye abduction or facial muscle control. *CP angle tumor* - A **cerebellopontine (CP) angle tumor** can affect cranial nerves VI and VII, but it typically presents with other symptoms like **vestibulocochlear nerve (VIII) dysfunction (hearing loss, vertigo)** early on due to its anatomical location. - While it can eventually compress the abducens and facial nerves, the combination of **isolated abducens and facial palsy** without VIII nerve involvement points more directly to an intraparenchymal pontine lesion. *Right pons* - A lesion in the **right pons** would cause **right-sided inability to abduct the eye** and **right-sided LMN facial palsy**, not left-sided as described in the case. - Brainstem lesions typically produce ipsilateral cranial nerve deficits due to the close proximity of the nuclei and fascicles before decussation of some pathways.
Question 3: All of the following can lead to increased intracranial pressure except -
- A. Meningitis
- B. Migraine (Correct Answer)
- C. Subdural hemorrhage
- D. Subarachnoid Hemorrhage
Explanation: ***Migraine*** - Migraine headaches typically *do not* cause **increased intracranial pressure (ICP)**; rather, they are primarily related to neurovascular dysfunction. - While severe migraines can mimic some symptoms of increased ICP (e.g., headache, nausea), they do not directly lead to a sustained rise in **intracranial pressure**. *Meningitis* - **Inflammation of the meninges** can lead to increased intracranial pressure by causing **cerebral edema**, hydrocephalus due to obstructed CSF flow, or increased CSF production [2]. - The inflammatory response can increase **vascular permeability**, leading to fluid accumulation within the brain tissue. *Subdural hemorrhage* - A **subdural hemorrhage** involves bleeding into the space between the dura mater and the arachnoid mater, leading to the formation of a **hematoma** [1]. - This mass lesion occupies space within the rigid skull, directly increasing **intracranial pressure** by compressing brain tissue [3]. *Subarachnoid Hemorrhage* - A **subarachnoid hemorrhage** is bleeding into the space between the arachnoid membrane and the pia mater, often due to an **aneurysm rupture**. - The presence of blood in the subarachnoid space can **obstruct CSF flow**, irritate meninges, and cause **cerebral vasospasm**, all contributing to increased intracranial pressure [4].
Question 4: Third nerve palsy causes all of the following except:
- A. Outward eye movement
- B. Diplopia
- C. Miosis (Correct Answer)
- D. Ptosis
Explanation: ***Miosis*** - A complete third nerve palsy typically presents with a **dilated pupil (mydriasis)** due to unopposed sympathetic innervation, not miosis [1]. - **Miosis** (pupil constriction) would occur if the parasympathetic fibers of the third nerve were overactive or due to sympathetic denervation (Horner's syndrome) [2]. *Ptosis* - The **levator palpebrae superioris muscle**, responsible for eyelid elevation, is innervated by the oculomotor (third) nerve [2]. - Damage to this nerve paralyzes the muscle, leading to **drooping of the eyelid (ptosis)** [1]. *Outward eye movement* - The oculomotor nerve innervates most extraocular muscles, including the medial, superior, and inferior rectus, and the inferior oblique. - When these muscles are paralyzed, the unopposed action of the **lateral rectus** (innervated by CN VI) causes the eye to deviate **laterally (outward)** [1]. *Diplopia* - Paralysis of the extraocular muscles innervated by the third nerve leads to **misalignment of the eyes** [1]. - This misalignment prevents the brain from fusing the images from both eyes, resulting in **double vision (diplopia)** [1].
Question 5: Opsoclonus-myoclonus syndrome is seen in -
- A. Melanoma
- B. Neuroblastoma (Correct Answer)
- C. Retinoblastoma
- D. Nephroblastoma
Explanation: ***Neuroblastoma*** - Opsoclonus-myoclonus syndrome (OMS) is a **rare paraneoplastic neurological syndrome** most commonly associated with **neuroblastoma** in children [1]. - The syndrome is characterized by **opsoclonus** (chaotic, multidirectional eye movements), **myoclonus** (brief, involuntary muscle jerks), and ataxia. *Melanoma* - Melanoma is rarely associated with OMS; however, it can be linked to other paraneoplastic syndromes like **Lambert-Eaton myasthenic syndrome** or **cerebellar degeneration** [2]. - Its primary neurological manifestations usually involve brain metastases rather than paraneoplastic syndromes. *Retinoblastoma* - Retinoblastoma is an intraocular malignancy of childhood that typically does not cause paraneoplastic syndromes like OMS. - Its neurological complications are usually related to **direct tumor extension** or leptomeningeal metastasis. *Nephroblastoma* - Nephroblastoma, also known as **Wilms' tumor**, is a kidney cancer in children. - It is not typically associated with paraneoplastic neurological syndromes like OMS; its paraneoplastic manifestations might include syndromes like **hypercalcemia** due to ectopic hormone production.
Question 6: Toxoplasma in children causes:
- A. Chorioretinitis (Correct Answer)
- B. Keratitis
- C. Papillitis
- D. Conjunctivitis
Explanation: ***Chorioretinitis*** - **Toxoplasmosis** is a significant cause of **chorioretinitis** in children, particularly congenital infections. - Ocular toxoplasmosis often presents with **retinal lesions** that can lead to vision loss. *Conjunctivitis* - **Conjunctivitis** is an inflammation of the conjunctiva, typically caused by bacterial or viral infections. - While it can occur in children, it is not a primary or characteristic manifestation of **Toxoplasma infection**. *Keratitis* - **Keratitis** is an inflammation of the cornea, often caused by bacterial, viral, or fungal infections, or sometimes trauma. - Although eyes are affected by **Toxoplasma**, **keratitis** is not the typical ophthalmic presentation; **chorioretinitis** is. *Papillitis* - **Papillitis** refers to inflammation of the optic disc (optic nerve head). - While **Toxoplasma** can rarely affect the optic nerve, **papillitis** is not the most common or specific ocular manifestation compared to **chorioretinitis**.
