Neurology Practice Questions

Q: A 1-year-old child presents with infantile spasms. EEG done shows: (Recent NEET Pattern 2016-17)

Hypsarrhythmia. ***Hypsarrhythmia*** - The provided EEG shows a chaotic, high-amplitude, and **disorganized background** activity with multifocal spikes and sharp waves, indicative of hypsarrhythmia. - This pattern is characteristic of **infantile spasms**, a severe epilepsy syndrome typically presenting in the first year of life with myoclonic seizures. *Normal record* - A normal EEG would show organized, age-appropriate background rhythms with no pathological discharges. - The presented EEG is clearly **abnormal and highly disorganized**, ruling out a normal record. *Periodic spike wave pattern* - Periodic spike-wave patterns, such as 3-Hz spike-and-wave, are typically seen in **absence seizures**, which manifest as brief staring spells rather than myoclonic seizures. - The EEG in the image is more **chaotic and polymorphous** than a rhythmic, generalized spike-wave pattern. *Burst suppression pattern* - **Burst suppression** is characterized by intermittent bursts of high-amplitude activity alternating with periods of relative electrical silence. - This pattern is typically seen in severe encephalopathies, deep anesthesia, or certain severe epilepsy syndromes like Ohtahara syndrome, but it does not match the **continuous, disorganized high-amplitude** pattern shown.

Q: A 6 month infant was brought with complaints of a failure to gain weight and a large head. On examination, increased head circumference, bounding pulses and features of heart failure were noted. On cranial auscultation loud cranial bruit was heard. MRI head shows? (Recent NEET Pattern 2018-19)

Vein of Galen malformation. ***Vein of Galen formation*** - The clinical presentation of **failure to thrive**, **macrocephaly**, **bounding pulses**, **heart failure**, and a **loud cranial bruit** in an infant is highly characteristic of a **Vein of Galen malformation (VOGM)**. The image would show a dilated vein of Galen. - VOGMs are high-flow arteriovenous malformations that can lead to significant hemodynamic stress on the heart and hydrocephalus due to obstruction of CSF pathways. *Arachnoid cyst* - While arachnoid cysts can cause **macrocephaly** and, less commonly, obstructive hydrocephalus, they generally do not present with **heart failure**, **bounding pulses**, or a **cranial bruit**. - MRI would show a CSF-filled cyst that follows CSF signal on all sequences and typically does not enhance. *Arnold-Chiari malformation* - Arnold-Chiari malformations involve downward displacement of cerebellar tonsils or vermis through the foramen magnum and are associated with hydrocephalus, but they do not typically cause **heart failure**, **bounding pulses**, or a **cranial bruit**. - Clinical features usually relate to brain stem compression or hydrocephalus, such as apnea, stridor, or feeding difficulties. *Dandy-Walker syndrome* - Dandy-Walker syndrome is characterized by hypoplasia of the cerebellar vermis and cystic dilation of the fourth ventricle, often leading to **hydrocephalus** and **macrocephaly**. - However, it does not explain the **bounding pulses**, **heart failure**, or **cranial bruit** seen in this patient, which point to a vascular anomaly.

Q: Which of the following are tools commonly used in the evaluation of children with cerebral palsy for motor function and spasticity assessment? I. Gross Motor Function Classification System II. Medical Research Council System III. Modified Connors Scale (Connors-II) IV. Modified Ashworth Scale Select the correct answer using the code given below:

I and IV. ***Correct: I and IV*** - The **Gross Motor Function Classification System (GMFCS)** is the gold standard tool to classify gross motor function in children with **cerebral palsy** based on self-initiated movement and functional limitations across five levels (I-V). - The **Modified Ashworth Scale** is the most widely used clinical tool for assessing **spasticity** and muscle tone in cerebral palsy, grading resistance to passive movement on a 0-4 scale. - These two tools directly address **motor function classification** and **spasticity assessment** as required in the question. *Incorrect: III and IV* - While the **Modified Ashworth Scale (IV)** is correctly identified for spasticity assessment, the **Modified Connors Scale (Connors-II)** is used exclusively for evaluating **Attention-Deficit/Hyperactivity Disorder (ADHD)**, not motor function or spasticity in cerebral palsy. *Incorrect: I and II* - The **GMFCS (I)** is correctly identified for motor function classification in cerebral palsy. - However, the **Medical Research Council (MRC) System** is primarily used for **muscle strength grading** (0-5 scale) in conditions like peripheral neuropathy, stroke, or myopathy—not for motor function classification or spasticity assessment specific to cerebral palsy. *Incorrect: II and III* - Both tools are inappropriate for the stated purpose: the **MRC System** assesses muscle strength (not CP-specific motor function classification), and the **Modified Connors Scale** evaluates ADHD symptoms. - Neither tool is standard for evaluating motor function or spasticity in cerebral palsy.

