Neurology Practice Questions

Q: Child was seen taking off clothes while watching TV; he suddenly closed eyes for some time and was lethargic after some time. He had probably

Temporal lobe epilepsy. ***Temporal lobe epilepsy*** - The behavior described, such as **taking off clothes** (automatisms) and subsequent **lethargy** (postictal state), is highly characteristic of a **complex partial seizure**, which often originates in the temporal lobe. - **Eyes closing** during the event and partial awareness suggest that the seizure involved altered consciousness without a complete loss of awareness, typical of focal seizures with impaired awareness. *febrile seizures* - Febrile seizures are typically generalized tonic-clonic seizures that occur in children with a **fever**, which is not mentioned in the scenario. - The described actions like taking off clothes and sudden eye closure are not typical presentations of a classic febrile seizure. *Absence seizures* - Absence seizures involve **brief staring spells** and a momentary loss of consciousness, without the complex motor behaviors (automatisms) like taking off clothes. - There is typically no significant postictal lethargy associated with absence seizures; the child usually resumes activity immediately after the event. *GTCS* - **Generalized tonic-clonic seizures (GTCS)** involve a complete loss of consciousness, followed by tonic (stiffening) and clonic (jerking) phases, often with a loud cry and tongue biting. - While GTCS can lead to postictal lethargy, the specific automatisms (taking off clothes) and the partial impairment of consciousness are more indicative of a focal seizure.

Q: Give the most probable diagnosis of a 1 yr. old child of normal intelligence with features of hypotonia. On examination there are tongue fasciculations and he keeps his body in a frog like position ?

Spinal muscular atrophy. ***Spinal muscular atrophy*** - The combination of **hypotonia**, **normal intelligence**, **tongue fasciculations**, and a **frog-leg position** in an infant is highly characteristic of **Spinal Muscular Atrophy (SMA)**, particularly Type 1 (Werdnig-Hoffmann disease). - **Tongue fasciculations** are a key diagnostic sign resulting from denervation of the tongue muscles, and the **frog-leg position** is a compensatory posture due to proximal muscle weakness. *Guillain-Barré Syndrome* - **Guillain-Barré Syndrome (GBS)** is an acute, post-infectious demyelinating polyneuropathy that typically presents with **rapidly progressive ascending paralysis** and areflexia. - While GBS can cause hypotonia, it is rare in infants under 1 year of age and usually presents with an acute onset, unlike the more chronic presentation implied by the frog-leg position often seen in SMA. *Limb girdle atrophy* - **Limb-girdle muscular dystrophies** typically manifest later in childhood or adolescence, not usually in a 1-year-old infant. - These conditions primarily affect the **proximal muscles** of the shoulders and hips, causing weakness, but **tongue fasciculations** are not a typical feature. *Down's syndrome* - **Down syndrome** is characterized by distinctive **facial features**, intellectual disability, and often congenital heart defects, which are not mentioned here. - While **hypotonia** is common in Down syndrome, **tongue fasciculations** are not a feature, and intelligence is not normal.

Q: A 9 year old female patient has come for routine dental examination. She is unable to articulate her words properly. General examination reveals muscle weakness and stiffness, irregular gait, uncoordinated and involuntary movements, chewing and swallowing difficulties as well as speech problems. Which of the following may not be an etiologic factor for this condition?

Hypothyroidism. ***Hypothyroidism*** - While **hypothyroidism** can cause developmental delays and neurological symptoms, it typically does not present with the specific constellation of **uncoordinated, involuntary movements** and **stiffness (spasticity)** characteristic of cerebral palsy. - The symptoms described are highly suggestive of **cerebral palsy**, and hypothyroidism is not considered a direct etiologic factor for this condition. *Head trauma* - **Head trauma**, particularly during infancy or childhood, can lead to **brain damage** that manifests as cerebral palsy-like symptoms. - The resulting neurological injury can cause **muscle weakness**, **spasticity**, and **uncoordinated movements**. *Premature birth* - **Premature birth** is a significant risk factor for **cerebral palsy** due to the increased vulnerability of the developing brain to injury before full term. - Complications such as **intraventricular hemorrhage** or **periventricular leukomalacia** are common in premature infants and can lead to the described neurological deficits. *Meningitis* - **Meningitis**, an infection of the membranes surrounding the brain and spinal cord, can cause **brain damage** and lead to long-term neurological sequelae. - The inflammation and potential for **ischemia** or **infarction** can result in motor deficits, **spasticity**, and **speech problems**, consistent with cerebral palsy.

