Neurology Practice Questions

Q: A 9-year-old boy is sent for neurological evaluation due to episodes of apparent inattention. Over the past year, the child has experienced episodes during which he develops a blank look on his face and his eyes blink for 15 seconds. He immediately resumes his previous activity. Which of the following therapies would be most appropriate for this patient?

Ethosuximide. ***Ethosuximide*** - The description of **brief episodes** of staring and blinking, followed by immediate resumption of activity, is classic for **absence seizures** (petit mal seizures). - **Ethosuximide** is the drug of choice for absence seizures due to its selective action on T-type calcium channels in the thalamus, which are implicated in the pathophysiology of these seizures. *Diazepam* - **Diazepam** is a benzodiazepine primarily used for acute seizure management, status epilepticus, or as an adjunctive treatment for certain refractory epilepsies. - It is not considered a first-line agent for the long-term management of absence seizures. *Observation without immediate treatment* - While some paroxysmal events in childhood can be benign, the recurrent nature and impact on attention suggest a true seizure disorder requiring intervention. - Untreated absence seizures can affect a child’s **learning** and **academic performance** due to frequent interruptions in consciousness. *Combination therapy with Carbamazepine and Primidone* - **Carbamazepine** is effective for focal (partial) seizures and generalized tonic-clonic seizures, but it can actually *exacerbate* absence seizures. - **Primidone** is an antiepileptic drug used for focal seizures and generalized tonic-clonic seizures, but it is not appropriate for absence seizures and is less commonly used as a first-line agent given its side effect profile.

Q: Factors that increase the risk of recurrence of febrile seizures include all except:

Temperature more than 40°C. ***Temperature more than 40°C*** - A **high fever** (over 40°C) during a febrile seizure **does not increase the risk of recurrence**. - In fact, **lower fever at onset** (<39°C) is actually associated with **higher recurrence risk**, as it suggests a lower seizure threshold. - The height of fever is **not a recognized predictor** of febrile seizure recurrence in major pediatric guidelines. *Initial seizure at less than 12 months of age* - **Younger age at the first febrile seizure** (especially under 12-18 months) is a significant predictor of future episodes. - The **immature nervous system** of younger infants makes them more susceptible to subsequent seizures with fever. - This is one of the strongest risk factors for recurrence. *Positive family history* - A **family history of febrile seizures** significantly increases a child's risk of recurrence. - This suggests a **genetic predisposition** to febrile seizures. - Children with affected first-degree relatives have approximately 2-3 times higher recurrence risk. *Complex seizures* - **Complex febrile seizures** (defined by prolonged duration >15 minutes, focal features, or multiple seizures within 24 hours) are associated with a higher risk of recurrence. - This indicates greater **neuronal excitability** or underlying susceptibility.

Q: Which among the following is not seen in Sturge Weber syndrome?

None of the options. ***None of the options*** - All listed features, including **railroad track appearance** (gyriform calcifications on neuroimaging), **intellectual disability**, and **seizures**, are characteristic manifestations of **Sturge-Weber syndrome**. - This indicates that the disease typically presents with a combination of these neurological and cutaneous findings. *Rail road track appearance* - This refers to the characteristic **gyriform calcifications** seen on imaging, which is a common and diagnostic finding in Sturge-Weber syndrome. - These calcifications develop in the cerebral cortex, often beneath the **leptomeningeal angioma**, and are a direct radiographic sign of the condition. *Intellectual disability* - **Intellectual disability** is a significant neurodevelopmental complication frequently observed in patients with Sturge-Weber syndrome, particularly those with extensive brain involvement. - The underlying cause is believed to be chronic **hypoperfusion** and structural brain abnormalities resulting from the leptomeningeal angioma. *Seizure* - **Seizures** are a very common and often the earliest neurological symptom in children with Sturge-Weber syndrome, occurring in 75-90% of affected individuals. - These seizures are typically **focal** and can be resistant to pharmacotherapy, caused by the underlying cortical dysplasia and abnormal vascularity.

Q: A 3 year old child is brought to you with history of frequent, violent and rapid swinging movement of the left arm for one week duration. What is the condition described in this scenario called?

Hemiballismus. ***Hemiballismus*** - **Hemiballismus** is characterized by sudden, involuntary, wild, rapid, and **flailing movements** of one side of the body, primarily affecting the proximal musculature such as the arm and leg. - The description of "frequent, violent and rapid swinging movement of the left arm" perfectly aligns with the clinical presentation of hemiballismus, often resulting from a lesion in the **subthalamic nucleus**. *Athetosis* - **Athetosis** involves slow, writhing, involuntary movements, especially in the distal parts of the limbs (fingers and toes). - This condition is typically slower and more sustained than the "violent and rapid swinging" described in the scenario. *Dystonia* - **Dystonia** is characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements or postures. - While it can involve forceful movements, it usually results in sustained postures or twisting movements rather than the rapid, flailing motion described. *Chorea* - **Chorea** refers to irregular, unpredictable, brief, and jerky movements that flow from one body part to another in a dance-like manner. - Unlike the violent, large-amplitude movements of hemiballismus, chorea typically involves more distal and less organized movements.

