Neurology Practice Questions

Q: All of the following are TRUE regarding Panayiotopoulos syndrome, EXCEPT:

Lifelong phenobarbitone is usually needed.. **Explanation** Panayiotopoulos syndrome (Early-onset Benign Occipital Epilepsy) is a common, benign childhood idiopathic focal epilepsy. The correct answer is **D** because this syndrome has an excellent prognosis; most children outgrow the condition by age 12, and long-term or lifelong antiepileptic therapy is almost never required. **Analysis of Options:** * **Option A (Focal Seizures):** This is **True**. Despite the prominent autonomic features, the syndrome is classified as a focal epilepsy. Seizures often involve eye deviation and may progress to hemiclonic or generalized tonic-clonic activity. * **Option B (Autonomic Symptoms):** This is **True**. Autonomic manifestations are the hallmark. **Vomiting** (ictal emesis) is the most common symptom, occurring in 70-80% of cases. Other signs include pallor, mydriasis, sweating, and incontinence. * **Option C (Prolonged Seizures):** This is **True**. A unique feature of this syndrome is that seizures are often prolonged, lasting >30 minutes (autonomic status epilepticus). Remarkably, these prolonged events do not cause permanent brain damage or neurological deficits. * **Option D (Lifelong Phenobarbitone):** This is **False**. Most patients experience very few seizures (often only 2-5 in a lifetime). Prophylactic medication is usually not indicated unless seizures are frequent or distressing. If treated, medication is typically tapered off after a few seizure-free years. **High-Yield Facts for NEET-PG:** * **Age of Onset:** Typically 3–6 years. * **EEG Finding:** High-amplitude spikes, often in the **occipital** regions, though they can be multifocal or shifting. * **Clinical Pearl:** If a child presents with nocturnal vomiting followed by eye deviation and a long duration of altered consciousness, think Panayiotopoulos syndrome. * **Prognosis:** Remission usually occurs within 2–3 years of onset.

Q: A newborn baby presents with a lumbar mass. What is the most likely diagnosis?

Lumbar meningomyelocele. ***Lumbar meningomyelocele*** - **Most common form** of open neural tube defect presenting as a **cystic mass** in the lumbar region containing **meninges, cerebrospinal fluid, and neural tissue**. - Results from failure of **neural tube closure** during embryogenesis (days 22-28), leading to herniation of spinal cord contents through a **vertebral defect**. *Myelocele* - Represents an **open neural tube defect** where **neural tissue is exposed** without a protective sac covering. - Less common than meningomyelocele and presents as **flat, non-cystic lesion** rather than a protruding mass. *Spina bifida* - This is a **broad umbrella term** encompassing all neural tube defects affecting the spine, including both **open and closed** forms. - **Too general** as a diagnosis since it includes spina bifida occulta (closed, often asymptomatic) and doesn't specify the **cystic mass** presentation described. *None of the above* - **Lumbar meningomyelocele** is indeed the most specific and accurate diagnosis for a newborn presenting with a **lumbar mass**. - Other causes of lumbar masses in newborns (like **teratomas** or **lipomas**) are much less common than neural tube defects.

Q: An 8-year-old boy presents with intellectual deterioration and myoclonus. What is the most likely diagnosis?

Subacute sclerosing panencephalitis (SSPE). **Explanation:** The clinical presentation of an 8-year-old boy with **intellectual deterioration** (cognitive decline) and **myoclonus** is a classic description of **Subacute Sclerosing Panencephalitis (SSPE)**. **1. Why SSPE is the Correct Answer:** SSPE is a progressive, fatal neurodegenerative disease caused by a persistent infection with a mutant **Measles virus**. It typically occurs 5–10 years after the initial measles infection. The disease progresses through four stages: * **Stage 1:** Behavioral changes and intellectual decline. * **Stage 2:** Motor symptoms, most characteristically **periodic myoclonus** (brief, sudden muscle jerks). * **Stage 3:** Extrapyramidal symptoms and dementia. * **Stage 4:** Vegetative state and death. **2. Why Other Options are Incorrect:** * **CJD and GSS syndrome:** While these are Prion diseases that cause dementia and myoclonus, they are extremely rare in the pediatric age group. CJD typically affects adults aged 50–70. * **Kuru:** This is a prion disease historically associated with ritualistic cannibalism in Papua New Guinea; it is now virtually extinct and does not fit the typical demographic or presentation. **3. NEET-PG High-Yield Pearls for SSPE:** * **EEG Finding:** Characterized by **periodic, high-voltage slow-wave complexes** (Radermecker complexes). * **CSF Analysis:** Shows significantly **elevated anti-measles antibody titers** and oligoclonal bands (but normal protein/pressure). * **MRI:** Shows diffuse white matter hyperintensities. * **Prevention:** The most effective "treatment" is prevention via the **Measles (MMR) vaccine**.

