Neurodegenerative Disorders — MCQs
Which of the following is typically not associated with upper motor neuron (UMN) lesions?
A 78-year-old woman presents with a progressive decline in daily activity. She gives a history of convulsions and visual hallucinations. She does not talk to anyone and keeps looking at the sky. Pathological examination shows the presence of Lewy bodies within the neurons. What is the most probable diagnosis?
False regarding Alzheimer's disease (AD) is:
Mad Cow disease (Bovine Spongioform Encephalopathy) is similar in man to
Which of the following is a pathognomonic feature of Alzheimer's disease?
In Huntington's chorea, which of the following is not typically associated?
Subcortical dementia is seen in all EXCEPT:
A 60-year-old man has resting tremor, pill-rolling movements, rigidity, and bradykinesia. Which of the following is most likely to be decreased in this man?
All of the following are Anticholinesterases used in Alzheimer's disease except
A 40-year-old man is brought to the doctor by his family for a rapid intellectual decline. Examination reveals fast, semi-purposive movements in his hands. His family history is significant for similar illnesses. What is the most likely diagnosis?
Neurodegenerative Disorders Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following is typically not associated with upper motor neuron (UMN) lesions?
- A. DTR increased
- B. Atrophy (Correct Answer)
- C. Spasticity
- D. Rigidity
Explanation: ***Atrophy*** - **Severe atrophy** (significant muscle wasting) is a hallmark of **lower motor neuron (LMN) lesions**, where the direct innervation to the muscle is interrupted, leading to denervation and subsequent muscle mass loss [1]. - While some disuse atrophy can occur with UMN lesions due to **immobility**, it is typically not as pronounced or rapid as that seen with LMN lesions [1]. *DTR increased* - **Increased deep tendon reflexes (DTRs)**, also known as hyperreflexia, are a classic sign of **upper motor neuron (UMN) lesions** due to the loss of inhibitory control from higher centers on spinal reflex arcs [1]. - The stretch reflex arc becomes more excitable without descending modulation, leading to exaggerated responses. *Spasticity* - **Spasticity** is characterized by a **velocity-dependent increase in muscle tone** with increased resistance to passive stretch, often accompanied by hyperreflexia [1]. - This is a cardinal sign of **upper motor neuron (UMN) lesions**, resulting from the disinhibition of spinal reflex mechanisms. *Rigidity* - **Rigidity** is a form of hypertonia characterized by a **constant resistance to passive movement** throughout the entire range of motion, independent of the speed of movement [2]. - While it can be seen in some UMN conditions involving the basal ganglia (such as Parkinson's disease), it is **not a typical or direct consequence of UMN tract lesions** in the way spasticity is [2],[3].
Question 2: A 78-year-old woman presents with a progressive decline in daily activity. She gives a history of convulsions and visual hallucinations. She does not talk to anyone and keeps looking at the sky. Pathological examination shows the presence of Lewy bodies within the neurons. What is the most probable diagnosis?
- A. Prion disease
- B. Huntington's disease
- C. Lewy body dementia (Correct Answer)
- D. Alzheimer's disease
Explanation: ***Lewy body dementia*** - The presence of **progressive cognitive decline**, **visual hallucinations**, and **convulsions** in an elderly patient is highly indicative of Lewy body dementia. - The definitive pathological finding of **Lewy bodies** within neurons confirms the diagnosis. *Prion disease* - Characterized by rapidly progressive dementia, **myoclonus**, and cerebellar ataxia, without typical visual hallucinations or convulsions. - Pathological examination typically shows **spongiform changes** and accumulation of abnormal prion protein, not Lewy bodies. *Huntington's disease* - Presents with a classic triad of **motor dysfunction** (chorea), psychiatric symptoms, and cognitive decline, typically with an earlier onset (30-50 years). - It is an inherited neurodegenerative disorder, and its pathology involves neuronal loss in the striatum, without Lewy bodies. *Alzheimer's disease* - The most common cause of dementia, characterized by **memory impairment** as an early and prominent feature. - Pathological findings include **amyloid plaques** and **neurofibrillary tangles**, not Lewy bodies, and visual hallucinations are less common or occur later in the disease.
