Neurology Practice Questions

Q: A child presents with ascending flaccid paralysis. There is subsequent respiratory muscle involvement. CSF examination shows albuminocytological dissociation. Treatment of choice is:

I.V. immunoglobulins. ***I.V. immunoglobulins*** - **Intravenous immunoglobulins (IVIg)** are a first-line treatment for **Guillain-Barré syndrome (GBS)**, which is strongly indicated by **ascending flaccid paralysis**, respiratory muscle involvement, and **albuminocytological dissociation** in CSF. - IVIg works by neutralizing pathogenic antibodies, modulating complement activation, and blocking Fc receptors, thereby reducing the autoimmune attack on peripheral nerves. *Cycloserine* - **Cycloserine** is an antibiotic primarily used to treat **tuberculosis**, especially in drug-resistant cases. - It has no role in the treatment of autoimmune neurological conditions like Guillain-Barré syndrome. *Oral prednisolone* - While corticosteroids like **prednisolone** are potent anti-inflammatory and immunosuppressive agents, they have been shown to be **ineffective** and potentially harmful in GBS. - Studies have demonstrated that oral steroids do not improve outcomes and may even prolong recovery in GBS. *I.V. methylprednisolone* - Similar to oral prednisolone, **intravenous methylprednisolone** is a corticosteroid that has **not been shown to be beneficial** in treating GBS. - High-dose corticosteroids are generally avoided in GBS due to lack of efficacy and potential side effects.

Q: What is the most common cause of hydrocephalus in children?

Congenital aqueductal stenosis. ***Congenital aqueductal stenosis*** - This is the **most common cause of hydrocephalus in children**, accounting for the majority of congenital hydrocephalus cases. - The **cerebral aqueduct** (connecting the third and fourth ventricles) is narrowed or blocked, preventing normal CSF flow. - Results in **obstructive hydrocephalus** with progressive ventricular enlargement. - Clinical presentation varies from neonatal period to childhood depending on severity. *Post inflammatory obstruction* - An important cause of **acquired hydrocephalus**, typically following meningitis or intraventricular hemorrhage. - Inflammation leads to **fibrosis and scarring** of CSF pathways, particularly affecting the arachnoid villi and basal cisterns. - More common in developing countries with higher rates of CNS infections. - While significant, it is not the overall most common cause in children. *Brain tumour* - Tumors can cause hydrocephalus by **obstructing CSF pathways**, particularly posterior fossa tumors blocking the fourth ventricle. - Common tumor types include medulloblastoma, ependymoma, and cerebellar astrocytoma. - Represents a **less common cause** compared to congenital malformations. *Perinatal injury* - Includes **intraventricular hemorrhage (IVH)** in premature infants and birth trauma. - IVH can lead to post-hemorrhagic hydrocephalus through blood clot obstruction or subsequent inflammation. - More relevant in **premature and low birth weight infants**, but not the most common cause overall.

Q: In a child with Rett syndrome, all of the following are seen except:

Macrocephaly. ***Macrocephaly*** - Rett syndrome is typically associated with **microcephaly**, not macrocephaly, due to slowed head growth after birth. - The brain size is smaller than average, reflecting impaired neural development. *Intellectual disability* - **Severe intellectual disability** is a core feature of Rett syndrome, as affected individuals experience significant cognitive impairment. - This cognitive decline often becomes apparent after a period of normal development. *Abnormal dendritic connections* - **Abnormal dendritic connections** and reduced dendritic complexity are characteristic neuropathological findings in Rett syndrome. - These structural changes in neurons contribute to the neurological dysfunction observed in the disorder. *Seizures* - **Seizures** are a very common symptom in children with Rett syndrome, affecting a majority of individuals at some point. - The type and frequency of seizures can vary widely among patients.

Q: A 1-year-old child presented with myoclonic jerks. What does the EEG show?

Hypsarrhythmia. ***Hypsarrhythmia*** - The EEG image displays a chaotic, high-amplitude, and disorganized background activity with multifocal spikes and sharp waves, which are characteristic features of **hypsarrhythmia**. - This pattern is classically associated with **West syndrome** (infantile spasms), which presents clinically with myoclonic jerks and developmental regression in infants, aligning with the 1-year-old child's presentation. *Normal record* - A normal EEG in a 1-year-old child would show organized background activity with appropriate developmental features for their age, lacking the pronounced disorganization seen here. - The presence of myoclonic jerks also makes a normal EEG an unlikely finding, as these are paroxysmal events suggestive of underlying brain dysfunction. *Periodic spike-wave pattern* - **Periodic spike-wave patterns** typically involve rhythmic, generalized discharge complexes occurring at regular intervals (e.g., 3 Hz spike-and-wave) and are characteristic of absence seizures or generalized tonic-clonic seizures. - The pattern observed in the image is much more irregular, chaotic, and lacks the distinct periodicity and morphology associated with typical spike-wave complexes. *Burst suppression pattern* - **Burst suppression** is characterized by bursts of high-amplitude generalized activity alternating with periods of profound reduction or suppression of electrical activity. - This pattern is seen in severe encephalopathies, deep anesthesia, or certain severe epilepsy syndromes, but it involves clear periods of suppression which are absent in the provided chaotic EEG.