Q: A 9 year old female patient has come for a routine dental examination. She is unable to articulate her words properly. General examination reveals muscle weakness and stiffness, irregular gait, uncoordinated and involuntary movements, chewing and swallowing difficulties as well as speech problems. Which of the following reflexes maybe absent in this patient?

Parachute reflex. ***Parachute reflex*** - The clinical presentation describes **Cerebral Palsy (CP)** with motor impairment, dysarthria, and dysphagia. - The **parachute reflex** is a **protective reflex** that appears at 8-9 months and should **persist throughout life**. - It involves automatic extension of arms when the child is tilted forward, protecting against falls. - In children with CP, this protective reflex may be **absent or impaired**, indicating significant motor dysfunction affecting protective responses. - At 9 years of age, absence of this reflex is **abnormal and clinically significant**. *Tonic labyrinthine reflex* - This is a **primitive reflex** present at birth that normally **integrates (disappears) by 4-6 months** of age. - Its **absence** at 9 years would be **normal** (it should already be gone). - In CP, the problem is **persistence** of this reflex beyond infancy, not its absence, leading to extensor or flexor tone changes with head position. *Startle reflex* - The startle reflex (Moro reflex in infants) is a **primitive reflex** that should **integrate by 3-6 months** of age. - Its **absence** at 9 years would be **normal and expected**. - In CP, the abnormality is **persistence** or exaggerated responses, not absence, contributing to hypertonicity and exaggerated startle responses. *Asymmetric tonic neck reflex (ATNR)* - This **primitive reflex** (fencing posture) should **integrate by 4-6 months** of age. - Its **absence** at 9 years would be **normal** (it should already be integrated). - In CP, the problem is **persistence** of ATNR beyond infancy, not its absence, which interferes with midline activities, rolling, and hand-to-mouth coordination.

Q: Febrile seizure persisting continuously beyond ____ should be treated with midazolam nasal spray:

5 minutes. ***5 minutes*** - Febrile seizures lasting longer than **5 minutes** are considered **prolonged** and require immediate intervention to prevent progression to status epilepticus. - **Midazolam nasal spray** is a convenient and effective first-line treatment for prolonged or status epilepticus due to its rapid absorption and anticonvulsant properties. *3 minutes* - While it's important to monitor febrile seizures, 3 minutes is generally too short a duration to initiate emergency medication for treatment. - Many febrile seizures self-terminate within this timeframe, and medical intervention is typically reserved for longer-lasting events. *10 minutes* - Waiting until 10 minutes to administer medication for a prolonged febrile seizure is generally too long and increases the risk of complications such as **status epilepticus** and potential neuronal damage. - Prompt intervention between 5-10 minutes is recommended to prevent these adverse outcomes. *8 minutes* - Similar to waiting 10 minutes, waiting 8 minutes to administer medication for a prolonged febrile seizure is generally too long. - Early intervention after the **5-minute mark** is crucial to improve outcomes and prevent further seizure activity.

Q: Which of the following is true about febrile convulsions?

Follows high temperature. ***Follows high temperature*** - Febrile convulsions are **directly associated with fever** and occur during febrile illness, typically when body temperature rises above **38°C (100.4°F)**. - By definition, a febrile seizure occurs in children aged **6 months to 5 years** during a **febrile illness** in the absence of CNS infection or other defined cause. - The seizure may occur during the **rising phase of fever** or when the temperature is already elevated, making this statement the most accurate characterization. *Recurrent in nature* - While febrile convulsions CAN recur, only approximately **30-40% of children** experience recurrent episodes. - The **majority (60-70%)** of children have only a **single episode**, so stating they are "recurrent in nature" is inaccurate. - Risk factors for recurrence include: young age at first episode, family history, and brief duration between fever onset and seizure. *No spontaneous remission* - This is incorrect; febrile convulsions have **excellent prognosis** with spontaneous resolution. - Individual episodes typically last only **a few minutes** and resolve spontaneously without intervention. - The condition itself remits as children grow older, with febrile seizures becoming **rare after age 5-6 years**. *Occurs at 6 years onwards* - This is incorrect; febrile convulsions occur in children between **6 months and 5 years of age**, with peak incidence around **18 months**. - Febrile seizures are **rare after age 6 years**, and a new-onset seizure with fever in an older child warrants investigation for other causes such as CNS infection or epilepsy.

