Neurology Practice Questions

Q: Which of the following is NOT true about Duchenne Muscular Dystrophy?

Distal muscle weakness is the primary finding.. **Explanation:** Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder caused by a mutation in the **dystrophin gene**, leading to progressive muscle degeneration. **Why Option C is correct:** In DMD, the primary finding is **proximal muscle weakness**, not distal. Weakness typically begins in the pelvic girdle and hip extensors, later progressing to the shoulder girdle. Distal muscle strength is usually preserved until the advanced stages of the disease. **Analysis of other options:** * **Option A:** **Calf pseudohypertrophy** is a hallmark sign. The calves appear enlarged, but this is due to the replacement of muscle tissue with fat and connective tissue (fibrosis), rather than true muscle hypertrophy. * **Option B:** **Gower’s sign** is a classic clinical manifestation. Due to weak hip extensors (proximal weakness), the child must use their hands to "walk up" their legs to achieve a standing position from the floor. * **Option D:** Dystrophin is also absent in cardiac muscle. Most patients develop **dilated cardiomyopathy** and arrhythmias, with heart failure or respiratory failure being the leading causes of mortality. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** X-linked recessive (affects males; females are carriers). * **Biochemical Marker:** Significantly elevated **Serum Creatine Kinase (CPK)** levels (often 10–100x normal). * **Diagnosis:** Genetic testing (deletion analysis) is the gold standard; muscle biopsy shows absent dystrophin. * **Maneuver:** Trendelenburg gait is often present early due to gluteus medius weakness. * **Becker Muscular Dystrophy (BMD):** A milder form where dystrophin is truncated/reduced rather than absent; it has a later onset.

Q: An 8-year-old boy presents with difficulty in walking. What is the most likely diagnosis?

Muscular dystrophy. ***Muscular dystrophy*** - **Duchenne muscular dystrophy** is the most common form in boys, typically presenting between ages 3-5 with progressive difficulty walking and **Gower's sign** (using hands to push up from floor). - The **X-linked recessive** inheritance pattern affects boys predominantly, causing **proximal muscle weakness** and **pseudohypertrophy** of calf muscles. *Neuropathy* - Peripheral neuropathy typically presents with **distal weakness** and **sensory loss** starting in hands and feet, not proximal muscle weakness. - Usually associated with **diabetes**, **vitamin deficiencies**, or **hereditary conditions** like Charcot-Marie-Tooth disease, less common in healthy 8-year-olds. *Guillain-Barré syndrome* - An **acute inflammatory demyelinating** condition that develops over days to weeks following an infection. - Characterized by **ascending paralysis**, **areflexia**, and often **respiratory involvement**, requiring immediate medical attention. *Polymyositis* - An **inflammatory myopathy** that typically affects adults, rarely seen in children under 10 years old. - Associated with **elevated muscle enzymes** (CPK, LDH), **muscle pain**, and often systemic symptoms like fever and rash.

Q: What is the commonest cause of non-communicating hydrocephalus in children?

Congenital anomaly. **Explanation:** Hydrocephalus is characterized by an imbalance between cerebrospinal fluid (CSF) production and absorption. **Non-communicating (obstructive) hydrocephalus** occurs when there is a physical blockage within the ventricular system, preventing CSF from reaching the subarachnoid space. **1. Why "Congenital Anomaly" is Correct:** Congenital malformations are the most frequent cause of obstructive hydrocephalus in the pediatric population. Among these, **Aqueductal Stenosis** (narrowing of the Aqueduct of Sylvius) is the single most common specific cause. Other congenital triggers include the Dandy-Walker malformation and Chiari malformations. **2. Analysis of Incorrect Options:** * **Perinatal injury:** While intraventricular hemorrhage (IVH) in premature infants is a major cause, it more frequently leads to *communicating* hydrocephalus due to blood products obliterating the arachnoid villi, though post-hemorrhagic clots can occasionally cause acute obstruction. * **Post-inflammatory obstruction:** Conditions like neonatal meningitis can cause adhesions. While significant, these typically result in communicating hydrocephalus due to basal cistern fibrosis. * **Brain tumors:** While a common cause of *acquired* obstructive hydrocephalus (e.g., medulloblastoma blocking the 4th ventricle), they are statistically less common than congenital structural defects in the overall pediatric age group. **NEET-PG High-Yield Pearls:** * **Most common cause overall:** Aqueductal stenosis (Non-communicating). * **Dandy-Walker Malformation:** Characterized by triad of cystic dilation of the 4th ventricle, cerebellar vermis hypoplasia, and enlarged posterior fossa. * **Clinical Sign:** "Setting-sun" eye phenomenon (downward gaze palsy due to pressure on the midbrain tectum). * **Macewen’s Sign:** "Cracked pot" sound on percussion of the skull due to separated sutures.

Q: The 'sunset sign' is observed in which of the following conditions?

