Neurology Practice Questions

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 statements regarding a medulloblastoma in children is true?

It is mostly infratentorial.. **Explanation:** Medulloblastoma is a highly malignant (WHO Grade 4) primitive neuroectodermal tumor and is the **most common malignant brain tumor in children**. **1. Why Option A is Correct:** Medulloblastomas characteristically arise from the **roof of the fourth ventricle** (vermis of the cerebellum). Since the cerebellum and fourth ventricle are located below the tentorium cerebelli, the tumor is **infratentorial**. In children, approximately 60-70% of all primary brain tumors are infratentorial, with medulloblastoma being a classic example. **2. Why the other options are Incorrect:** * **Option B & D:** Because these tumors arise in the posterior fossa, they frequently compress the fourth ventricle. This leads to obstructive **hydrocephalus** and increased intracranial pressure (ICP). Consequently, **papilledema** (a sign of raised ICP) is a very common clinical finding, not rare. * **Option C:** While it is the most common *malignant* brain tumor, the most common brain tumor overall in children is the **Pilocytic Astrocytoma** (which is typically benign/Grade 1). **High-Yield Clinical Pearls for NEET-PG:** * **Homer-Wright Rosettes:** A classic histopathological finding (pseudorosettes). * **Drop Metastasis:** Medulloblastoma has a high propensity to spread via CSF; therefore, imaging of the entire craniospinal axis is mandatory. * **Clinical Presentation:** Presents with truncal ataxia (due to vermis involvement) and signs of raised ICP (morning headaches, vomiting). * **Genetic Subtypes:** WNT (best prognosis), SHH, Group 3 (worst prognosis), and Group 4.

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: Periventricular leukomalacia most commonly causes which of the following conditions?

Spastic diplegia. **Explanation:** **Periventricular Leukomalacia (PVL)** is the most common ischemic brain injury in premature infants. It is characterized by necrosis of the white matter adjacent to the external angles of the lateral ventricles. **Why Spastic Diplegia is the Correct Answer:** The white matter in the periventricular region contains the **descending corticospinal tract fibers**. Specifically, the fibers supplying the lower extremities are located closest to the ventricles (medial position), while fibers for the upper extremities and face are located more laterally. Because PVL occurs in this specific periventricular zone, the fibers destined for the legs are most vulnerable to damage. This results in **Spastic Diplegia**, where motor impairment is significantly more pronounced in the legs than in the arms. **Analysis of Incorrect Options:** * **A. Spastic Quadriplegia:** This usually results from more extensive, diffuse brain injury or severe multicystic encephalomalacia affecting both the medial and lateral motor fibers. * **B. Hypotonic Cerebral Palsy:** This is a rare form of CP often associated with genetic syndromes or cerebellar injury, rather than periventricular white matter damage. * **D. Dyskinetic Cerebral Palsy:** This is typically caused by damage to the **basal ganglia** (specifically the bilirubin-induced injury in kernicterus or profound perinatal asphyxia), not periventricular white matter. **High-Yield Clinical Pearls for NEET-PG:** * **Strongest Risk Factor:** Prematurity (especially <32 weeks gestation). * **Imaging of Choice:** Cranial Ultrasonography (initial) or MRI (most sensitive for identifying "flaring" or cystic changes). * **Pathogenesis:** Vulnerability of "pre-oligodendrocytes" to oxidative stress and ischemia in the watershed zones. * **Classic Presentation:** "Scissoring gait" due to increased tone in the adductor muscles of the legs.

Q: What is true about Erb's Palsy?

C5, C6 roots affected. **Erb’s Palsy** is a common brachial plexus injury occurring at **Erb’s point** (the junction of C5 and C6 nerve roots). It typically results from birth trauma involving lateral traction on the neck during a breech delivery or shoulder dystocia. ### **Explanation of Options:** * **Correct Answer (C):** Erb’s palsy specifically involves the **C5 and C6 nerve roots**. These roots supply the muscles of the shoulder and upper arm, leading to the characteristic clinical presentation. * **Option A (Incorrect):** The limb is held in **Adduction** and Internal rotation. Abduction is lost because of paralysis of the deltoid and supraspinatus. * **Option B (Incorrect):** **Claw hand** is a feature of **Klumpke’s Palsy**, which involves the lower roots (**C8-T1**). * **Option D (Incorrect):** **Pronation** is seen, not supination. The loss of the biceps brachii and supinator muscles causes the forearm to remain fixed in pronation. ### **Clinical Presentation (The "Policeman’s Tip" or "Waiter’s Tip" Position):** The affected limb hangs by the side with: 1. **Arm:** Adducted and Internally rotated (Loss of Abductors/External rotators). 2. **Forearm:** Extended and Pronated (Loss of Biceps/Brachialis). 3. **Wrist:** Flexed. ### **High-Yield Pearls for NEET-PG:** * **Moro Reflex:** Asymmetrical or absent on the affected side. * **Grasp Reflex:** **Present** (since C8-T1 are intact), distinguishing it from Klumpke’s. * **Associated Nerve Injury:** If C4 is involved, **Phrenic nerve palsy** (diaphragmatic paralysis) may occur. * **Muscle Involvement:** Primarily Deltoid, Biceps, Brachialis, and Brachioradialis.

