Neurology Practice Questions

Q: What are the first-line disease-modifying treatments for Guillain-Barre Syndrome (GBS) in a child?

Intravenous Immunoglobulin (IV Ig) and Plasmapheresis. ***Intravenous Immunoglobulin (IV Ig) and Plasmapheresis*** - Both **IV Ig** and **plasmapheresis** are equally effective first-line disease-modifying treatments for GBS in children - **IV Ig** works by neutralizing pathogenic antibodies and modulating the immune response - **Plasmapheresis** removes circulating antibodies and inflammatory mediators from the plasma - Both treatments reduce the severity and duration of paralysis and accelerate recovery - They are equally effective with **no significant difference in outcomes**; choice depends on availability, contraindications, and patient factors *Intravenous Immunoglobulin (IV Ig) alone* - While IV Ig is indeed a first-line treatment, it is not the only one - The question asks for treatments (plural), and plasmapheresis is equally effective *Mechanical Ventilation alone* - This is a **supportive measure** for respiratory failure, not a disease-modifying treatment - About 20-30% of GBS patients require mechanical ventilation due to respiratory muscle weakness - It manages complications but does not treat the underlying immune-mediated neuropathy *Plasmapheresis alone* - While plasmapheresis is indeed a first-line treatment, it is not the only one - The question asks for treatments (plural), and IV Ig is equally effective

Q: A child presents with ascending flaccid paralysis. There is subsequent respiratory muscle involvement. CSF examination shows albuminocytological dissociation. Treatment of choice is:

I.V. immunoglobulins. ***I.V. immunoglobulins*** - **Intravenous immunoglobulins (IVIg)** are a first-line treatment for **Guillain-Barré syndrome (GBS)**, which is strongly indicated by **ascending flaccid paralysis**, respiratory muscle involvement, and **albuminocytological dissociation** in CSF. - IVIg works by neutralizing pathogenic antibodies, modulating complement activation, and blocking Fc receptors, thereby reducing the autoimmune attack on peripheral nerves. *Cycloserine* - **Cycloserine** is an antibiotic primarily used to treat **tuberculosis**, especially in drug-resistant cases. - It has no role in the treatment of autoimmune neurological conditions like Guillain-Barré syndrome. *Oral prednisolone* - While corticosteroids like **prednisolone** are potent anti-inflammatory and immunosuppressive agents, they have been shown to be **ineffective** and potentially harmful in GBS. - Studies have demonstrated that oral steroids do not improve outcomes and may even prolong recovery in GBS. *I.V. methylprednisolone* - Similar to oral prednisolone, **intravenous methylprednisolone** is a corticosteroid that has **not been shown to be beneficial** in treating GBS. - High-dose corticosteroids are generally avoided in GBS due to lack of efficacy and potential side effects.

Q: What is the most common cause of hydrocephalus in children?

Congenital aqueductal stenosis. ***Congenital aqueductal stenosis*** - This is the **most common cause of hydrocephalus in children**, accounting for the majority of congenital hydrocephalus cases. - The **cerebral aqueduct** (connecting the third and fourth ventricles) is narrowed or blocked, preventing normal CSF flow. - Results in **obstructive hydrocephalus** with progressive ventricular enlargement. - Clinical presentation varies from neonatal period to childhood depending on severity. *Post inflammatory obstruction* - An important cause of **acquired hydrocephalus**, typically following meningitis or intraventricular hemorrhage. - Inflammation leads to **fibrosis and scarring** of CSF pathways, particularly affecting the arachnoid villi and basal cisterns. - More common in developing countries with higher rates of CNS infections. - While significant, it is not the overall most common cause in children. *Brain tumour* - Tumors can cause hydrocephalus by **obstructing CSF pathways**, particularly posterior fossa tumors blocking the fourth ventricle. - Common tumor types include medulloblastoma, ependymoma, and cerebellar astrocytoma. - Represents a **less common cause** compared to congenital malformations. *Perinatal injury* - Includes **intraventricular hemorrhage (IVH)** in premature infants and birth trauma. - IVH can lead to post-hemorrhagic hydrocephalus through blood clot obstruction or subsequent inflammation. - More relevant in **premature and low birth weight infants**, but not the most common cause overall.