Hydrocephalus. **Explanation:** The **'Sunset Sign'** (or setting-sun phenomenon) is a classic clinical finding in pediatric neurology, most commonly associated with **Hydrocephalus**. **Why Hydrocephalus is correct:** The sign is characterized by the downward deviation of the eyes, where the sclera is visible above the iris, making the eyes look like a "setting sun." This occurs due to increased intracranial pressure (ICP) causing pressure on the **midbrain tectum** and the **periaqueductal region**. This pressure leads to upward gaze palsy (Parinaud’s syndrome) and lid retraction (Collier’s sign). In infants, it is a critical early indicator of hydrocephalus or shunt malfunction, often appearing before significant head circumference enlargement. **Why the other options are incorrect:** * **Optic neuritis:** Typically presents with sudden vision loss, pain with eye movement, and a relative afferent pupillary defect (RAPD), but does not affect eye positioning. * **Thyroid ophthalmopathy:** While it causes lid retraction (Dalrymple sign) and proptosis, it is an autoimmune inflammatory condition of the extraocular muscles, not related to ICP or the sunset sign. * **Orbital cellulitis:** Presents with proptosis, ophthalmoplegia, and fever due to infection, but lacks the specific downward deviation characteristic of the sunset sign. **High-Yield Clinical Pearls for NEET-PG:** * **Macewen’s Sign (Cracked-pot sign):** Resonant percussion note over the skull, also seen in hydrocephalus. * **Parinaud’s Syndrome:** The triad of upward gaze palsy, convergence-retraction nystagmus, and pupillary light-near dissociation (often due to pineal gland tumors). * **Transillumination test:** Useful in infants to differentiate hydrocephalus from hydranencephaly.

Q: A child with mental retardation and a skin rash on the cheeks undergoes a CT scan. What is the most likely diagnosis?

Tuberous sclerosis. ***Tuberous sclerosis*** - The combination of **mental retardation** (intellectual disability) and **skin rash on the cheeks** (adenoma sebaceum) forms part of the classic triad of tuberous sclerosis. - CT scan typically shows **subependymal calcified nodules** (candle-guttering appearance) and **cortical tubers**, which are pathognomonic findings. *Von Hippel-Lindau disease* - Primarily characterized by **retinal angiomas** and **cerebellar hemangioblastomas**, not skin rashes on cheeks. - Associated with **renal cell carcinoma** and **pheochromocytoma**, but intellectual disability is not a typical feature. *Sturge-Weber syndrome* - Features a **port-wine stain** (capillary malformation) typically in the **trigeminal nerve distribution**, not a rash on cheeks. - CT shows **tramtrack calcifications** in the occipital cortex, not subependymal nodules. *Ataxia-telangiectasia* - Characterized by **progressive cerebellar ataxia** and **telangiectasias** on bulbar conjunctiva and skin creases. - Associated with **immunodeficiency** and increased cancer risk, but facial rashes are not typical presentations.

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.

Question 1

Which of the following is NOT true about Duchenne Muscular Dystrophy?

Accepted Answer

Distal muscle weakness is the primary finding.

Answer

Calf pseudohypertrophy is present.

Answer

The patient uses their hands to climb up their body when rising from a seated position (Gower's sign).

Answer

Cardiomyopathy and congestive heart failure are common complications.

Question 2

Which of the following statements about Duchenne muscular dystrophy is false?

Accepted Answer

Duchenne muscular dystrophy is less severe than Becker's dystrophy.

Answer

It is an X-linked recessive disorder.

Answer

Dilated cardiomyopathy is a common complication.

Answer

Intellectual impairment is frequently observed.

Question 3

An 8-year-old boy presents with difficulty in walking. What is the most likely diagnosis?

Accepted Answer

Muscular dystrophy

Answer

Neuropathy

Answer

Guillain-Barré syndrome

Answer

Polymyositis

Question 4

What is the commonest cause of non-communicating hydrocephalus in children?

Accepted Answer

Congenital anomaly

Answer

Perinatal injury

Answer

Post-inflammatory obstruction

Answer

Brain tumors

Question 5

The 'sunset sign' is observed in which of the following conditions?

Accepted Answer

Hydrocephalus

Answer

Optic neuritis

Answer

Thyroid ophthalmopathy

Answer

Orbital cellulitis

Question 6

A child with mental retardation and a skin rash on the cheeks undergoes a CT scan. What is the most likely diagnosis?

Accepted Answer

Tuberous sclerosis

Answer

Von Hippel-Lindau disease

Answer

Sturge-Weber syndrome

Answer

Ataxia-telangiectasia

Question 7

Hung up reflex is seen in?

Accepted Answer

Chorea

Answer

Cerebral palsy

Answer

Athetosis

Answer

None of the above

Question 8

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 9

All of the following statements regarding Sturge Weber syndrome are true, EXCEPT:

Accepted Answer

Mental retardation is seen in only less than 15% of individuals

Answer

Characterized by nevus flammeus

Answer

Diffuse choroidal hemangioma may be seen in up to 40% of the cases

Answer

Up to 85% of individuals may suffer from seizure disorder

Question 10

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

Neurology — MCQs

Neurology — MCQs

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