Neurology — MCQs
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Hung up reflex is seen in?
What is the best prophylaxis for a 4-year-old male child experiencing febrile seizures?
An 8-year-old boy presents with behavioral changes, intellectual deterioration, and "laziness." Over several months, he develops increasing clumsiness and periodic, involuntary, jerky movements every 3 to 6 seconds. Visual deterioration is apparent on visual field testing, and optic atrophy is evident on funduscopic examination. Cerebrospinal fluid studies show oligoclonal bands of IgG on electrophoresis but no significant pleocytosis. The electroencephalogram reveals periodic discharges synchronous with the myoclonus. Computed tomography (CT) of the head demonstrates low-density white matter lesions and cerebral atrophy. The boy dies at age 10. Prior infection with which of the following agents was most likely related to this patient's condition?
Which of the following is the most common cause of congenital hydrocephalus?
A 5-year-old boy presents with a 10-day history of fever, for which he received some medication. For the past 3 days, he has experienced anorexia and vomiting, and for the past day, altered sensorium. One day prior to presentation, the child had two episodes of seizures. On examination, the child is hemodynamically stable, with no pallor or icterus, and no meningeal signs. The liver is palpable 2 cm below the right costal margin. Blood glucose is 40 mg%, Hb is 11 g/dl, TLC is 8300, platelet count is 2.8 lac/mm3, and PT is 58/12 sec. What is the most likely diagnosis?
A 4-year-old child presented with symptoms of unsteady gait, tremors, frequent shivering, headaches, and dizziness for the past 6 months. There was a history of frequent chest infections and a few episodes of seizures in the past. The child was normal at birth. On examination, the child had speech, hearing, and visual impairment and discoloration of areas of skin exposed to sunlight. MRI brain with MR spectroscopy showed a choline peak. The gene responsible for the above condition is present on which of the following chromosomes?
A 7-year-old male patient presented with skin lesions on the face and the lumbosacral region. The patient also has a history of frequent seizures. Which of the following chromosomes is involved in the disease?
Which of the following disorders is NOT associated with the sign shown in the image?
A 7-year-old child presents with a 10-day history of fever, early morning headache, and vomiting, along with focal neurological deficits. A CT scan is performed. What is the management for this condition?
What is the investigation of choice for the diagnostic evaluation of hydrocephalus in a one-month-old child?
Neurology Indian Medical PG Practice Questions and MCQs
Question 151: Hung up reflex is seen in?
- A. Chorea (Correct Answer)
- B. Cerebral palsy
- C. Athetosis
- D. None of the above
Explanation: **Explanation:** The **"Hung-up reflex"** is a classic clinical sign of **Sydenham’s Chorea** (a major manifestation of Rheumatic Fever). It refers to a sustained or prolonged relaxation phase of a deep tendon reflex, most commonly observed in the knee jerk. 1. **Why Chorea is Correct:** In Chorea, there is a state of **hypotonia** combined with involuntary, jerky movements. When the patellar tendon is tapped, the initial contraction occurs, but the leg remains extended for a few seconds before returning to the neutral position. This occurs because an involuntary choreiform contraction of the quadriceps muscle superimposes on the normal reflex arc, "hanging" the leg in mid-air. 2. **Why other options are incorrect:** * **Cerebral Palsy (CP):** Depending on the type, CP usually presents with either spasticity (brisk reflexes) or dyskinesia. It does not typically feature the specific "hung-up" relaxation phase. * **Athetosis:** This involves slow, writhing, continuous movements. While it is a basal ganglia disorder like chorea, it does not manifest with the specific hung-up reflex phenomenon. **Clinical Pearls for NEET-PG:** * **Other signs of Chorea:** * **Milkmaid’s Grip:** Irregular contractions of hand muscles while shaking hands. * **Jack-in-the-box tongue:** Inability to keep the tongue protruded (Trombone tongue). * **Pronator Sign:** Turning out of the palms when arms are raised above the head. * **Pathophysiology:** Caused by molecular mimicry where antibodies against Group A Beta-hemolytic Streptococcus cross-react with the **Basal Ganglia** (specifically the caudate and subthalamic nuclei).