Question 3: False regarding Alzheimer's disease (AD) is:
- A. Number of neurofibrillary tangles is associated with the severity of dementia
- B. Number of senile (neuritic) plaques correlates (increases) with age
- C. Presence of tau protein suggest neurodegeneration
- D. Extracellular inclusion (lesion) can occur in the absence of intracellular inclusions to make pathological diagnosis of AD (Correct Answer)
Explanation: ***Extracellular inclusion (lesion) can occur in the absence of intracellular inclusions to make pathological diagnosis of AD*** - A definitive pathological diagnosis of **Alzheimer's disease** requires both the presence of **extracellular amyloid plaques** and **intracellular neurofibrillary tangles** [1]. - Neither inclusion type alone is sufficient for the diagnosis, as amyloid plaques can be found in non-demented elderly individuals [1]. *Number of neurofibrillary tangles is associated with the severity of dementia* - The **density and distribution of neurofibrillary tangles** (NFTs) directly correlate with the severity of cognitive impairment and **dementia** in AD [1]. - Tangles are composed of hyperphosphorylated **tau protein** and disrupt neuronal function, leading to neurodegeneration [2]. *Number of senile (neuritic) plaques correlates (increases) with age* - The accumulation of **senile (neuritic) plaques**, composed primarily of **beta-amyloid protein**, generally increases with age, even in cognitively normal individuals [1]. - While plaques are a hallmark of AD, their mere presence is not always diagnostic of clinical dementia [1]. *Presence of tau protein suggest neurodegeneration* - The presence of **hyperphosphorylated tau protein**, especially when forming **neurofibrillary tangles**, is a strong indicator of **neurodegeneration** [2]. - **Tauopathy** is a key pathological feature in AD and other neurodegenerative diseases [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1294. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722.
Question 4: Mad Cow disease (Bovine Spongioform Encephalopathy) is similar in man to
- A. Huntington's chorea
- B. Alzheimer's Disease
- C. Creutzfeldt Jakob Disease (Correct Answer)
- D. Pick's Disease
Explanation: ***Creutzfeldt Jakob Disease*** - **Creutzfeldt-Jakob Disease (CJD)** in humans is a **transmissible spongiform encephalopathy (TSE)**, caused by **prions**, which is pathophysiologically similar to Bovine Spongiform Encephalopathy (BSE) or "Mad Cow Disease" in cattle [2]. - The **variant CJD (vCJD)**, specifically linked to consuming beef products from BSE-infected cattle, shares similar pathological features, including widespread **spongiform changes** in the brain [1], [2]. *Huntington's chorea* - **Huntington's chorea** is an **autosomal dominant neurodegenerative disorder** caused by a genetic mutation leading to an expansion of CAG trinucleotide repeats, resulting in the aggregation of **huntingtin protein**. - Its pathology involves specific neuronal loss in the **striatum** and cortex, lacking the prion-induced spongiform changes seen in CJD. *Alzheimer's Disease* - **Alzheimer's Disease** is characterized by the accumulation of **amyloid plaques** (extracellular deposits of beta-amyloid protein) and **neurofibrillary tangles** (intracellular aggregates of hyperphosphorylated tau protein) [4]. - It is not a prion disease and does not involve the characteristic spongiform changes or transmission pathways associated with CJD [3]. *Pick's Disease* - **Pick's Disease** is a type of **frontotemporal dementia** distinguished by the presence of **Pick bodies**, which are intracellular aggregates of **hyperphosphorylated tau protein**. - Unlike CJD, it is not a prion disease and primarily affects the frontal and temporal lobes, causing specific types of dementia and behavioral changes without spongiform encephalopathy.
Question 5: Which of the following is a pathognomonic feature of Alzheimer's disease?
- A. Plaques and tangles (Correct Answer)
- B. Presence of Lewy bodies
- C. Presence of Pick bodies
- D. Red neuronal degeneration
Explanation: ***Plaques and tangles*** - The presence of **amyloid plaques** (extracellular deposits of beta-amyloid protein) and **neurofibrillary tangles** (intracellular aggregates of hyperphosphorylated tau protein) are the defining neuropathological hallmarks of Alzheimer's disease [1]. These are considered **pathognomonic** features upon post-mortem examination. - While other neurological conditions can have some tauopathy or amyloid deposition, the specific combination, distribution, and extensive nature of these two pathologies are unique to Alzheimer's [1]. [3] *Presence of Lewy bodies* - **Lewy bodies** are abnormal aggregates of alpha-synuclein protein that are characteristic of **Parkinson's disease** and **Lewy body dementia** [3]. - Their presence indicates a distinct neurodegenerative process, separate from Alzheimer's disease. *Presence of Pick bodies* - **Pick bodies** are cytoplasmic inclusions composed primarily of tau protein, but they are characteristic of **Pick's disease**, a type of frontotemporal dementia [2]. - Pick's disease has a different clinical presentation and neuropathological profile than Alzheimer's disease [2]. *Red neuronal degeneration (general feature)* - **Red neuronal degeneration** refers to the morphological changes seen in neurons undergoing acute irreversible ischemic injury, such as during a **stroke**. - It is a general feature of acute neuronal death and is not specific to, nor a pathognomonic feature of, Alzheimer's disease. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1292-1294. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1294-1295. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 721-722.
Question 6: In Huntington's chorea, which of the following is not typically associated?
