Neurology Practice Questions

Q: What is the typical maintenance dose of Levetiracetam in pediatric patients?

30 - 40 mg/kg/day. ***30 - 40 mg/kg/day*** - Levetiracetam is often initiated with a lower dose (e.g., 10-15 mg/kg/day) and **titrated upwards** to achieve seizure control. - The typical maintenance dose for Levetiracetam, especially in pediatric patients, is generally considered to be in the range of **30-40 mg/kg/day** administered in two divided doses. *10 - 20 mg/kg/day* - This range is more typical for the **initial starting dose** of Levetiracetam, rather than the maintenance dose. - While sometimes used as a maintenance dose in specific adult cases or for mild seizure control, it is often considered **sub-therapeutic** for many patients. *20 - 30 mg/kg/day* - This dose range can be an **intermediate dose** during titration, or a maintenance dose for some patients, but it is generally at the lower end of the effective maintenance range. - Many patients require a higher dose to achieve **optimal seizure control** without intolerable side effects. *40 - 50 mg/kg/day* - Doses in this range are often associated with an **increased risk of side effects**, such as somnolence, asthenia, and behavioral changes [1]. - While some patients may require these higher doses under careful supervision, they are generally **above the typical maintenance dose** and should be considered only when lower doses are ineffective.

Q: A 1-day-old male baby delivered by LSCS presents with swelling over the back in the midline. What is the most likely diagnosis?

Spina bifida. ***Spina bifida*** - A **midline swelling over the back in a newborn** is the classic presentation of **spina bifida**, which refers to incomplete closure of the vertebral column. - This can manifest as **meningocele** (herniation of meninges alone) or **myelomeningocele** (herniation of meninges and neural tissue), both presenting as visible midline swellings. - Spina bifida is the **most appropriate clinical diagnosis** for this presentation and is what would be documented in medical records and managed by pediatric neurosurgeons. - Associated features may include a **translucent sac**, leakage of CSF, and neurological deficits depending on the type. *Neural tube defect* - While technically correct as spina bifida is a type of neural tube defect, this term is too **broad and non-specific** for clinical diagnosis. - Neural tube defects include anencephaly, encephalocele, and spina bifida - the term is more useful for **epidemiological or embryological discussions** rather than clinical diagnosis. - In clinical practice, we specify the **exact type** (spina bifida, anencephaly, etc.) rather than using the umbrella term. *Meningocele* - Meningocele is a **specific subtype of spina bifida** where only meninges protrude through the vertebral defect without neural tissue. - Without imaging or surgical exploration, we cannot definitively distinguish meningocele from myelomeningocele based on external appearance alone. - Therefore, **"spina bifida"** is the more appropriate initial diagnosis, with the specific subtype determined by further evaluation. *Dermal sinus* - A dermal sinus is a **tract connecting the skin to deeper structures**, typically presenting as a small **pit or dimple** rather than a swelling. - It may have a tuft of hair or discharge but does not present as a prominent midline swelling as described in this case.

Q: What is the recurrence rate of febrile seizures?

30-50%. ***30-50%*** - The recurrence rate of febrile seizures is approximately **30-50%**, with an average of about **33% (one-third)** of children experiencing another febrile seizure. - The rate varies with age: children ** 12 months** have approximately **20-30% risk**. - Other risk factors for recurrence include a **lower temperature threshold** for seizures, a **family history** of febrile seizures, and a **short duration** between fever onset and seizure occurrence. *10-20%* - This range is **too low** for the overall recurrence rate of febrile seizures. - While this might represent recurrence in older children with no risk factors, it does not reflect the average population risk. *20-30%* - This percentage represents a **lower estimate** than the generally accepted overall recurrence rate. - This range may apply to children **>12 months** at first seizure, but the overall average is higher at approximately **33%**. *50-70%* - This range is **too high** for the overall recurrence rate of febrile seizures. - While children with **multiple risk factors** (very young age, family history, low fever threshold) may approach **50%** recurrence, rates of 70% are not supported by evidence.

Q: A 10-month-old infant was brought with complaints of jerking movements of limbs towards the body. On examination, there is regression of developmental milestones. Electroencephalogram shows hypsarrhythmia. What is the most appropriate first-line treatment?