Q: Which of the following are characteristic features of cerebral palsy? I. Disorder of movement II. Permanent nature III. Progressive course IV. Disorder of posture Select the correct answer using the code given below :

I, II and IV. ***Correct: I, II and IV*** - Cerebral palsy is defined as a group of **permanent disorders** that affect the development of **movement and posture**, causing activity limitations. - It is attributed to **non-progressive disturbances** that occurred in the developing fetal or infant brain. - Key characteristics: **Disorder of movement (I)** + **Permanent nature (II)** + **Disorder of posture (IV)** + Non-progressive course. *Incorrect: I, II and III* - While cerebral palsy is a **disorder of movement** and **permanent**, it is explicitly characterized by its **non-progressive** nature. - The underlying brain lesion does not worsen over time, though clinical manifestations may change as the child develops. - Statement III (Progressive course) is incorrect. *Incorrect: I, III and IV* - Cerebral palsy is fundamentally a **disorder of movement** and **posture**. - However, a key distinguishing characteristic is its **non-progressive** nature; the brain injury itself does not advance or worsen. - Statement III (Progressive course) contradicts the definition of cerebral palsy. *Incorrect: II, III and IV* - Cerebral palsy is **permanent** and affects **posture**, but it has a **non-progressive** course, not progressive. - It is also fundamentally a **disorder of movement**, which is a primary manifestation. - Statement III (Progressive course) is incorrect, making this combination wrong.

Q: Which one of the following childhood epileptic disorders often needs long term treatment with antiepileptic drugs?

Juvenile myoclonic epilepsy. ***Juvenile myoclonic epilepsy*** - This is a **genetic generalized epilepsy syndrome** that typically emerges during adolescence and often requires **lifelong treatment** with antiepileptic drugs due to a high risk of relapse if treatment is discontinued. - Characterized by **myoclonic jerks**, particularly in the morning, along with generalized tonic-clonic and absence seizures. *Febrile seizures* - These are **age-dependent seizures** that occur in association with fever and typically have a **benign prognosis**, rarely requiring long-term antiepileptic drug treatment. - They usually resolve spontaneously by **age 5-6 years**, and the risk of developing epilepsy is low unless there are complex features or underlying neurological conditions. *Benign rolandic epilepsy* - This is an **idiopathic focal epilepsy** of childhood that is characterized by seizures occurring primarily during sleep, with **motor or sensory symptoms** involving the face and mouth. - It has a very **favorable prognosis**, with spontaneous remission typically occurring by adolescence, and many children do not require antiepileptic drugs or only short-term treatment. *Benign neonatal seizures* - This refers to a group of self-limited epileptic syndromes that occur in the first few weeks of life, often with a **hereditary component**, but they typically **resolve spontaneously** within weeks or months. - **Long-term antiepileptic drug treatment** is generally not needed, and the **neurodevelopmental outcome is usually excellent**.

Q: Neural tube defects have which one of the following inheritance patterns ?

Multi-factorial. ***Multi-factorial*** - Neural tube defects (NTDs) are considered **multi-factorial**, meaning they result from a complex interaction between multiple genetic predispositions and environmental factors. - While there are genetic components, no single gene mutation typically explains the recurrence risk, and external factors like **folic acid deficiency** play a significant role. *X-linked recessive* - This inheritance pattern typically affects males more severely and exclusively, with females often being carriers, which is not the primary pattern observed in NTDs. - Conditions like **Duchenne muscular dystrophy** exhibit X-linked recessive inheritance. *Autosomal dominant* - A single copy of an altered gene on a non-sex chromosome is sufficient to cause the condition, resulting in a 50% chance of transmission to offspring, which does not match the observed inheritance pattern for NTDs. - Examples include **Huntington's disease** and **Marfan syndrome**. *Autosomal recessive* - Both copies of a gene on a non-sex chromosome must be altered for the condition to manifest, meaning parents are often carriers but unaffected, which isn't the primary inheritance pattern for NTDs. - Conditions like **cystic fibrosis** and **sickle cell anemia** follow autosomal recessive inheritance.

