Neurology Practice Questions

Q: What is the diagnosis of this patient?

Muscular dystrophy. ***Muscular dystrophy*** - Characterized by **pseudohypertrophy of calves** and **proximal muscle weakness**, particularly affecting hip and shoulder girdles. - **Gower's sign** (using hands to climb up legs when rising from floor) is a classic clinical feature indicating proximal weakness. *Myasthenia gravis* - Presents with **fluctuating weakness** that worsens with activity and improves with rest, particularly affecting **ocular** and **bulbar muscles**. - Associated with **acetylcholine receptor antibodies** and shows improvement with **anticholinesterase medications**. *Congenital myopathy* - Usually presents in **infancy** with **hypotonia** and delayed motor milestones, not progressive weakness. - Muscle biopsy shows specific **structural abnormalities** like central cores or nemaline rods, without pseudohypertrophy. *Inclusion body myositis* - Typically affects **elderly patients** (>50 years) with **asymmetric weakness** involving both proximal and distal muscles. - Characterized by **rimmed vacuoles** on muscle biopsy and poor response to immunosuppressive therapy.

Q: A 16-year-old male presents with complaints of seeing wave-like visual disturbances and sudden monocular blindness, occurring while watching movies. What is the most likely diagnosis?

Migraine. ### **Explanation** **Correct Option: A. Migraine** The clinical presentation of wave-like visual disturbances (scotomas or fortification spectra) followed by sudden monocular blindness (amaurosis fugax) is characteristic of **Retinal Migraine** or migraine with aura. In adolescents, visual triggers such as flickering lights from a movie screen are well-known precipitants for migraine attacks. The "wave-like" disturbances represent the spreading depression of cortical or retinal activity, leading to transient neurological deficits. **Analysis of Incorrect Options:** * **B. Exhibitionism:** This is a paraphilic disorder involving the urge to expose one's genitals to unsuspecting strangers. It has no neurological or visual symptoms and is entirely unrelated to the clinical vignette. * **C. Temporal Lobe Epilepsy (TLE):** While TLE can present with visual hallucinations (usually complex scenes or "deja vu"), it typically involves automatisms, altered consciousness, or olfactory/gustatory sensations. It does not typically cause monocular blindness. * **D. Grand Mal Epilepsy (Tonic-Clonic Seizure):** This involves a sudden loss of consciousness followed by generalized tonic and clonic muscle contractions. While a "prodrome" may exist, the focal visual symptoms described are more specific to migraine or focal occipital seizures, not generalized grand mal epilepsy. **NEET-PG High-Yield Pearls:** * **Retinal Migraine:** Defined by fully reversible monocular visual phenomena (scintillations, scotoma, or blindness) associated with a headache. * **Acephalgic Migraine:** Migraine aura occurring without the subsequent headache, common in the pediatric/adolescent age group. * **Visual Triggers:** Photosensitivity is a hallmark of both migraine and certain types of epilepsy (reflex epilepsy), but the specific description of "wave-like" patterns strongly favors migraine.

Q: All of the following are true about Juvenile Myoclonic Epilepsy, except:

Focal seizures. Explanation: Juvenile Myoclonic Epilepsy (JME), also known as Janz Syndrome, is the most common form of **Idiopathic Generalized Epilepsy (IGE)**. By definition, generalized epilepsies involve both cerebral hemispheres from the onset, making **focal seizures** (Option A) inconsistent with the diagnosis [1], [3]. While JME patients may rarely exhibit focal EEG abnormalities, the clinical seizure semiology remains generalized. * **Why Option A is correct:** JME is a generalized epilepsy syndrome. Focal seizures originate from a localized network in one hemisphere and are not a characteristic feature of JME [3]. * **Why Option B is incorrect:** Generalized Tonic-Clonic Seizures (GTCS) are seen in nearly 90% of JME patients, typically occurring shortly after awakening [2]. * **Why Option C is incorrect:** Myoclonic jerks (sudden, involuntary muscle contractions) are the hallmark of JME [2]. They characteristically involve the upper extremities and occur most frequently in the morning. * **Why Option D is incorrect:** Sodium Valproate is the **drug of choice** for JME, providing excellent seizure control in over 80% of patients [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Age of Onset:** Typically between 12–18 years [2]. * **Classic Triad:** Myoclonic jerks (100%), GTCS (90%), and Absence seizures (one-third of patients) [2]. * **Triggers:** Sleep deprivation, alcohol consumption, and flickering lights (photosensitivity) [2]. * **EEG Findings:** Characteristic **4–6 Hz polyspike-and-wave** discharges [1], [2]. * **Prognosis:** Excellent control with medication, but usually requires **lifelong treatment** as relapse rates are high (up to 90%) upon drug withdrawal [2].