Question 152: What is the best prophylaxis for a 4-year-old male child experiencing febrile seizures?
- A. Paracetamol every 6 hours
- B. Paracetamol and diazepam
- C. Diazepam (Correct Answer)
- D. Phenobarbitone
Explanation: **Explanation:** The management of febrile seizures focuses on distinguishing between continuous and intermittent prophylaxis. For a child with simple febrile seizures, **intermittent prophylaxis** is the preferred approach. **Why Diazepam is correct:** Oral or rectal **Diazepam (0.3 mg/kg/dose)** is the drug of choice for intermittent prophylaxis. It is administered only during the onset of a febrile illness (when the temperature rises) for the first 48–72 hours. This strategy effectively reduces the risk of seizure recurrence during that specific febrile episode while avoiding the chronic side effects of daily anticonvulsants. **Analysis of Incorrect Options:** * **A & B (Paracetamol):** While antipyretics like Paracetamol improve the child's comfort, clinical studies have shown they **do not prevent** the occurrence or recurrence of a febrile seizure. They do not cross the blood-brain barrier to alter the seizure threshold. * **D (Phenobarbitone):** This was previously used for **continuous prophylaxis**. However, it is no longer recommended for simple febrile seizures due to its significant side effects, including hyperactivity, irritability, and potential cognitive impairment in developing children. **High-Yield Clinical Pearls for NEET-PG:** * **Simple Febrile Seizure:** Generalized, lasts <15 minutes, occurs once in 24 hours. * **Complex Febrile Seizure:** Focal, lasts >15 minutes, or recurs within 24 hours. * **Indication for Continuous Prophylaxis:** Only considered in cases of atypical/complex seizures or significant neurological deficits (Drug of choice: Sodium Valproate). * **Age Group:** Most common between 6 months and 5 years (Peak: 18 months). * **Prognosis:** Excellent; it does not typically lead to a decrease in IQ or academic performance.
Question 153: An 8-year-old boy presents with behavioral changes, intellectual deterioration, and "laziness." Over several months, he develops increasing clumsiness and periodic, involuntary, jerky movements every 3 to 6 seconds. Visual deterioration is apparent on visual field testing, and optic atrophy is evident on funduscopic examination. Cerebrospinal fluid studies show oligoclonal bands of IgG on electrophoresis but no significant pleocytosis. The electroencephalogram reveals periodic discharges synchronous with the myoclonus. Computed tomography (CT) of the head demonstrates low-density white matter lesions and cerebral atrophy. The boy dies at age 10. Prior infection with which of the following agents was most likely related to this patient's condition?
- A. Measles virus (Correct Answer)
- B. Mumps virus
- C. Papillomavirus
- D. Poliovirus
Explanation: ### Explanation The clinical presentation describes **Subacute Sclerosing Panencephalitis (SSPE)**, a rare, progressive, and fatal neurodegenerative disease caused by a persistent, mutated **Measles virus** infection. #### 1. Why Measles Virus is Correct SSPE typically occurs 5–10 years after an initial measles infection (often in children infected before age 2). The pathogenesis involves a defective measles virus that lacks the **M-protein**, allowing it to persist in the CNS without being cleared. * **Clinical Stages:** It begins with subtle behavioral changes and intellectual decline (Stage 1), progressing to characteristic **periodic myoclonic jerks** (Stage 2), and eventually leading to dementia, optic atrophy, and death (Stage 3/4). * **Diagnostic Hallmarks:** * **EEG:** Periodic, high-voltage, slow-wave complexes (Radermecker complexes) synchronous with myoclonus. * **CSF:** Markedly elevated measles antibody titers and **oligoclonal bands** of IgG (without pleocytosis). * **Imaging:** Diffuse white matter changes and cerebral atrophy. #### 2. Why Other Options are Incorrect * **Mumps virus:** Primarily causes parotitis, orchitis, and aseptic meningitis; it does not cause a chronic, progressive panencephalitis. * **Papillomavirus:** Associated with warts and cervical cancer. While the **JC virus** (a Polyomavirus) causes Progressive Multifocal Leukoencephalopathy (PML), it typically occurs in immunocompromised adults and lacks the periodic myoclonus seen here. * **Poliovirus:** Affects the anterior horn cells of the spinal cord, leading to acute flaccid paralysis, not a chronic dementing illness. #### 3. High-Yield Pearls for NEET-PG * **SSPE Risk Factor:** Early age of primary measles infection (<2 years). * **CSF Finding:** "Intrathecal synthesis of measles-specific IgG." * **EEG Pattern:** Periodic complexes (Burst-suppression-like) every 5–15 seconds. * **Treatment:** No definitive cure; Isoprinosine and intrathecal Interferon-alpha may slow progression. * **Prevention:** The most effective strategy is vaccination with the **MMR vaccine**.
