Neurology Practice Questions

Q: Which of the following conditions is associated with vestibular schwannoma, spinal cord astrocytoma, and meningioma?

Neurofibromatosis - 2. **Explanation** **Neurofibromatosis Type 2 (NF-2)**, also known as MISME syndrome (Multiple Inherited Schwannomas, Meningiomas, and Ependymomas), is the correct answer. It is an autosomal dominant neurocutaneous syndrome caused by a mutation in the **merlin gene** on **chromosome 22**. The hallmark of NF-2 is **bilateral vestibular schwannomas** (acoustic neuromas), which typically present with sensorineural hearing loss and tinnitus. Beyond the 8th cranial nerve, patients are highly predisposed to other CNS tumors, specifically **meningiomas** and intramedullary spinal tumors, most commonly **ependymomas** and **astrocytomas**. **Why other options are incorrect:** * **Tuberous Sclerosis (TSC):** Characterized by the triad of seizures, mental retardation, and adenoma sebaceum. Associated tumors include Subependymal Giant Cell Astrocytomas (SEGA), cortical tubers, and cardiac rhabdomyomas. * **Neurofibromatosis-1 (NF-1):** Linked to chromosome 17. It presents with Lisch nodules, Café-au-lait spots, and neurofibromas. The classic CNS tumor associated with NF-1 is **Optic Nerve Glioma**, not vestibular schwannoma. * **Von Hippel-Lindau (VHL):** Associated with chromosome 3. It is characterized by **hemangioblastomas** (cerebellum, retina, spinal cord), renal cell carcinoma, and pheochromocytoma. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for NF-2:** Remember **"22"**—Chromosome **22**, **2** ears (bilateral schwannomas), and **2** eyes (juvenile posterior subcapsular lenticular opacities/cataracts). * **Diagnostic Hallmark:** Bilateral vestibular schwannomas on MRI are pathognomonic for NF-2. * **NF-1 vs. NF-2:** NF-1 is more common and primarily involves peripheral nerves and skin; NF-2 is rarer and primarily involves central nervous system tumors.

Q: Which of the following is NOT true about conus medullaris syndrome?

Knee and ankle jerks are absent.. **Explanation:** **Conus Medullaris Syndrome (CMS)** occurs due to injury to the terminal end of the spinal cord (usually at the L1-L2 vertebral level). Understanding the distinction between CMS and Cauda Equina Syndrome (CES) is a high-yield topic for NEET-PG. **Why Option B is the correct answer (The False Statement):** In CMS, the lesion is located at the **sacral segments** of the spinal cord. The **Knee jerk (L2-L4)** is mediated by segments above the conus; therefore, it is typically **preserved/normal**. While the ankle jerk (S1-S2) may be affected, the statement that "both knee and ankle jerks are absent" is incorrect. In contrast, both are usually absent in Cauda Equina Syndrome because it involves multiple nerve roots. **Analysis of other options:** * **Option A:** CMS specifically involves the sacral (S3-S5) and coccygeal segments of the spinal cord. * **Option C:** Since CMS is technically a spinal cord (CNS) lesion, the plantar reflex remains **flexor** (normal) or is absent. An extensor response (Babinski sign) is rare but more likely here than in CES, which is a pure lower motor neuron (LMN) lesion. * **Option D:** **Saddle anesthesia** (sensory loss over S3-S5 dermatomes) is a hallmark of CMS and is typically **symmetrical** and bilateral. **High-Yield Clinical Pearls for NEET-PG:** 1. **Level of Lesion:** CMS occurs at **L1-L2**; CES occurs below **L2**. 2. **Onset:** CMS has a **sudden, bilateral** onset; CES is often gradual and asymmetrical. 3. **Autonomic Dysfunction:** Early and severe urinary/fecal incontinence and impotence are characteristic of **Conus Medullaris Syndrome**. 4. **Pain:** Radicular pain is severe in CES but mild/absent in CMS.

Q: Which medication can be administered to reduce the frequency of future headache episodes in a 26-year-old female patient who experiences one-sided pulsating headaches accompanied by nausea, vomiting, and sensitivity to light and finds relief in a dimly lit environment?