Q: The congenital abnormality which is invariably lethal is:

Anencephaly. ***Anencephaly*** - **Anencephaly** is a severe form of **neural tube defect** where the brain and skull do not develop properly, resulting in the absence of a major part of the brain and skull. - Infants born with anencephaly are either stillborn or die shortly after birth due to the lack of vital brain structures, making it an **invariably lethal** condition. *Transposition of great vessel* - **Transposition of the great arteries (TGA)** is a serious congenital heart defect where the two main arteries leaving the heart (aorta and pulmonary artery) are connected to the wrong ventricles. - While TGA is a life-threatening condition requiring immediate medical intervention, including surgery, it is **not invariably lethal** with modern treatment and advances in pediatric cardiology. *Cleft lip* - A **cleft lip** is a birth defect characterized by a split or opening in the upper lip, which may extend into the nose. - While it requires surgical correction and can cause feeding difficulties or speech problems if untreated, it is **not life-threatening** and has no direct impact on infant mortality. *Down Syndrome* - **Down syndrome** (Trisomy 21) is a chromosomal disorder characterized by intellectual disability, distinctive facial features, and often associated with other medical conditions like heart defects or gastrointestinal abnormalities. - Individuals with Down syndrome have a **reduced life expectancy** compared to the general population; however, advancements in medical care have significantly improved their lifespan, and it is **not an invariably lethal** condition.

Q: A child presents with myoclonic jerks and decreasing school performance. The child had a history of fever and rash at the age of 1 year. What is the most likely diagnosis?

Subacute sclerosing panencephalitis (SSPE). ***Subacute sclerosing panencephalitis (SSPE)*** - SSPE is a **rare, progressive, and fatal neurodegenerative disease** caused by persistent measles virus infection of the central nervous system. - The presentation of **myoclonic jerks** (stage 2 SSPE) and **decreasing school performance** (stage 1 - cognitive decline) in a child with a history of measles (fever and rash) at 1 year of age is **pathognomonic** for SSPE. - SSPE typically manifests **7-10 years after measles infection**, with male predominance (M:F = 2-4:1). - EEG shows characteristic **Radermecker complexes** (periodic high-amplitude slow wave complexes). - CSG findings include elevated measles antibodies with high CSF:serum antibody ratio. *Measles* - While the child had measles in infancy, the current neurological symptoms are **not acute measles** but rather a late complication (SSPE). - Acute measles is a self-limiting illness; these progressive neurological symptoms occurring years later indicate SSPE, not active measles. *Mesial temporal sclerosis* - This is the most common cause of **drug-resistant temporal lobe epilepsy** characterized by hippocampal atrophy. - Presents with **focal seizures with impaired awareness**, not generalized myoclonic jerks. - **No link to prior measles infection** and typically associated with history of febrile seizures or status epilepticus in childhood. - Progressive cognitive decline is not a primary feature. *Polio* - Polio is caused by **poliovirus** affecting anterior horn cells of the spinal cord, causing **acute flaccid paralysis**. - Presents with **asymmetric lower motor neuron weakness**, not myoclonic jerks or cognitive decline. - **No association with measles** or rash in history. - Would not cause the progressive encephalopathy seen in this case.

Q: Adrenocorticotropic hormone is the drug of choice in which of the following conditions?

West syndrome. ***West syndrome*** - **Adrenocorticotropic hormone (ACTH)** is considered the drug of choice for West syndrome, also known as infantile spasms, due to its efficacy in reducing spasm frequency and improving neurodevelopmental outcomes. - **Steroid therapy**, including ACTH, is thought to work by modulating cortisol levels and neurotransmitter activity, suppressing the abnormal brain activity characteristic of these seizures. *Juvenile myoclonic epilepsy* - The drug of choice for juvenile myoclonic epilepsy is typically **valproate**, or newer antiepileptic drugs like levetiracetam or lamotrigine, known for their efficacy against myoclonic seizures. - ACTH is not used in this condition, as it is generally effective for specific types of **epileptic encephalopathies** and not generalized idiopathic epilepsies like JME. *Rolandic epilepsy* - **Rolandic epilepsy** (benign epilepsy with centrotemporal spikes) often resolves spontaneously and may not require treatment, but if seizures are frequent, drugs like gabapentin or carbamazepine are used. - ACTH has no established role in the treatment of Rolandic epilepsy, which is a **focal idiopathic epilepsy** with a good prognosis. *Lennox Gastaut syndrome* - While a severe epileptic encephalopathy, the initial management for **Lennox-Gastaut syndrome** (LGS) typically involves a combination of antiepileptic drugs such as valproate, lamotrigine, clobazam, or rufinamide. - ACTH may be considered as an adjunctive treatment in refractory cases of LGS, but it is not the **first-line drug of choice**, unlike in West syndrome where it holds a primary role.