Question 154: Which of the following is the most common cause of congenital hydrocephalus?
- A. Craniosynostosis
- B. Intrauterine meningitis
- C. Congenital aqueductal stenosis (Correct Answer)
- D. Vein of Galen malformation
Explanation: **Explanation:** **Congenital Aqueductal Stenosis** is the most common cause of congenital hydrocephalus, accounting for approximately 43% of cases. The Aqueduct of Sylvius is the narrowest point in the ventricular system, connecting the third and fourth ventricles. Obstruction here (due to gliosis, forking, or septum formation) leads to **non-communicating (obstructive) hydrocephalus**, characterized by dilation of the lateral and third ventricles with a normal-sized fourth ventricle. **Analysis of Incorrect Options:** * **Craniosynostosis (A):** This is the premature closure of cranial sutures. While it can lead to increased intracranial pressure and occasionally secondary hydrocephalus, it is primarily a disorder of skull growth, not a primary cause of congenital hydrocephalus. * **Intrauterine Meningitis (B):** This is a common cause of *acquired* or post-inflammatory hydrocephalus. It typically results in communicating hydrocephalus due to the obliteration of subarachnoid cisterns or impaired resorption at arachnoid villi. * **Vein of Galen Malformation (D):** This is a rare arteriovenous malformation. While it can cause hydrocephalus (via compression of the aqueduct or high venous pressure), it is far less common than idiopathic aqueductal stenosis. **NEET-PG High-Yield Pearls:** * **X-linked Hydrocephalus:** About 5% of aqueductal stenosis cases are X-linked (L1CAM mutation), often associated with thumb adduction (spastic paraplegia). * **Dandy-Walker Malformation:** Characterized by a triad of cystic dilation of the 4th ventricle, cerebellar vermis hypoplasia, and hydrocephalus. * **Chiari II Malformation:** Almost always associated with myelomeningocele and is a major cause of hydrocephalus in neonates. * **Clinical Sign:** "Setting sun" eye sign (downward gaze) due to pressure on the midbrain tectum.
Question 155: A 5-year-old boy presents with a 10-day history of fever, for which he received some medication. For the past 3 days, he has experienced anorexia and vomiting, and for the past day, altered sensorium. One day prior to presentation, the child had two episodes of seizures. On examination, the child is hemodynamically stable, with no pallor or icterus, and no meningeal signs. The liver is palpable 2 cm below the right costal margin. Blood glucose is 40 mg%, Hb is 11 g/dl, TLC is 8300, platelet count is 2.8 lac/mm3, and PT is 58/12 sec. What is the most likely diagnosis?
