Neurology Practice Questions

Q: A 25-year-old woman presents with intermittent double vision and fatigue. Her symptoms are worse at the end of the day. She reports no other focal muscle weakness or sensory symptoms. On examination her eye movements, motor strength, and reflexes in the upper and lower limbs are normal. Repetitive handgrip exercises cause loss of strength in the grip. A CXR reveals an anterior mediastinal mass. Which of the following is the most likely diagnosis of the anterior mediastinal mass?

thymoma. ***thymoma*** - The patient's symptoms of **intermittent double vision** and **fatigue, worsening at the end of the day**, are highly suggestive of **myasthenia gravis** [1]. - Approximately 15-20% of patients with myasthenia gravis have a **thymoma**, which is typically found as an **anterior mediastinal mass** on CXR [2]. *lymphoma* - While lymphoma can present as an anterior mediastinal mass, it is not typically associated with the specific neurological symptoms of **myasthenia gravis**. - Lymphoma often presents with systemic symptoms like **fever, night sweats, and weight loss**, which are not described here. *teratoma* - A teratoma is a germ cell tumor that can occur in the anterior mediastinum but does not typically cause **neuromuscular symptoms** like those seen in myasthenia gravis. - They can sometimes be associated with paraneoplastic syndromes, but not usually with symptoms directly pointing to **acetylcholine receptor antibodies** [2]. *thyroid* - An ectopic thyroid gland or thyroid mass in the mediastinum is rare and would not explain the patient's **myasthenic symptoms** [2]. - Thyroid masses are usually asymptomatic unless they cause **compression symptoms** or are hormonally active.

Q: Most common clinical presentation of juvenile myoclonic epilepsy -

Myoclonus. ***Myoclonus*** - **Juvenile myoclonic epilepsy (JME)** is classically characterized by sudden, brief, involuntary muscle jerks, particularly in the morning or upon awakening [1]. - These **myoclonic jerks** are often the first and most prominent symptom reported by patients. *Generalised tonic clonic seizures in awake state* - While **generalized tonic-clonic seizures (GTCS)** do occur in JME, they typically appear later in the disease course and are often triggered by sleep deprivation or alcohol [1]. - The initial and most common presentation, however, remains the **myoclonic jerks**. *Generalised tonic clonic seizures in sleep* - **GTCS** in JME are more common upon **awakening** rather than during sleep, and often follow initial myoclonic jerks [1]. - Seizures occurring predominantly during sleep would suggest other epilepsy syndromes. *Absence seizures* - **Absence seizures** are seen in other forms of generalized epilepsy, such as **childhood absence epilepsy**, but are not a primary or most common feature of JME [1, 2]. - While some patients with JME might experience absence-like phenomena, it's not the defining or most frequent presentation [1].

Q: Absence seizures are characterized on EEG by:

3 Hz spike and wave. ***3 Hz spike and wave*** - **Absence seizures** are classically characterized by a **generalized, synchronous 3 Hz spike-and-wave discharge** pattern on EEG. - This pattern is seen bilaterally and symmetrically, reflecting the generalized nature of the seizure. *Generalized polyspikes* - **Generalized polyspikes** (multiple spikes) are often associated with other types of generalized seizures, such as **myoclonic seizures**, rather than typical absence seizures. - While reflecting generalized activity, the specific **3 Hz spike-and-wave** is the hallmark of absence seizures. *Hypsarrhythmia* - **Hypsarrhythmia** is a chaotic, high-amplitude, irregular pattern seen in **infantile spasms** (West syndrome), not absence seizures. - It is characterized by random high-voltage slow waves and spikes in all derivations. *1-2 Hz spike & wave* - A **slow spike-and-wave pattern (1-2.5 Hz)** is typically associated with **Lennox-Gastaut syndrome**, a severe epileptic encephalopathy. - This differs significantly from the faster **3 Hz spike-and-wave** characteristic of typical absence seizures.

Q: All of the following occur in botulism except:

Diarrhoea. ***Diarrhoea*** - Botulism typically causes **constipation**, not diarrhoea, due to the paralysis of smooth muscles in the gastrointestinal tract [1]. - The neurotoxin produced by *Clostridium botulinum* blocks acetylcholine release, affecting both skeletal and autonomic nervous system functions [1]. *Diplopia* - **Diplopia** is a common early symptom of botulism, resulting from paralysis of the extraocular muscles [1]. - It is part of the characteristic descending flaccid paralysis that begins with cranial nerve involvement. *Dysphagia* - **Dysphagia** (difficulty swallowing) is a classic symptom of botulism due to paralysis of the pharyngeal and laryngeal muscles [1]. - This can lead to aspiration and is a significant concern in severe cases. *Dysarthria* - **Dysarthria** (difficulty speaking) is frequently observed in botulism as the cranial nerves controlling speech muscles become affected [1]. - This symptom, along with dysphagia and diplopia, indicates bulbar paralysis.

