Neurology Practice Questions

Q: A 49-year-old man presents with progressive weakness in his arms and legs over 24 months. He has bulbar symptoms including dysphagia and dysarthria. EMG shows widespread denervation. What is the most important prognostic factor?

Bulbar involvement. ***Bulbar involvement*** - The presence of **bulbar symptoms** (dysphagia and dysarthria) at presentation or early in the disease course is the single most important predictor of shorter survival in **Amyotrophic Lateral Sclerosis (ALS)**. - This is primarily because bulbar dysfunction leads quickly to difficulty swallowing, increasing the risk of **aspiration pneumonia** and malnutrition, and often precedes necessary respiratory intervention. *Age at onset* - While a factor, **older age (>65 years)** at onset is associated with a worse prognosis but is generally considered less influential than the pattern of motor neuron involvement. - Patients with **younger onset** (e.g., in their 40s) tend to have a slightly better prognosis than those with onset in their 70s. *Rate of progression* - The **rate of progression** directly determines the prognosis (survival time) itself, reflecting the speed of motor neuron loss. - However, **bulbar involvement** is often the underlying *predictor* or characteristic that drives a rapid rate of progression, making the bulbar site the more fundamental prognostic factor. *Site of onset* - The specific **site of onset** is critical, distinguishing between **limb-onset** and **bulbar-onset** ALS. - Because bulbar onset conveys the worst prognosis, selecting the specific involvement (**bulbar**) is more precise than selecting the general concept of

Q: A 28-year-old woman presents with sudden onset severe headache during exercise. She vomits and has neck stiffness. CT head shows subarachnoid hemorrhage. What is the most likely cause?

Berry aneurysm. ***Berry aneurysm*** - The sudden onset of a severe, explosive **thunderclap headache** coupled with signs of meningeal irritation (vomiting, neck stiffness) and the finding of **subarachnoid hemorrhage (SAH)** on CT head is the classic presentation of a ruptured saccular (**berry**) aneurysm. - Exertion (exercise) acts as a common trigger for aneurysm rupture due to the sudden increase in **intracranial pressure** and transmural wall stress. *Arteriovenous malformation* - AVMs more commonly present with **intraparenchymal hemorrhage** (ICH) or seizures, rather than isolated SAH, especially when triggered by exertion. - While AVMs can cause SAH, they are a less frequent cause of widespread spontaneous SAH compared to ruptured **berry aneurysms**. *Carotid dissection* - Carotid artery dissection typically manifests as severe neck/facial pain, headache, and signs of **cerebral ischemia** (stroke symptoms) due to vessel stenosis or occlusion. - Dissections rarely cause primary, widespread SAH; they are more often associated with **subintimal hemorrhage** leading to stroke or pseudoaneurysm formation. *Hypertensive hemorrhage* - Hemorrhage due to chronic or severe hypertension (hypertensive hemorrhage) almost exclusively causes deep **intraparenchymal hemorrhage** (ICH), usually in the basal ganglia, thalamus, or brainstem. - The primary finding of SAH, rather than ICH and the patient's young age, makes uncontrolled hypertension highly unlikely as the underlying cause. *Trauma* - Although trauma is the single most frequent overall cause of SAH, the clinical history describes a **spontaneous event** triggered by exertion (exercise) without any external injury. - Traumatic SAH is clinically differentiated by the history of a specific injury and usually involves bleeding adjacent to the site of impact.

Q: A 41-year-old man presents with progressive weakness in his hands and arms over 12 months. He has muscle fasciculations and hyperreflexia. EMG shows both acute and chronic denervation changes. What is the prognosis?

