Neurology — MCQs

A 9-month-old previously healthy infant presented with a 12-hour history of fever, rhinorrhea, and a single episode of generalized tonic-clonic seizure lasting 34 minutes. The infant was brought to the emergency department within 1 hour and was observed to be alert, active, and playful after the fever subsided. What is the most probable diagnosis in this infant?

Q15Easy

Which of the following statements is true regarding Duchenne muscular dystrophy?

Q16Medium

What is seen in the knee jerk reflex in scurvy?

Q17Easy

All are causes of infantile tremor syndrome EXCEPT:

Q18Medium

Which of the following is NOT typically seen in metachromatic leukodystrophy?

Q19Medium

A 7-day-old neonate presents with recurrent seizures. On examination, tachycardia and S3 gallop are noted, along with a bruit in the anterior fontanelle. Blood investigations are normal. Neurosonogram shows a hypoechoic lesion. What is the diagnosis?

Q20Easy

Gower's maneuver is classically seen in:

Neurology Indian Medical PG Practice Questions and MCQs

Question 11: Which of the following epileptic syndromes has the worst prognosis?

A. Rolandic epilepsy
B. Versive epilepsy
C. Absence epilepsy
D. Infantile spasm (Correct Answer)

Explanation: **Explanation:** **Infantile Spasms (West Syndrome)** is associated with the worst prognosis among the options provided due to its strong association with significant neurodevelopmental regression and long-term cognitive impairment. It is characterized by the classic triad of infantile spasms, **hypsarrhythmia** on EEG, and developmental delay. Even with prompt treatment (using ACTH or Vigabatrin), many children progress to other refractory seizure types, such as Lennox-Gastaut Syndrome, and suffer from permanent intellectual disabilities. **Analysis of Incorrect Options:** * **Rolandic Epilepsy (Benign Rolandic Epilepsy):** As the name suggests, it is "benign" and has an excellent prognosis. It is age-dependent, typically occurring between 3–13 years, and almost always remits spontaneously by puberty without long-term deficits. * **Absence Epilepsy (Childhood Absence Epilepsy):** This generally has a good prognosis. Most children respond well to Ethosuximide or Valproate, and approximately 80% outgrow the seizures by adolescence without cognitive decline. * **Versive Epilepsy:** This refers to a focal seizure involving head and eye deviation. The prognosis depends entirely on the underlying structural lesion, but it is not inherently a "catastrophic" epileptic encephalopathy like Infantile Spasms. **NEET-PG High-Yield Pearls:** * **EEG Hallmark:** Hypsarrhythmia (high-voltage, chaotic, disorganized background). * **Drug of Choice (DOC):** **ACTH** is the overall DOC; however, **Vigabatrin** is the DOC if the spasms are associated with **Tuberous Sclerosis**. * **Age of Onset:** Typically between 4 to 8 months of age. * **Evolution:** Often evolves into **Lennox-Gastaut Syndrome** (characterized by slow spike-wave discharges <2.5 Hz and multiple seizure types).

Question 12: In pediatric patients, what factor significantly increases the risk of developing post-traumatic epilepsy?

A. Brief loss of consciousness
B. Acute intracranial hemorrhage (Correct Answer)
C. Retrograde amnesia
D. Post-traumatic vomiting

Explanation: **Explanation:** Post-traumatic epilepsy (PTE) is defined as recurrent unprovoked seizures occurring more than one week after a traumatic brain injury (TBI). The risk of developing PTE is directly proportional to the severity of the initial brain insult. **Why Acute Intracranial Hemorrhage is Correct:** Intracranial hemorrhage (such as subdural, epidural, or intracerebral hematoma) and cerebral contusions are the strongest predictors of PTE. The underlying pathophysiology involves the breakdown of hemoglobin, which releases **hemosiderin and free iron** into the brain parenchyma. These substances act as potent pro-convulsants by generating free radicals and causing oxidative stress, leading to the formation of an epileptogenic focus. **Why the Other Options are Incorrect:** * **Brief Loss of Consciousness (LOC):** While LOC is a marker of concussion, a brief duration (typically <30 minutes) without associated structural damage is considered a "mild" TBI and does not significantly elevate the long-term risk of epilepsy. * **Retrograde Amnesia:** Amnesia is a common feature of mild-to-moderate TBI. Unless it is prolonged (Post-Traumatic Amnesia >24 hours), it is not a primary independent risk factor for PTE. * **Post-traumatic Vomiting:** This is a common non-specific symptom in pediatric head injuries due to increased intracranial pressure or vestibular upset. It does not correlate with the development of a chronic seizure disorder. **High-Yield Facts for NEET-PG:** * **Risk Factors for PTE:** Penetrating brain injuries (highest risk), depressed skull fractures, GCS <8, and acute intracranial hematomas. * **Classification:** * *Immediate:* Within 24 hours. * *Early:* Within 1 week (increased risk of late PTE). * *Late:* After 1 week (defines Post-traumatic Epilepsy). * **Prophylaxis:** Prophylactic anti-epileptic drugs (e.g., Phenytoin or Levetiracetam) are indicated to prevent **early** seizures but have **not** been proven to prevent the development of late PTE.