Question 7: The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?
- A. Vomiting
- B. Papilledema
- C. Normal head circumference (Correct Answer)
- D. Bulging fontanel
Explanation: ***Normal head circumference*** - **Raised intracranial tension (RIC)** in infants often leads to an **increased head circumference** if the sutures have not yet fused, making a normal circumference *less likely* for RIC. - A persistent increase in head circumference is a key indicator of **hydrocephalus** or other conditions causing RIC in infants. *Bulging fontanel* - A **full or bulging fontanel** is a classic sign of RIC in infants because the open fontanelle provides a direct route for pressure to manifest. - This occurs due to increased pressure within the skull pushing the brain and cerebrospinal fluid outwards. *Papilledema* - **Papilledema**, or swelling of the optic disc, indicates increased pressure transmitted to the optic nerve. - While it can be harder to detect in infants than in older children, it is a significant sign of RIC when present. *Vomiting* - **Vomiting**, especially projectile vomiting without associated nausea, is a common non-specific symptom of RIC in infants and children. - This is thought to be due to pressure on the **brainstem's emetic center**.
Question 8: What type of diplopia is typically associated with sixth cranial nerve palsy?
- A. Crossed diplopia
- B. Uncrossed diplopia (Correct Answer)
- C. No diplopia
- D. Vertical diplopia
Explanation: ***Uncrossed diplopia*** - **Sixth cranial nerve palsy** affects the **lateral rectus muscle**, causing the affected eye to deviate inwards (**esotropia**) [1], [2]. - When the eye is turned inward, the image from the affected eye falls on the **nasal retina**, which is perceived as coming from the temporal visual field, leading to **uncrossed diplopia** (the image from the right eye is seen on the right, and the image from the left eye is seen on the left) [1]. *Crossed diplopia* - **Crossed diplopia** occurs when the eyes are exotropic (turned outward), such as in **third cranial nerve palsy** with loss of medial rectus function [1], [2]. - In this case, the image from the right eye is seen on the left, and the image from the left eye is seen on the left. *Vertical diplopia* - **Vertical diplopia** is typically associated with **fourth cranial nerve palsy**, which affects the **superior oblique muscle**, or sometimes with ocular motor nerve palsies (third nerve) affecting superior and inferior recti [2]. - This results in a vertical separation of images, where one image appears above the other [1]. *No diplopia* - **Diplopia** is a hallmark symptom of **ocular motor nerve palsies** because the eyes are misaligned, causing the brain to receive two distinct images [1]. - The absence of diplopia would indicate properly aligned eyes or a mechanism of suppression in chronic conditions.
Question 9: A patient with ptosis has the upper 4 mm of cornea covered by the upper eyelid. What is the grade of ptosis?
- A. Moderate (Correct Answer)
- B. Profound
- C. Severe
- D. Mild
Explanation: ***Moderate*** - **Moderate ptosis** is defined as **3-4 mm of lid drooping** below the normal position - In this case, the upper eyelid covers **4 mm of the cornea**, which falls into the moderate category - The lid margin is typically **at or slightly below the superior limbus** in moderate ptosis - This degree of ptosis is **functionally significant** and may warrant surgical correction *Mild* - **Mild ptosis** is defined as **2 mm or less** of lid drooping - The upper lid margin is **above the superior limbus** but below the normal position - This patient has 4 mm coverage, which **exceeds the mild category** *Severe* - **Severe ptosis** is defined as **5 mm or more** of lid drooping below the normal position - The upper lid typically **covers the pupillary axis significantly** and causes marked visual obstruction - This patient's 4 mm coverage **does not reach severe criteria** *Profound* - **"Profound"** is not a standard term in ptosis grading systems - The standard classification uses **mild, moderate, and severe** as the three grades - If used, it would refer to extreme cases where the lid almost completely covers the pupil
Question 10: In blowout fractures, which of the following is seen?
- A. Bulbar hemorrhage
- B. None of the options
- C. Enophthalmos (Correct Answer)
- D. Exophthalmos
Explanation: ***Enophthalmos*** - A **blowout fracture** of the orbit typically involves the orbital floor or medial wall from direct trauma to the eye or periorbital region. - The fracture allows orbital contents (fat and muscle) to herniate into the maxillary sinus or ethmoid sinuses, **increasing orbital volume**. - This increased volume causes the eye to recede backward into the orbit, resulting in **enophthalmos** (sunken eye appearance). - **Key clinical features**: enophthalmos, diplopia (due to inferior rectus/medial rectus entrapment), restricted eye movements, infraorbital nerve hypoesthesia. *Exophthalmos* - **Exophthalmos** (proptosis) is forward protrusion of the eye, occurring when orbital volume is **decreased** or orbital contents are **increased** (e.g., thyroid eye disease, orbital tumors, orbital hemorrhage). - This is the **opposite** of enophthalmos and would not occur in a blowout fracture where orbital volume increases. *Bulbar hemorrhage* - **Subconjunctival hemorrhage** may occur as an associated finding from ocular trauma but is not a characteristic or defining feature of blowout fractures. - Many types of blunt ocular trauma can cause conjunctival hemorrhage without orbital fracture. *None of the options* - Incorrect because **enophthalmos** is the classic and characteristic finding of orbital blowout fractures.
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