Neurology Practice Questions

Q: All are true about Miller Fisher syndrome except?

Muscle weakness. **Miller Fisher Syndrome (MFS)** is a rare, localized variant of **Guillain-Barré Syndrome (GBS)**. It is classically defined by a clinical triad, and understanding this triad is the key to solving this question. ### Why "Muscle Weakness" is the Correct Answer Unlike the classic form of GBS (Acute Inflammatory Demyelinating Polyradiculoneuropathy), which presents with prominent ascending paralysis and limb weakness, **Miller Fisher Syndrome typically spares the limb muscles.** While patients may feel "unsteady," this is due to sensory ataxia rather than motor loss. Significant limb weakness is generally absent; if it occurs, the diagnosis often shifts to an "MFS-GBS overlap syndrome." ### Explanation of Incorrect Options (The Classic Triad) The hallmark of MFS is the triad of: * **Ataxia (Option A):** Patients present with a profound gait ataxia. This is sensory in nature, caused by the involvement of large-diameter sensory fibers. * **Areflexia (Option B):** There is a generalized loss of deep tendon reflexes, similar to classic GBS, due to peripheral nerve involvement. * **Ophthalmoplegia (Option D):** This is often the presenting feature. It manifests as external ophthalmoplegia (paralysis of eye muscles), frequently starting with diplopia. ### High-Yield Clinical Pearls for NEET-PG * **Antibody Marker:** The most specific marker for MFS is the **Anti-GQ1b antibody** (present in >90% of cases). These antibodies target gangliosides highly concentrated in the oculomotor nerves and muscle spindles. * **Preceding Infection:** Like GBS, it often follows a respiratory or gastrointestinal infection (commonly *Campylobacter jejuni*). * **CSF Findings:** Shows **albuminocytologic dissociation** (elevated protein with a normal cell count), though this may be absent in the first week. * **Prognosis:** Generally carries a good prognosis with spontaneous recovery over weeks to months. Treatment (IVIG or Plasmapheresis) is similar to GBS.

Q: A patient with traumatic paraplegia due to injury of the thoracic cord at T3 level is observed to have a blood pressure of 210/120 mmHg. What is the initial management?

Nifedipine. **Explanation:** The patient is presenting with **Autonomic Dysreflexia (AD)**, a life-threatening medical emergency occurring in patients with spinal cord injuries at or above the **T6 level**. **1. Why Nifedipine is Correct:** Autonomic Dysreflexia is triggered by noxious stimuli (e.g., distended bladder, fecal impaction) below the level of the lesion. This causes a massive, uninhibited sympathetic discharge leading to severe hypertension, bradycardia (reflexive), and pounding headaches. The **initial management** involves sitting the patient upright (to induce orthostatic pressure drop) and identifying/removing the trigger. If blood pressure remains dangerously high (as in this case, 210/120 mmHg), rapid-acting antihypertensives are indicated. **Nifedipine (immediate-release)** or Nitroglycerin paste are the preferred agents to prevent intracranial hemorrhage or seizures. **2. Why Other Options are Incorrect:** * **A. LMWH:** Used for DVT prophylaxis in spinal cord injuries, but it does not address the acute hypertensive crisis. * **B. Steroids:** High-dose methylprednisolone was previously used for acute spinal cord injury to reduce edema, but it is no longer the standard of care due to complications and has no role in managing acute hypertension. * **D. Normal Saline/Dextrose:** Fluid resuscitation is contraindicated here as it could further worsen the hypertension and volume load. **High-Yield Clinical Pearls for NEET-PG:** * **Level of Lesion:** AD typically occurs in injuries at **T6 or above** (above the splanchnic outflow). * **Classic Triad:** Hypertension, Bradycardia, and Flushing/Sweating *above* the level of injury. * **Most Common Trigger:** Bladder distension (check the Foley catheter first!). * **Management Priority:** 1. Sit the patient up → 2. Loosen tight clothing → 3. Empty bladder/bowel → 4. Pharmacotherapy (Nifedipine) if BP >150 mmHg systolic.

Q: Examination of a patient revealed ipsilateral third nerve paresis and cerebellar ataxia. What is the MOST likely diagnosis?