Question 13: A 15-year-old boy complains of jerky movements that are precipitated upon awakening in the morning. There is no history of loss of consciousness. EEG showed a 4-6 Hz spike and wave pattern. What is the child suffering from?

A. Lennox-Gastaut syndrome
B. Juvenile Myoclonic Epilepsy (Correct Answer)
C. Rolandic epilepsy
D. West syndrome

Explanation: ### Explanation **Correct Answer: B. Juvenile Myoclonic Epilepsy (JME)** The clinical presentation is classic for **Juvenile Myoclonic Epilepsy (JME)**, also known as Janz syndrome. The key diagnostic features in this case are: 1. **Age of onset:** Typically occurs between 12–18 years. 2. **Seizure type:** Myoclonic jerks (sudden, brief, involuntary muscle contractions) without loss of consciousness. 3. **Circadian rhythm:** Characteristically occurs shortly **after awakening** (often exacerbated by sleep deprivation). 4. **EEG findings:** Shows generalized, symmetric **4–6 Hz polyspike-and-slow-wave** discharges. --- ### Why the other options are incorrect: * **A. Lennox-Gastaut Syndrome:** This is a severe childhood epilepsy (onset 3–5 years) characterized by a triad of multiple seizure types (atonic, tonic), intellectual disability, and a slow EEG pattern (**<2.5 Hz spike-and-wave**). * **C. Rolandic Epilepsy (Benign Childhood Epilepsy with Centrotemporal Spikes):** This presents in younger children (5–10 years) with nocturnal or early morning focal seizures involving the face and oropharynx (drooling, speech arrest). EEG shows **centrotemporal spikes**. * **D. West Syndrome:** This occurs in infants (3–12 months) and is characterized by infantile spasms, developmental arrest, and an EEG pattern of **hypsarrhythmia**. --- ### NEET-PG High-Yield Pearls: * **Drug of Choice (DOC):** Valproate is the DOC for JME. In females of childbearing age, Levetiracetam is preferred due to the teratogenicity of Valproate. * **Prognosis:** JME usually requires **lifelong treatment**; relapse rates are high (>90%) if medication is discontinued. * **Triggers:** Sleep deprivation, alcohol consumption, and flickering lights (photosensitivity) are common precipitants.

Question 14: A 9-month-old previously healthy infant presented with a 12-hour history of fever, rhinorrhea, and a single episode of generalized tonic-clonic seizure lasting 34 minutes. The infant was brought to the emergency department within 1 hour and was observed to be alert, active, and playful after the fever subsided. What is the most probable diagnosis in this infant?

A. Febrile convulsions (Correct Answer)
B. Transient myoclonic epilepsy
C. Hypocalcemia
D. Hypernatremia

Explanation: ### Explanation **Correct Answer: A. Febrile convulsions** The clinical presentation is classic for a **Complex Febrile Seizure**. Febrile convulsions occur in children aged 6 months to 5 years in the presence of fever, without evidence of intracranial infection or a defined metabolic cause. While a "Simple" febrile seizure lasts <15 minutes and is generalized, a **Complex** febrile seizure (as seen here) is defined by a duration >15 minutes, focal features, or recurrence within 24 hours. Despite the 34-minute duration (status epilepticus), the infant’s rapid return to an alert, active, and playful state once the fever subsided is the hallmark of febrile seizures, distinguishing it from more serious conditions like meningitis or encephalitis. **Why Incorrect Options are Wrong:** * **B. Transient Myoclonic Epilepsy:** This typically presents with brief, sudden myoclonic jerks in infancy without fever. It does not present as a prolonged tonic-clonic episode triggered by pyrexia. * **C. Hypocalcemia:** While it can cause seizures in infants, it usually presents with jitteriness, tetany, or laryngospasm. It is not specifically triggered by fever, and the infant would likely not be "alert and playful" without metabolic correction. * **D. Hypernatremia:** This usually occurs in the context of severe dehydration (e.g., diarrhea). Seizures occur during rapid shifts in osmolarity; however, the presence of rhinorrhea and fever points more strongly toward a viral prodrome triggering a febrile seizure. **NEET-PG High-Yield Pearls:** * **Age range:** 6 months to 5 years (Peak: 18 months). * **Simple vs. Complex:** Simple is <15 mins, generalized, and occurs once in 24 hours. Complex is >15 mins, focal, or multiple episodes in 24 hours. * **Management:** Most are self-limiting. For active seizures >5 mins, **IV Lorazepam** (0.1 mg/kg) or **Rectal Diazepam** (0.5 mg/kg) is the drug of choice. * **Prognosis:** Excellent; however, the risk of recurrence is ~30%. Risk of future epilepsy is low (~1-2% for simple, slightly higher for complex).

