Neurology Practice Questions

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**.

Q: Myasthenia gravis is a disorder of:

Neuromuscular junction. Neuromuscular junction - **Myasthenia gravis** is an **autoimmune disorder** where antibodies attack **acetylcholine receptors** at the **neuromuscular junction**, impairing nerve-to-muscle communication [1], [2]. - This leads to characteristic **muscle weakness** that worsens with activity and improves with rest [2]. *Spinal cord* - Disorders of the **spinal cord** typically involve sensory, motor, and autonomic deficits below the level of the lesion, often characterized by **spasticity** and **hyperreflexia**. - **Myasthenia gravis** does not directly affect the spinal cord, and its symptoms are distinct from spinal cord pathologies. *Motor neuron* - **Motor neuron diseases** (e.g., **ALS**) primarily affect the motor neurons in the brain and spinal cord, leading to progressive **muscle atrophy**, **fasciculations**, and **weakness** due to **denervation**. - Myasthenia gravis does not involve motor neuron degeneration; the neurons themselves are healthy. *Peripheral nerve* - **Peripheral nerve disorders** (e.g., **Guillain-Barré syndrome**) involve damage to nerves outside the brain and spinal cord, causing sensory changes (numbness, tingling), muscle weakness, and often **diminished reflexes**. - While myasthenia gravis causes weakness, it is due to a problem at the junction between the nerve and muscle, not within the nerve itself.

Q: True about myasthenia gravis:

Skeletal muscle function improves after rest. ***Skeletal muscle function improves after rest*** - Myasthenia gravis is characterized by **fatigue and weakness of skeletal muscles** that worsens with activity and improves with rest [1]. - This fluctuating weakness is a hallmark symptom, distinguishing it from other neuromuscular disorders. *Increased acetylcholine receptors* - Myasthenia gravis involves an **autoimmune attack** against **postsynaptic acetylcholine receptors (AChRs)** at the neuromuscular junction, leading to their **destruction and reduction**, not an increase [2]. - The decreased number of functional AChRs impairs neuromuscular transmission. *Associated with thymoma in 3/4 of cases* - While there is a strong association with thymic abnormalities, especially **thymic hyperplasia** (in 65-70% of cases) and **thymoma** (in 10-15% of cases), stating 3/4 (75%) is an **overestimate** for thymoma [2]. - Thymoma is present in a significant minority, not the vast majority, of myasthenia gravis patients. *Incremental response seen with repetitive electric stimuli* - Repetitive nerve stimulation in myasthenia gravis typically shows a **decremental (fatiguing) response** in the compound muscle action potential (CMAP). - An incremental response is more characteristic of conditions like **Lambert-Eaton myasthenic syndrome**, where presynaptic calcium channel dysfunction is dominant [3].

Q: A female patient of childbearing age is on valproate for JME. Which drug should be used to replace valproate and can be prescribed as monotherapy?

Levetiracetam. ***Levetiracetam*** - **Levetiracetam** is recommended as a safer alternative to valproate in women of childbearing potential due to its **favorable pregnancy safety profile** and broad-spectrum efficacy against generalized seizures, including those seen in **juvenile myoclonic epilepsy (JME)** [1]. - It can be used as **monotherapy** and has a generally well-tolerated side effect profile [1], making it a suitable long-term option.*Carbamazepine* - **Carbamazepine** is primarily effective for **focal (partial) seizures** and is generally not recommended for **generalized epilepsy syndromes** like JME due to the risk of worsening myoclonic or absence seizures. - It also has significant **teratogenic risks**, including neural tube defects, making it unsuitable for women of childbearing age when safer alternatives exist [2].*Phenytoin* - **Phenytoin** is effective for focal and tonic-clonic seizures but can **exacerbate myoclonic seizures** in JME. - It carries a significant risk of **teratogenicity**, including fetal hydantoin syndrome, making it an inappropriate choice for women of childbearing potential [2].*Zonisamide* - **Zonisamide** is a broad-spectrum antiepileptic drug, but it is often reserved as an **add-on therapy** for refractory epilepsy rather than a first-line monotherapy, particularly if there are safer and more established first-line options. - While generally considered less teratogenic than valproate, its safety profile in pregnancy has fewer established data compared to levetiracetam.

Q: Which of the following is the only drug found to be beneficial in halting the progression on EDSS of multiple sclerosis?

Natalizumab. ***Natalizumab*** - **Natalizumab** is a monoclonal antibody that targets the **α4-integrin subunit** on leukocytes, preventing their migration across the blood-brain barrier [1]. - It has been shown to significantly reduce relapse rates and slow the progression of disability (as measured by **EDSS**) in **relapsing-remitting multiple sclerosis (RRMS)**, making it one of the most efficacious disease-modifying therapies. *Glatiramer* - **Glatiramer acetate** is a synthetic polypeptide that acts as a **decoy myelin basic protein**, inducing T-cell responses that may reduce inflammation in the CNS [1]. - While it reduces relapse rates and may slow disability progression, its efficacy in halting progression on the **EDSS** is generally considered less potent than natalizumab. *IFN-beta* - **Interferon-beta (IFN-beta)** modulates the immune system by reducing inflammation and preventing auto-reactive T-cells from attacking myelin. - Although it reduces relapse rates and slows the accumulation of lesions, its impact on completely halting **EDSS** progression is limited, especially compared to newer, more potent therapies. *Methotrexate* - **Methotrexate** is an **immunosuppressant** and **anti-inflammatory** drug, often used in other autoimmune diseases like rheumatoid arthritis. - While it can suppress immune activity, it is **not a primary treatment** for multiple sclerosis and has not shown significant benefit in halting **EDSS** progression in MS.

Question 1

All of the following occur in botulism except:

Accepted Answer

Diarrhoea

Answer

Diplopia

Answer

Dysphagia

Answer

Dysarthria

Question 2

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 3

Uhthoff's sign is seen in:

Accepted Answer

Multiple sclerosis

Answer

Parkinsonism

Answer

Alzheimer's disease

Answer

Cerebral palsy

Question 4

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 5

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 6

The treatment of post traumatic epilepsy is -

Accepted Answer

Long term anticonvulsants

Answer

Long term corticosteroids

Answer

Immediate corticosteroids

Answer

Mannitol infusion

Question 7

Myasthenia gravis is a disorder of:

Accepted Answer

Neuromuscular junction

Answer

Spinal cord

Answer

Motor neuron

Answer

Peripheral nerve

Question 8

True about myasthenia gravis:

Accepted Answer

Skeletal muscle function improves after rest

Answer

Increased acetylcholine receptors

Answer

Associated with thymoma in 3/4 of cases

Answer

Incremental response seen with repetitive electric stimuli

Question 9

A female patient of childbearing age is on valproate for JME. Which drug should be used to replace valproate and can be prescribed as monotherapy?

Accepted Answer

Levetiracetam

Answer

Carbamazepine

Answer

Phenytoin

Answer

Zonisamide

Question 10

Which of the following is the only drug found to be beneficial in halting the progression on EDSS of multiple sclerosis?

Accepted Answer

Natalizumab

Answer

Glatiramer

Answer

IFN-beta

Answer

Methotrexate

Neurology — MCQs

Neurology — MCQs

On this page

Practice by Chapter

Want unlimited practice?