Neurology Practice Questions

Q: Treatment of simple febrile convulsion is based on

Control of fever. ***Control of fever*** - Among the given options, **control of fever** is the most appropriate answer as it represents the **immediate supportive care** for a child with a simple febrile seizure. - Management includes using antipyretics like **paracetamol** or **ibuprofen** to reduce fever and improve comfort. - **Important note:** While fever control is good supportive care, evidence shows that antipyretics do **NOT prevent recurrence** of febrile seizures. The actual cornerstone of management is **reassurance and parental education**. - According to AAP guidelines, simple febrile seizures are benign, self-limited events that require no specific anticonvulsant treatment. *Rectal diazepam* - **Rectal diazepam** is used for **acute termination** of prolonged seizures (>5 minutes) or as rescue therapy for recurrent episodes. - It is NOT indicated for routine management of simple febrile seizures, which typically last <15 minutes and resolve spontaneously. - May be prescribed for home use in select cases with recurrent seizures. *CSF finding* - **CSF analysis** is a **diagnostic procedure**, not a treatment basis. - It is indicated only when there is clinical suspicion of meningitis or meningoencephalitis (e.g., altered sensorium, meningeal signs, complex seizure features). - NOT routinely required for simple febrile seizures in well-appearing children. *Blood reports* - **Blood investigations** are diagnostic, not treatment-guiding for simple febrile seizures. - They may be considered to identify the source of fever or rule out electrolyte abnormalities, but are not the basis of seizure management itself. - Simple febrile seizures do not require routine laboratory workup.

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: What is correct about febrile seizures

Normal EEG. ***Normal EEG*** - An **electroencephalogram (EEG)** is generally **not recommended** after a simple febrile seizure because these seizures are due to the brain's response to fever, not an underlying epileptic disorder. - The **EEG typically appears normal** following a simple febrile seizure, as there is no intrinsic cerebral pathology to detect. - Simple febrile seizures are benign events that do not require routine EEG investigation. *Focal deficits* - **Focal neurological deficits** (e.g., weakness on one side of the body) are **not characteristic** of **simple febrile seizures** and would suggest a more complex neurological issue or an underlying etiology. - The presence of focal deficits would prompt further investigation for complex febrile seizures or other neurological conditions. *Repeated seizure* - While **recurrence of febrile seizures** is common (about 30-35% of children experience a second seizure), this refers to a **risk factor** for recurrence rather than a defining characteristic of febrile seizures. - Risk factors for recurrence include young age at first seizure, family history of febrile seizures, low fever at onset, and brief duration between fever onset and seizure. *Abnormal EEG* - An **abnormal EEG** in the context of a febrile seizure would raise concerns for an **underlying epileptic syndrome** or other neurological pathology, which is not typical for **simple febrile seizures**. - Routine EEG is not indicated for simple febrile seizures as it is unlikely to show abnormalities and is not predictive of future epilepsy.

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 tuft of hair over the lumbosacral region in a newborn is most commonly suggestive of:

Spina bifida occulta. ***Spina bifida occulta*** - A **lumbosacral hair tuft** (hypertrichosis) is a classic **cutaneous stigmata** indicating underlying **spinal dysraphism**. - **Spina bifida occulta** is the **most common association** with this finding—it represents incomplete fusion of the vertebral arches without herniation of neural elements. - This is often asymptomatic but requires **imaging evaluation** (ultrasound in infants <6 months, MRI if older) to rule out tethered cord or other occult spinal anomalies. - Other cutaneous markers include dimples (above gluteal crease), lipomas, hemangiomas, and dermal sinuses. *Tumor* - While subcutaneous tumors like **lipomas** or **lipomyelomeningoceles** can present with overlying hair tufts, they are typically palpable masses and less common than spina bifida occulta. - These would also fall under the spectrum of spinal dysraphism requiring imaging. *Sinus tract* - A **dermal sinus tract** can be associated with a hair tuft but more commonly presents with a visible **dimple or opening** above the gluteal crease. - This carries risk of CNS infection (meningitis) and requires surgical intervention. *Dermoid cyst* - Dermoid cysts can present with overlying skin changes but are not the most common cause of isolated hair tufts in the lumbosacral region.

Question 1

Treatment of simple febrile convulsion is based on

Accepted Answer

Control of fever

Answer

Rectal diazepam

Answer

CSF finding

Answer

Blood reports

Question 2

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 3

What is correct about febrile seizures

Accepted Answer

Normal EEG

Answer

Focal deficits

Answer

Repeated seizure

Answer

Abnormal EEG

Question 4

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 5

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 6

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 7

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 8

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 9

A tuft of hair over the lumbosacral region in a newborn is most commonly suggestive of:

Accepted Answer

Spina bifida occulta

Answer

Dermoid cyst

Answer

Tumor

Answer

Sinus tract

Question 10

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

Neurology — MCQs

Neurology — MCQs

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