Neurology Practice Questions

Q: Hung up reflex is seen in?

Chorea. **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).

Q: What is the best prophylaxis for a 4-year-old male child experiencing febrile seizures?

Diazepam. **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, 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.

Q: Which of the following is the most common cause of congenital hydrocephalus?

Congenital aqueductal stenosis. **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.

Q: 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?

11q. ### 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.

Q: Which of the following disorders is NOT associated with the sign shown in the image?

Spinal muscular atrophy. ***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**.

Q: 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?

Acyclovir for 10 days. ***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.

Q: What is the investigation of choice for the diagnostic evaluation of hydrocephalus in a one-month-old child?

USG. **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**.

Question 1

Hung up reflex is seen in?

Accepted Answer

Chorea

Answer

Cerebral palsy

Answer

Athetosis

Answer

None of the above

Question 2

What is the best prophylaxis for a 4-year-old male child experiencing febrile seizures?

Accepted Answer

Diazepam

Answer

Paracetamol every 6 hours

Answer

Paracetamol and diazepam

Answer

Phenobarbitone

Question 3

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?

Accepted Answer

Measles virus

Answer

Mumps virus

Answer

Papillomavirus

Answer

Poliovirus

Question 4

Which of the following is the most common cause of congenital hydrocephalus?

Accepted Answer

Congenital aqueductal stenosis

Answer

Craniosynostosis

Answer

Intrauterine meningitis

Answer

Vein of Galen malformation

Question 5

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?

Accepted Answer

Reye syndrome

Answer

Tyrosinemia

Answer

Alagille syndrome

Answer

Acute Viral Hepatitis

Question 6

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?

Accepted Answer

11q

Answer

11p

Answer

12q

Answer

12p

Question 7

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?

Accepted Answer

22q

Answer

17q

Answer

9p

Answer

9q

Question 8

Which of the following disorders is NOT associated with the sign shown in the image?

Accepted Answer

Spinal muscular atrophy

Answer

Duchenne muscular dystrophy

Answer

Becker's muscular dystrophy

Answer

Landouzy-Dejerine disease

Question 9

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?

Accepted Answer

Acyclovir for 10 days

Answer

Anti-tubercular therapy for 6-9 months

Answer

Surgical removal of a tumor

Answer

None of the above

Question 10

What is the investigation of choice for the diagnostic evaluation of hydrocephalus in a one-month-old child?

Accepted Answer

USG

Answer

X-ray skull

Answer

CT

Answer

MRI

Neurology — MCQs

Neurology — MCQs

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