Question 15: Which of the following statements is true regarding Duchenne muscular dystrophy?

A. It is autosomal dominant.
B. It has an onset in the second decade of life.
C. There is a universal increase in creatine kinase. (Correct Answer)
D. Cardiac muscle fibers are normal.

Explanation: **Explanation:** **Duchenne Muscular Dystrophy (DMD)** is the most common and severe form of muscular dystrophy, caused by a mutation in the *DMD* gene on the X chromosome, leading to a complete absence of the protein **dystrophin**. 1. **Why Option C is Correct:** Dystrophin acts as a mechanical stabilizer for the sarcolemma (muscle cell membrane). Its absence leads to membrane instability, resulting in chronic muscle fiber necrosis and leakage of intracellular enzymes into the blood. Consequently, **Serum Creatine Kinase (CK)** levels are universally elevated (often 50–100 times the upper limit of normal) from birth, even before clinical symptoms appear. 2. **Why Other Options are Incorrect:** * **Option A:** DMD follows an **X-linked recessive** inheritance pattern, not autosomal dominant. It primarily affects males. * **Option B:** The onset is typically in **early childhood (3–5 years)**. Patients usually present with frequent falls, difficulty climbing stairs, and a positive Gower’s sign. Onset in the second decade is characteristic of *Becker Muscular Dystrophy*, which is a milder form. * **Option D:** Cardiac involvement is nearly universal. Absence of dystrophin leads to **Dilated Cardiomyopathy** and arrhythmias, which are major causes of mortality. **High-Yield Clinical Pearls for NEET-PG:** * **Gower’s Sign:** Use of hands to "climb up" one's own body to stand from a supine position due to proximal muscle weakness. * **Pseudohypertrophy:** The calves appear large but are actually composed of fat and connective tissue (fibrosis) replacing lost muscle. * **Diagnosis:** Gold standard is **Genetic Testing** (Multiplex PCR for deletions). Muscle biopsy shows absent dystrophin staining. * **Treatment:** Glucocorticoids (e.g., Prednisolone/Deflazacort) are the mainstay to prolong ambulation and respiratory function.

Question 16: What is seen in the knee jerk reflex in scurvy?

A. Exaggerated
B. Decreased
C. May be normal initially, later increases
D. May be normal initially, later decreases (Correct Answer)

Explanation: **Explanation:** In **Scurvy (Vitamin C deficiency)**, the primary pathology involves defective collagen synthesis, leading to capillary fragility and subperiosteal hemorrhages. The changes in the knee jerk reflex are not due to primary neurological damage, but rather due to the **mechanical and painful complications** of the disease. 1. **Why Option D is correct:** Initially, the reflexes are normal because the nerves and muscles are intact. However, as the disease progresses, massive **subperiosteal hemorrhages** occur, particularly at the lower end of the femur and upper end of the tibia. This leads to intense pain and exquisite tenderness (pseudoparalysis). Additionally, intramuscular bleeds and subsequent fibrosis or disuse atrophy of the quadriceps muscle can occur. Consequently, the elicitation of the knee jerk becomes difficult or the response becomes **decreased/diminished** due to muscle weakness and the physical restriction caused by pain and hematomas. 2. **Why other options are incorrect:** * **Exaggerated (A):** This indicates an Upper Motor Neuron (UMN) lesion. Scurvy does not affect the pyramidal tracts. * **Decreased (B):** While reflexes do decrease, they are not decreased from the onset; the "initially normal" phase is a key clinical distinction. * **Increases later (C):** There is no physiological mechanism in Vitamin C deficiency that would lead to hyperreflexia. **Clinical Pearls for NEET-PG:** * **Earliest sign of Scurvy:** Follicular hyperkeratosis. * **Radiological signs:** Wimberger’s ring sign (epiphysis), Frankel’s line (white line of Frenkel), and Pelkan spur. * **Position:** "Frog-leg position" due to extreme pain on movement (pseudoparalysis). * **Gums:** Swollen, spongy, and bleeding gums (only seen if teeth have erupted).