Propranolol. ***Propranolol***- This is a non-selective **beta-blocker** and is considered a first-line prophylactic agent for the reduction of frequency and severity in patients with **episodic migraine** [1].- The patient's symptoms (unilateral, pulsating headache, **nausea, vomiting, and photophobia**) [2] are classic features of migraine, making **Propranolol** an appropriate choice for maintenance therapy [3].*Alprazolam*- This medication is a **short-acting benzodiazepine** primarily indicated for the acute management of anxiety and panic disorders [3].- It has **no established role** in the long-term prophylactic management of migraine and carries risks of sedation and dependence.*Diazepam*- This is a **long-acting benzodiazepine** commonly used as a muscle relaxant, anxiolytic, and for acute seizure management.- Similar to Alprazolam, it is **not recommended** for headache prophylaxis due to lack of efficacy and significant abuse potential [3].*Fluoxetine*- This drug is a selective serotonin reuptake inhibitor (**SSRI**) primarily used to treat **major depressive disorder** and anxiety [3].- While some antidepressants (like TCAs) are used for migraine prophylaxis, **Fluoxetine** is generally not considered a standard or preferred first-line agent for preventing migraine attacks.

Q: A patient presents with contralateral face, arm, and leg weakness. NCCT shows intracerebral hemorrhage (ICH). What is the most likely location of the CNS bleed?

Basal ganglia. ***Basal ganglia*** - The **basal ganglia**, particularly the **putamen**, is the most common site for hypertensive intracerebral hemorrhage, which aligns perfectly with the deep parenchymal bleed seen on the provided NCCT scan. - A lesion in this location classically damages the adjacent **internal capsule**, where motor fibers for the face, arm, and leg are tightly packed, resulting in **contralateral hemiparesis**. *Midbrain* - A midbrain hemorrhage would present with distinct cranial nerve deficits, most notably **oculomotor nerve (CN III) palsy**, causing a "down and out" eye position, ptosis, and a dilated pupil. - Other characteristic signs include altered consciousness and **vertical gaze palsy** (Parinaud syndrome), which are absent in this patient's pure motor presentation. *Pons* - Pontine hemorrhages have a catastrophic presentation, typically causing a rapid descent into **coma**, **quadriplegia**, and classic **pinpoint pupils** due to disruption of descending sympathetic pathways. - The patient's presentation of contralateral hemiparesis without coma or pupillary changes is inconsistent with a pontine bleed. *Medulla* - Medullary hemorrhages are rare and cause specific brainstem syndromes like **Wallenberg syndrome**, characterized by vertigo, nystagmus, dysphagia, and ipsilateral facial numbness. - These syndromes involve complex crossed sensory and motor deficits, not the uniform contralateral motor weakness described in the question.

Q: A 30-year-old male presents with deep-seated retro-orbital pain from cluster headache. What is the best management?

Oxygen. ***Oxygen*** - **High-flow oxygen** (100% at 7-15 L/min) is the safest and most effective **first-line abortive treatment** for an acute cluster headache attack [1] - It works rapidly to cause **vasoconstriction** and often terminates the severe retro-orbital pain within **15 to 20 minutes** - This is the preferred treatment for acute attacks due to its rapid onset and excellent safety profile *Verapamil* - Verapamil is a calcium channel blocker and is considered the **first-line prophylactic agent** for both episodic and chronic cluster headache, not acute treatment [1] - Its mechanism of action requires time, rendering it ineffective for terminating an ongoing acute pain episode - Used to prevent future attacks, not to abort current ones *Lithium* - Lithium is a prophylactic treatment reserved mainly for patients with **chronic cluster headache** or those who do not respond to Verapamil [1] - It has a slow onset of action and is not used to manage the acute pain of a cluster attack - Requires therapeutic monitoring due to narrow therapeutic window [1] *Topiramate* - Topiramate is an established prophylactic treatment primarily for **migraine**, and occasionally used as a second-line prophylactic agent for cluster headache - As a prophylactic medication, it plays **no role** in the acute termination or abortive management of a cluster headache episode

Q: A patient presents with symmetrical leg weakness and areflexia. He had developed diarrhoea 2 weeks ago. He was diagnosed as having GBS. What is the cause?