Q: Which of the following is not typical of Simple Febrile Seizures?

The seizures are followed by Todd's paralysis. ***The seizures are followed by Todd's paralysis*** - **Todd's paralysis** (postictal hemiparesis) is a focal neurological deficit typically seen after a **focal seizure**, rather than a generalized simple febrile seizure. - Simple febrile seizures are **generalized tonic-clonic events** and do not typically involve focal neurological deficits such as Todd's paralysis. *The age of onset is usually between 6 months and 6 years* - This statement accurately describes the characteristic **age range** for simple febrile seizures, which primarily affect young children. - The developing brain's susceptibility to fever-induced seizures is highest during this period. *The seizures are more likely to occur if the body temperature rises rapidly* - A **rapid rise in body temperature** is a recognized trigger for simple febrile seizures, often more so than the absolute temperature itself. - This sudden change is thought to overwhelm the immature thermoregulatory system and neuronal excitability in young children. *The seizures last for less than 15 minutes* - One of the defining characteristics of a **simple febrile seizure** is its short duration, typically lasting **less than 15 minutes**. - Seizures lasting longer or occurring in multiple episodes are classified as **complex febrile seizures**.

Question 1

A 1-year-old child presents with infantile spasms. EEG done shows: (Recent NEET Pattern 2016-17)

Accepted Answer

Hypsarrhythmia

Answer

Normal record

Answer

Periodic spike wave pattern

Answer

Burst suppression pattern

Question 2

A 6 month infant was brought with complaints of a failure to gain weight and a large head. On examination, increased head circumference, bounding pulses and features of heart failure were noted. On cranial auscultation loud cranial bruit was heard. MRI head shows? (Recent NEET Pattern 2018-19)

Accepted Answer

Vein of Galen malformation

Answer

Arachnoid cyst

Answer

Arnold-Chiari malformation

Answer

Dandy-Walker syndrome

Question 3

Which of the following are tools commonly used in the evaluation of children with cerebral palsy for motor function and spasticity assessment?
I. Gross Motor Function Classification System
II. Medical Research Council System
III. Modified Connors Scale (Connors-II)
IV. Modified Ashworth Scale

Select the correct answer using the code given below:

Accepted Answer

I and IV

Answer

III and IV

Answer

I and II

Answer

II and III

Question 4

Which of the following are characteristic features of cerebral palsy?
I. Disorder of movement
II. Permanent nature
III. Progressive course
IV. Disorder of posture

Select the correct answer using the code given below :

Accepted Answer

I, II and IV

Answer

I, II and III

Answer

I, III and IV

Answer

II, III and IV

Question 5

Which one of the following childhood epileptic disorders often needs long term treatment with antiepileptic drugs?

Accepted Answer

Juvenile myoclonic epilepsy

Answer

Febrile seizures

Answer

Benign rolandic epilepsy

Answer

Benign neonatal seizures

Question 6

Neural tube defects have which one of the following inheritance patterns ?

Accepted Answer

Multi-factorial

Answer

X-linked recessive

Answer

Autosomal dominant

Answer

Autosomal recessive

Question 7

The congenital abnormality which is invariably lethal is:

Accepted Answer

Anencephaly

Answer

Transposition of great vessels

Answer

Cleft lip

Answer

Down Syndrome

Question 8

A child presents with myoclonic jerks and decreasing school performance. The child had a history of fever and rash at the age of 1 year. What is the most likely diagnosis?

Accepted Answer

Subacute sclerosing panencephalitis (SSPE)

Answer

Measles

Answer

Mesial temporal sclerosis

Answer

Polio

Question 9

Adrenocorticotropic hormone is the drug of choice in which of the following conditions?

Accepted Answer

West syndrome

Answer

Juvenile myoclonic epilepsy

Answer

Rolandic epilepsy

Answer

Lennox Gastaut syndrome

Question 10

Which of the following is not typical of Simple Febrile Seizures?

Accepted Answer

The seizures are followed by Todd's paralysis

Answer

The age of onset is usually between 6 months and 6 years

Answer

The seizures are more likely to occur if the body temperature rises rapidly

Answer

The seizures last for less than 15 minutes

Neurology — MCQs

Neurology — MCQs

On this page

Practice by Chapter

Want unlimited practice?