- A. Tyrosinemia
- B. Reye syndrome (Correct Answer)
- C. Alagille syndrome
- D. Acute Viral Hepatitis
Explanation: **Explanation:** The clinical presentation is classic for **Reye Syndrome**, a rapidly progressive non-inflammatory encephalopathy associated with fatty degeneration of the liver. **Why Reye Syndrome is correct:** The diagnosis is based on the characteristic **triad of antecedent viral illness (fever), acute encephalopathy (altered sensorium/seizures), and hepatic dysfunction without jaundice.** * **Clinical Clues:** The history of fever followed by a "latent period" of vomiting and rapid neurological deterioration is hallmark. The absence of icterus (jaundice) despite significant coagulopathy (PT 58/12) and hypoglycemia (40 mg%) strongly points toward Reye syndrome rather than typical hepatitis. * **Pathophysiology:** It involves mitochondrial dysfunction, often triggered by **salicylate (aspirin)** use during viral infections like Influenza or Varicella. **Why other options are incorrect:** * **Tyrosinemia:** Usually presents in infancy with failure to thrive, rickets, and chronic liver failure; it would not present as an acute encephalopathic event in a previously healthy 5-year-old. * **Alagille Syndrome:** A genetic disorder characterized by neonatal cholestasis, "butterfly" vertebrae, and distinct facial features. It presents with chronic jaundice and pruritus. * **Acute Viral Hepatitis:** While it causes vomiting and fever, it is almost always accompanied by **icterus (jaundice)** and significant hepatomegaly. The "white liver" (no jaundice) with severe coagulopathy is the differentiating factor here. **High-Yield Pearls for NEET-PG:** * **Biochemical hallmarks:** Elevated serum ammonia, hypoglycemia, and prolonged PT with **normal bilirubin.** * **Liver Biopsy:** Shows **microvesicular steatosis** (small fat droplets). * **Management:** Supportive, focusing on managing cerebral edema (Mannitol) and correcting hypoglycemia. * **Aspirin Link:** Always look for a history of "medication for fever" in the vignette. Use of aspirin in children is now restricted to specific conditions like Kawasaki disease to prevent Reye syndrome.
Question 156: A 4-year-old child presented with symptoms of unsteady gait, tremors, frequent shivering, headaches, and dizziness for the past 6 months. There was a history of frequent chest infections and a few episodes of seizures in the past. The child was normal at birth. On examination, the child had speech, hearing, and visual impairment and discoloration of areas of skin exposed to sunlight. MRI brain with MR spectroscopy showed a choline peak. The gene responsible for the above condition is present on which of the following chromosomes?
- A. 11q (Correct Answer)
- B. 11p
- C. 12q
- D. 12p
Explanation: ### Explanation The clinical presentation points toward **Ataxia-Telangiectasia (Louis-Bar Syndrome)**. This is an autosomal recessive multisystem disorder characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, and immune deficiency. **Why Option A (11q) is correct:** The diagnosis is confirmed by the constellation of symptoms: * **Neurological:** Unsteady gait (ataxia), tremors, and seizures. * **Immunological:** Frequent chest infections due to combined B and T-cell deficiency (low IgA, IgG2, and IgE). * **Dermatological:** Discoloration/telangiectasia in sun-exposed areas. * **Biochemical/Imaging:** MRI shows cerebellar atrophy; MR spectroscopy shows a **choline peak** (indicating membrane turnover/demyelination). * **Genetics:** The defective gene is the **ATM (Ataxia-Telangiectasia Mutated) gene**, located on **chromosome 11q22.3**. This gene is responsible for DNA double-strand break repair. **Why other options are incorrect:** * **11p:** Associated with the **WT1 gene** (Wilms tumor, Aniridia, Genitourinary anomalies, and Range of developmental delays—WAGR syndrome). * **12q:** Associated with Vitamin D-dependent rickets Type 1A (*CYP27B1* gene) or Phenylketonuria (*PAH* gene). * **12p:** Associated with certain leukemias (ETV6 gene) but not with primary neuro-immunological syndromes. **Clinical Pearls for NEET-PG:** 1. **Diagnostic Marker:** Elevated **Alpha-fetoprotein (AFP)** levels are seen in >95% of patients after age 2. 2. **Radiosensitivity:** Patients are highly sensitive to ionizing radiation (X-rays/CT) due to defective DNA repair. 3. **Malignancy Risk:** Increased risk of lymphomas and leukemias. 4. **Ocular Sign:** Oculomotor apraxia (difficulty initiating voluntary eye movements) is a common early sign.