Poor with 3-5 year survival. ***Poor with 3-5 year survival*** - The presentation with **progressive weakness**, **muscle fasciculations** (lower motor neuron sign), and **hyperreflexia** (upper motor neuron sign), along with mixed **acute and chronic denervation** on EMG, is highly characteristic of **Amyotrophic Lateral Sclerosis (ALS)**. - **ALS** is a rapidly progressive neurodegenerative disease, and the typical survival time from diagnosis is **3 to 5 years**, often due to respiratory failure. *Excellent with treatment* - **ALS** is an incurable disease, and current treatments such as **Riluzole** or **Edaravone** only offer a modest slowing of disease progression or symptom management, not an excellent prognosis. - There is no known treatment that can halt or reverse the neurodegeneration characteristic of **ALS**. *Good with supportive care* - While **supportive care** (e.g., respiratory support, physical therapy, nutritional guidance) is essential for managing symptoms and improving quality of life in **ALS**, it does not alter the underlying progressive and fatal nature of the disease. - The relentless degeneration of motor neurons continues despite comprehensive supportive measures, leading to eventual paralysis and death. *Variable depending on subtype* - While there are different clinical presentations (e.g., bulbar vs. limb onset) and genetic forms of **ALS**, the overall prognosis for classic **ALS** is uniformly poor. - Although a small percentage of patients may have a longer survival, the vast majority follow a progressive course with a limited life expectancy, making a

Q: A 57-year-old woman presents with progressive memory loss and behavioral changes. MRI shows frontotemporal atrophy. What is the most likely initial symptom domain affected?

Behavioral changes. ***Behavioral changes***- **Frontotemporal dementia (FTD)**, indicated by **frontotemporal atrophy** on MRI, typically presents with prominent **behavioral or language changes** in its initial stages. - In **behavioral variant FTD (bvFTD)**, early symptoms include progressive changes in personality, social conduct, and executive function, such as **disinhibition**, **apathy**, or **loss of empathy**.*Memory loss*- While memory loss can occur in FTD, it is typically **not the initial or most prominent symptom** in the behavioral variant, differentiating it from Alzheimer's disease.- In bvFTD, **episodic memory** is often relatively preserved in the early stages, with more significant deficits in executive function and social cognition.*Language difficulties*- **Primary progressive aphasia (PPA)** is another major subtype of FTD where language difficulties are the initial and prominent symptom.- However, the question highlights "behavioral changes" as a key presenting feature, which is characteristic of the behavioral variant of FTD, where language issues are usually not primary.*Motor symptoms*- Motor symptoms like parkinsonism, amyotrophy (ALS-like symptoms), or supranuclear gaze palsy can be associated with specific FTD subtypes (e.g., FTD with **motor neuron disease**).- These are generally **later manifestations** or indicate distinct clinical syndromes, not typically the initial domain affected in the most common presentations of bvFTD.*Visual problems*- Visual problems are **not a typical initial symptom domain** in frontotemporal dementia.- Such symptoms are more commonly associated with posterior cortical atrophy (a variant of Alzheimer's disease) or other neurodegenerative conditions affecting visual pathways.

Question 1

A 49-year-old man presents with progressive weakness in his arms and legs over 24 months. He has bulbar symptoms including dysphagia and dysarthria. EMG shows widespread denervation. What is the most important prognostic factor?

Accepted Answer

Bulbar involvement

Answer

Age at onset

Answer

Rate of progression

Answer

Site of onset

Answer

Family history

Question 2

A 28-year-old woman presents with sudden onset severe headache during exercise. She vomits and has neck stiffness. CT head shows subarachnoid hemorrhage. What is the most likely cause?

Accepted Answer

Berry aneurysm

Answer

Arteriovenous malformation

Answer

Carotid dissection

Answer

Hypertensive hemorrhage

Answer

Trauma

Question 3

A 41-year-old man presents with progressive weakness in his hands and arms over 12 months. He has muscle fasciculations and hyperreflexia. EMG shows both acute and chronic denervation changes. What is the prognosis?

Accepted Answer

Poor with 3-5 year survival

Answer

Excellent with treatment

Answer

Good with supportive care

Answer

Variable depending on subtype

Answer

Stable with medication

Question 4

A 62-year-old man presents with progressive weakness in his arms and legs over 18 months. He has difficulty speaking and swallowing. EMG shows widespread denervation. What is the most important prognostic factor?

Accepted Answer

Bulbar involvement

Answer

Age at onset

Answer

Site of onset

Answer

Rate of progression

Answer

Genetic factors

Question 5

A 57-year-old woman presents with progressive memory loss and behavioral changes. MRI shows frontotemporal atrophy. What is the most likely initial symptom domain affected?

Accepted Answer

Behavioral changes

Answer

Memory loss

Answer

Language difficulties

Answer

Motor symptoms

Answer

Visual problems

Neurology — MCQs

Neurology — MCQs

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