Q: A newborn is admitted for high-output cardiac failure after presenting with seizures. CT shows dilated lateral ventricles suggestive of hydrocephalus. What is the most probable diagnosis?

Vein of Galen Malformation. **Explanation:** The clinical triad of **high-output cardiac failure (HOCF)**, **seizures**, and **hydrocephalus** in a newborn is classic for a **Vein of Galen Malformation (VOGM)**. **Why B is correct:** VOGM is an arteriovenous malformation where cerebral arteries drain directly into a dilated embryonic precursor of the Vein of Galen. This creates a massive **left-to-right shunt**. The high volume of blood returning to the right heart leads to volume overload and HOCF. Hydrocephalus occurs due to the dilated venous sac compressing the Aqueduct of Sylvius, obstructing CSF flow. Seizures result from "steal phenomenon" (ischemia to surrounding brain tissue) or venous hypertension. **Why the other options are incorrect:** * **A. Cavernous Malformation:** These are low-flow vascular lesions. They typically present with seizures or focal deficits in older children/adults but do not cause systemic heart failure. * **C. Moyamoya Disease:** This is a chronic occlusive cerebrovascular disease characterized by "puff of smoke" collateral vessels. It typically presents with strokes or TIAs in older children, not HOCF in neonates. * **D. Central Venous Angioma:** These are common, benign venous anomalies (Developmental Venous Anomalies) that are usually asymptomatic and do not cause high-flow shunting. **NEET-PG High-Yield Pearls:** * **Gold Standard Diagnosis:** Digital Subtraction Angiography (DSA). * **Initial Screening:** Cranial Ultrasonography (Doppler shows turbulent flow). * **Management:** Endovascular embolization is the treatment of choice (usually delayed until 5-6 months if the child is stable). * **Clinical Sign:** A continuous bruit may be heard over the anterior fontanelle.

Q: Which of the following is not used in the prophylaxis of febrile seizures?

Carbamazepine. **Explanation:** The management of febrile seizures focuses on distinguishing between drugs that are effective for prophylaxis and those that are ineffective or even counterproductive. **1. Why Carbamazepine is the Correct Answer:** **Carbamazepine** (and Phenytoin) are notoriously **ineffective** in preventing the recurrence of febrile seizures. Clinical studies have shown that these drugs do not reduce the risk of further febrile episodes. Furthermore, in some pediatric seizure syndromes (like Dravet syndrome, which can present with febrile seizures), Carbamazepine can actually exacerbate seizures. **2. Analysis of Incorrect Options:** * **Diazepam (Option D):** This is the drug of choice for **intermittent prophylaxis**. It is administered only during the onset of a fever (0.3 mg/kg every 8 hours) to prevent a seizure from occurring. * **Phenobarbitone (Option C) & Sodium Valproate (Option A):** Both are effective for **continuous prophylaxis**. While they significantly reduce the risk of recurrence, they are rarely used today due to their side-effect profiles (behavioral issues with Phenobarbitone and hepatotoxicity/weight gain with Valproate). **3. Clinical Pearls for NEET-PG:** * **Definition:** Febrile seizures occur between **6 months and 5 years** of age associated with fever, in the absence of CNS infection. * **Prophylaxis Indications:** Generally not recommended for "Simple" febrile seizures. It is considered only for "Complex" seizures (prolonged >15 mins, focal, or recurrent within 24 hours) or significant parental anxiety. * **Drug of Choice for Acute Episode:** Per-rectal or Intravenous Diazepam (or Midazolam). * **Gold Standard for Intermittent Prophylaxis:** Oral/Rectal Diazepam.

Q: A 1-day-old male baby delivered by LSCS has swelling over the back in the midline. Which of the following is the most likely cause?