Question 157: A 7-year-old male patient presented with skin lesions on the face and the lumbosacral region. The patient also has a history of frequent seizures. Which of the following chromosomes is involved in the disease?
- A. 22q (Correct Answer)
- B. 17q
- C. 9p
- D. 9q
Explanation: **Explanation:** The clinical presentation of facial skin lesions (likely **Adenoma Sebaceum/Angiofibromas**), a lumbosacral lesion (likely a **Shagreen patch**), and a history of seizures is classic for **Tuberous Sclerosis Complex (TSC)**. TSC is an autosomal dominant neurocutaneous syndrome caused by mutations in two possible genes: 1. **TSC1:** Located on chromosome **9q34** (encodes the protein Hamartin). 2. **TSC2:** Located on chromosome **16p13.3** (encodes the protein Tuberin). **Wait, let’s re-evaluate the provided key:** In standard medical genetics, TSC involves 9q and 16p. However, if the question identifies **22q** as the correct answer in a specific exam context, it typically refers to **Neurofibromatosis Type 2 (NF2)**, where the *MERLIN* gene is located on **22q12**. NF2 presents with bilateral acoustic neuromas and skin plaques, though seizures are less common than in TSC. *Note: If the question strictly describes TSC (Shagreen patch + Seizures), the standard answer should be 9q or 16p. If 22q is the designated key, it implies the examiner is testing NF2 or there is a typographical error in the provided options/key.* **Analysis of Options:** * **A. 22q (Correct per key):** Location of the **NF2 gene**. While TSC is the better clinical fit for the description, 22q is a high-yield "phakomatosis" chromosome. * **B. 17q:** Location of the **NF1 gene** (17q11.2). NF1 presents with Café-au-lait spots and Lisch nodules. * **D. 9q:** Location of **TSC1**. This is the classic association for Tuberous Sclerosis (Hamartin). **High-Yield Clinical Pearls for TSC:** * **Vogt’s Triad:** Seizures, Intellectual disability, and Adenoma sebaceum (only in <30% of cases). * **Ash-leaf spots:** Earliest sign (hypopigmented macules visible under Wood’s lamp). * **Cardiac Rhabdomyoma:** Most common fetal/neonatal cardiac tumor; often regresses. * **Renal Angiomyolipoma:** Common bilateral renal finding. * **Subependymal Giant Cell Astrocytoma (SEGA):** Characteristic brain lesion near the Foramen of Monro.
Question 158: Which of the following disorders is NOT associated with the sign shown in the image?
- A. Duchenne muscular dystrophy
- B. Becker's muscular dystrophy
- C. Landouzy-Dejerine disease
- D. Spinal muscular atrophy (Correct Answer)
Explanation: ***Spinal muscular atrophy*** - **Gower's sign** is NOT typically associated with SMA as it primarily affects **anterior horn cells** rather than muscle fibers directly, leading to different patterns of weakness. - SMA patients usually present with **hypotonia** and **areflexia** from infancy, often never achieving independent ambulation in severe forms. *Duchenne muscular dystrophy* - **Gower's sign** is a classic presentation due to severe **proximal muscle weakness** affecting the pelvic girdle and thighs. - Progressive **muscle fiber degeneration** caused by **dystrophin deficiency** leads to the characteristic climbing maneuver to stand. *Becker's muscular dystrophy* - Shows **Gower's sign** similar to DMD but with milder symptoms due to **partially functional dystrophin** production. - Patients typically have later onset and slower progression compared to Duchenne, but still exhibit **proximal weakness**. *Landouzy-Dejerine disease* - Also known as **facioscapulohumeral dystrophy (FSHD)**, can present with **Gower's sign** when **pelvic girdle muscles** are involved. - Though primarily affecting **facial** and **shoulder girdle muscles** initially, progression often includes **hip girdle weakness**.
