Neurology Practice Questions

Q: Which among the following is a neural tube defect?

Diastematomyelia. **Explanation:** **Diastematomyelia** is a type of **occult spinal dysraphism** (Neural Tube Defect). It is characterized by a longitudinal splitting of the spinal cord into two hemicords, usually separated by a fibrous, cartilaginous, or osseous septum. This occurs due to an embryological error during **primary neurulation** or gastrulation, where an abnormal neurenteric canal persists, leading to the split. **Analysis of Incorrect Options:** * **B. Corpus callosal agenesis:** This is a **commissural anomaly** resulting from a failure of the axons to cross the midline between the two cerebral hemispheres. It is a disorder of prosencephalic midline development, not a failure of neural tube closure. * **C. Holoprosencephaly:** This is a **diverticulation defect** where the embryonic forebrain (prosencephalon) fails to divide into two distinct cerebral hemispheres. It is frequently associated with facial midline defects (e.g., cyclopia, cleft lip). * **D. Schizencephaly:** This is a **disorder of neuronal migration** (or a post-migratory encephaloclastic insult) characterized by gray matter-lined clefts extending from the pial surface to the ventricles. **High-Yield Clinical Pearls for NEET-PG:** * **Neural Tube Defects (NTDs)** are broadly classified into **Open** (e.g., Myelomeningocele, Anencephaly) and **Closed/Occult** (e.g., Spina bifida occulta, Diastematomyelia, Lipomyelomeningocele). * **Diastematomyelia** is often associated with **Tethered Cord Syndrome** and cutaneous markers like a tuft of hair (hypertrichosis) or a dimple over the spine. * **Prevention:** Periconceptional **Folic acid (0.4 mg/day)** reduces the risk of NTDs by 70%. For women with a previous affected child, the dose is increased to **4 mg/day**. * **Screening:** Elevated **Alpha-fetoprotein (AFP)** in maternal serum and amniotic fluid is seen in *open* NTDs, but is typically normal in closed defects like Diastematomyelia.

Q: A 1.5-year-old female child presents with a history of delayed developmental milestones. The child was normal until 5 months of age, after which milestones became delayed, and some attained milestones have regressed. On examination, the head circumference is smaller than expected, and the child has impaired coordination. What is the most likely diagnosis?

Rett disorder. **Explanation:** The clinical presentation of a **1.5-year-old female** with a period of normal development followed by **regression of milestones**, **acquired microcephaly** (smaller head circumference), and **impaired coordination** (ataxia) is classic for **Rett Syndrome**. **1. Why Rett Disorder is Correct:** Rett syndrome is an X-linked dominant neurodevelopmental disorder (mostly affecting females due to male lethality in utero) caused by a mutation in the **MECP2 gene**. * **Key Stages:** Infants typically develop normally until **6–18 months**, followed by a plateau and then rapid regression. * **Hallmarks:** Loss of purposeful hand skills (replaced by stereotypic **hand-wringing**), deceleration of head growth (acquired microcephaly), and gait abnormalities/ataxia. **2. Why Other Options are Incorrect:** * **Viral Encephalitis:** Presents acutely with fever, altered sensorium, and seizures, rather than a chronic, progressive developmental regression starting at 6 months. * **Reye’s Syndrome:** An acute metabolic emergency (associated with Aspirin use during viral illness) characterized by fatty liver and encephalopathy; it does not cause progressive microcephaly or developmental regression over months. * **Shakhnovich Syndrome:** This is a rare clinical sign (intermittent stupor/hypersomnia) associated with metabolic or neurological conditions, not a developmental regression disorder. **NEET-PG High-Yield Pearls:** * **Genetics:** MECP2 gene mutation on the X chromosome. * **Gender:** Almost exclusively seen in **females**. * **Classic Sign:** Repetitive, purposeless **hand-wringing** or "hand-washing" movements. * **Head Growth:** "Acquired microcephaly" is a high-yield diagnostic clue. * **Differential:** Often misdiagnosed as Autism, but the specific regression and microcephaly distinguish Rett.