Q: In a child with Rett syndrome, all of the following are seen except:

Macrocephaly. ***Macrocephaly*** - Rett syndrome is typically associated with **microcephaly**, not macrocephaly, due to slowed head growth after birth. - The brain size is smaller than average, reflecting impaired neural development. *Intellectual disability* - **Severe intellectual disability** is a core feature of Rett syndrome, as affected individuals experience significant cognitive impairment. - This cognitive decline often becomes apparent after a period of normal development. *Abnormal dendritic connections* - **Abnormal dendritic connections** and reduced dendritic complexity are characteristic neuropathological findings in Rett syndrome. - These structural changes in neurons contribute to the neurological dysfunction observed in the disorder. *Seizures* - **Seizures** are a very common symptom in children with Rett syndrome, affecting a majority of individuals at some point. - The type and frequency of seizures can vary widely among patients.

Q: A 1-year-old child presented with myoclonic jerks. What does the EEG show?

Hypsarrhythmia. ***Hypsarrhythmia*** - The EEG image displays a chaotic, high-amplitude, and disorganized background activity with multifocal spikes and sharp waves, which are characteristic features of **hypsarrhythmia**. - This pattern is classically associated with **West syndrome** (infantile spasms), which presents clinically with myoclonic jerks and developmental regression in infants, aligning with the 1-year-old child's presentation. *Normal record* - A normal EEG in a 1-year-old child would show organized background activity with appropriate developmental features for their age, lacking the pronounced disorganization seen here. - The presence of myoclonic jerks also makes a normal EEG an unlikely finding, as these are paroxysmal events suggestive of underlying brain dysfunction. *Periodic spike-wave pattern* - **Periodic spike-wave patterns** typically involve rhythmic, generalized discharge complexes occurring at regular intervals (e.g., 3 Hz spike-and-wave) and are characteristic of absence seizures or generalized tonic-clonic seizures. - The pattern observed in the image is much more irregular, chaotic, and lacks the distinct periodicity and morphology associated with typical spike-wave complexes. *Burst suppression pattern* - **Burst suppression** is characterized by bursts of high-amplitude generalized activity alternating with periods of profound reduction or suppression of electrical activity. - This pattern is seen in severe encephalopathies, deep anesthesia, or certain severe epilepsy syndromes, but it involves clear periods of suppression which are absent in the provided chaotic EEG.

Q: Which of the following is NOT a feature of Moebius syndrome?

Decreased chest movements. ***Decreased chest movements*** - **Decreased chest movements** are not a characteristic feature of **Moebius syndrome**, which primarily affects cranial nerves, particularly the **facial and abducens nerves**. - While other systemic issues can coexist, respiratory problems like decreased chest movements are not considered a direct or defining symptom of this condition. *Bilateral facial paralysis* - **Bilateral facial paralysis** is a hallmark of **Moebius syndrome**, resulting from congenital underdevelopment or absence of the **facial (VII) cranial nerves**. - This leads to a characteristic **mask-like facial expression**, difficulty with smiling, frowning, and other facial movements. *Impaired lateral eye movement* - **Impaired lateral eye movement** is a common feature due to involvement of the **abducens (VI) cranial nerves**, which control the **lateral rectus muscle**. - Patients often present with **esotropia** (crossed eyes) and are unable to move their eyes past the midline when looking to the side. *Unilateral or bilateral abducens nerve involvement* - **Unilateral or bilateral abducens (VI) nerve involvement** is a core diagnostic criterion for **Moebius syndrome**. - This leads to the characteristic deficit in **lateral gaze**, as the abducens nerve innervates the **lateral rectus muscle**.

Q: A 1-year-old child was brought to the outpatient department with a soft and compressible swelling on the nose that increases on coughing. Which of the following is most likely the diagnosis?

Meningoencephalocele. ***Meningoencephalocele*** - A soft, compressible nasal swelling that increases with **coughing** or **straining** is highly suggestive of a meningoencephalocele due to increased intracranial pressure. - This condition involves a **herniation of brain tissue** (encephalocele) and meninges through a bony defect, often in the nasal region. *Lacrimal sac cyst* - A lacrimal sac cyst would typically present as a swelling in the **medial canthal region** and is usually associated with **tear duct obstruction**, not directly on the nose increasing with coughing. - While soft, it is not usually **compressible** or affected by changes in intracranial pressure in the same way. *Arteriovenous malformation* - An arteriovenous malformation (AVM) would typically present as a **pulsatile** mass with a **bruit**, and might cause warmth or discoloration. - It would not characteristically increase in size with **coughing** as a result of intracranial pressure changes. *Ethmoid cyst* - An ethmoid cyst is a fluid-filled sac originating from the **ethmoid sinuses**. While it can cause nasal obstruction or swelling, it usually presents as a firm, non-pulsatile mass. - It would not typically exhibit **compressibility** with an increase in size when coughing, differentiating it from an intracranial connection.