Nothnagel's syndrome. **Explanation:** The clinical presentation of **ipsilateral 3rd nerve palsy** combined with **cerebellar ataxia** is the hallmark of **Nothnagel’s syndrome**. This midbrain stroke syndrome occurs due to a lesion in the **superior cerebellar peduncle** and the emerging fibers of the **oculomotor nerve (CN III)**. **Why Nothnagel’s syndrome is correct:** The lesion is typically located in the dorsal midbrain (tectum). The involvement of the superior cerebellar peduncle results in ipsilateral (or sometimes bilateral) cerebellar ataxia, while the involvement of the CN III fascicles leads to ipsilateral ophthalmoplegia and ptosis. **Analysis of Incorrect Options:** * **Weber’s Syndrome:** Characterized by ipsilateral 3rd nerve palsy and **contralateral hemiparesis** (due to involvement of the crus cerebri/pyramidal tract) [1]. * **Claude’s Syndrome:** Characterized by ipsilateral 3rd nerve palsy and **contralateral cerebellar ataxia** (due to involvement of the red nucleus and dentatothalamic tract) [1]. *Note: Nothnagel's is often distinguished by the presence of gait ataxia and its more dorsal location.* * **Benedikt’s Syndrome:** Characterized by ipsilateral 3rd nerve palsy and **contralateral tremors/involuntary movements** (chorea/athetosis) due to involvement of the red nucleus and substantia nigra. **High-Yield Clinical Pearls for NEET-PG:** * **Rule of Thumb:** All midbrain syndromes involve the **ipsilateral 3rd nerve**. The differentiating factor is the motor/sensory deficit [1]: * **Weber:** Hemiplegia (Motor) * **Benedikt:** Tremors (Extrapyramidal) * **Claude:** Ataxia (Cerebellar) * **Nothnagel:** Ataxia + Ocular signs (often associated with pineal tumors or tectal lesions). * The most common cause of these syndromes is an infarct in the branches of the **Posterior Cerebral Artery (PCA)**.

Q: Epilepsy is defined as?

Two unprovoked seizures. The definition of epilepsy is primarily clinical and focuses on the enduring predisposition of the brain to generate spontaneous seizures [1]. According to the **International League Against Epilepsy (ILAE)**, epilepsy is defined by any of the following conditions [1]: 1. At least **two unprovoked (or reflex) seizures** occurring more than 24 hours apart. 2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years. 3. Diagnosis of an epilepsy syndrome. **Why Option B is correct:** Historically and clinically, a single seizure does not constitute epilepsy. The occurrence of two unprovoked seizures signifies a permanent threshold shift, indicating a chronic neurological disorder rather than a transient insult [1]. **Why other options are incorrect:** * **Option A:** A single unprovoked seizure is termed a "provoked" or "isolated" seizure. While it requires investigation, it does not meet the criteria for epilepsy unless there is a high risk of recurrence (e.g., structural brain abnormality on MRI). * **Options C & D:** While having three or four seizures certainly confirms epilepsy, the minimum diagnostic threshold is two. Waiting for a third or fourth event would unnecessarily delay treatment. **High-Yield Clinical Pearls for NEET-PG:** * **Provoked Seizures:** These are caused by temporary factors (hypoglycemia, hyponatremia, alcohol withdrawal, or high fever) and are **not** classified as epilepsy. * **Resolved Epilepsy:** Epilepsy is considered resolved if an individual remains seizure-free for 10 years, with at least the last 5 years off anti-seizure medications. * **Status Epilepticus:** Defined as >5 minutes of continuous seizure activity or two or more discrete seizures without full recovery of consciousness between them.

Q: A 72-year-old man complains of drooping of eyelids for the past 2 months. On awakening, he is able to open his eyes, but a few hours later his ptosis progressively worsens with increasing diplopia. What is the most likely diagnosis?

Myasthenia gravis. ### Explanation **Correct Answer: C. Myasthenia Gravis** The clinical hallmark of **Myasthenia Gravis (MG)** is **fatigability**. The patient’s symptoms (ptosis and diplopia) are absent or minimal in the morning (after rest) but worsen as the day progresses (with activity). This occurs due to autoimmune-mediated destruction of acetylcholine receptors at the neuromuscular junction, leading to failure of neuromuscular transmission upon repetitive stimulation. **Why the other options are incorrect:** * **A. Thyroid Ophthalmopathy:** Typically presents with **lid retraction** (Dalrymple sign) and proptosis, rather than ptosis. While it causes diplopia due to muscle infiltration, it does not show diurnal variation. * **B. Horner’s Syndrome:** Characterized by a triad of **mild ptosis** (due to Mueller’s muscle involvement), miosis, and anhidrosis. It is a sympathetic nerve palsy and does not feature diplopia or fatigability. * **D. 3rd Nerve Palsy:** Presents with **complete ptosis**, a "down and out" eye position, and often pupillary involvement. It is a static neurological deficit and does not fluctuate with rest or activity. **High-Yield Clinical Pearls for NEET-PG:** * **Cogan’s Lid Twitch:** A brief upward overshoot of the eyelid when the patient shifts gaze from downward to primary position; highly suggestive of MG. * **Ice Pack Test:** Placing ice over the eyelid for 2 minutes improves ptosis in MG (cold inhibits acetylcholinesterase). * **Simpson’s Test:** Worsening of ptosis on sustained upward gaze (demonstrates fatigability). * **Associated Conditions:** Always screen for **Thymoma** (Chest CT) in patients diagnosed with MG. * **Ocular Myasthenia:** In 50-60% of cases, MG starts with ocular symptoms; 90% of these will progress to systemic involvement within 2 years.