Meningomyelocele. **Explanation:** The clinical presentation of a midline swelling over the back in a newborn is a classic sign of a **Neural Tube Defect (NTD)**. **Why Meningomyelocele is correct:** Meningomyelocele is the most common and severe form of open spinal dysraphism. It involves the herniation of both the **meninges and the spinal cord/nerve roots** through a vertebral defect. It typically presents at birth as a fluid-filled sac, often lacking skin cover (placode exposed), making it the most likely diagnosis for a visible midline swelling in a neonate. **Analysis of Incorrect Options:** * **A. Spinal dysraphism:** This is a broad "umbrella term" that encompasses all forms of spinal malformations (including spina bifida occulta and cystica). While technically true, "Meningomyelocele" is the specific clinical diagnosis for the described lesion. * **C. Lipomyelomeningocele:** This is a form of closed spinal dysraphism where a lipoma is attached to the spinal cord. While it presents as a swelling, it is usually skin-covered and less common than a classic meningomyelocele in general neonatal presentations. * **D. Dermoid cyst:** These are slow-growing tumors that may occur along the neuraxis but are rarely the primary cause of a prominent midline swelling noted immediately at birth compared to NTDs. **High-Yield Clinical Pearls for NEET-PG:** * **Prevention:** Periconceptional **Folic Acid (400 mcg/day)** reduces the risk of NTDs by 70%. For women with a previous affected child, the dose is **4 mg/day**. * **Screening:** Elevated **Maternal Serum Alpha-Fetoprotein (MSAFP)** at 15-20 weeks is a screening marker; diagnosis is confirmed via "Lemon sign" or "Banana sign" on ultrasound. * **Association:** Meningomyelocele is frequently associated with **Arnold-Chiari Malformation Type II** and hydrocephalus.

Question 1

All of the following are TRUE regarding Panayiotopoulos syndrome, EXCEPT:

Accepted Answer

Lifelong phenobarbitone is usually needed.

Answer

It includes focal seizures.

Answer

Autonomic symptoms may predominate.

Answer

Seizures may last hours without causing permanent brain damage.

Question 2

Which of the following is NOT true about migraine in children?

Accepted Answer

Daily headache with vomiting upon waking in the morning, improving when the child is out of bed.

Answer

Preceding aura is common.

Answer

Recurrent headache lasting 2-72 hours per episode.

Answer

Headache is often bilateral.

Question 3

A newborn baby presents with a lumbar mass. What is the most likely diagnosis?

Accepted Answer

Lumbar meningomyelocele

Answer

Myelocele

Answer

Spina bifida

Answer

None of the above

Question 4

An 8-year-old boy presents with intellectual deterioration and myoclonus. What is the most likely diagnosis?

Accepted Answer

Subacute sclerosing panencephalitis (SSPE)

Answer

Gerstmann-Sträussler-Scheinker (GSS) syndrome

Answer

Kuru

Answer

Creutzfeldt-Jakob disease (CJD)

Question 5

A patient diagnosed with febrile convulsions is in the pediatric emergency. Which of the following can be used for the treatment of this patient?

Accepted Answer

Rectal diazepam

Answer

Intramuscular phenobarbitone

Answer

Intravenous phenytoin

Answer

Oral sodium valproate

Question 6

A 5-year-old male presented with a chief complaint of difficulty in climbing stairs and getting up from a sitting position. There was a history of a maternal uncle having the same illness. On examination, there was pseudohypertrophy of the calf muscle. A muscle biopsy was performed. What is your diagnosis?

Accepted Answer

Duchenne muscular dystrophy

Answer

Myotonic dystrophy

Answer

Facioscapulohumeral muscular dystrophy

Answer

Nemaline myopathy

Question 7

What is the most common clinical sign or symptom for the diagnosis of Tuberous sclerosis?

Accepted Answer

An infant with infantile spasms, a hypsarrhythmic EEG pattern, and ash-leaf depigmentation on her back

Answer

A 6-month-old child with blindness on the same side as a large facial lesion

Answer

An 18-year-old patient with a history of fractures and optic gliomas, who now has developed a malignant schwannoma

Answer

A 2-year-old child with multiple episodes of skin infection and failure to thrive

Question 8

A newborn is admitted for high-output cardiac failure after presenting with seizures. CT shows dilated lateral ventricles suggestive of hydrocephalus. What is the most probable diagnosis?

Accepted Answer

Vein of Galen Malformation

Answer

Cavernous Malformation

Answer

Moyamoya Disease

Answer

Central Venous angioma

Question 9

Which of the following is not used in the prophylaxis of febrile seizures?

Accepted Answer

Carbamazepine

Answer

Sodium valproate

Answer

Phenobarbitone

Answer

Diazepam

Question 10

A 1-day-old male baby delivered by LSCS has swelling over the back in the midline. Which of the following is the most likely cause?

Accepted Answer

Meningomyelocele

Answer

Spinal dysraphism

Answer

Lipomyelomeningocele

Answer

Dermoid cyst

Neurology — MCQs

Neurology — MCQs

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