Question 17: All are causes of infantile tremor syndrome EXCEPT:

A. Malnutrition
B. Magnesium deficiency
C. Infections
D. Vitamin B1 deficiency (Correct Answer)

Explanation: **Explanation:** **Infantile Tremor Syndrome (ITS)** is a clinical disorder characterized by a classic tetrad of symptoms: **tremors, anemia, pigmentary skin changes, and regression of mental development.** It is primarily seen in infants aged 6 to 24 months who are exclusively breastfed by malnourished, vegetarian mothers. **Why Vitamin B1 deficiency is the correct answer:** The primary nutritional etiology of ITS is **Vitamin B12 (Cobalamin) deficiency**, not Vitamin B1 (Thiamine). Vitamin B12 is essential for myelin synthesis and DNA maturation; its deficiency leads to the characteristic neurological symptoms and megaloblastic anemia seen in ITS. Vitamin B1 deficiency causes Beriberi, which presents differently (congestive heart failure or peripheral neuropathy). **Analysis of other options:** * **Malnutrition:** This is the foundational risk factor. Most affected infants have Protein-Energy Malnutrition (PEM) and are born to mothers with poor nutritional status. * **Magnesium deficiency:** Hypomagnesemia has been documented in several cases of ITS and is thought to contribute to the irritability and tremors observed in these infants. * **Infections:** Acute infections (respiratory or gastrointestinal) often act as a "trigger" that precipitates the onset of tremors in a nutritionally vulnerable child. **NEET-PG High-Yield Pearls:** * **Classic Triad:** Tremors, skin hyperpigmentation (knuckle pigmentation), and mental regression. * **The "Plump Baby" Appearance:** Infants often appear well-nourished or "chubby" due to edema, despite underlying malnutrition. * **Hematology:** Peripheral smear typically shows **Megaloblastic Anemia**. * **Treatment:** Vitamin B12 supplementation is the mainstay of therapy. Tremors may transiently worsen after starting treatment (phenobarbital can be used for control).

Question 18: Which of the following is NOT typically seen in metachromatic leukodystrophy?

A. Mental retardation
B. Optic atrophy
C. Decerebrate posture
D. Exaggerated tendon reflexes (Correct Answer)

Explanation: **Explanation:** Metachromatic Leukodystrophy (MLD) is an autosomal recessive lysosomal storage disorder caused by a deficiency of the enzyme **Arylsulfatase A**. This leads to the accumulation of sulfatides, which are toxic to the myelin-producing cells of both the **Central Nervous System (CNS)** and the **Peripheral Nervous System (PNS)**. **Why "Exaggerated tendon reflexes" is the correct answer:** In MLD, there is significant peripheral nerve involvement (demyelinating polyneuropathy). In any neurological condition where the peripheral nerves are affected, the deep tendon reflexes are typically **diminished or absent (hyporeflexia/areflexia)**. Therefore, exaggerated reflexes (hyperreflexia), which indicate an isolated Upper Motor Neuron (UMN) lesion, are not typically seen in MLD. **Analysis of Incorrect Options:** * **Mental retardation:** As a leukodystrophy, MLD causes progressive white matter destruction leading to cognitive decline, loss of speech, and intellectual disability. * **Optic atrophy:** Demyelination of the optic nerve is a common feature in the later stages of the disease, leading to visual loss. * **Decerebrate posture:** As the disease progresses to advanced stages, extensive cortical and subcortical destruction leads to motor posturing, including decerebrate (extensor) rigidity. **NEET-PG High-Yield Pearls for MLD:** * **Enzyme Deficiency:** Arylsulfatase A. * **Accumulated Substance:** Cerebroside sulfate (sulfatide). * **Diagnosis:** Gold standard is measuring enzyme activity in leukocytes; MRI shows "tigroid" pattern of demyelination. * **Histology:** Sural nerve biopsy shows metachromasia (nerves stain brown/purple with toluidine blue). * **Clinical Triad:** Progressive motor symptoms + Cognitive decline + Peripheral neuropathy (absent reflexes).