Q: The electromyogram (EMG) is least useful for the diagnosis of:

Cerebral palsy. ***Cerebral palsy*** - Cerebral palsy is a **disorder of movement and posture** caused by non-progressive brain damage, primarily affecting the **upper motor neurons**. - While EMG can assess muscle activity, its primary role is in evaluating **lower motor neuron diseases** and **neuromuscular junction disorders**, making it less directly useful for diagnosing a central nervous system disorder like cerebral palsy. *Myasthenia gravis* - EMG, particularly **repetitive nerve stimulation**, is highly useful for diagnosing myasthenia gravis by revealing a **decremental response** in the compound muscle action potential due to impaired neuromuscular transmission [1]. - It directly assesses the function of the **neuromuscular junction**, which is the site of pathology in myasthenia gravis [1]. *Charcot-Marie-Tooth disease* - EMG and **nerve conduction studies (NCS)** are crucial for diagnosing Charcot-Marie-Tooth disease by demonstrating **abnormal nerve conduction velocities** (demyelinating forms) or **reduced amplitude** of compound muscle action potentials (axonal forms) which indicate peripheral nerve damage. - The findings help characterize the type and severity of **peripheral neuropathy**, a hallmark of this condition. *Spinal muscular atrophy* - EMG is essential for diagnosing spinal muscular atrophy by showing **denervation and reinnervation changes** in muscles, such as **fibrillations**, **positive sharp waves**, and **large-amplitude, long-duration motor unit potentials** [1]. - These findings reflect the loss of **anterior horn cells** and subsequent attempts by surviving motor neurons to reinnervate muscle fibers.

Q: Uhthoff's sign is seen in:

Multiple sclerosis. ***Multiple sclerosis*** - **Uhthoff's sign** is a classic symptom of multiple sclerosis, characterized by a **temporary worsening of neurological symptoms** in response to an increase in body temperature. - This phenomenon is due to demyelinated nerve fibers being particularly sensitive to heat, which further impairs **nerve conduction**. - Recent MRI studies in multiple sclerosis often show multiple high-signal lesions in the paraventricular region, and active inflammation is characterized by gadolinium enhancement [1]. *Parkinsonism* - Parkinsonism is a neurodegenerative disorder primarily affecting **motor control**, leading to tremors, rigidity, bradykinesia, and postural instability. - Its symptoms do not typically worsen with increases in body temperature; rather, the core pathology involves **dopaminergic neuron degeneration** in the substantia nigra. *Alzheimer's disease* - Alzheimer's disease is a progressive neurodegenerative disorder leading to **memory loss** and cognitive decline, primarily due to the accumulation of **amyloid plaques** and **tau tangles**. - Its progression is not influenced by body temperature fluctuations in the way described by Uhthoff's sign. *Cerebral palsy* - Cerebral palsy is a group of permanent movement disorders that appear in early childhood, caused by **abnormal brain development** or damage to the developing brain. - While various factors can exacerbate symptoms, the classic temperature-sensitive worsening described by Uhthoff's sign is not characteristic of cerebral palsy.

Q: In juvenile myoclonic epilepsy (JME), which is the most common presentation?

Myoclonus. ***Myoclonus*** - **Myoclonic jerks** are the hallmark and defining feature of JME, present in virtually all patients - These typically occur bilaterally, symmetrically, and primarily involve the **upper extremities** shortly after waking up - Most common and earliest seizure manifestation in JME *Absence seizures* - While **absence seizures** can occur in JME, they are present in only 10-30% of patients - They are characterized by **brief periods of impaired consciousness** without motor convulsions - When present, they usually manifest earlier in childhood *GTCS on awake state* - **Generalized tonic-clonic seizures (GTCS)** occur in 85-95% of JME patients and are often triggered by sleep deprivation - They typically occur shortly after awakening and usually develop after myoclonic jerks have already begun - GTCS are not the *most common* or defining seizure type in JME *GTCS during sleep* - GTCS in JME are characteristically associated with awakening, not during sleep itself - While GTCS can occur, they are less common than myoclonic jerks as the presenting feature - The typical pattern is seizures occurring shortly *after* waking up rather than *during* sleep

