Neurology Practice Questions

Q: First-line acute management of febrile convulsion in hospital setting includes

IV lorazepam administration. ***IV lorazepam administration*** - **Intravenous lorazepam** is a first-line treatment for acute febrile convulsions, especially in a hospital setting, due to its rapid onset and effectiveness in terminating seizures. - As a **benzodiazepine**, it enhances the effect of GABA, leading to CNS depression and seizure cessation. *Immediate IV phenytoin loading* - **Phenytoin** is typically used for long-term seizure control or in refractory status epilepticus, not as a first-line acute agent for febrile convulsions. - Its slower onset of action makes it less suitable for immediate seizure termination compared to benzodiazepines. *Rectal diazepam* - **Rectal diazepam** is often used for acute seizure management in the **pre-hospital setting** or at home when IV access is not readily available. - In a hospital setting, IV administration of benzodiazepines is preferred due to more precise dosing and faster action. *Oral carbamazepine prophylaxis* - **Carbamazepine** is an anti-epileptic drug used for **long-term prevention** of certain types of seizures, but it is not indicated for acute management of febrile convulsions. - It is not routinely recommended for prophylaxis of recurrent febrile seizures.

Q: A mother brought her child who has got a vascular plaque like lesion over the lateral aspect of forehead mainly involving ophthalmic and maxillary division of trigeminal nerve. Mother says that the lesion has remained unchanged since birth. Also mother gives a history that the child is on valproate for seizure disorder. What is the MOST probable diagnosis?

Sturge weber syndrome. ***Sturge weber syndrome*** - The classic presentation of a **vascular plaque-like lesion** (port-wine stain) in the distribution of the **trigeminal nerve** (ophthalmic and maxillary divisions) from birth, combined with a history of **seizure disorder**, strongly points to Sturge-Weber syndrome. - This neurocutaneous disorder is characterized by a **facial cutaneous angioma**, **leptomeningeal angioma** (leading to seizures), and often **ocular involvement** like glaucoma. *Infantile hemangioma* - While also a vascular lesion, **infantile hemangiomas** typically proliferate in the first few months of life and then involute, rather than being present as a stable "plaque" from birth. - They also don't typically follow a specific trigeminal nerve distribution and are not directly associated with a primary **seizure disorder** as a core feature. *Tuberous sclerosis* - Tuberous sclerosis presents with characteristic clinical features, including **facial angiofibromas** (adenoma sebaceum), **shagreen patches**, and **ash-leaf spots**, which are distinct from a flat vascular plaque. - Although seizures are common in tuberous sclerosis due to **cortical tubers**, the facial skin lesion described does not fit the typical dermatological manifestations of this condition. *Incontinentia pigmenti* - This condition presents with highly characteristic **skin lesions** that evolve through distinct stages, including vesicular, verrucous, and hyperpigmented (swirl-like patterns), which do not match the description of a vascular plaque. - While it can be associated with neurological issues like **seizures**, the dermatological findings are the primary differentiating factor here.

Q: MRI scan of a child with intellectual disability, recurrent seizures and hemangioma. Diagnosis is?

Encephalofacial angiomatosis. ***Encephalofacial angiomatosis*** - This syndrome, also known as **Sturge-Weber syndrome**, is characterized by a "port-wine stain" **facial hemangioma**, leptomeningeal angioma, and ocular involvement. The combination of mental retardation, recurrent seizures, and hemangioma points directly to this diagnosis. - The **leptomeningeal angioma** often calcifies ("tram-track" calcifications) and can be seen on imaging, contributing to the neurological symptoms like seizures and mental retardation. *Epiloias* - Epiloias is another name for **Tuberous Sclerosis Complex**, which presents with facial angiofibromas (not hemangiomas), hypopigmented macules, and neurological symptoms such as seizures and mental retardation. - Imaging typically shows **cortical tubers**, subependymal nodules, and subependymal giant cell astrocytomas, which are distinct from leptomeningeal angiomas. *Neuronal ceroid lipofuscinoses* - This is a group of **neurodegenerative lysosomal storage disorders** characterized by progressive intellectual and motor decline, seizures, and visual failure. - While it causes mental retardation and seizures, it does not involve angiomas or hemangiomas and has different imaging findings (cerebral atrophy, often with thalami and cerebellum spared initially). *Louis Bar syndrome* - Also known as **ataxia-telangiectasia**, this is a rare, neurodegenerative, inherited disease characterized by progressive cerebellar ataxia, **oculocutaneous telangiectasias**, and immunodeficiency. - While it can involve neurological symptoms, the key clinical features are ataxia and telangiectasias, not hemangiomas or the specific imaging findings associated with Sturge-Weber syndrome.

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.

Question 1

First-line acute management of febrile convulsion in hospital setting includes

Accepted Answer

IV lorazepam administration

Answer

Immediate IV phenytoin loading

Answer

Rectal diazepam

Answer

Oral carbamazepine prophylaxis

Question 2

A mother brought her child who has got a vascular plaque like lesion over the lateral aspect of forehead mainly involving ophthalmic and maxillary division of trigeminal nerve. Mother says that the lesion has remained unchanged since birth. Also mother gives a history that the child is on valproate for seizure disorder. What is the MOST probable diagnosis?

Accepted Answer

Sturge weber syndrome

Answer

Infantile hemangioma

Answer

Tuberous sclerosis

Answer

Incontinentia pigmenti

Question 3

MRI scan of a child with intellectual disability, recurrent seizures and hemangioma. Diagnosis is?

Accepted Answer

Encephalofacial angiomatosis

Answer

Epiloias

Answer

Neuronal ceroid lipofuscinoses

Answer

Louis Bar syndrome

Question 4

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 5

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 6

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 7

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 8

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 9

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 10

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

Neurology — MCQs

Neurology — MCQs

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