Q: A patient presents with intractable convulsions, mental defect, and a facial nevus. What is the most likely diagnosis?

Sturge-Weber syndrome. ### Explanation The clinical triad of **intractable convulsions (seizures)**, **mental retardation**, and a **facial nevus** (specifically a Port-wine stain) is the classic presentation of **Sturge-Weber Syndrome (SWS)**, also known as Encephalotrigeminal Angiomatosis. **1. Why Sturge-Weber Syndrome is Correct:** SWS is a neurocutaneous disorder characterized by a congenital capillary malformation. * **Facial Nevus:** A Port-wine stain (Nevus Flammeus) usually follows the distribution of the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve. * **Neurological involvement:** Leptomeningeal angiomas (usually ipsilateral to the skin lesion) lead to cortical ischemia, resulting in refractory seizures and progressive intellectual disability. * **Imaging:** Skull X-rays or CT scans often show "tram-track" calcifications in the cerebral cortex. **2. Why the Other Options are Incorrect:** * **Tuberous Sclerosis:** Presents with the "Vogt triad" of seizures, mental retardation, and **Adenoma Sebaceum** (angiofibromas). It does not feature a flat facial nevus. Other signs include Ash-leaf spots and Shagreen patches. * **Von-Hippel-Lindau (VHL) Disease:** Characterized by hemangioblastomas of the cerebellum and retina, and renal cell carcinoma. It lacks the classic facial nevus and early-onset intractable seizures. * **Von Recklinghausen Disease (NF-1):** Characterized by Café-au-lait spots, Lisch nodules, and neurofibromas. It does not present with a Port-wine stain. **Clinical Pearls for NEET-PG:** * **Inheritance:** SWS is **sporadic** (not inherited), caused by a somatic mutation in the *GNAQ* gene. * **Ocular finding:** Glaucoma is the most common ocular manifestation (present in ~50% of cases). * **Radiology:** Look for the keyword **"Tram-track calcifications"** on CT/X-ray. * **Treatment:** Focuses on seizure control and managing intraocular pressure; Port-wine stains are treated with Pulse Dye Laser.

Q: A 4-month-old child presents with excessive irritability and crying, unexplained hyperpyrexia, vomiting, and difficulty feeding for the last 15 days. On admission, the child has rigidity and visual inattentiveness. A CT scan of the brain shows specific findings. What is the probable diagnosis?

Krabbe disease. ***Krabbe disease*** - Classic **infantile onset** at 3-6 months with **excessive irritability**, **hyperpyrexia**, and **feeding difficulties** strongly suggests Krabbe disease. - CT shows characteristic **hyperdense thalami** and **basal ganglia** with **periventricular white matter changes** and **rigidity** with **visual inattentiveness** are hallmark features. *Alexander disease* - Typically presents with **macrocephaly** and **developmental delay** rather than acute irritability and fever. - CT shows **frontal lobe predominance** with **white matter changes** and **Rosenthal fibers** on histology, not the thalamic hyperdensity seen here. *Metachromatic leukodystrophy* - Usually presents **later** (18-24 months) with **progressive motor regression** and **gait abnormalities**. - CT shows **symmetric periventricular demyelination** without the **hyperdense thalami** characteristic of Krabbe disease. *Adrenoleukodystrophy* - Typically affects **school-age boys** (4-8 years) with **behavioral changes** and **adrenal insufficiency**. - CT demonstrates **occipital-parietal white matter lesions** with **contrast enhancement**, distinct from the basal ganglia involvement in this case.

Question 1

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 2

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 3

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 4

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 5

Which of the following statements regarding a medulloblastoma in children is true?

Accepted Answer

It is mostly infratentorial.

Answer

Papilledema is rare.

Answer

It is the most common tumor in children.

Answer

Hydrocephalus is rare.

Question 6

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

Accepted Answer

Carbamazepine

Answer

Sodium valproate

Answer

Phenobarbitone

Answer

Diazepam

Question 7

Periventricular leukomalacia most commonly causes which of the following conditions?

Accepted Answer

Spastic diplegia

Answer

Spastic quadriplegia

Answer

Hypotonic cerebral palsy

Answer

Dyskinetic cerebral palsy

Question 8

What is true about Erb's Palsy?

Accepted Answer

C5, C6 roots affected

Answer

Abduction and Internal rotation

Answer

Claw hand is seen

Answer

Supination seen

Question 9

A patient presents with intractable convulsions, mental defect, and a facial nevus. What is the most likely diagnosis?

Accepted Answer

Sturge-Weber syndrome

Answer

Tuberous sclerosis

Answer

Von-Hippel-Lindau disease

Answer

Von Recklinghausen disease

Question 10

A 4-month-old child presents with excessive irritability and crying, unexplained hyperpyrexia, vomiting, and difficulty feeding for the last 15 days. On admission, the child has rigidity and visual inattentiveness. A CT scan of the brain shows specific findings. What is the probable diagnosis?

Accepted Answer

Krabbe disease

Answer

Alexander disease

Answer

Metachromatic leukodystrophy

Answer

Adrenoleukodystrophy

Neurology — MCQs

Neurology — MCQs

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