Q: A CT scan of a child presenting with CNS pathology reveals a subdural effusion. Which of the following conditions commonly leads to subdural effusion in children?

Bacterial meningitis. **Explanation:** **Subdural effusion** is a common complication of **Bacterial Meningitis** in infants and young children, occurring in approximately 10–30% of cases. It is characterized by an accumulation of sterile, proteinaceous fluid in the subdural space. The underlying mechanism involves increased permeability of the meningeal vessels due to intense inflammation, leading to fluid leakage. It is most frequently associated with *Haemophilus influenzae* type b (Hib) and *Streptococcus pneumoniae*. Clinically, it should be suspected if a child with meningitis fails to improve after 48–72 hours of appropriate antibiotics or develops a bulging fontanelle and enlarging head circumference. **Analysis of Incorrect Options:** * **A. Tuberculous Meningitis:** Typically presents with basal exudates, hydrocephalus, and infarcts rather than subdural effusions. * **B. Trauma:** While trauma can cause subdural *hematomas* (blood), it is not the primary cause of the sterile *effusions* typically discussed in pediatric CNS infections. * **D. Viral Encephalitis:** Primarily affects the brain parenchyma. It does not typically involve the significant meningeal inflammation required to produce a subdural effusion. **NEET-PG High-Yield Pearls:** * **Most common organism:** Historically *H. influenzae* type b; currently *S. pneumoniae* (post-vaccination era). * **Diagnosis:** Transillumination of the skull (in infants) or CT/MRI showing crescentic fluid collections. * **Management:** Most effusions are asymptomatic and resolve spontaneously. Surgical drainage is indicated only if there are signs of increased intracranial pressure (e.g., seizures, persistent vomiting, or focal neurological deficits). * **Distinction:** If the fluid becomes infected, it is termed a **subdural empyema**.

Q: Regarding Dandy-Walker syndrome, which of the following statements are true and which are false? 1. It is the most common posterior fossa malformation. 2. It consists of a cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia. 3. The classic triad includes hypoplasia of the vermis, cephalad rotation of the vermian remnant, and cystic dilatation of the 4th ventricle extending posteriorly with a small posterior fossa. 4. The most common manifestation is macrocephaly. 5. Management involves shunting the cystic cavity.

1-True, 2-True, 3-False, 4-True, 5-True. **Dandy-Walker Malformation (DWM)** is the most common congenital malformation of the posterior fossa. It results from the developmental failure of the roof of the fourth ventricle. ### **Analysis of Statements:** 1. **True:** It is indeed the most common posterior fossa malformation. 2. **True:** The core pathology involves a cystic expansion of the 4th ventricle and varying degrees of cerebellar vermis hypoplasia. 3. **False:** While the triad includes vermian hypoplasia/rotation and cystic 4th ventricle dilatation, the **posterior fossa is characteristically ENLARGED**, not small. The tentorium and transverse sinuses are displaced superiorly (torcular-herophili inversion). 4. **True:** Macrocephaly is the most common clinical presentation (found in ~80% of cases), often accompanied by signs of increased intracranial pressure due to associated hydrocephalus. 5. **True:** Management focuses on treating hydrocephalus, typically via a **cystoperitoneal shunt** or ventriculoperitoneal shunt. ### **Why Option B is Correct:** Option B correctly identifies that Statement 3 is false because DWM is defined by an **enlarged posterior fossa**. A small posterior fossa is a hallmark of Chiari II malformation, not Dandy-Walker. ### **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** 1. Agenesis/Hypoplasia of cerebellar vermis; 2. Cystic dilatation of the 4th ventricle; 3. Enlarged posterior fossa. * **Associated Findings:** Hydrocephalus (present in 90%), Agenesis of Corpus Callosum (20-25%), and intellectual disability. * **Radiology Sign:** "Torcular-lambdoid inversion" (the torcular herophili lies above the lambdoid suture on X-ray/CT). * **Differential:** **Blake’s Pouch Cyst** (normal vermis, non-communicating) and **Mega Cisterna Magna** (normal vermis and 4th ventricle).

Q: Which of the following is NOT typically found in cerebral palsy?