Question 159: A 7-year-old child presents with a 10-day history of fever, early morning headache, and vomiting, along with focal neurological deficits. A CT scan is performed. What is the management for this condition?
- A. Anti-tubercular therapy for 6-9 months
- B. Acyclovir for 10 days (Correct Answer)
- C. Surgical removal of a tumor
- D. None of the above
Explanation: ***Acyclovir for 10 days*** - The presentation of **fever, early morning headache, vomiting, and focal neurological deficits** in a child strongly suggests **HSV encephalitis**, which commonly affects the **temporal lobe**. - **Acyclovir** is the first-line antiviral treatment that inhibits **viral DNA polymerase**, effectively treating HSV encephalitis when started promptly. *Anti-tubercular therapy for 6-9 months* - **Tubercular meningitis** typically presents with **gradual onset** over weeks to months, not the acute 10-day history described. - CT findings in TB meningitis show **basal enhancement** and **hydrocephalus**, whereas HSV encephalitis shows **temporal lobe hemorrhagic necrosis**. *Surgical removal of a tumor* - **Brain tumors** in children usually present with **gradually progressive symptoms** and **raised intracranial pressure** over weeks to months. - The **acute febrile illness** with focal deficits is more consistent with **infectious encephalitis** rather than a space-occupying lesion. *None of the above* - This option is incorrect as **acyclovir therapy** is the established standard treatment for **HSV encephalitis**. - Early initiation of acyclovir significantly reduces **mortality and morbidity** associated with HSV encephalitis in pediatric patients.
Question 160: What is the investigation of choice for the diagnostic evaluation of hydrocephalus in a one-month-old child?
- A. X-ray skull
- B. USG (Correct Answer)
- C. CT
- D. MRI
Explanation: **Explanation:** The investigation of choice for a one-month-old child with suspected hydrocephalus is **Ultrasonography (USG) of the head (Transfontanellar USG)**. **Why USG is the Correct Answer:** In infants under the age of 6–12 months, the **anterior fontanelle** remains open, serving as an acoustic window. This allows high-frequency sound waves to visualize the ventricular system, brain parenchyma, and subarachnoid spaces clearly. USG is preferred because it is non-invasive, cost-effective, bedside-accessible, and, most importantly, involves **no ionizing radiation** or sedation, which is critical in the neonatal and early infancy period. **Analysis of Incorrect Options:** * **A. X-ray skull:** This is obsolete for diagnosing hydrocephalus. While it may show "copper beaten appearance" or suture diastasis in chronic cases, it cannot visualize brain tissue or ventricular size. * **C. CT Scan:** While CT provides excellent detail, it involves significant radiation exposure. It is generally reserved for emergencies (e.g., acute hemorrhage) or when USG is inconclusive and MRI is unavailable. * **D. MRI:** MRI is the **gold standard** for defining the precise etiology (e.g., aqueductal stenosis) and detailed anatomy. However, it is not the *initial* investigation of choice because it requires sedation in infants and is less accessible than USG. **NEET-PG Clinical Pearls:** * **Screening Tool:** USG is the best screening and monitoring tool for Intraventricular Hemorrhage (IVH) in preterm neonates. * **Closure of Fontanelles:** The anterior fontanelle typically closes by 18 months; once closed, USG is no longer technically feasible, making CT/MRI necessary. * **Gold Standard:** If the question asks for the "most accurate" or "gold standard" for etiology, the answer is **MRI**. For "initial/investigation of choice" in an infant, it is **USG**.
Practice by Chapter
Seizure Disorders and Epilepsy
Practice Questions
Febrile Seizures
Practice Questions
Headache Disorders
Practice Questions
Cerebral Palsy
Practice Questions
Neural Tube Defects
Practice Questions
Neuromuscular Disorders
Practice Questions
Neurodegenerative Disorders
Practice Questions
CNS Infections
Practice Questions
Hydrocephalus
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Movement Disorders
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Traumatic Brain Injury
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Neuroimaging in Pediatrics
Practice Questions
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