Adrenocorticotropic hormone (ACTH). ***Adrenocorticotropic hormone (ACTH)*** - **ACTH** is considered the **first-line treatment** for infantile spasms due to its high efficacy in controlling spasms and improving developmental outcomes. - Its mechanism involves directly modulating **cortical excitability** and neurotransmitter function. *Phenytoin* - **Phenytoin** is primarily used for **focal and tonic-clonic seizures** but is generally ineffective for infantile spasms. - Its mechanism of action involves **blocking voltage-gated sodium channels**, which is not the primary pathophysiology target for infantile spasms. *Levetiracetam* - **Levetiracetam** is an **adjunctive therapy** for various seizure types and can be used in some cases of infantile spasms, but it is not typically the first-line treatment. - It acts by binding to the **synaptic vesicle protein 2A (SV2A)**, modulating neurotransmitter release. *Phenobarbitone* - **Phenobarbitone** is an older anticonvulsant that can be used for various seizure types, including some forms of infantile epilepsy, but it is **not the preferred first-line agent** for infantile spasms due to potential side effects and lower efficacy compared to ACTH. - It works by **enhancing GABAergic inhibition** in the brain.

Q: Which of the following is not a known cause of neuroregression in children?

ADHD. ***ADHD*** - **Attention-deficit/hyperactivity disorder (ADHD)** is a neurodevelopmental disorder characterized by persistent patterns of inattention, hyperactivity, and impulsivity. It is **not** a cause of neuroregression. - While ADHD can impact cognitive and behavioral functioning, it does not involve a loss of previously acquired developmental milestones or skills. *Wilson's disease* - **Wilson's disease** is an inherited disorder that causes **copper accumulation** in organs, particularly the liver and brain. - Neurological symptoms, including **neuroregression**, can occur due to copper toxicity in the central nervous system. *Vitamin B12 deficiency* - **Vitamin B12 deficiency** can lead to neurological complications such as **subacute combined degeneration** of the spinal cord and peripheral neuropathy. - In children, severe or prolonged deficiency can impair brain development and lead to **developmental regression**. *Ataxia telangiectasia* - **Ataxia telangiectasia** is a rare, neurodegenerative, inherited disease that affects multiple body systems. - It is characterized by progressive **cerebellar ataxia**, leading to **neuroregression** and intellectual disability over time.

Q: What is the preferred medication for treating severe cases of rheumatic chorea that do not respond to standard treatments?

Haloperidol. **Haloperidol** - **Haloperidol**, a **dopamine receptor antagonist**, is a highly effective medication for severe rheumatic chorea that is refractory to standard treatments like carbamazepine or valproate. - Its mechanism of action involves blocking dopamine receptors in the brain, thereby reducing the **hyperkinetic movements** characteristic of chorea. *Valproate* - **Valproate** is a commonly used antiepileptic drug that can be effective for managing chorea, especially in milder cases. - However, it is generally considered a second-line agent for severe, refractory cases of **rheumatic chorea** compared to haloperidol. *Diazepam* - **Diazepam** is a benzodiazepine, primarily used for its **anxiolytic** and **sedative** properties due to its effect on GABA receptors. - While it can help alleviate some motor restlessness, it is not a first-line treatment for the involuntary movements of **chorea** and lacks the specific anti-chorea efficacy of antipsychotics. *Probenecid* - **Probenecid** is a uricosuric drug used to treat **gout** by increasing the excretion of uric acid. - It has no known role or efficacy in the management of **rheumatic chorea**.

Q: Which of the following statements about encephalocoele is false?

Common in the parietal region. ***Common in the parietal region*** - This statement is **false** because encephaloceles are **rarely found in the parietal region** (only 10-15% of cases). - **Occipital encephaloceles** are most common in Western populations (75-80%), while **frontal/sincipital encephaloceles** are most common in Southeast Asia including India (40-60%). - **Parietal encephaloceles** represent only a small minority of cases globally, making this statement incorrect. *It is a neural tube defect* - **Encephalocele** is indeed a type of **neural tube defect (NTD)**, resulting from incomplete closure of the neural tube during embryonic development. - Specifically, it involves a defect in the skull that allows for protrusion of brain tissue and/or meninges. *Can be associated with hydrocephalus* - **Hydrocephalus**, or the accumulation of cerebrospinal fluid in the brain, is a known complication and associated condition with encephaloceles. - The abnormal brain development and structural defects can disrupt normal CSF flow and absorption, particularly with posterior encephaloceles. *It is protrusion of neural tissue through a defect* - This is the defining characteristic of an **encephalocele**: the **herniation of intracranial contents**, such as brain tissue, meninges, or both, through a congenital **bony defect** in the skull. - The contents of the sac can vary (meninges only = meningocele; brain tissue included = meningoencephalocele), influencing clinical presentation and prognosis.

Q: A young child presents with episodes of inappropriate laughter. What is the most likely diagnosis?