Q: A 7-year old boy presents with a right-sided hemangioma and left-sided focal seizures. The most likely diagnosis is:

Sturge-Weber disease. ***Sturge-Weber disease*** - The classic presentation of a **facial hemangioma** (port-wine stain) and **focal seizures** on the contralateral side is highly characteristic of Sturge-Weber disease. - This neurocutaneous syndrome involves a **leptomeningeal angioma** on the same side as the facial hemangioma, leading to neurological symptoms. *Incontinentia pigmenti* - This X-linked dominant disorder primarily affects females and presents with **skin lesions** that evolve through vesicular, verrucous, and hyperpigmented stages, often in a **linear pattern**. - While it can cause neurological symptoms, a unilateral hemangioma and contralateral focal seizures are not typical. *Neurofibromatosis* - Neurofibromatosis Type 1 (NF1) is characterized by **café-au-lait spots**, **axillary/inguinal freckling**, and **neurofibromas**. - While seizures can occur, the combination of a unilateral hemangioma and contralateral focal seizures is not the hallmark presentation of neurofibromatosis. *Hypermelanosis of Ito* - This condition is characterized by **streaky or whorled hyperpigmentation** patterns on the skin, often following Blaschko's lines. - While neurological abnormalities can be associated, the primary cutaneous manifestation is diffuse hyperpigmentation, not a focal hemangioma.

Q: All are characteristic features of cerebral palsy except

Erb's palsy. ***Erb's palsy*** - **Erb's palsy** is a form of brachial plexus palsy, characterized by injury to the **upper brachial plexus** (C5-C6 nerve roots), typically occurring during birth. - It results in a characteristic **"waiter's tip" position** of the arm and is a distinct peripheral nerve injury, not a characteristic feature of **cerebral palsy**, which is a central neurological disorder. *Hypotonia* - While many forms of cerebral palsy present with **spasticity**, some individuals, particularly those with **ataxic cerebral palsy** or specific types of dyskinetic cerebral palsy, can exhibit **hypotonia** (low muscle tone). - Hypotonia can also be an early manifestation before the development of more prominent hypertonia or spasticity, making it an associated feature. *Epilepsy* - **Epilepsy** and seizure disorders are common co-morbidities seen in children with **cerebral palsy**, particularly in those with severe brain damage or certain types of CP. - The underlying brain injury that causes cerebral palsy can also disrupt normal electrical activity in the brain, leading to seizures. *Spasticity* - **Spasticity** is the most common motor type of **cerebral palsy**, affecting approximately 80% of individuals. - It is characterized by **increased muscle tone** and **hyperreflexia**, resulting in stiff, tight muscles and exaggerated reflexes, due to damage to the motor cortex or pyramidal tracts.

Q: Which of the following is NOT true regarding rheumatic chorea in children?

Within 8-12 weeks of disease. ***Within 8-12 weeks of disease*** - Rheumatic chorea, or **Sydenham's chorea**, typically manifests several weeks to months (often 1-6 months) after the initial **Group A Streptococcus (GAS)** infection. - It is not usually limited to an 8-12 week timeframe from the onset of the entire rheumatic fever disease process but rather represents a delayed manifestation. *Chorea disappears during sleep* - This is a true characteristic of Sydenham's chorea and other forms of chorea; the involuntary movements **cease during sleep**. - This cessation during sleep helps differentiate chorea from other movement disorders like some forms of **myoclonus** or tics, which may persist. *Remits spontaneously* - Sydenham's chorea is generally **self-limited**, with symptoms often resolving spontaneously over weeks to months, although resolution can take up to a year or more. - While it may recur in some cases, the natural course is one of **spontaneous remission**. *Rapid jerky movements of distal extremities* - Sydenham's chorea is characterized by **involuntary, abrupt, purposeless, and rapid jerky movements**, which are often more prominent in the distal extremities and face. - These movements can involve any part of the body, leading to difficulties with coordination, gait, and fine motor tasks.

