Neurology Practice Questions

Q: The 7-year-old boy, who is now in your office, was last seen 2 weeks ago with a mild viral upper respiratory tract infection. However, he presents today with fever, ataxia, weakness, headache, and vomiting. In the office, he experiences a 3-minute left-sided tonic-clonic seizure. After sending him to the hospital and ordering a magnetic resonance imaging (MRI) with contrast of the brain, the results of which are shown, his likely diagnosis is which of the following?

Acute disseminated encephalomyelitis (ADEM). ***Acute disseminated encephalomyelitis (ADEM)*** - This condition typically presents with **acute neurological deficits** (ataxia, weakness, seizures) following a **viral illness** (URI 2 weeks prior) in children. - MRI findings in ADEM often show **widespread, inflammatory, demyelinating lesions**, consistent with the described image in the context of post-infectious neurological symptoms. *Malignant astrocytoma* - While it can cause neurological deficits and seizures, a malignant astrocytoma would typically manifest as a **single, discrete mass lesion** on MRI with significant mass effect. - The rapid onset of widespread symptoms after a viral illness is less characteristic of a primary brain tumor. *Bacterial meningitis* - This would present with more prominent signs of infection such as **high-grade fever**, nuchal rigidity, and altered mental status, and the MRI would likely show **leptomeningeal enhancement** or hydrocephalus, not widespread demyelinating lesions. - The antecedent viral illness and specific neurological symptom profile are more suggestive of a post-infectious demyelinating process. *Multiple sclerosis (MS)* - While MS is a demyelinating disease, it is far **less common in a 7-year-old child** with such an acute presentation following a viral infection. - MS typically has a **relapsing-remitting course** with lesions evolving over time, and usually presents in young adults.

Q: The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?

Normal head circumference. ***Normal head circumference*** - **Raised intracranial tension (RIC)** in infants often leads to an **increased head circumference** if the sutures have not yet fused, making a normal circumference *less likely* for RIC. - A persistent increase in head circumference is a key indicator of **hydrocephalus** or other conditions causing RIC in infants. *Bulging fontanel* - A **full or bulging fontanel** is a classic sign of RIC in infants because the open fontanelle provides a direct route for pressure to manifest. - This occurs due to increased pressure within the skull pushing the brain and cerebrospinal fluid outwards. *Papilledema* - **Papilledema**, or swelling of the optic disc, indicates increased pressure transmitted to the optic nerve. - While it can be harder to detect in infants than in older children, it is a significant sign of RIC when present. *Vomiting* - **Vomiting**, especially projectile vomiting without associated nausea, is a common non-specific symptom of RIC in infants and children. - This is thought to be due to pressure on the **brainstem's emetic center**.

Q: Which of the following is the most common cause of a bulging anterior fontanelle in infants?

Hydrocephalus. ***Hydrocephalus*** - **Hydrocephalus** is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the brain’s ventricles, leading to increased intracranial pressure. - This increased pressure frequently manifests as a **bulging anterior fontanelle** in infants due to the expandable nature of their skull. *Bacterial Meningitis* - While **bacterial meningitis** can cause a bulging fontanelle due to inflammation and increased intracranial pressure, it is typically accompanied by other severe symptoms like fever, lethargy, and nuchal rigidity, which are not mentioned as the most common cause in isolation. - Meningitis is a serious, acute infection, often less common than hydrocephalus as a primary cause for a bulging fontanelle. *Subdural Hematoma* - A **subdural hematoma** is a collection of blood between the dura mater and arachnoid mater, usually resulting from trauma. - While it can cause increased intracranial pressure and a bulging fontanelle, it is not the most common cause and would be associated with a history of injury or specific neurological deficits. *Benign Intracranial Hypertension* - **Benign intracranial hypertension (BIH)**, also known as pseudotumor cerebri, is rare in infants and typically affects older children or adults. - It usually presents with symptoms like headache and visual disturbances, which are not primary features of an isolated bulging fontanelle in an infant.

Q: Febrile seizures occur most frequently in children of which age group?

Between six months and five years of age. ***Between six months and five years of age*** - Febrile seizures primarily occur in this age range when the developing brain is most susceptible to fever-induced neuronal hyperexcitability. - This period aligns with the peak incidence of common childhood infections that cause fever. *In the first month of life* - Seizures in the **neonatal period** (first month) are typically due to serious underlying neurological conditions, infections, or metabolic disturbances, rather than simple fever. - The brain's thermoregulatory mechanisms and seizure thresholds are different in neonates. *In the first six months of life* - While possible, febrile seizures are **less common** during the initial six months of life compared to the 6-month to 5-year window. - Seizures during this early period often warrant a more thorough investigation for other etiologies. *Between five and ten years of age* - The incidence of febrile seizures significantly **decreases** after age five, as the brain matures and becomes less prone to seizure activity in response to fever. - Seizures occurring in this age group, especially if recurrent with fever, might suggest an underlying epilepsy syndrome.

Q: Which of the following is a symptom of cerebral palsy?

Ataxia. ***Ataxia*** - **Ataxia** (lack of voluntary coordination of muscle movements) is one of the primary **motor symptoms of cerebral palsy**, specifically seen in **ataxic cerebral palsy** which accounts for 5-10% of CP cases. - Ataxic CP presents with **poor coordination, tremors, and balance difficulties**, representing a distinct motor presentation pattern. - It is a direct neurological symptom resulting from **cerebellar or basal ganglia involvement**. *Hypotonia* - **Hypotonia** (decreased muscle tone) can occur in cerebral palsy, particularly as an **early finding in infants**. - However, hypotonia often **evolves into other motor patterns** (spasticity, dyskinesia) as the child develops, making it less specific as a defining symptom. - While present in some forms, it's more transitional than a consistent motor symptom across CP types. *Microcephaly* - **Microcephaly** (abnormally small head) is not a symptom of cerebral palsy itself, but rather a potential **associated condition or underlying cause**. - It suggests **abnormal brain development** which *could* lead to cerebral palsy, rather than being a neurological motor symptom *of* CP. *Flaccid paralysis* - **Flaccid paralysis** involves complete **loss of muscle tone and voluntary movement**, characteristic of **lower motor neuron lesions**, **spinal cord injury**, or certain **neuromuscular diseases**. - Cerebral palsy is an **upper motor neuron disorder** typically presenting with **spasticity, dyskinesia, or ataxia**, rather than pure flaccid paralysis.