Demyelination of A, B-fibers. ***Demyelination of A, B-fibers***- GBS is an acute **demyelinating polyneuropathy** primarily affecting the myelin sheath of peripheral nerves and nerve roots (PNS involvement) [1].- Demyelination of large myelinated **A-fibers** (motor and sensory) explains the severe **symmetrical weakness** and **areflexia** [1]; demyelination of **B-fibers** contributes to common **autonomic dysfunction**.*Demyelination of B-fibers*- B-fibers are primarily **preganglionic autonomic fibers**; their demyelination causes autonomic instability (e.g., blood pressure fluctuation) but does not account for the profound motor weakness and areflexia that define GBS [2].- The cardinal features of GBS (weakness and areflexia) result from damage to the larger **A-fibers** (motor nerves) [1]. *Demyelination of the spinal cord*- GBS is a disease of the **Peripheral Nervous System (PNS)**, affecting nerve roots and peripheral nerves [2].- Demyelination confined to the spinal cord is characteristic of **Central Nervous System (CNS)** demyelinating diseases, such as **Transverse Myelitis** or **Multiple Sclerosis** [3]. *Cord inflammation*- **Spinal cord inflammation** or myelitis is a CNS process and typically results in upper motor neuron signs (spasticity, brisk reflexes) or distinct sensory levels, which are not seen in GBS.- GBS involves inflammation and attack on the **peripheral nerve myelin** or axons, specifically in the nerve roots and distal peripheral nerves [1].

Q: A 35-year-old female with progressive limb weakness for the past 1 year. On examination, muscle fasciculations and hypertonia are noted, and the sensory system is intact. What is the diagnosis?

ALS. ***Correct: ALS (Amyotrophic Lateral Sclerosis)*** - This patient presents with the classic triad of ALS: **progressive limb weakness, fasciculations (LMN sign), and hypertonia/spasticity (UMN sign)** [1] - The combination of **both upper and lower motor neuron signs** is pathognomonic for ALS [1] - **Sensory sparing** is characteristic - ALS affects motor neurons only [1] - Age and progressive course over 1 year fits the typical presentation - El Escorial criteria require UMN + LMN signs in multiple regions *Incorrect: Multifocal motor neuropathy* - This is a **pure lower motor neuron disorder** with conduction block - Would present with fasciculations and weakness but **NO hypertonia** (no UMN involvement) [1] - Treatable with immunotherapy, making it an important differential to exclude [1] *Incorrect: Becker muscular dystrophy* - This is a **primary muscle disease**, not a motor neuron disorder - **No fasciculations** would be present (fasciculations indicate motor neuron pathology) [2] - Predominantly affects males (X-linked), presents with proximal weakness and calf pseudohypertrophy - Elevated CK levels would be expected *Incorrect: Spinal muscular atrophy* - This is a **pure lower motor neuron disorder** due to anterior horn cell degeneration - Would NOT present with **hypertonia or UMN signs** - Typically presents in childhood/early life with symmetric proximal weakness - Associated with SMN1 gene mutations

Q: A young female presents with ptosis showing diurnal variation. CT chest shows an anterior mediastinal mass identified as thymus gland cancer. Which receptor is affected in this patient?

Post-synaptic ACh Receptor. ***Post-synaptic ACh Receptor*** - The clinical presentation of **ptosis** with **diurnal variation** (worsening weakness throughout the day) and an associated **thymoma** are hallmark features of **Myasthenia Gravis** [1]. - Myasthenia Gravis is an autoimmune disorder characterized by antibodies that bind to and block or destroy **post-synaptic nicotinic acetylcholine (ACh) receptors** at the neuromuscular junction, leading to impaired muscle contraction [2]. *Presynaptic ACh Receptor* - Autoantibodies in common neuromuscular junction disorders do not target presynaptic ACh receptors. These receptors are typically involved in modulating neurotransmitter release via feedback mechanisms. - The primary presynaptic autoimmune disorder, **Lambert-Eaton Myasthenic Syndrome**, involves antibodies against voltage-gated calcium channels, not ACh receptors [3]. *Voltage-gated calcium channels at the NMJ* - This is the pathological target in **Lambert-Eaton Myasthenic Syndrome (LEMS)**, where antibodies inhibit presynaptic calcium influx, reducing acetylcholine release [3]. - LEMS is clinically distinct, often presenting with weakness that **improves with activity** (incremental response) and is strongly associated with **small cell lung cancer** [3]. *Acetylcholinesterase* - **Acetylcholinesterase (AChE)** is the enzyme responsible for degrading acetylcholine in the synaptic cleft; it is not the target of autoimmunity in Myasthenia Gravis. - In fact, **AChE inhibitors** (e.g., pyridostigmine) are a primary treatment for Myasthenia Gravis, as they increase the concentration and duration of ACh in the synapse to better activate the reduced number of receptors [2].