Question 19: A 7-day-old neonate presents with recurrent seizures. On examination, tachycardia and S3 gallop are noted, along with a bruit in the anterior fontanelle. Blood investigations are normal. Neurosonogram shows a hypoechoic lesion. What is the diagnosis?

A. Arachnoid cyst
B. Vein of Galen malformation (Correct Answer)
C. Dilated ventricles
D. Intraventricular hemorrhage

Explanation: **Explanation:** The clinical presentation of a neonate with seizures, high-output heart failure (tachycardia, S3 gallop), and a cranial bruit is classic for a **Vein of Galen Malformation (VGAM)**. **Why the correct answer is right:** VGAM is an arteriovenous malformation where cerebral arteries drain directly into an embryonic precursor of the Vein of Galen. This creates a massive **left-to-right shunt**, leading to high-output cardiac failure (the most common presentation in neonates). The rapid blood flow creates a **bruit** audible over the anterior fontanelle. On neurosonogram, it appears as a midline **hypoechoic/anechoic cystic mass** posterior to the third ventricle, which shows turbulent flow on Doppler. **Why the incorrect options are wrong:** * **Arachnoid cyst:** While hypoechoic on ultrasound, these are static fluid collections that do not cause heart failure or bruits. * **Dilated ventricles (Hydrocephalus):** Though VGAM can cause secondary hydrocephalus (due to venous hypertension or aqueductal compression), hydrocephalus alone does not explain the S3 gallop or cranial bruit. * **Intraventricular hemorrhage (IVH):** Typically presents in preterm neonates with a drop in hematocrit and bulging fontanelle; it does not cause high-output heart failure or a bruit. **High-Yield Pearls for NEET-PG:** * **Triad of VGAM:** High-output heart failure + Cranial bruit + Hydrocephalus. * **Best Initial Investigation:** Neurosonogram with Color Doppler. * **Gold Standard Investigation:** Digital Subtraction Angiography (DSA). * **Management:** Endovascular embolization (usually delayed until 5–6 months of age if the child is stable). * **Differential:** Always consider VGAM in a neonate with unexplained heart failure and a normal structurally intact heart.

Question 20: Gower's maneuver is classically seen in:

A. Cerebral palsy
B. Friedreich's ataxia
C. Duchenne muscular dystrophy (Correct Answer)
D. Parkinsonism

Explanation: **Explanation:** **Gower’s maneuver** is a clinical sign where a patient has to use their hands and arms to "climb up" their own body to transition from a sitting or supine position to a standing position. **1. Why Duchenne Muscular Dystrophy (DMD) is correct:** DMD is an X-linked recessive disorder caused by a deficiency of the protein **dystrophin**. This leads to progressive muscle degeneration. Gower’s maneuver specifically indicates **proximal muscle weakness**, particularly of the pelvic girdle and hip extensors (gluteus maximus). Because the patient cannot extend the hips effectively, they use their upper extremities to compensate for the lack of strength in the lower limbs. **2. Why the other options are incorrect:** * **Cerebral Palsy:** This is a non-progressive upper motor neuron (UMN) disorder characterized by spasticity and movement coordination issues, rather than the specific proximal muscle wasting seen in DMD. * **Friedreich’s Ataxia:** This is a spinocerebellar degeneration presenting with gait ataxia, loss of deep tendon reflexes, and sensory loss. While it affects mobility, it does not typically present with the "climbing up" sign of proximal weakness. * **Parkinsonism:** This is an extrapyramidal disorder characterized by tremors, rigidity, and bradykinesia. Patients have difficulty initiating movement (festinating gait), but not the specific proximal weakness required for Gower's sign. **Clinical Pearls for NEET-PG:** * **Pseudohypertrophy:** In DMD, the calves appear large but are actually weak because muscle is replaced by fat and connective tissue. * **Becker Muscular Dystrophy:** A milder form of dystrophinopathy; Gower’s sign may appear later in childhood compared to DMD (which usually presents by age 3–5). * **Laboratory Gold Standard:** Elevated **Serum Creatine Kinase (CK)** levels (often 10–100x normal) are the initial screening test; Genetic testing for the *DMD* gene is the definitive diagnosis.

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Seizure Disorders and Epilepsy

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Febrile Seizures

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Headache Disorders

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Cerebral Palsy

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Neural Tube Defects

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Neuromuscular Disorders

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Neurodegenerative Disorders

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CNS Infections

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Hydrocephalus

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Movement Disorders

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Traumatic Brain Injury

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Neuroimaging in Pediatrics

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Neurology — MCQs

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