Q: In a 9-month-old child, which of the following reflexes is considered most abnormal?

Asymmetric tonic neck reflex (ATNR). ***Asymmetric tonic neck reflex (ATNR)*** - The **ATNR** (fencing reflex) typically **disappears by 6 months of age**. Persistence beyond this age, especially at 9 months, is a critical indicator of potential neurological dysfunction or developmental delay. - Its presence can hinder normal development, such as rolling, crawling, and reaching milestones like bringing hands to midline, and is therefore considered the **most abnormal** at this age. *Parachute reflex* - The **parachute reflex** (forward protective extension) typically emerges between **6 to 9 months of age** and persists throughout life. - Its presence at 9 months indicates a normally developing protective mechanism and is therefore **normal**, not abnormal. *Righting reflex* - The **righting reflex** (which includes various head and body righting reactions), allows the infant to maintain an upright head position and orient the body relative to the head and gravity. - These reflexes gradually develop and are often well-established by **6-12 months**, being crucial for independent sitting and balance, making its presence at 9 months **expected and normal**. *None of the options* - This option is incorrect because the **Asymmetric Tonic Neck Reflex (ATNR)** is indeed considered abnormal if present at 9 months of age, indicating a potential developmental concern.

Q: The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?

Normal head circumference. ***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 1

What are the first-line disease-modifying treatments for Guillain-Barre Syndrome (GBS) in a child?

Accepted Answer

Intravenous Immunoglobulin (IV Ig) and Plasmapheresis

Answer

Intravenous Immunoglobulin (IV Ig) alone

Answer

Mechanical Ventilation alone

Answer

Plasmapheresis

Question 2

A child presents with ascending flaccid paralysis. There is subsequent respiratory muscle involvement. CSF examination shows albuminocytological dissociation. Treatment of choice is:

Accepted Answer

I.V. immunoglobulins

Answer

Oral prednisolone

Answer

I.V. methylprednisolone

Answer

Cycloserine

Question 3

What is the most common cause of hydrocephalus in children?

Accepted Answer

Congenital aqueductal stenosis

Answer

Post inflammatory obstruction

Answer

Brain tumour

Answer

Perinatal injury

Question 4

In a child with Rett syndrome, all of the following are seen except:

Accepted Answer

Macrocephaly

Answer

Abnormal dendritic connections

Answer

Intellectual disability

Answer

Seizures

Question 5

A 1-year-old child presented with myoclonic jerks. What does the EEG show?

Accepted Answer

Hypsarrhythmia

Answer

Normal record

Answer

Periodic spike-wave pattern

Answer

Burst suppression pattern

Question 6

Which of the following is NOT a feature of Moebius syndrome?

Accepted Answer

Decreased chest movements

Answer

Bilateral facial paralysis

Answer

Impaired lateral eye movement

Answer

Unilateral or bilateral abducens nerve involvement

Question 7

A 2-week-old female infant has a head circumference of 40 cm, which is greater than the 98th percentile, along with a large, tense fontanelle and downward deviation of the eyes. She has been vomiting her formula and is irritable. Which of the following is the least likely cause of her symptoms?

Accepted Answer

Intraventricular hemorrhage

Answer

Meningitis

Answer

Aqueductal stenosis

Answer

Brain tumor

Question 8

A 1-year-old child was brought to the outpatient department with a soft and compressible swelling on the nose that increases on coughing. Which of the following is most likely the diagnosis?

Accepted Answer

Meningoencephalocele

Answer

Arteriovenous malformation

Answer

Lacrimal sac cyst

Answer

Ethmoid cyst

Question 9

In a 9-month-old child, which of the following reflexes is considered most abnormal?

Accepted Answer

Asymmetric tonic neck reflex (ATNR)

Answer

Parachute reflex

Answer

Righting reflex

Answer

None of the options

Question 10

The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?

Accepted Answer

Normal head circumference

Answer

Vomiting

Answer

Papilledema

Answer

Bulging fontanel

Neurology — MCQs

Neurology — MCQs

On this page

Practice by Chapter

Want unlimited practice?