Neurology Practice Questions

Q: Factors that increase the risk of recurrence of febrile seizures include all except:

Temperature more than 40°C. ***Temperature more than 40°C*** - A **high fever** (over 40°C) during a febrile seizure **does not increase the risk of recurrence**. - In fact, **lower fever at onset** (<39°C) is actually associated with **higher recurrence risk**, as it suggests a lower seizure threshold. - The height of fever is **not a recognized predictor** of febrile seizure recurrence in major pediatric guidelines. *Initial seizure at less than 12 months of age* - **Younger age at the first febrile seizure** (especially under 12-18 months) is a significant predictor of future episodes. - The **immature nervous system** of younger infants makes them more susceptible to subsequent seizures with fever. - This is one of the strongest risk factors for recurrence. *Positive family history* - A **family history of febrile seizures** significantly increases a child's risk of recurrence. - This suggests a **genetic predisposition** to febrile seizures. - Children with affected first-degree relatives have approximately 2-3 times higher recurrence risk. *Complex seizures* - **Complex febrile seizures** (defined by prolonged duration >15 minutes, focal features, or multiple seizures within 24 hours) are associated with a higher risk of recurrence. - This indicates greater **neuronal excitability** or underlying susceptibility.

Q: A 3 year old child is brought to you with history of frequent, violent and rapid swinging movement of the left arm for one week duration. What is the condition described in this scenario called?

Hemiballismus. ***Hemiballismus*** - **Hemiballismus** is characterized by sudden, involuntary, wild, rapid, and **flailing movements** of one side of the body, primarily affecting the proximal musculature such as the arm and leg. - The description of "frequent, violent and rapid swinging movement of the left arm" perfectly aligns with the clinical presentation of hemiballismus, often resulting from a lesion in the **subthalamic nucleus**. *Athetosis* - **Athetosis** involves slow, writhing, involuntary movements, especially in the distal parts of the limbs (fingers and toes). - This condition is typically slower and more sustained than the "violent and rapid swinging" described in the scenario. *Dystonia* - **Dystonia** is characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements or postures. - While it can involve forceful movements, it usually results in sustained postures or twisting movements rather than the rapid, flailing motion described. *Chorea* - **Chorea** refers to irregular, unpredictable, brief, and jerky movements that flow from one body part to another in a dance-like manner. - Unlike the violent, large-amplitude movements of hemiballismus, chorea typically involves more distal and less organized movements.

Q: All of the following are true of febrile seizures except-

Almost invariably develop into epilepsy. ***Almost invariably develop into epilepsy*** - This statement is **false**; only a small percentage (2-7%) of children with febrile seizures later develop **epilepsy**. - Febrile seizures are generally considered **benign** and do not typically lead to a diagnosis of epilepsy. *Prognosis is good* - The **prognosis for simple febrile seizures is excellent**, with no long-term neurological sequelae or cognitive impairment. - The risk of recurrence is present but usually decreases with age, and **most children outgrow them**. *Does not last more than 15 minutes* - **Simple febrile seizures** are defined as generalized tonic-clonic seizures lasting **less than 15 minutes**. - Seizures lasting longer than 15 minutes are considered **complex febrile seizures**, which have a slightly higher risk of recurrence but do not necessarily change the overall good prognosis. *Most commonly seen between 9 months and 5 years* - Febrile seizures typically occur in children between **6 months and 5 years of age**, with a peak incidence around **18 months**. - This age range reflects the developing brain's susceptibility to febrile illness and its ability to generate a generalized seizure response.

Q: Duchenne's muscular dystrophy affects which group of muscles commonly:

Proximal muscles. ***Proximal muscles*** - Duchenne's muscular dystrophy (DMD) is characterized by **progressive proximal muscle weakness**, which is the hallmark feature of this X-linked recessive disorder. - The weakness typically begins in the **pelvic girdle muscles** (hip flexors, quadriceps) and later progresses to involve the **shoulder girdle muscles**. - This proximal pattern of weakness leads to classic clinical features: difficulty **climbing stairs**, **rising from the floor** (Gower's sign), **waddling gait**, and difficulty **raising arms overhead**. - The term "proximal muscles" accurately encompasses the characteristic distribution of muscle involvement in DMD, making it the most correct answer. *Shoulder muscles* - While shoulder girdle muscles are indeed affected in DMD, they are typically involved **after the pelvic girdle muscles**. - This option is too specific and does not capture the **generalized proximal muscle weakness** that defines DMD. - Shoulder muscle weakness becomes prominent as the disease progresses, but it is not the initial or most characteristic presentation. *Calf muscles* - Calf muscles may show **pseudohypertrophy** (apparent enlargement due to replacement of muscle with fat and connective tissue), which is a characteristic finding in DMD. - However, pseudohypertrophy does not represent true muscle strength, and the calves are **distal muscles**, not the primary site of weakness. - DMD is a **proximal myopathy**, not a distal myopathy. *Respiratory muscles* - **Respiratory muscle weakness** (diaphragm and intercostal muscles) is a serious and life-threatening complication of DMD. - However, respiratory involvement occurs **later in the disease course**, typically in the second decade of life. - Progressive respiratory failure is a major cause of morbidity and mortality in DMD but is not the early or most characteristic muscle group affected.