Q: Which of the following is the most common neurocutaneous syndrome?

Neurofibromatosis. ***Neurofibromatosis*** - **Neurofibromatosis type 1 (NF1)**, also known as von Recklinghausen disease, is the most common of all neurocutaneous syndromes, affecting about 1 in 3,000 live births. - It is characterized by **café-au-lait spots**, **axillary/groin freckling**, **neurofibromas**, **Lisch nodules**, and optic pathway gliomas. *Sturge Weber syndrome* - This syndrome is characterized by a **port-wine stain (facial angioma)**, leptomeningeal angioma, and glaucoma; it is less common than neurofibromatosis. - It is a **sporadic disorder** and does not show an inherited pattern like neurofibromatosis. *Von Hippel Lindau disease* - This is a rare, **autosomal dominant** inherited disorder characterized by various tumors such as **hemangioblastomas** in the cerebellum, retina, and spinal cord, as well as renal cysts and pheochromocytomas. - Its prevalence is significantly lower than neurofibromatosis. *Tuberous sclerosis* - This is an **autosomal dominant** disorder marked by the growth of **benign tumors** in the brain, heart, kidneys, skin, and other organs, such as **facial angiofibromas** and **epilepsy**. - While significant, its incidence is approximately 1 in 6,000 to 1 in 10,000 live births, making it less common than neurofibromatosis.

Question 1

Child was seen taking off clothes while watching TV; he suddenly closed eyes for some time and was lethargic after some time. He had probably

Accepted Answer

Temporal lobe epilepsy

Answer

febrile seizures

Answer

Absence seizures

Answer

GTCS

Question 2

Give the most probable diagnosis of a 1 yr. old child of normal intelligence with features of hypotonia. On examination there are tongue fasciculations and he keeps his body in a frog like position ?

Accepted Answer

Spinal muscular atrophy

Answer

Guillain-Barré Syndrome

Answer

Limb girdle atrophy

Answer

Down's syndrome

Question 3

A 9 year old female patient has come for routine dental examination. She is unable to articulate her words properly. General examination reveals muscle weakness and stiffness, irregular gait, uncoordinated and involuntary movements, chewing and swallowing difficulties as well as speech problems. Which of the following may not be an etiologic factor for this condition?

Accepted Answer

Hypothyroidism

Answer

Head trauma

Answer

Premature birth

Answer

Meningitis

Question 4

A 9 year old female patient has come for a routine dental examination. She is unable to articulate her words properly. General examination reveals muscle weakness and stiffness, irregular gait, uncoordinated and involuntary movements, chewing and swallowing difficulties as well as speech problems. Which of the following reflexes maybe absent in this patient?

Accepted Answer

Parachute reflex

Answer

Tonic labyrinthine reflex

Answer

Startle reflex

Answer

Asymmetric tonic neck reflex

Question 5

Febrile seizure persisting continuously beyond ____ should be treated with midazolam nasal spray:

Accepted Answer

5 minutes

Answer

3 minutes

Answer

10 minutes

Answer

8 minutes

Question 6

Which of the following is true about febrile convulsions?

Accepted Answer

Follows high temperature

Answer

Recurrent in nature

Answer

Occurs at 6 years onwards

Answer

No spontaneous remission

Question 7

A 7-year old boy presents with a right-sided hemangioma and left-sided focal seizures. The most likely diagnosis is:

Accepted Answer

Sturge-Weber disease

Answer

Incontinentia pigmenti

Answer

Neurofibromatosis

Answer

Hypermelanosis of Ito

Question 8

All are characteristic features of cerebral palsy except

Accepted Answer

Erb's palsy

Answer

Hypotonia

Answer

Epilepsy

Answer

Spasticity

Question 9

Which of the following is NOT true regarding rheumatic chorea in children?

Accepted Answer

Within 8-12 weeks of disease

Answer

Chorea disappears during sleep

Answer

Remits spontaneously

Answer

Rapid jerky movements of distal extremities

Question 10

Which of the following is the most common neurocutaneous syndrome?

Accepted Answer

Neurofibromatosis

Answer

Sturge Weber syndrome

Answer

Von Hippel Lindau disease

Answer

Tuberous sclerosis

Neurology — MCQs

Neurology — MCQs

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