Neurology Practice Questions

Q: A 7-year-old girl is easily distracted in class and exhibits poor scholastic performance. EEG shows? (AIIMS May 2014)

Absence seizures. ***Absence seizures*** - The clinical presentation of a young girl who is **easily distracted** and has **poor scholastic performance** is highly suggestive of absence seizures. These are brief episodes of impaired consciousness often mistaken for daydreaming. - The EEG image shows a characteristic **generalized 3-Hz spike-and-wave discharge** pattern, which is the hallmark of typical absence seizures. *Myoclonic seizures* - Myoclonic seizures involve **sudden, brief muscle jerks** or twitches, which are not described in the patient's presentation. - The EEG in myoclonic seizures typically shows **polyspike-and-wave discharges**, not the 3-Hz spike-and-wave pattern seen here. *Atonic seizures* - Atonic seizures, also known as **drop attacks**, cause a sudden loss of muscle tone leading to falls, which is not consistent with the described symptoms. - While EEG may show generalized spike-and-wave activity or diffuse slow waves during an atonic seizure, the 3-Hz rhythmic pattern is less typical for this seizure type. *Myoclonia* - Myoclonia refers to **involuntary, sudden muscle contractions**, which can be physiological (e.g., hiccups, sleep starts) or pathological. - While myoclonic seizures are a type of myoclonia, the term "myoclonia" alone is too general and does not specifically align with the classic EEG pattern and subtle clinical signs of absence seizures.

Q: The EEG of an infant with early morning jerks shows?

Hypsarrhythmia. ***Hypsarrhythmia*** - This EEG pattern is characterized by **chaotic, high-amplitude, irregular slow waves and spikes originating from multiple foci**. It is often seen in infants with infantile spasms, which manifest as **early morning jerks** or subtle head nods. - The provided image clearly shows a disorganised, high-amplitude, and multifocal discharge pattern consistent with hypsarrhythmia. *Burst and suppression pattern* - This pattern consists of alternating periods of **high-amplitude, fast activity (bursts)** and periods of **relative electrical inactivity (suppression)**. - It is typically associated with severe encephalopathy and some forms of epilepsy in infants, but not primarily with infantile spasms. *Periodic spike and wave pattern* - This pattern involves **repetitive, rhythmic sequences of a spike followed by a slow wave**, usually occurring every 1-3 seconds. - It is classically seen in **Lennox-Gastaut Syndrome**, a severe childhood epilepsy syndrome, rather than the early morning jerks characteristic of infantile spasms. *Rademecker complex* - Also known as **triphasic waves**, these are specific EEG abnormalities characterized by a typical three-phase morphology (positive-negative-positive or negative-positive-negative). - They are often associated with **metabolic encephalopathies**, particularly hepatic encephalopathy, and are not characteristic of infantile spasms.

Q: Comment on the diagnosis based on the image shown below. (Recent NEET Pattern 2016-17)

Plexiform neurofibromatosis. ***Plexiform neurofibromatosis*** - The image exhibits significant facial disfigurement with **nodular and soft tissue overgrowth**, particularly around the eye and midface, consistent with a **plexiform neurofibroma**. - This presentation is a classic feature of **Neurofibromatosis Type 1 (NF1)**, where plexiform neurofibromas can be extensive and cause severe cosmetic and functional issues. *Proteus syndrome* - Proteus syndrome is characterized by **asymmetric overgrowth** of various tissues including bone, skin, and fat, often leading to patchy or mosaic patterns of disproportionate growth. - While it involves overgrowth, the specific appearance in the image with its nodular, bag-like masses is more typical of plexiform neurofibroma than the broader, more generalized overgrowth seen in Proteus syndrome. *Rhabdomyosarcoma* - Rhabdomyosarcoma is a **malignant tumor of skeletal muscle**, typically presenting as a rapidly growing mass. - The lesions in the image appear more chronic and diffuse, lacking the aggressive, distinct tumoral characteristics of a typical rhabdomyosarcoma. *Malignant fibrous histiocytosis* - Malignant fibrous histiocytosis (now often classified under **undifferentiated pleomorphic sarcoma**) is a **high-grade soft tissue sarcoma** primarily affecting older adults, typically presenting as a rapidly enlarging mass. - Similar to rhabdomyosarcoma, this is a distinct malignant tumor and does not match the diffuse, chronic, and somewhat benign-appearing overgrowth seen in the picture.

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.

Question 1

A 7-year-old girl is easily distracted in class and exhibits poor scholastic performance. EEG shows? (AIIMS May 2014)

Accepted Answer

Absence seizures

Answer

Myoclonic seizures

Answer

Atonic seizures

Answer

Myoclonia

Question 2

The EEG of an infant with early morning jerks shows?

Accepted Answer

Hypsarrhythmia

Answer

Burst and suppression pattern

Answer

Periodic spike and wave pattern

Answer

Rademecker complex

Question 3

Comment on the diagnosis based on the image shown below. (Recent NEET Pattern 2016-17)

Accepted Answer

Plexiform neurofibromatosis

Answer

Proteus syndrome

Answer

Rhabdomyosarcoma

Answer

Malignant fibrous histiocytosis

Question 4

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 5

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 6

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 7

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 8

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 9

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 10

The congenital abnormality which is invariably lethal is:

Accepted Answer

Anencephaly

Answer

Transposition of great vessels

Answer

Cleft lip

Answer

Down Syndrome

Neurology — MCQs

Neurology — MCQs

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