Q: All the following are Causes of spasticity in a 2 year old child except

Congenital muscular dystrophy. ***Congenital muscular dystrophy*** - This condition is characterized by **muscle weakness and hypotonia**, not spasticity, due to primary muscle pathology. - Spasticity is a sign of **upper motor neuron involvement**, whereas muscular dystrophies are disorders of peripheral muscle fibers resulting in **flaccid weakness**. *Cerebral Palsy* - This is the **most common cause of spasticity in children**, resulting from **non-progressive brain injury during development**. - **Spastic cerebral palsy** accounts for 70-80% of CP cases, characterized by increased muscle tone, hyperreflexia, and extensor plantar responses. *Kernicterus* - **Bilirubin encephalopathy** from severe neonatal hyperbilirubinemia causes **basal ganglia damage**. - Results in **extrapyramidal cerebral palsy** with spasticity, choreoathetosis, and developmental delays. *Birth asphyxia* - **Hypoxic-ischemic encephalopathy** from perinatal asphyxia causes **upper motor neuron damage**. - Leads to spastic quadriplegia or diplegia with increased tone, hyperreflexia, and developmental impairment.

Q: A 4-year-old male child presents with muscle weakness. His mother reports that her child has difficulty in climbing stairs and getting up from the floor. On muscle biopsy, small degenerated muscle fibers and absence of dystrophin were found. What is the diagnosis?

Duchenne muscular dystrophy. ***Duchenne muscular dystrophy*** - The classic presentation of a young boy with **progressive muscle weakness**, difficulty climbing stairs (**Gowers' sign**), and **absent dystrophin** on muscle biopsy is characteristic of Duchenne muscular dystrophy. - It is an **X-linked recessive disorder** that leads to severe muscle degeneration and weakness due to a complete lack of functional dystrophin protein. *Myotonic dystrophy* - This condition is characterized by **myotonia** (delayed relaxation of muscles after contraction) and typically affects adults, although congenital forms exist. - While it causes muscle weakness, the primary differentiating feature of **myotonia** and its later onset are not present in this case. *Becker's muscle dystrophy* - Becker's muscular dystrophy (BMD) is also an X-linked recessive disorder and a milder form of muscular dystrophy, caused by a **reduced but still functional dystrophin** protein. - Patients with BMD typically present later in childhood or adolescence with slower progression and **some dystrophin** presence, unlike the absent dystrophin and early onset here. *Limb-girdle muscular dystrophy* - This is a group of muscular dystrophies that primarily affect the **pelvic and shoulder girdle muscles**. - It can present with similar weakness, but the **complete absence of dystrophin** found on biopsy points specifically to Duchenne muscular dystrophy, not typical limb-girdle dystrophy, which involves other genetic defects.

Q: A 6-year-old with recurrent febrile seizures presents lethargic with a high fever. What is the most appropriate next step in management?

Perform lumbar puncture. ***Perform lumbar puncture*** - The combination of **lethargy**, high fever, and a history of recurrent febrile seizures in a 6-year-old child raises suspicion for **meningitis or encephalitis**, necessitating a prompt **lumbar puncture** to analyze **cerebrospinal fluid (CSF)**. - While febrile seizures alone are benign, **altered mental status (lethargy)** in conjunction with fever is a red flag for **central nervous system infection**. *Consider using antipyretics for comfort* - **Antipyretics** can help reduce fever and improve comfort but do not address the underlying cause of lethargy and potential CNS infection. - Delaying definitive diagnostic steps like a **lumbar puncture** while waiting for antipyretics to work could worsen the patient's prognosis if a serious infection is present. *Start anticonvulsants* - **Anticonvulsants** are primarily used for managing ongoing seizures or preventing recurrent non-febrile seizures but are **not indicated as a first-line diagnostic or emergency treatment** for a child presenting with **fever and lethargy without active seizures**. - There is no clinical indication of current seizure activity, and the immediate concern is detecting a potential **CNS infection**. *Order urgent EEG* - An **EEG (electroencephalogram)** is useful for evaluating seizure disorders or encephalopathy but is **not the most appropriate initial diagnostic step** when a **serious CNS infection like meningitis** is suspected. - A **lumbar puncture** is crucial for diagnosing or ruling out meningitis, which requires immediate treatment.

