Neurology Practice Questions

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: 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 **Analysis of Incorrect Options:** * **A. X-ray skull:** This is obsolete for diagnosing hydrocephalus.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**.

Q: A 9-year-old boy presented with difficulty in climbing stairs and combing. On examination, his calves are swollen and the child uses his feet to stand up on his legs. What is the next diagnostic step?

Creatine kinase levels. ### Explanation **Clinical Diagnosis: Duchenne Muscular Dystrophy (DMD)** The clinical presentation of a young boy with proximal muscle weakness (difficulty climbing stairs and combing hair), **pseudohypertrophy of the calves**, and a positive **Gowers’ sign** (using hands/feet to "walk up" his own body to stand) is classic for Duchenne Muscular Dystrophy. **1. Why Creatine Kinase (CK) is the next step:** In any suspected primary myopathy, especially DMD, the serum **Creatine Kinase (CK) level** is the most important initial screening test. In DMD, CK levels are characteristically massively elevated (often 10 to 100 times the upper limit of normal) even in the early stages of the disease, reflecting ongoing muscle fiber necrosis. **2. Why other options are incorrect:** * **Electromyography (EMG):** While it can differentiate between neurogenic and myopathic processes (showing small, polyphasic potentials in myopathy), it is invasive and no longer the first-line investigation when clinical signs strongly point to DMD. * **Nerve Conduction Velocity (NCV):** This assesses peripheral nerves. It would be normal in DMD as the pathology lies in the muscle, not the nerves. * **Rheumatoid Factor (RA factor):** This is a marker for autoimmune inflammatory arthritis (like JIA), which presents with joint pain and swelling rather than painless proximal muscle weakness and calf hypertrophy. **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Genetic testing (Multiplex PCR) for **Dystrophin gene deletion** (Xp21). * **Muscle Biopsy:** Shows "dystrophic changes" (variation in fiber size, central nuclei, and fibrosis); Immunohistochemistry shows absence of Dystrophin. * **Prognosis:** Most children are wheelchair-bound by age 12; death usually occurs in the 20s due to respiratory failure or dilated cardiomyopathy. * **Becker Muscular Dystrophy:** A milder variant where dystrophin is reduced/abnormal rather than absent.

Q: Continuous prophylactic anticonvulsant therapy is not needed in a child with febrile convulsions with which of the following conditions?

Typical simple febrile seizures. **Explanation:** The management of febrile seizures focuses on distinguishing between **Simple** and **Complex** febrile seizures. **Why Option C is correct:** A **Simple Febrile Seizure** is defined as a generalized tonic-clonic seizure lasting <15 minutes, occurring once in 24 hours in a neurologically normal child. These are benign, do not cause brain damage, and have a very low risk of progressing to epilepsy. Current clinical guidelines (AAP and IAP) strongly recommend **against** continuous prophylactic anticonvulsants for simple febrile seizures because the potential side effects of drugs (like Phenobarbital or Valproate) outweigh the benefits of preventing a benign recurrence. **Why other options are incorrect:** Options A, B, and D are considered **risk factors** for the development of future epilepsy or represent **Complex Febrile Seizures**. * **A & D (Developmental delay/Neurological deficit):** Children with pre-existing brain abnormalities are at a higher risk for recurrent and prolonged seizures. * **B (Family history of epilepsy):** This increases the genetic predisposition to non-febrile seizures, often necessitating closer monitoring or prophylaxis in specific high-risk scenarios. **NEET-PG High-Yield Pearls:** * **Drug of Choice (Acute):** Per-rectal Diazepam (0.5 mg/kg) or IV Lorazepam (0.1 mg/kg). * **Intermittent Prophylaxis:** Oral Diazepam (0.3 mg/kg) is given only during the onset of fever to prevent recurrence. * **Continuous Prophylaxis:** Indicated only if there are frequent recurrences (>3 in 6 months), prolonged seizures (>15 mins), or significant parental anxiety despite counseling. * **Age Group:** Typically occurs between 6 months and 5 years (Peak: 18 months).

Q: A child presented to the casualty with seizures. On examination, oval hypo-pigmented macules were noted on the trunk, along with stib-noo:il. What is the probable diagnosis in this child?