Q: Which medication is considered a treatment option for juvenile myoclonic epilepsy during pregnancy?

Levetiracetam. **Explanation:** **Juvenile Myoclonic Epilepsy (JME)** is a generalized epilepsy syndrome characterized by myoclonic jerks, generalized tonic-clonic seizures (GTCS), and absence seizures. While **Valproate** is the drug of choice for JME, it is highly **teratogenic** (associated with neural tube defects and cognitive impairment) and is generally avoided in women of childbearing age. **Why Levetiracetam is correct:** Levetiracetam is a broad-spectrum antiepileptic drug (AED) effective against myoclonic seizures. It is considered a preferred alternative in pregnancy because it has a **favorable safety profile** with a significantly lower risk of major congenital malformations compared to Valproate or Phenytoin. **Why the other options are incorrect:** * **Carbamazepine:** This is a narrow-spectrum AED. It is primarily used for focal seizures and can actually **exacerbate** myoclonic and absence seizures in JME patients. * **Vigabatrin:** It is primarily used for infantile spasms (West Syndrome) and refractory focal seizures. It is associated with permanent **visual field defects** and is not a standard treatment for JME. * **Phenytoin:** Like Carbamazepine, it can worsen myoclonic jerks. Furthermore, it is associated with **Fetal Hydantoin Syndrome** (cleft lip/palate, digital hypoplasia) when used during pregnancy. **NEET-PG High-Yield Pearls:** * **Drug of Choice for JME:** Valproate (in males/non-pregnant females). * **Safest AEDs in Pregnancy:** Levetiracetam and Lamotrigine. * **Lamotrigine caveat:** While safe in pregnancy, it may also worsen myoclonic seizures in some JME patients; hence, Levetiracetam is often preferred for this specific syndrome. * **Supplementation:** All pregnant women on AEDs should receive high-dose **Folic acid (5mg)** to reduce the risk of neural tube defects.

Q: Pseudocoma most commonly results from infarction or hemorrhage in which of the following structures?

Pons. **Explanation:** **Pseudocoma**, also known as **Locked-in Syndrome (LIS)**, is a clinical state where a patient is conscious and alert but suffers from total paralysis of all voluntary muscles except for vertical eye movements and blinking. 1. **Why Pons is Correct:** The most common cause of Locked-in Syndrome is a lesion (infarction due to basilar artery occlusion or hemorrhage) in the **ventral (anterior) portion of the Pons**. This lesion destroys the descending **corticospinal and corticobulbar tracts**, resulting in quadriplegia and paralysis of the lower cranial nerves. However, the **tegmentum of the pons** (containing the Reticular Activating System responsible for consciousness) and the **midbrain** (controlling vertical eye movements via the oculomotor nerve) are spared. 2. **Why Other Options are Incorrect:** * **Mid-brain:** Lesions here typically cause "Top of the Basilar" syndrome, often resulting in coma or third-nerve palsies, rather than the specific sparing of consciousness seen in LIS. * **Medulla:** Lesions in the medulla (e.g., Wallenberg syndrome) cause specific sensory and autonomic deficits but do not typically result in the global motor paralysis characteristic of pseudocoma. * **Hypothalamus:** Damage here usually leads to disturbances in autonomic function, temperature regulation, or sleep-wake cycles (Akinetic Mutism), not the de-efferented state of LIS. **Clinical Pearls for NEET-PG:** * **Classic Triad:** Quadriplegia, aphonia (inability to speak), and preserved consciousness. * **Preserved Function:** Vertical eye movement and blinking (controlled by the midbrain). * **EEG Findings:** Normal (reflecting preserved cortical activity/wakefulness). * **Differential Diagnosis:** Must be distinguished from **Akinetic Mutism** (lesion in the frontal lobes/hypothalamus where the patient *lacks the will* to move) and **Persistent Vegetative State** (where consciousness is lost).

Q: Which of the following is a true statement about medial medullary syndrome?

