Neurology Practice Questions

Q: Which of the following statements concerning spina bifida is True?

Spina bifida occulta occurs most often at L5-S1 level. **Spina bifida occulta occurs most often at L5-S1 level** - **Spina bifida occulta** is the mildest form of spina bifida, characterized by an incomplete closure of the vertebral arch without protrusion of the spinal cord or meninges. - The most common location for spina bifida occulta is the **L5-S1 vertebral level**, which often presents as a skin dimple, tuft of hair, or discolored patch in the lumbar region. *Chromosomal abnormalities can be identified in approximately 50% of patients* - **Chromosomal abnormalities** are found in a minority of patients with spina bifida, typically less than 10%, when evaluating for genetic causes. - While single-gene mutations or environmental factors contribute, spina bifida is most often considered a **multifactorial condition** rather than solely chromosomal. *A myelomeningocele is the most common type of spina bifida* - A **myelomeningocele** is the most severe and a common type of spina bifida where the spinal cord and meninges protrude through an opening in the spine. - However, **spina bifida occulta** is statistically the most common type overall, though it is often asymptomatic and may go undiagnosed. *Additional congenital abnormalities are rare findings* - **Additional congenital abnormalities** are not rare; they are frequently associated with spina bifida, particularly with myelomeningocele. - These can include **hydrocephalus**, **Chiari malformations**, urinary tract anomalies, and orthopedic issues like clubfoot, reflecting a systemic developmental disruption.

Q: Simple febrile seizure is defined as

Generalized seizure <15 min, within 24 hours of fever onset, in a child 6mo-5yr. ***Generalized seizure <15 min, within 24 hours of fever onset, in a child 6mo-5yr*** - A **simple febrile seizure** is characterized by a **generalized tonic-clonic appearance**, lasting less than 15 minutes, and occurring within 24 hours of the onset of fever. - It typically affects children between the ages of **6 months and 5 years** who do not have a history of afebrile seizures or other neurological conditions. *All of the options* - This option is incorrect because simple febrile seizures must meet **specific criteria** regarding duration, number of episodes, and seizure type to be classified as simple. - While some individual components might be correct, the definition requires a **combination of specific features**, not a general 'all of the options' approach. *Seizure lasts < 15 min* - While a duration of less than 15 minutes is a **necessary criterion** for a simple febrile seizure, it is not sufficient on its own. - Other factors such as the type of seizure (generalized), the number of seizures (single in 24 hours), and the child's age are also crucial for the definition. *Focal seizure* - A **focal seizure**, characterized by originating in a specific area of the brain and often manifesting with localized symptoms, is a hallmark of a **complex febrile seizure**, not a simple one. - Simple febrile seizures are by definition **generalized**, meaning they affect both sides of the brain from the outset.

Q: A child presents with an open anterior fontanelle and an audible cranial bruit. Imaging reveals a midline lesion in the brain. What is the most likely diagnosis?

Malformation of vein of Galen. ***Malformation of vein of Galen*** - A **malformation of the vein of Galen** is a type of arteriovenous malformation that can cause greatly increased blood flow, leading to heart failure and a characteristic intracranial **bruit** audible over the skull. - The increased blood flow and often associated hydrocephalus can lead to **macrocephaly** and delayed closure of the **anterior fontanelle**. *Down syndrome* - While individuals with **Down syndrome** may have cardiac defects, a prominent intracranial bruit is not a typical feature unless associated with another condition. - The primary diagnostic features relate to distinct facial features, intellectual disability, and specific chromosomal abnormalities, not primarily a cranial bruit or specific midline brain lesion with this presentation. *Rickets* - **Rickets** is a bone disorder caused by vitamin D deficiency, leading to softening and weakening of bones, which can cause delayed fontanelle closure. - However, rickets does not cause a **cranial bruit** or a specific **midline brain lesion** as described. *Congenital hydrocephalus* - **Congenital hydrocephalus** can cause a persistently open **anterior fontanelle** and can lead to **macrocephaly** due to increased intracranial pressure. - However, a prominent **bruit** is not a characteristic feature of hydrocephalus itself; if a bruit is present, it suggests an underlying vascular anomaly like a vein of Galen malformation as the cause.

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.

Question 1

Which of the following statements concerning spina bifida is True?

Accepted Answer

Spina bifida occulta occurs most often at L5-S1 level

Answer

Chromosomal abnormalities can be identified in approximately 50% of patients

Answer

A myelomeningocele is the most common type of spina bifida

Answer

Additional congenital abnormalities are rare findings

Question 2

Simple febrile seizure is defined as

Accepted Answer

Generalized seizure <15 min, within 24 hours of fever onset, in a child 6mo-5yr

Answer

All of the options

Answer

Seizure lasts < 15 min

Answer

Focal seizure

Question 3

A child presents with an open anterior fontanelle and an audible cranial bruit. Imaging reveals a midline lesion in the brain. What is the most likely diagnosis?

Accepted Answer

Malformation of vein of Galen

Answer

Down syndrome

Answer

Rickets

Answer

Congenital hydrocephalus

Question 4

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 5

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 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 –

Accepted Answer

Midline posterior fossa tumour

Answer

Suprasellar tumour

Answer

Hydrocephalus

Answer

Brain stem tumour

Question 7

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 8

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 9

Brain abscess in Cyanotic heart disease is commonly located in

Accepted Answer

Frontal lobe

Answer

Cerebellar hemisphere

Answer

Parietal lobe

Answer

Temporal lobe

Question 10

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

Neurology — MCQs

Neurology — MCQs

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