Q: A 2-year-old boy presents with generalized tonic-clonic seizures, and his development has been normal. Which investigation should be performed first?

Electrolyte levels. ***Electrolyte levels*** - This is the **first-line investigation** for new-onset seizures, especially in children, to rule out common and easily treatable metabolic causes. - **Hypoglycemia**, **hypocalcemia**, and **hyponatremia** can all trigger seizures and are readily identifiable with a basic metabolic panel. *CT scan of the head* - While useful for acute structural abnormalities like **hemorrhage** or large tumors, it exposes the child to **radiation** and is usually reserved for cases where other causes have been ruled out or if there are focal neurological signs. - It provides less detail than an MRI for soft tissue and subtle lesions. *EEG* - An **EEG** is performed to characterize the **epileptic discharges** and classify the type of seizure or epilepsy syndrome, and hence is useful for guiding long-term management. - It is not typically the first investigation for an **unexplained acute seizure**, as it doesn't identify the immediate underlying cause in most emergent situations. *MRI* - **MRI** provides much more detailed imaging of brain structure than CT and is preferred for investigating **structural causes of epilepsy**, especially for subtle lesions, congenital malformations, or hippocampal sclerosis. - However, it is a more time-consuming and expensive test, often requiring **sedation** in young children, and is usually performed after ruling out acute metabolic causes.

Question 1

Factors that increase the risk of recurrence of febrile seizures include all except:

Accepted Answer

Temperature more than 40°C

Answer

Initial seizure at less than 12 months of age

Answer

Positive family history

Answer

Complex seizures

Question 2

A 10 year old child presented with headache, vomiting, gait instability and diplopia. On examination he had papilledema and gait ataxia. The most probable diagnosis is –

Accepted Answer

Midline posterior fossa tumour

Answer

Suprasellar tumour

Answer

Hydrocephalus

Answer

Brain stem tumour

Question 3

A 3 year old child is brought to you with history of frequent, violent and rapid swinging movement of the left arm for one week duration. What is the condition described in this scenario called?

Accepted Answer

Hemiballismus

Answer

Athetosis

Answer

Dystonia

Answer

Chorea

Question 4

All of the following are true of febrile seizures except-

Accepted Answer

Almost invariably develop into epilepsy

Answer

Prognosis is good

Answer

Does not last more than 15 minutes

Answer

Most commonly seen between 9 months and 5 years

Question 5

Duchenne's muscular dystrophy affects which group of muscles commonly:

Accepted Answer

Proximal muscles

Answer

Calf muscles

Answer

Shoulder muscles

Answer

Respiratory muscles

Question 6

All the following are Causes of spasticity in a 2 year old child except

Accepted Answer

Congenital muscular dystrophy

Answer

Cerebral Palsy

Answer

Kernicterus

Answer

Birth asphyxia

Question 7

A 4-year-old male child presents with muscle weakness. His mother reports that her child has difficulty in climbing stairs and getting up from the floor. On muscle biopsy, small degenerated muscle fibers and absence of dystrophin were found. What is the diagnosis?

Accepted Answer

Duchenne muscular dystrophy

Answer

Myotonic dystrophy

Answer

Becker's muscle dystrophy

Answer

Limb-girdle muscular dystrophy

Question 8

A 6-year-old with recurrent febrile seizures presents lethargic with a high fever. What is the most appropriate next step in management?

Accepted Answer

Perform lumbar puncture

Answer

Consider using antipyretics for comfort

Answer

Start anticonvulsants

Answer

Order urgent EEG

Question 9

A 7-year-old child presents with ataxia and slurred speech. An MRI shows a mass in the cerebellum. What is the most likely diagnosis?

Accepted Answer

Astrocytoma

Answer

Brainstem glioma

Answer

Arachnoid cyst

Answer

Medulloblastoma

Question 10

A 2-year-old boy presents with generalized tonic-clonic seizures, and his development has been normal. Which investigation should be performed first?

Accepted Answer

Electrolyte levels

Answer

CT scan of the head

Answer

EEG

Answer

MRI

Neurology — MCQs

Neurology — MCQs

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