Microcephaly. **Explanation:** Cerebral Palsy (CP) is a non-progressive permanent disorder of movement and posture caused by an insult to the developing fetal or infant brain. **Why Microcephaly is the correct answer:** While microcephaly is a common **associated finding** in children with CP (due to underlying brain malformation or injury), it is not a **defining motor feature** of the condition itself. In the context of this question, the other options represent specific motor types or manifestations of CP. Furthermore, many children with CP have normal head circumferences, making microcephaly a frequent comorbidity rather than a diagnostic requirement or a "typical" finding in the same category as motor tone abnormalities. **Analysis of Incorrect Options:** * **Hypotonicity (A):** This is common in the early stages of CP (the "hypotonic phase") before spasticity develops, and is the hallmark of **Atonic CP**. * **Ataxia (C):** This is a recognized clinical subtype (**Ataxic CP**), usually resulting from cerebellar injury, characterized by loss of coordination and balance. * **Flaccid paralysis (D):** While CP is primarily an Upper Motor Neuron (UMN) lesion (leading to spasticity), severe "floppy" infants can present with flaccid-like weakness. However, it is important to note that if the question implies "pure" Lower Motor Neuron (LMN) paralysis, it wouldn't be CP; but in many exams, "flaccid" is used interchangeably with the initial hypotonic phase of CP. **High-Yield Clinical Pearls for NEET-PG:** * **Most common type:** Spastic CP (specifically Spastic Diplegia, often associated with Periventricular Leukomalacia in preterms). * **Most common cause:** Prematurity (not birth asphyxia, which accounts for only ~10%). * **Early Sign:** Hand preference before age 1 year. * **Diagnosis:** Primarily clinical; MRI is the preferred imaging to identify the timing and extent of the brain insult.

Q: Which type of neonatal seizure has the best prognosis?

Focal. **Explanation:** The prognosis of neonatal seizures is primarily determined by the underlying etiology and the specific seizure semiology. **1. Why Focal Seizures are the Correct Answer:** Focal seizures (specifically focal clonic seizures) are associated with the best neurological prognosis. This is because they are often localized and frequently associated with focal brain lesions or transient metabolic disturbances that may not involve global encephalopathy. In the neonatal period, focal clonic seizures have a high correlation with localized pathology, which carries a much better long-term outcome (approx. 50-80% normal development) compared to generalized or fragmented patterns. **2. Why the Other Options are Incorrect:** * **Myoclonic Seizures:** These carry the **worst prognosis**. They are often associated with severe underlying brain dysfunction, such as Inborn Errors of Metabolism (e.g., Non-ketotic hyperglycinemia) or Early Myoclonic Encephalopathy (EME). * **Tonic Seizures:** These are frequently seen in premature infants with Intraventricular Hemorrhage (IVH) or severe Hypoxic-Ischemic Encephalopathy (HIE), indicating significant brainstem involvement and a poor prognosis. * **Opsoclonus:** This is not a seizure type but a movement disorder (rapid, involuntary, multivectorial eye movements). In pediatrics, it is classically associated with Neuroblastoma (Opsoclonus-Myoclonus-Ataxia Syndrome). **High-Yield Clinical Pearls for NEET-PG:** * **Most common type** of neonatal seizure: **Subtle seizures** (e.g., bicycling, rowing, apnea, lip-smacking). * **Best prognosis:** Focal clonic seizures. * **Worst prognosis:** Myoclonic seizures. * **First-line drug:** Phenobarbital remains the drug of choice for neonatal seizures (Levetiracetam is an emerging alternative). * **Most common cause:** Hypoxic-Ischemic Encephalopathy (HIE) is the #1 cause of seizures in term neonates.

Q: Which of the following radiographic findings in a child may indicate increased intracranial tension?