Q: A patient presents with unilateral painful ophthalmoplegia. Imaging revealed an enlargement of the cavernous sinus on the affected side. What is the likely diagnosis?

Tolosa-Hunt syndrome. **Explanation:** **Tolosa-Hunt Syndrome (THS)** is the correct diagnosis. It is characterized by idiopathic, non-specific granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbital apex. * **Clinical Presentation:** Patients typically present with **unilateral, boring orbital pain** and **ophthalmoplegia** (palsy of CN III, IV, and VI). * **Imaging:** MRI often shows enlargement or "fullness" of the cavernous sinus due to inflammatory tissue. * **Pathognomonic Feature:** A dramatic clinical response to systemic **corticosteroids** (usually within 48–72 hours) is a diagnostic hallmark. **Why the other options are incorrect:** * **Gradenigo Syndrome:** Characterized by the triad of abducens nerve palsy (CN VI), deep ear pain (trigeminal neuralgia), and suppurative otitis media (petrous apicitis). It does not involve the cavernous sinus. * **Cavernous Sinus Thrombosis (CST):** While it presents with painful ophthalmoplegia, it is typically an acute, life-threatening infectious process (often from a "danger area" facial infection) presenting with high fever, proptosis, and chemosis. * **Orbital Pseudotumor:** This is also an idiopathic inflammatory condition, but it primarily involves the **orbit** (extraocular muscles or lacrimal gland), leading to proptosis and chemosis rather than primary cavernous sinus enlargement. **High-Yield Pearls for NEET-PG:** * **THS is a diagnosis of exclusion:** Always rule out tumors or CST first. * **Nerves involved:** CN III, IV, VI, and the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve. * **Steroid Response:** If the pain does not resolve rapidly with steroids, reconsider the diagnosis (likely a malignancy).

Q: The conus medullaris syndrome differs from the cauda equina syndrome in that the former shows which of the following?

Symmetric weakness of lower limbs. ### Explanation The differentiation between **Conus Medullaris Syndrome (CMS)** and **Cauda Equina Syndrome (CES)** is a high-yield neuroanatomy topic. **Why Option C is Correct:** The **Conus Medullaris** is the terminal, tapered end of the spinal cord (ending at L1-L2). Because it is a compact part of the central nervous system, a lesion here typically affects the spinal segments (S3-Co) bilaterally and simultaneously. This results in **symmetric** motor weakness and sensory loss. In contrast, the Cauda Equina consists of individual nerve roots; a lesion here is often eccentric, leading to asymmetric involvement. **Analysis of Incorrect Options:** * **A. Radicular Pain:** This is a hallmark of **Cauda Equina Syndrome**. Since CES involves compression of nerve roots, severe, asymmetric radicular pain is prominent. In CMS, pain is usually less severe and localized to the lower back (perianal/saddle area). * **B. Lower Motor Neuron (LMN) Injury:** While both can show LMN signs, CMS often presents with a **mix of UMN and LMN signs** (e.g., increased Achilles reflex but absent anal wink) because it involves the cord itself. CES is purely a peripheral nerve root (LMN) injury. * **D. Urinary Retention:** Both syndromes present with bladder and bowel dysfunction. However, in CMS, autonomic involvement occurs **early and suddenly**, whereas in CES, it is often a late-stage finding. **NEET-PG High-Yield Pearls:** 1. **Level of Lesion:** CMS occurs at **L1-L2**; CES occurs below L2. 2. **Saddle Anesthesia:** In CMS, it is **symmetric** and perianal; in CES, it is **asymmetric** and involves the saddle area. 3. **Reflexes:** In CMS, the Knee jerk (L2-L4) is preserved, but the Ankle jerk (S1) may be affected. In CES, both may be absent depending on the roots involved. 4. **Onset:** CMS has a sudden, bilateral onset; CES is typically gradual and unilateral/asymmetric.

Q: What is the characteristic EEG pattern observed in?