Tuberous sclerosis. ### Explanation The clinical presentation of seizures associated with specific cutaneous markers is a classic "spotter" for Neurocutaneous syndromes in NEET-PG. **1. Why Tuberous Sclerosis (TSC) is correct:** The presence of **oval hypo-pigmented macules** (commonly known as **Ash-leaf spots**) is the earliest cutaneous sign of Tuberous Sclerosis. The term "stib-noo:il" in the question refers to **Subungual fibromas** (Koenen tumors), which are pathognomonic fleshy growths emerging from the nail beds. Seizures in TSC often manifest as infantile spasms or focal seizures due to cortical tubers. **2. Why other options are incorrect:** * **Neurofibromatosis (NF-1):** Characterized by *hyper*-pigmented lesions (**Café-au-lait spots**), Lisch nodules, and neurofibromas, rather than hypo-pigmented macules. * **Sturge Weber Syndrome:** Typically presents with a **Port-wine stain** (Nevus Flammeus) in the V1/V2 distribution of the trigeminal nerve and leptomeningeal angiomas. It does not feature hypo-pigmented macules. * **Incontinentia Pigmenti:** Follows a distinct four-stage skin evolution (vesicular, verrucous, hyperpigmented, and finally atrophic/hypopigmented "swirls"). It is X-linked dominant and usually lethal in males. **3. Clinical Pearls for NEET-PG:** * **Vogt’s Triad (TSC):** Seizures, Mental retardation, and Adenoma sebaceum (facial angiofibromas). * **Shagreen patch:** A leathery, "orange-peel" textured plaque usually found on the lumbosacral area (highly specific for TSC). * **Cardiac involvement:** Rhabdomyomas (often regress spontaneously). * **Renal involvement:** Angiomyolipomas (AML). * **Genetics:** Mutations in *TSC1* (Hamartin) or *TSC2* (Tuberin) genes.

Q: A 7-year-old girl presents with recurrent staring spells lasting a few seconds, after which she returns to her previous activities. Her EEG shows 3 Hz spike-and-wave discharges. Which of the following drugs is NOT indicated for treating this child?

Phenytoin. **Explanation:** The clinical presentation of recurrent staring spells lasting seconds, followed by an immediate return to baseline, is classic for **Childhood Absence Epilepsy (CAE)**. The pathognomonic EEG finding of **3 Hz spike-and-wave discharges** confirms the diagnosis. **Why Phenytoin is the Correct Answer:** Phenytoin is a sodium channel blocker that is highly effective for focal seizures and generalized tonic-clonic seizures. However, it is **contraindicated** in absence epilepsy because it can paradoxically **exacerbate** absence seizures and increase the frequency of staring spells. Other drugs that can worsen absence seizures include Carbamazepine, Vigabatrin, and Tiagabine. **Analysis of Incorrect Options:** * **Ethosuximide (Option A):** This is the **drug of choice** for isolated absence seizures. It works by inhibiting T-type calcium channels in the thalamus. * **Valproate (Option B):** This is a broad-spectrum antiepileptic and is the drug of choice if absence seizures are associated with generalized tonic-clonic seizures (GTCS). * **Lamotrigine (Option C):** This is considered a second-line or adjunctive treatment for absence epilepsy. **NEET-PG High-Yield Pearls:** * **First-line for Absence:** Ethosuximide (if only absence); Valproate (if absence + GTCS). * **EEG Hallmark:** 3 Hz spike-and-wave (generalized, symmetrical). * **Provocation:** Absence seizures can be induced in the clinic by **hyperventilation**. * **Drugs that worsen Absence:** "CPV" (Carbamazepine, Phenytoin, Vigabatrin).

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.

Question 1

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 2

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

Question 3

A 9-year-old boy presented with difficulty in climbing stairs and combing. On examination, his calves are swollen and the child uses his feet to stand up on his legs. What is the next diagnostic step?

Accepted Answer

Creatine kinase levels

Answer

Electromyography (EMG)

Answer

Nerve conduction velocity

Answer

Rheumatoid factor (RA factor)

Question 4

Continuous prophylactic anticonvulsant therapy is not needed in a child with febrile convulsions with which of the following conditions?

Accepted Answer

Typical simple febrile seizures

Answer

Developmental delay

Answer

Family history of epilepsy

Answer

Persistent neurological deficit

Question 5

Atypical febrile seizures are associated with which of the following?

Accepted Answer

Complex partial seizures

Answer

No postictal deficit

Answer

Neurodegeneration

Answer

Raised intracranial pressure

Question 6

A child presented to the casualty with seizures. On examination, oval hypo-pigmented macules were noted on the trunk, along with stib-noo:il. What is the probable diagnosis in this child?

Accepted Answer

Tuberous sclerosis

Answer

Neurofibromatosis

Answer

Sturge Weber syndrome

Answer

Incontinenta pigmenti

Question 7

A 7-year-old girl presents with recurrent staring spells lasting a few seconds, after which she returns to her previous activities. Her EEG shows 3 Hz spike-and-wave discharges. Which of the following drugs is NOT indicated for treating this child?

Accepted Answer

Phenytoin

Answer

Ethosuximide

Answer

Valproate

Answer

Lamotrigine

Question 8

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 9

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 10

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

Neurology — MCQs

Neurology — MCQs

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