Q: The drug of choice for infantile spasms in a patient with tuberous sclerosis is:

Vigabatrin. ***Vigabatrin*** - **Vigabatrin** is considered the **first-line treatment** for **infantile spasms** associated with **tuberous sclerosis complex (TSC)**. - Its mechanism of action involves **irreversibly inhibiting GABA transaminase**, thereby increasing GABA levels in the brain. - It has shown **superior efficacy** in TSC-related infantile spasms compared to other antiepileptic drugs, with response rates of 70-95% in this specific population. - While ACTH (adrenocorticotropic hormone) is an alternative first-line agent for non-TSC infantile spasms, vigabatrin is specifically preferred in TSC due to its effectiveness and the underlying pathophysiology. *Lamotrigine* - While an effective **antiepileptic drug** for various seizure types, **lamotrigine** is not the primary choice for infantile spasms, especially in the context of TSC. - It works by blocking **voltage-gated sodium channels** and modulating calcium channels, but its efficacy for infantile spasms is generally lower than first-line options. - Lamotrigine is more commonly used for focal seizures and generalized tonic-clonic seizures. *Levetiracetam* - **Levetiracetam** is a broad-spectrum antiepileptic drug, but it is typically used as a **second-line or adjunctive therapy** for infantile spasms. - Its primary mechanism involves binding to the **synaptic vesicle protein 2A (SV2A)**, modulating neurotransmitter release. - While well-tolerated, it has not demonstrated the same level of efficacy as vigabatrin or ACTH for infantile spasms. *Tiagabine* - **Tiagabine** works by blocking the reuptake of **GABA** through GABA transporter 1 (GAT-1), thereby increasing GABAergic transmission. - It is **not indicated for infantile spasms** and is primarily used for focal seizures. - Tiagabine can sometimes precipitate or worsen non-convulsive status epilepticus and spike-wave stupor, making it inappropriate for this condition.

Q: What is the first-line treatment for West syndrome in a child with Tuberous Sclerosis?

Vigabatrin. ***Vigabatrin*** - For infants with **West syndrome** and confirmed **tuberous sclerosis complex (TSC)**, **vigabatrin** is the recommended first-line treatment. - It targets the **GABAergic system** and is particularly effective due to its mechanism of action related to the pathogenesis of seizures in TSC. *Valproate* - While a broad-spectrum **antiepileptic drug**, **valproate** is not the first-line choice for West syndrome, especially in the context of TSC. - It is associated with potential adverse effects, including **hepatic toxicity**, which can be a concern in young children. *ACTH* - **Adrenocorticotropic hormone (ACTH)** is a highly effective treatment for **West syndrome** in general, especially when the etiology is unknown or not related to TSC. - However, due to its significant side effects and the specific efficacy of vigabatrin in TSC, **ACTH** is typically considered second-line for TSC-associated West syndrome. *Lamotrigine* - **Lamotrigine** is an antiepileptic drug that is generally not used as a first-line treatment for **West syndrome**, regardless of the underlying etiology. - It can sometimes **exacerbate infantile spasms** in some cases, making it an unsuitable initial choice.

Question 1

The 7-year-old boy, who is now in your office, was last seen 2 weeks ago with a mild viral upper respiratory tract infection. However, he presents today with fever, ataxia, weakness, headache, and vomiting. In the office, he experiences a 3-minute left-sided tonic-clonic seizure. After sending him to the hospital and ordering a magnetic resonance imaging (MRI) with contrast of the brain, the results of which are shown, his likely diagnosis is which of the following?

Accepted Answer

Acute disseminated encephalomyelitis (ADEM)

Answer

Malignant astrocytoma

Answer

Bacterial meningitis

Answer

Multiple sclerosis (MS)

Question 2

The following are recognized signs and symptoms of raised intracranial tension in a 9-month-old infant, except which of the following?

Accepted Answer

Normal head circumference

Answer

Vomiting

Answer

Papilledema

Answer

Bulging fontanel

Question 3

Which of the following is the most common cause of a bulging anterior fontanelle in infants?

Accepted Answer

Hydrocephalus

Answer

Benign Intracranial Hypertension

Answer

Subdural Hematoma

Answer

Bacterial Meningitis

Question 4

Febrile seizures occur most frequently in children of which age group?

Accepted Answer

Between six months and five years of age

Answer

In the first month of life

Answer

In the first six months of life

Answer

Between five and ten years of age

Question 5

Which of the following is a symptom of cerebral palsy?

Accepted Answer

Ataxia

Answer

Microcephaly

Answer

Hypotonia

Answer

Flaccid paralysis

Question 6

The drug of choice for infantile spasms in a patient with tuberous sclerosis is:

Accepted Answer

Vigabatrin

Answer

Lamotrigine

Answer

Levetiracetam

Answer

Tiagabine

Question 7

What is the first-line treatment for West syndrome in a child with Tuberous Sclerosis?

Accepted Answer

Vigabatrin

Answer

Valproate

Answer

ACTH

Answer

Lamotrigine

Neurology — MCQs

Neurology — MCQs

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