Q: Which of the following is NOT a feature of Moebius syndrome?

Decreased chest movements. ***Decreased chest movements*** - **Decreased chest movements** are not a characteristic feature of **Moebius syndrome**, which primarily affects cranial nerves, particularly the **facial and abducens nerves**. - While other systemic issues can coexist, respiratory problems like decreased chest movements are not considered a direct or defining symptom of this condition. *Bilateral facial paralysis* - **Bilateral facial paralysis** is a hallmark of **Moebius syndrome**, resulting from congenital underdevelopment or absence of the **facial (VII) cranial nerves**. - This leads to a characteristic **mask-like facial expression**, difficulty with smiling, frowning, and other facial movements. *Impaired lateral eye movement* - **Impaired lateral eye movement** is a common feature due to involvement of the **abducens (VI) cranial nerves**, which control the **lateral rectus muscle**. - Patients often present with **esotropia** (crossed eyes) and are unable to move their eyes past the midline when looking to the side. *Unilateral or bilateral abducens nerve involvement* - **Unilateral or bilateral abducens (VI) nerve involvement** is a core diagnostic criterion for **Moebius syndrome**. - This leads to the characteristic deficit in **lateral gaze**, as the abducens nerve innervates the **lateral rectus muscle**.

Q: A tuft of hair over the lumbosacral region in a newborn is most commonly suggestive of:

Spina bifida occulta. ***Spina bifida occulta*** - A **lumbosacral hair tuft** (hypertrichosis) is a classic **cutaneous stigmata** indicating underlying **spinal dysraphism**. - **Spina bifida occulta** is the **most common association** with this finding—it represents incomplete fusion of the vertebral arches without herniation of neural elements. - This is often asymptomatic but requires **imaging evaluation** (ultrasound in infants <6 months, MRI if older) to rule out tethered cord or other occult spinal anomalies. - Other cutaneous markers include dimples (above gluteal crease), lipomas, hemangiomas, and dermal sinuses. *Tumor* - While subcutaneous tumors like **lipomas** or **lipomyelomeningoceles** can present with overlying hair tufts, they are typically palpable masses and less common than spina bifida occulta. - These would also fall under the spectrum of spinal dysraphism requiring imaging. *Sinus tract* - A **dermal sinus tract** can be associated with a hair tuft but more commonly presents with a visible **dimple or opening** above the gluteal crease. - This carries risk of CNS infection (meningitis) and requires surgical intervention. *Dermoid cyst* - Dermoid cysts can present with overlying skin changes but are not the most common cause of isolated hair tufts in the lumbosacral region.

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.

Question 1

A child presents with ascending flaccid paralysis. There is subsequent respiratory muscle involvement. CSF examination shows albuminocytological dissociation. Treatment of choice is:

Accepted Answer

I.V. immunoglobulins

Answer

Oral prednisolone

Answer

I.V. methylprednisolone

Answer

Cycloserine

Question 2

What is the most common cause of hydrocephalus in children?

Accepted Answer

Congenital aqueductal stenosis

Answer

Post inflammatory obstruction

Answer

Brain tumour

Answer

Perinatal injury

Question 3

In a child with Rett syndrome, all of the following are seen except:

Accepted Answer

Macrocephaly

Answer

Abnormal dendritic connections

Answer

Intellectual disability

Answer

Seizures

Question 4

A 1-year-old child presented with myoclonic jerks. What does the EEG show?

Accepted Answer

Hypsarrhythmia

Answer

Normal record

Answer

Periodic spike-wave pattern

Answer

Burst suppression pattern

Question 5

Which of the following is NOT a feature of Moebius syndrome?

Accepted Answer

Decreased chest movements

Answer

Bilateral facial paralysis

Answer

Impaired lateral eye movement

Answer

Unilateral or bilateral abducens nerve involvement

Question 6

A tuft of hair over the lumbosacral region in a newborn is most commonly suggestive of:

Accepted Answer

Spina bifida occulta

Answer

Dermoid cyst

Answer

Tumor

Answer

Sinus tract

Question 7

A 2-week-old female infant has a head circumference of 40 cm, which is greater than the 98th percentile, along with a large, tense fontanelle and downward deviation of the eyes. She has been vomiting her formula and is irritable. Which of the following is the least likely cause of her symptoms?

Accepted Answer

Intraventricular hemorrhage

Answer

Meningitis

Answer

Aqueductal stenosis

Answer

Brain tumor

Question 8

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 9

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 10

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

Neurology — MCQs

Neurology — MCQs

On this page

Practice by Chapter

Want unlimited practice?