Q: Locked-in state is characterized by -

Paralysis of all voluntary muscles except ocular muscles. Paralysis of all voluntary muscles except ocular muscles - In locked-in syndrome, patients are conscious and aware but are unable to move any voluntary muscles except for blinking or vertical eye movements. - This condition typically results from damage to the pons, which disconnects higher brain centers from motor pathways and cranial nerves. Paralysis of all voluntary muscles with unconsciousness - This description aligns more with a vegetative state or coma, where there is both extensive paralysis and a lack of awareness or consciousness [1], [2]. - In a locked-in state, despite the severe paralysis, the patient retains full consciousness and cognitive function [2]. Injury to medulla - While significant medullary injury can cause profound neurological deficits, including respiratory failure and quadriparesis, it typically presents with distinct symptoms from locked-in syndrome. - Locked-in syndrome primarily stems from damage to the pons, sparing the reticular activating system and oculomotor pathways, which is different from typical medullary lesions. Loss of cognitive function - Loss of cognitive function is characteristic of conditions like dementia, severe brain injury impacting cortical areas, or certain comatose states [1]. - Patients in a locked-in state retain full cognitive abilities and are completely aware and oriented, despite their inability to communicate verbally or through body movements [2].

Q: The treatment of post traumatic epilepsy is -

Long term anticonvulsants. ***Long term anticonvulsants*** - **Post-traumatic epilepsy** is a chronic condition characterized by recurrent seizures following a head injury. Long-term **anticonvulsant medications** are the primary treatment to prevent these recurrent seizures. - The choice of anticonvulsant depends on the seizure type and patient-specific factors, aiming to achieve seizure control with minimal side effects. *Long term corticosteroids* - **Corticosteroids** are not indicated for the long-term management or prevention of post-traumatic epilepsy. - Their primary use would be in reducing inflammation or edema, which is not the long-term goal of epilepsy treatment. *Immediate corticosteroids* - **Immediate corticosteroids** might be used in acute head injury to reduce cerebral edema, but they do not prevent or treat subsequent **epilepsy**. - They do not address the underlying neuronal hyperexcitability that leads to seizures in post-traumatic epilepsy. *Mannitol infusion* - **Mannitol** is an osmotic diuretic used acutely to reduce **intracranial pressure** in severe head injuries. - It does not have any role in the long-term treatment or prevention of **epilepsy**.

Question 1

A 25-year-old woman presents with intermittent double vision and fatigue. Her symptoms are worse at the end of the day. She reports no other focal muscle weakness or sensory symptoms. On examination her eye movements, motor strength, and reflexes in the upper and lower limbs are normal. Repetitive handgrip exercises cause loss of strength in the grip. A CXR reveals an anterior mediastinal mass. Which of the following is the most likely diagnosis of the anterior mediastinal mass?

Accepted Answer

thymoma

Answer

lymphoma

Answer

teratoma

Answer

thyroid

Question 2

Most common clinical presentation of juvenile myoclonic epilepsy -

Accepted Answer

Myoclonus

Answer

Generalised tonic clonic seizures in awake state

Answer

Generalised tonic clonic seizures in sleep

Answer

Absence seizures

Question 3

Absence seizures are characterized on EEG by:

Accepted Answer

3 Hz spike and wave

Answer

Generalized polyspikes

Answer

Hypsarrhythmia

Answer

1-2 Hz spike & wave

Question 4

In benign intracranial hypertension-

Accepted Answer

Normal or small ventricles are characteristic findings

Answer

Brain scan is not required in young women as sagittal sinus thrombosis is rare

Answer

There is a restriction of upgaze

Answer

Optic nerve fenestration is one of the treatment options that should be considered early to prevent vision loss in benign intracranial hypertension

Question 5

All of the following occur in botulism except:

Accepted Answer

Diarrhoea

Answer

Diplopia

Answer

Dysphagia

Answer

Dysarthria

Question 6

The electromyogram (EMG) is least useful for the diagnosis of:

Accepted Answer

Cerebral palsy

Answer

Myasthenia gravis

Answer

Charcot-Marie-Tooth disease

Answer

Spinal muscular atrophy

Question 7

Uhthoff's sign is seen in:

Accepted Answer

Multiple sclerosis

Answer

Parkinsonism

Answer

Alzheimer's disease

Answer

Cerebral palsy

Question 8

In juvenile myoclonic epilepsy (JME), which is the most common presentation?

Accepted Answer

Myoclonus

Answer

Absence seizures

Answer

GTCS on awake state

Answer

GTCS during sleep

Question 9

Locked-in state is characterized by -

Accepted Answer

Paralysis of all voluntary muscles except ocular muscles

Answer

Paralysis of all voluntary muscles with unconsciousness

Answer

Injury to medulla

Answer

Loss of cognitive function

Question 10

The treatment of post traumatic epilepsy is -

Accepted Answer

Long term anticonvulsants

Answer

Long term corticosteroids

Answer

Immediate corticosteroids

Answer

Mannitol infusion

Neurology — MCQs

Neurology — MCQs

On this page

Practice by Chapter

Want unlimited practice?