Creutzfeldt-Jakob Disease. ### Explanation **Correct Option: B. Creutzfeldt-Jakob Disease (CJD)** The characteristic EEG finding in CJD is **Periodic Sharp Wave Complexes (PSWCs)**, typically occurring at a frequency of 0.5 to 2.0 Hz [3]. These are seen in about 60-80% of patients, usually in the later stages of the disease. The underlying pathophysiology involves the rapid, widespread neuronal loss and spongiform changes caused by prion proteins, leading to synchronized, repetitive electrical discharges [3]. **Analysis of Incorrect Options:** * **A. Hepatic Encephalopathy:** The hallmark EEG finding is **Triphasic waves** (high-voltage, symmetrical, slow waves). While characteristic, they are not pathognomonic and can be seen in other metabolic encephalopathies (e.g., uremia). * **C. Generalized Tonic-Clonic Seizure (GTCS):** The EEG typically shows **generalized 10-12 Hz spikes** during the tonic phase, followed by **spike-and-slow-wave** discharges during the clonic phase [1], [2]. * **D. Herpes Simplex Encephalitis:** The classic finding is **Periodic Lateralized Epileptiform Discharges (PLEDs)**, usually localized to the temporal lobes, reflecting the focal necrotizing nature of the virus. **High-Yield Clinical Pearls for NEET-PG:** * **CJD Triad:** Rapidly progressive dementia, myoclonus (often startle-induced), and the characteristic PSWC on EEG [3]. * **Other CJD Markers:** Elevated **14-3-3 protein** in CSF and the **"Pulvinar sign"** (hyperintensity of the posterior thalamus) or **"Hockey stick sign"** on MRI (DWI/FLAIR sequences). * **Subacute Sclerosing Panencephalitis (SSPE):** Often confused with CJD; it shows **Radermecker complexes** (periodic high-voltage slow-wave complexes with longer intervals of 4-15 seconds).

Question 1

What is the diagnosis of this patient?

Accepted Answer

Muscular dystrophy

Answer

Myasthenia gravis

Answer

Congenital myopathy

Answer

Inclusion body myositis

Question 2

Which of the following is NOT true regarding Frontotemporal Dementia (FTD)?

Accepted Answer

FTD typically presents with a rapid onset and static course.

Answer

Semantic dementia can be a subtype of FTD.

Answer

Nonfluent aphasia can be a feature of FTD.

Answer

Apathetic or disinhibited personality changes can occur in FTD.

Question 3

A 23-year-old man complains of intermittent double vision and fatigue. The symptoms are worse at the end of the day, and he has stopped working as a courier because of easy leg fatigue and weakness. He finds that the symptoms improve with rest. On physical examination, there is weakness of the eyelids, masticatory muscles, and thigh flexors. Having him look up at the ceiling for a long time brings on his double vision. There is no sensory abnormality, and reflexes are normal. Which of the following treatments is contraindicated in this patient?

Accepted Answer

Anticholinergic drugs

Answer

Surgery

Answer

Plasmapheresis

Answer

Cyclophosphamide

Question 4

In herpes encephalitis, all of the following are true, except?

Accepted Answer

CT scan is better than MRI

Answer

Focal symptoms are common

Answer

The temporal lobe is involved

Answer

MRI is diagnostic

Question 5

A 16-year-old male presents with complaints of seeing wave-like visual disturbances and sudden monocular blindness, occurring while watching movies. What is the most likely diagnosis?

Accepted Answer

Migraine

Answer

Exhibitionism

Answer

Temporal lobe epilepsy

Answer

Grand mal epilepsy

Question 6

All of the following are true about Juvenile Myoclonic Epilepsy, except:

Accepted Answer

Focal seizures

Answer

Generalized seizures

Answer

Myoclonus

Answer

Responses to sodium valproate

Question 7

A patient presents with unilateral painful ophthalmoplegia. Imaging revealed an enlargement of the cavernous sinus on the affected side. What is the likely diagnosis?

Accepted Answer

Tolosa-Hunt syndrome

Answer

Gradenigo syndrome

Answer

Cavernous sinus thrombosis

Answer

Orbital pseudotumor

Question 8

The conus medullaris syndrome differs from the cauda equina syndrome in that the former shows which of the following?

Accepted Answer

Symmetric weakness of lower limbs

Answer

Radicular pain

Answer

Lower motor neuron injury

Answer

Urinary retention

Question 9

A 50-year-old right-handed female presented with right-sided hemiparesis, sensory loss, and right homonymous hemianopia. Neurological examination revealed dysarthria, global aphasia, alexia, agraphia, acalculia, and apraxia. Which artery is most likely involved in this condition?

Accepted Answer

Sylvian cistern

Answer

Cerebellomedullary cistern

Answer

Cisterna pontis

Answer

Quadrigeminal cistern

Question 10

What is the characteristic EEG pattern observed in?

Accepted Answer

Creutzfeldt-Jakob Disease

Answer

Hepatic encephalopathy

Answer

Generalized tonic-clonic seizure (GTCS)

Answer

Herpes simplex encephalitis

Neurology — MCQs

Neurology — MCQs

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