Q: Brain abscess in Cyanotic heart disease is commonly located in

Frontal lobe. ***Frontal lobe*** - The **frontal lobe** is the most common location for brain abscesses in patients with cyanotic heart disease. - This is attributed to the **rich blood supply** and relatively straight vascular path from the heart to the frontal region. *Cerebellar hemisphere* - While cerebellar abscesses can occur, they are **less common** than those in the cerebral hemispheres. - They are more typically associated with **direct spread from ear infections** rather than hematogenous spread. *Parietal lobe* - The parietal lobe can be affected, but it is **not the most frequent site** compared to the frontal lobe. - Abscesses in this region may lead to **sensory or motor deficits**. *Temporal lobe* - Temporal lobe abscesses are often seen with **otogenic infections** (e.g., otitis media, mastoiditis). - They tend to be **less common** as a primary site of hematogenous spread in cyanotic heart disease.

Q: All of the following are true of febrile seizures except-

Almost invariably develop into epilepsy. ***Almost invariably develop into epilepsy*** - This statement is **false**; only a small percentage (2-7%) of children with febrile seizures later develop **epilepsy**. - Febrile seizures are generally considered **benign** and do not typically lead to a diagnosis of epilepsy. *Prognosis is good* - The **prognosis for simple febrile seizures is excellent**, with no long-term neurological sequelae or cognitive impairment. - The risk of recurrence is present but usually decreases with age, and **most children outgrow them**. *Does not last more than 15 minutes* - **Simple febrile seizures** are defined as generalized tonic-clonic seizures lasting **less than 15 minutes**. - Seizures lasting longer than 15 minutes are considered **complex febrile seizures**, which have a slightly higher risk of recurrence but do not necessarily change the overall good prognosis. *Most commonly seen between 9 months and 5 years* - Febrile seizures typically occur in children between **6 months and 5 years of age**, with a peak incidence around **18 months**. - This age range reflects the developing brain's susceptibility to febrile illness and its ability to generate a generalized seizure response.

Q: A pediatric patient presents with a 45-minute history of continuous convulsions. The senior resident (SR) recommends IV lorazepam, but the junior resident (JR) is unable to secure IV access. What is the next best step in management?

Rectal diazepam. ***Rectal diazepam*** - Rectal diazepam is an **effective first-line alternative** when IV access cannot be obtained in status epilepticus - It has a **rapid onset of action** (within 2-5 minutes) and can be easily administered in emergency settings - **Widely available** and part of established pediatric seizure protocols globally - Both rectal diazepam and IM midazolam are acceptable alternatives per current guidelines *Intramuscular midazolam* - IM midazolam is **equally effective** and increasingly preferred in many modern protocols when IV access is unavailable - The RAMPART trial demonstrated **faster seizure cessation** with IM midazolam compared to rectal diazepam in prehospital settings - **Both IM midazolam and rectal diazepam** are considered first-line alternatives per WHO and major pediatric emergency guidelines - Either option is appropriate depending on local protocols and availability *Intramuscular phenobarbital* - Phenobarbital has a **slower onset of action** when given intramuscularly (15-30 minutes) - Typically reserved for **refractory status epilepticus** or as a second-line agent after benzodiazepines have failed - Not preferred as an immediate alternative to IV lorazepam *IV phenytoin* - IV phenytoin **requires IV access**, which is specifically unavailable in this scenario - It is a second-line antiepileptic for status epilepticus, used after benzodiazepines - Requires **cardiac monitoring** due to risk of hypotension and arrhythmias

Q: Duchenne's muscular dystrophy affects which group of muscles commonly:

Proximal muscles. ***Proximal muscles*** - Duchenne's muscular dystrophy (DMD) is characterized by **progressive proximal muscle weakness**, which is the hallmark feature of this X-linked recessive disorder. - The weakness typically begins in the **pelvic girdle muscles** (hip flexors, quadriceps) and later progresses to involve the **shoulder girdle muscles**. - This proximal pattern of weakness leads to classic clinical features: difficulty **climbing stairs**, **rising from the floor** (Gower's sign), **waddling gait**, and difficulty **raising arms overhead**. - The term "proximal muscles" accurately encompasses the characteristic distribution of muscle involvement in DMD, making it the most correct answer. *Shoulder muscles* - While shoulder girdle muscles are indeed affected in DMD, they are typically involved **after the pelvic girdle muscles**. - This option is too specific and does not capture the **generalized proximal muscle weakness** that defines DMD. - Shoulder muscle weakness becomes prominent as the disease progresses, but it is not the initial or most characteristic presentation. *Calf muscles* - Calf muscles may show **pseudohypertrophy** (apparent enlargement due to replacement of muscle with fat and connective tissue), which is a characteristic finding in DMD. - However, pseudohypertrophy does not represent true muscle strength, and the calves are **distal muscles**, not the primary site of weakness. - DMD is a **proximal myopathy**, not a distal myopathy. *Respiratory muscles* - **Respiratory muscle weakness** (diaphragm and intercostal muscles) is a serious and life-threatening complication of DMD. - However, respiratory involvement occurs **later in the disease course**, typically in the second decade of life. - Progressive respiratory failure is a major cause of morbidity and mortality in DMD but is not the early or most characteristic muscle group affected.

Q: All the following are Causes of spasticity in a 2 year old child except

Congenital muscular dystrophy. ***Congenital muscular dystrophy*** - This condition is characterized by **muscle weakness and hypotonia**, not spasticity, due to primary muscle pathology. - Spasticity is a sign of **upper motor neuron involvement**, whereas muscular dystrophies are disorders of peripheral muscle fibers resulting in **flaccid weakness**. *Cerebral Palsy* - This is the **most common cause of spasticity in children**, resulting from **non-progressive brain injury during development**. - **Spastic cerebral palsy** accounts for 70-80% of CP cases, characterized by increased muscle tone, hyperreflexia, and extensor plantar responses. *Kernicterus* - **Bilirubin encephalopathy** from severe neonatal hyperbilirubinemia causes **basal ganglia damage**. - Results in **extrapyramidal cerebral palsy** with spasticity, choreoathetosis, and developmental delays. *Birth asphyxia* - **Hypoxic-ischemic encephalopathy** from perinatal asphyxia causes **upper motor neuron damage**. - Leads to spastic quadriplegia or diplegia with increased tone, hyperreflexia, and developmental impairment.

Question 1

A 9-year-old boy is sent for neurological evaluation due to episodes of apparent inattention. Over the past year, the child has experienced episodes during which he develops a blank look on his face and his eyes blink for 15 seconds. He immediately resumes his previous activity. Which of the following therapies would be most appropriate for this patient?

Accepted Answer

Ethosuximide

Answer

Diazepam

Answer

Observation without immediate treatment

Answer

Combination therapy with Carbamazepine and Primidone

Question 2

Factors that increase the risk of recurrence of febrile seizures include all except:

Accepted Answer

Temperature more than 40°C

Answer

Initial seizure at less than 12 months of age

Answer

Positive family history

Answer

Complex seizures

Question 3

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 –

Accepted Answer

Midline posterior fossa tumour

Answer

Suprasellar tumour

Answer

Hydrocephalus

Answer

Brain stem tumour

Question 4

Which among the following is not seen in Sturge Weber syndrome?

Accepted Answer

None of the options

Answer

Rail road track appearance

Answer

Intellectual disability

Answer

Seizure

Question 5

A 3 year old child is brought to you with history of frequent, violent and rapid swinging movement of the left arm for one week duration. What is the condition described in this scenario called?

Accepted Answer

Hemiballismus

Answer

Athetosis

Answer

Dystonia

Answer

Chorea

Question 6

Brain abscess in Cyanotic heart disease is commonly located in

Accepted Answer

Frontal lobe

Answer

Cerebellar hemisphere

Answer

Parietal lobe

Answer

Temporal lobe

Question 7

All of the following are true of febrile seizures except-

Accepted Answer

Almost invariably develop into epilepsy

Answer

Prognosis is good

Answer

Does not last more than 15 minutes

Answer

Most commonly seen between 9 months and 5 years

Question 8

A pediatric patient presents with a 45-minute history of continuous convulsions. The senior resident (SR) recommends IV lorazepam, but the junior resident (JR) is unable to secure IV access. What is the next best step in management?

Accepted Answer

Rectal diazepam

Answer

Intramuscular phenobarbital

Answer

Intramuscular midazolam

Answer

IV phenytoin

Question 9

Duchenne's muscular dystrophy affects which group of muscles commonly:

Accepted Answer

Proximal muscles

Answer

Calf muscles

Answer

Shoulder muscles

Answer

Respiratory muscles

Question 10

All the following are Causes of spasticity in a 2 year old child except

Accepted Answer

Congenital muscular dystrophy

Answer

Cerebral Palsy

Answer

Kernicterus

Answer

Birth asphyxia

Neurology — MCQs

Neurology — MCQs

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