All of the above. Increased intracranial pressure (ICP) in children manifests differently depending on the age of the patient and the patency of the cranial sutures. The correct answer is **All of the above** because each finding represents a compensatory or pathological response to elevated pressure within the rigid or semi-rigid skull. ### **Explanation of Findings:** * **Separation of the Sutures (Sutural Diastasis):** In infants and young children, the cranial sutures are not yet fused. When ICP rises, the pressure pushes the cranial bones apart. Radiographically, a suture width of >2 mm is generally considered abnormal. * **Tense Anterior Fontanelle:** The fontanelle acts as a "pressure valve." While a bulging fontanelle is primarily a clinical finding, it indicates increased volume within the vault. On imaging (like lateral X-rays or ultrasound), this appears as an outward convexity of the soft tissue at the bregma. * **Silver Beaten Appearance (Copper Beaten Skull):** This refers to prominent gyral impressions on the inner table of the skull. It is caused by the chronic pulsation of the cerebral gyri against the bone due to long-standing increased ICP. While it can be a normal variant in children aged 4–10, its presence in a symptomatic child is highly suggestive of pathology (e.g., craniosynostosis or obstructive hydrocephalus). ### **Clinical Pearls for NEET-PG:** * **Macewen’s Sign (Cracked Pot Sign):** A clinical sign where percussion of the skull over separated sutures produces a resonant sound. * **Sunset Phenomenon:** Downward deviation of the eyes seen in infants with hydrocephalus/increased ICP. * **Cushing’s Triad:** A late sign of increased ICP consisting of hypertension, bradycardia, and irregular respirations. * **Gold Standard:** While X-rays show these signs, **CT or MRI** is the investigation of choice for diagnosing the underlying cause of increased ICP.

Q: Infantile spasms are associated with what finding?

A hypsarrhythmic pattern on EEG. **Explanation:** **Infantile Spasms (West Syndrome)** is a specific seizure disorder of infancy characterized by the triad of infantile spasms, developmental regression, and a pathognomonic EEG pattern. 1. **Why Option A is Correct:** The hallmark EEG finding in West Syndrome is **Hypsarrhythmia**. This is described as a "chaotic" pattern consisting of high-voltage, disorganized slow waves and multi-focal spikes against a background of no identifiable rhythm. It is most prominent during non-REM sleep. Identifying this pattern is crucial for diagnosis. 2. **Why the other options are incorrect:** * **Option B:** The prognosis is generally **poor**. Up to 80–90% of children experience significant intellectual disability and many go on to develop other seizure types, such as Lennox-Gastaut Syndrome. * **Option C:** Phenobarbital is not the treatment of choice. The first-line treatments are **ACTH (Adrenocorticotropic hormone)** or oral prednisolone. In cases associated with Tuberous Sclerosis, **Vigabatrin** is the drug of choice. * **Option D:** Hypocalcemia typically presents as jitteriness or tetany in neonates, not as infantile spasms. West Syndrome is more commonly associated with structural brain anomalies, metabolic disorders, or genetic conditions (e.g., Tuberous Sclerosis). **High-Yield Clinical Pearls for NEET-PG:** * **Triad of West Syndrome:** Infantile spasms (salaam seizures), developmental arrest/regression, and hypsarrhythmia. * **Age of onset:** Typically between 4 to 8 months. * **Drug of Choice (General):** ACTH. * **Drug of Choice (Tuberous Sclerosis):** Vigabatrin (Watch for visual field defects as a side effect). * **Evolution:** Infantile spasms often evolve into **Lennox-Gastaut Syndrome** (characterized by slow spike-wave discharges on EEG).

Q: What is the drug of choice for infantile spasms in a child with Tuberous sclerosis?