Ipsilateral 12th nerve palsy. **Explanation:** **Medial Medullary Syndrome (Dejerine Syndrome)** occurs due to the occlusion of the **Anterior Spinal Artery** or the paramedian branches of the vertebral artery. This results in an "alternating hemiplegia" pattern involving specific structures in the medial medulla. **Why Option C is correct:** The **Hypoglossal nerve (CN XII) nucleus and its exiting fibers** are located medially in the medulla. Damage to these fibers results in **ipsilateral LMN-type paralysis of the tongue**, causing the tongue to deviate toward the side of the lesion when protruded. **Analysis of Incorrect Options:** * **Option A (3rd nerve palsy):** This is a feature of **Weber’s Syndrome**, which involves the midbrain, not the medulla. * **Option B (6th nerve palsy):** This is seen in **Foville’s Syndrome** or **Millard-Gubler Syndrome**, which involve the pons. * **Option D (Ipsilateral hemiplegia):** The syndrome involves the **Medullary Pyramids**. Since the corticospinal fibers decussate *below* this level (at the cervicomedullary junction), damage here results in **contralateral hemiplegia**, not ipsilateral. **High-Yield Clinical Pearls for NEET-PG:** * **The Medial Medullary Triad:** 1. **Ipsilateral CN XII palsy** (Tongue deviation). 2. **Contralateral Hemiplegia** (Pyramidal tract involvement). [1] 3. **Contralateral loss of vibration/proprioception** (Medial Lemniscus involvement). * **Contrast with Lateral Medullary Syndrome (Wallenberg):** Wallenberg involves the PICA and presents with sensory loss, ataxia, and dysphagia, but **spares** the motor tracts and CN XII.

Question 1

All are true about Miller Fisher syndrome except?

Accepted Answer

Muscle weakness

Answer

Ataxia

Answer

Areflexia

Answer

Ophthalmoplegia

Question 2

A patient with traumatic paraplegia due to injury of the thoracic cord at T3 level is observed to have a blood pressure of 210/120 mmHg. What is the initial management?

Accepted Answer

Nifedipine

Answer

Subcutaneous low molecular weight heparin

Answer

Steroids

Answer

Normal saline/Dextrose

Question 3

Examination of a patient revealed ipsilateral third nerve paresis and cerebellar ataxia. What is the MOST likely diagnosis?

Accepted Answer

Nothnagel's syndrome

Answer

Weber's syndrome

Answer

Claude's syndrome

Answer

Benedikt's syndrome

Question 4

Epilepsy is defined as?

Accepted Answer

Two unprovoked seizures

Answer

One unprovoked seizure

Answer

Three unprovoked seizures

Answer

Four unprovoked seizures

Question 5

A 72-year-old man complains of drooping of eyelids for the past 2 months. On awakening, he is able to open his eyes, but a few hours later his ptosis progressively worsens with increasing diplopia. What is the most likely diagnosis?

Accepted Answer

Myasthenia gravis

Answer

Thyroid ophthalmopathy

Answer

Horner's syndrome

Answer

3rd nerve Palsy

Question 6

Which medication is considered a treatment option for juvenile myoclonic epilepsy during pregnancy?

Accepted Answer

Levetiracetam

Answer

Carbamazepine

Answer

Vigabatrin

Answer

Phenytoin

Question 7

A 70-year-old hypertensive male presents complaining of a brief episode of slurred speech and left-sided weakness the previous evening. He gives a history of two transient episodes of sudden loss of vision in his right eye last month. His neurological examination is normal now. What is the most appropriate next diagnostic test?

Accepted Answer

Carotid Doppler

Answer

CT Scan

Answer

Diffusion-weighted MRI

Answer

Cerebral Angiography

Question 8

Pseudocoma most commonly results from infarction or hemorrhage in which of the following structures?

Accepted Answer

Pons

Answer

Mid-brain

Answer

Medulla

Answer

Hypothalamus

Question 9

A 15-year-old boy experiences 10-12 complex partial seizures daily despite being on adequate four-drug antiepileptic therapy. He has a history of recurrent high-grade fevers in childhood. An MRI performed with an epilepsy protocol shows a normal brain scan. What is the best non-invasive strategy to establish a definitive diagnosis for epilepsy surgery preparation?

Accepted Answer

Video EEG with ictal 99mTc-HMPAO Brain SPECT

Answer

Interictal scalp EEG

Answer

Video EEG

Answer

Interictal 18F-FDG PET

Question 10

Which of the following is a true statement about medial medullary syndrome?

Accepted Answer

Ipsilateral 12th nerve palsy

Answer

3rd nerve palsy

Answer

6th nerve palsy

Answer

Ipsilateral hemiplegia

Neurology — MCQs

Neurology — MCQs

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