Hypothalamic hamartoma. ***Hypothalamic hamartoma*** - **Gelastic seizures**, characterized by episodes of inappropriate laughter, are the hallmark symptom of a **hypothalamic hamartoma**. - These benign brain malformations are located near the **mammillary bodies** and can disrupt normal brain function, leading to these characteristic seizures. *Tetralogy of Fallot* - This is a **congenital heart defect** involving four specific abnormalities, leading to cyanosis. - Its symptoms include **"tet spells"** (bluish skin during crying or feeding), shortness of breath, and clubbing of fingers, not inappropriate laughter. *Nitrous oxide poisoning* - Inhalation of nitrous oxide can lead to euphoria, dizziness, and motor incoordination, but it does not typically cause recurrent episodes of involuntary, inappropriate laughter as a primary neurological symptom. - Chronic exposure can cause **vitamin B12 deficiency** and neurological dysfunction like neuropathy. *Seizure disorder* - While inappropriate laughter can be a symptom of a seizure (gelastic seizure), a general diagnosis of "seizure disorder" is too broad and does not pinpoint the specific, highly localized cause implied by this particular presentation. - The type of seizure (gelastic) specifically points to a lesion in the **hypothalamus**.

Q: What is the significance of the persistence of the asymmetric tonic neck reflex in a 9-month-old infant?

Increased muscle tone. ***Increased muscle tone*** - The **asymmetric tonic neck reflex (ATNR)** should integrate by **6 months of age**, and its persistence beyond this period is a sign of **neurological dysfunction**. - Persistent primitive reflexes, including ATNR, are often associated with **upper motor neuron lesions** and can manifest as increased muscle tone or **spasticity**. *Decreased muscle tone* - **Decreased muscle tone**, or **hypotonia**, is typically associated with **lower motor neuron lesions** or certain genetic conditions, not the persistence of primitive reflexes. - While some neurological conditions can cause hypotonia, persistent ATNR is a hallmark of problems leading to **hypertonia**. *Normal phenomenon* - The persistence of the ATNR beyond **6 months of age** is considered abnormal and indicates a potential developmental delay or neurological issue. - In a **9-month-old**, the reflex should have fully integrated, and its presence warrants further investigation. *None of the options* - As the persistence of the ATNR is indeed a significant finding, associated with increased muscle tone, this option is incorrect.

Question 1

What is the typical maintenance dose of Levetiracetam in pediatric patients?

Accepted Answer

30 - 40 mg/kg/day

Answer

10 - 20 mg/kg/day

Answer

20 - 30 mg/kg/day

Answer

40 - 50 mg/kg/day

Question 2

A 1-day-old male baby delivered by LSCS presents with swelling over the back in the midline. What is the most likely diagnosis?

Accepted Answer

Spina bifida

Answer

Neural tube defect

Answer

Dermal sinus

Answer

Meningocele

Question 3

What is the recurrence rate of febrile seizures?

Accepted Answer

30-50%

Answer

10-20%

Answer

20-30%

Answer

50-70%

Question 4

A 10-month-old infant was brought with complaints of jerking movements of limbs towards the body. On examination, there is regression of developmental milestones. Electroencephalogram shows hypsarrhythmia. What is the most appropriate first-line treatment?

Accepted Answer

Adrenocorticotropic hormone (ACTH)

Answer

Phenytoin

Answer

Levetiracetam

Answer

Phenobarbitone

Question 5

An 8-year old child has difficulty walking and getting up from a squatting position. A muscle    biopsy was done and is as shown in the image. Which of the following is true about this    condition?

Accepted Answer

Death occurs in the 3rd decade for certain types of muscular dystrophy.

Answer

Previous history of viral prodrome

Answer

It is a mitochondrial storage disorder

Answer

Early treatment has excellent prognosis

Question 6

Which of the following is not a known cause of neuroregression in children?

Accepted Answer

ADHD

Answer

Vitamin B12 deficiency

Answer

Ataxia telangiectasia

Answer

Wilson's disease

Question 7

What is the preferred medication for treating severe cases of rheumatic chorea that do not respond to standard treatments?

Accepted Answer

Haloperidol

Answer

Diazepam

Answer

Probenecid

Answer

Valproate

Question 8

Which of the following statements about encephalocoele is false?

Accepted Answer

Common in the parietal region

Answer

It is a neural tube defect

Answer

Can be associated with hydrocephalus

Answer

It is protrusion of neural tissue through a defect

Question 9

A young child presents with episodes of inappropriate laughter. What is the most likely diagnosis?

Accepted Answer

Hypothalamic hamartoma

Answer

Tetralogy of Fallot

Answer

Nitrous oxide poisoning

Answer

Seizure disorder

Question 10

What is the significance of the persistence of the asymmetric tonic neck reflex in a 9-month-old infant?

Accepted Answer

Increased muscle tone

Answer

Decreased muscle tone

Answer

Normal phenomenon

Answer

None of the options

Neurology — MCQs

Neurology — MCQs

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