Vigabatrin. **Explanation:** **Infantile Spasms (West Syndrome)** is characterized by the triad of infantile spasms, hypsarrhythmia on EEG, and developmental regression. While the standard first-line treatment for idiopathic cases is **ACTH**, the management changes significantly when the underlying etiology is **Tuberous Sclerosis Complex (TSC)**. **Why Vigabatrin is the Correct Answer:** In children with Tuberous Sclerosis, **Vigabatrin** is the drug of choice (DOC). It is an irreversible inhibitor of GABA-transaminase, increasing GABA levels in the brain. It has shown superior efficacy specifically in TSC-associated spasms, often achieving complete cessation of seizures. **Analysis of Incorrect Options:** * **ACTH (Adrenocorticotropic Hormone):** This is the drug of choice for infantile spasms **not** associated with Tuberous Sclerosis. In TSC patients, Vigabatrin is preferred due to higher specific response rates. * **Diazepam:** While a benzodiazepine used for status epilepticus, it has no role as a primary maintenance therapy for infantile spasms. * **Levetiracetam:** This is a broad-spectrum anticonvulsant used for focal or generalized tonic-clonic seizures, but it is not effective for the specific pathophysiology of hypsarrhythmia in West Syndrome. **High-Yield Clinical Pearls for NEET-PG:** * **Side Effect Alert:** The most significant side effect of Vigabatrin is **permanent peripheral visual field defects** (concentric visual field contraction). Regular ophthalmological monitoring is mandatory. * **TSC Triad (Vogt’s Triad):** Adenoma sebaceum (facial angiofibromas), mental retardation, and seizures. * **EEG Finding:** The classic EEG pattern for West Syndrome is **Hypsarrhythmia** (high-amplitude, disorganized, chaotic background). * **Summary:** * Infantile Spasms (General) → **ACTH** * Infantile Spasms (with Tuberous Sclerosis) → **Vigabatrin**

Question 1

Which among the following is a neural tube defect?

Accepted Answer

Diastematomyelia

Answer

Corpus callosal agenesis

Answer

Holoprosencephaly

Answer

Schizencephaly

Question 2

A 1.5-year-old female child presents with a history of delayed developmental milestones. The child was normal until 5 months of age, after which milestones became delayed, and some attained milestones have regressed. On examination, the head circumference is smaller than expected, and the child has impaired coordination. What is the most likely diagnosis?

Accepted Answer

Rett disorder

Answer

Viral encephalitis

Answer

Reye's syndrome

Answer

Shakhnovich syndrome

Question 3

What is the preferred treatment for Rolandic epilepsy?

Accepted Answer

Carbamazepine

Answer

Phenytoin

Answer

Lamotrigine

Answer

ACTH

Question 4

A CT scan of a child presenting with CNS pathology reveals a subdural effusion. Which of the following conditions commonly leads to subdural effusion in children?

Accepted Answer

Bacterial meningitis

Answer

Tuberculous meningitis

Answer

Trauma

Answer

Viral encephalitis

Question 5

Regarding Dandy-Walker syndrome, which of the following statements are true and which are false? 1. It is the most common posterior fossa malformation. 2. It consists of a cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia. 3. The classic triad includes hypoplasia of the vermis, cephalad rotation of the vermian remnant, and cystic dilatation of the 4th ventricle extending posteriorly with a small posterior fossa. 4. The most common manifestation is macrocephaly. 5. Management involves shunting the cystic cavity.

Accepted Answer

1-True, 2-True, 3-False, 4-True, 5-True

Answer

1-True, 2-True, 3-True, 4-True, 5-True

Answer

1-True, 2-False, 3-False, 4-True, 5-True

Answer

1-True, 2-True, 3-True, 4-False, 5-False

Question 6

Which of the following is NOT typically found in cerebral palsy?

Accepted Answer

Microcephaly

Answer

Hypotonicity

Answer

Ataxia

Answer

Flaccid paralysis

Question 7

Which type of neonatal seizure has the best prognosis?

Accepted Answer

Focal

Answer

Myoclonic

Answer

Tonic clonic

Answer

Opsoclonus

Question 8

Which of the following radiographic findings in a child may indicate increased intracranial tension?

Accepted Answer

All of the above

Answer

Separation of the sutures

Answer

Tense anterior fontanelle

Answer

Silver beaten appearance of the skull bones

Question 9

Infantile spasms are associated with what finding?

Accepted Answer

A hypsarrhythmic pattern on EEG

Answer

A favorable outlook regarding neurologic and intellectual development

Answer

A response to treatment with Phenobarbital

Answer

Hypocalcemia

Question 10

What is the drug of choice for infantile spasms in a child with Tuberous sclerosis?

Accepted Answer

Vigabatrin

Answer

Diazepam

Answer

ACTH

Answer

Levetiracetam

Neurology — MCQs

Neurology — MCQs

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