Q: A patient presents with unilateral throbbing pain, photophobia, nausea, and vomiting. The symptoms improve after taking sumatriptan. What is the most likely diagnosis?

Migraine. ***Migraine*** - The presentation of **unilateral**, **throbbing pain** combined with associated symptoms like **photophobia**, **nausea**, and **vomiting** constitutes the classic criteria for migraine [1]. - Dramatic symptomatic relief after taking **sumatriptan** (a **triptan**) is highly characteristic, as these drugs are specific abortive treatments for acute **migraine** attacks. *Tension headache* - This headache is typically **bilateral**, described as a **tightening** or **band-like** pressure, and is non-throbbing [1]. - It usually lacks associated features such as **nausea**, **vomiting**, or severe photophobia, and does not typically respond well to triptans. *Cluster headache* - While also highly painful and unilateral, cluster headaches are characterized by **excruciating, non-throbbing** pain, often localized to the **periorbital** or retro-orbital region [1]. - Key associated features are **autonomic**, including ipsilateral **lacrimation**, ptosis, miosis, and rhinorrhea, features not mentioned in this presentation. *Sinus headache* - This diagnosis is associated with symptoms of **sinusitis**, such as facial pressure, pain over the sinuses, fever, and purulent nasal discharge. - The pain is usually localized to the **maxillary** or **frontal** regions and is not typically a severe, throbbing pain that uniquely responds to triptans.

Question 1

Which of the following conditions is associated with vestibular schwannoma, spinal cord astrocytoma, and meningioma?

Accepted Answer

Neurofibromatosis - 2

Answer

Tuberous sclerosis

Answer

Neurofibromatosis - 1

Answer

Von Hippel-Lindau syndrome

Question 2

Brain dead individuals have all of the following features EXCEPT?

Accepted Answer

Only pain is preserved

Answer

Dolls eye movement is absent

Answer

Oculo-vestibular reflex is absent

Answer

Corneal reflex is absent

Question 3

Which of the following is NOT true about conus medullaris syndrome?

Accepted Answer

Knee and ankle jerks are absent.

Answer

It involves segments from the lower 3 sacral and coccygeal segments.

Answer

Plantar reflex is flexor.

Answer

Saddle anesthesia is present.

Question 4

Which medication can be administered to reduce the frequency of future headache episodes in a 26-year-old female patient who experiences one-sided pulsating headaches accompanied by nausea, vomiting, and sensitivity to light and finds relief in a dimly lit environment?

Accepted Answer

Propranolol

Answer

Diazepam

Answer

Fluoxetine

Answer

Alprazolam

Question 5

A patient presents with contralateral face, arm, and leg weakness. NCCT shows intracerebral hemorrhage (ICH). What is the most likely location of the CNS bleed?

Accepted Answer

Basal ganglia

Answer

Pons

Answer

Midbrain

Answer

Medulla

Question 6

A 30-year-old male presents with deep-seated retro-orbital pain from cluster headache. What is the best management?

Accepted Answer

Oxygen

Answer

Verapamil

Answer

Lithium

Answer

Topiramate

Question 7

A patient presents with symmetrical leg weakness and areflexia. He had developed diarrhoea 2 weeks ago. He was diagnosed as having GBS. What is the cause?

Accepted Answer

Demyelination of A, B-fibers

Answer

Cord inflammation

Answer

Demyelination of the spinal cord

Answer

Demyelination of B-fibers

Question 8

A 35-year-old female with progressive limb weakness for the past 1 year. On examination, muscle fasciculations and hypertonia are noted, and the sensory system is intact. What is the diagnosis?

Accepted Answer

ALS

Answer

Multifocal motor neuropathy

Answer

Becker muscular dystrophy

Answer

Spinal muscular atrophy

Question 9

A young female presents with ptosis showing diurnal variation. CT chest shows an anterior mediastinal mass identified as thymus gland cancer. Which receptor is affected in this patient?

Accepted Answer

Post-synaptic ACh Receptor

Answer

Acetylcholinesterase

Answer

Voltage-gated calcium channels at the NMJ

Answer

Presynaptic ACh Receptor

Question 10

A patient presents with unilateral throbbing pain, photophobia, nausea, and vomiting. The symptoms improve after taking sumatriptan. What is the most likely diagnosis?

Accepted Answer

Migraine

Answer

Cluster headache

Answer

Sinus headache

Answer

Tension headache

Neurology — MCQs

Neurology — MCQs

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