- A. Slurred speech
- B. Dysmetria (Correct Answer)
- C. Degeneration of striatal GABA-ergic neurons
- D. Progressive dementia
Explanation: ***Dysmetria*** - **Dysmetria**, an inability to control the distance, speed, or coordination of movements, is more characteristic of **cerebellar dysfunction**, not typically a primary feature of Huntington's chorea [3]. - While some motor incoordination can occur, profound dysmetria specifically points away from the classic basal ganglia pathology of Huntington's disease. *Slurred speech* - **Dysarthria**, or slurred speech, is a very common symptom in Huntington's chorea due to the involuntary movements affecting the muscles of articulation. - The progressive neurological degeneration impacts various motor functions, including speech production. *Progressive dementia* - **Progressive dementia** is a hallmark feature of Huntington's chorea, manifesting as cognitive decline, executive dysfunction, and memory impairment. - This cognitive deterioration is often a significant and debilitating aspect of the disease. *Degeneration of striatal GABA-ergic neurons* - **Degeneration of striatal GABA-ergic neurons** within the caudate and putamen is the primary neuropathological finding in Huntington's chorea [1]. - This loss of inhibitory neurons leads to the characteristic hyperkinetic movements, such as chorea [2].
Question 7: Subcortical dementia is seen in all EXCEPT:
- A. Wilson's disease
- B. Parkinsonism
- C. Alzheimer's disease (Correct Answer)
- D. Huntington's chorea
Explanation: ***Alzheimer's disease*** - This is primarily a **cortical dementia**, characterized by widespread **cortical atrophy**, particularly in the **hippocampus** and temporal and parietal lobes [1]. - Its clinical presentation typically involves significant **memory impairment**, **aphasia**, **agnosia**, and **apraxia**, which are hallmarks of cortical dysfunction [1]. *Wilson's disease* - This is a **subcortical neurodegenerative disorder** caused by impaired copper metabolism leading to copper accumulation in the **basal ganglia**. - It often presents with **dysarthria**, **dystonia**, **ataxia**, and **behavioral changes**, consistent with subcortical involvement. *Parkinsonism* - Characterized by degeneration of the **substantia nigra** and loss of dopaminergic neurons, affecting the **basal ganglia**. - Commonly associated with **bradykinesia**, **rigidity**, **tremor**, and **postural instability**, and cognitive deficits often manifest as subcortical dementia. *Huntington's chorea* - This is a **neurodegenerative disorder** primarily affecting the **basal ganglia**, especially the caudate nucleus and putamen. - It presents with **chorea**, **psychiatric disturbances**, and **subcortical dementia**, including issues with executive function and slowed thought processes.
Question 8: A 60-year-old man has resting tremor, pill-rolling movements, rigidity, and bradykinesia. Which of the following is most likely to be decreased in this man?
- A. GABA neurons in the caudate nucleus and putamen
- B. Serotonin neurons in the raphe nuclei
- C. Acetylcholine neurons in the forebrain
- D. Dopamine neurons in the substantia nigra (Correct Answer)
Explanation: ***Dopamine neurons in the substantia nigra*** - The symptoms described—**resting tremor**, **pill-rolling movements**, **rigidity**, and **bradykinesia**—are classic signs of **Parkinson's disease** [5]. - Parkinson's disease is pathologically characterized by the degeneration of **dopamine-producing (dopaminergic) neurons** in the **substantia nigra pars compacta**, leading to decreased dopamine levels in the **striatum** [1]. *GABA neurons in the caudate nucleus and putamen* - **GABAergic neurons** in the **caudate nucleus and putamen** are primarily affected in conditions like **Huntington's disease**, where their degeneration leads to increased involuntary movements (chorea) [4]. - While there can be secondary changes in these neurons in Parkinson's, the primary deficit is not in GABA but in dopamine. *Serotonin neurons in the raphe nuclei* - **Serotonin neurons** in the **raphe nuclei** are involved in mood regulation, sleep, and appetite, and their dysfunction is primarily linked to conditions like **depression**, anxiety, and certain sleep disorders [3]. - While some serotonergic involvement can occur in Parkinson's, it is not the primary neurological deficit explaining the motor symptoms. *Acetylcholine neurons in the forebrain* - **Acetylcholine neurons** in the **nucleus basalis of Meynert** (part of the forebrain) are primarily implicated in **Alzheimer's disease**, where their degeneration contributes to cognitive decline [2]. - While some cholinergic deficits may be present in advanced Parkinson's, they are not the hallmark pathology or the initial cause of the characteristic motor symptoms.
Question 9: All of the following are Anticholinesterases used in Alzheimer's disease except
- A. Donepezil
- B. Rivastigmine
- C. Memantine (Correct Answer)
- D. Galantamine
Explanation: ***Memantine*** - While used in Alzheimer's disease, **memantine** is an **NMDA receptor antagonist**, not an anticholinesterase. - It works by blocking excessive **glutamatergic stimulation**, which is implicated in neurodegeneration. *Donepezil* - **Donepezil** is an **acetylcholinesterase inhibitor** used to treat mild to moderate Alzheimer's disease. - It increases the availability of **acetylcholine** in the synaptic cleft, improving cognitive function. *Rivastigmine* - **Rivastigmine** is a **cholinesterase inhibitor** that acts on both **acetylcholinesterase** and **butyrylcholinesterase**. - It is approved for the treatment of mild to moderate Alzheimer's disease and Parkinson's disease dementia. *Galantamine* - **Galantamine** is an **acetylcholinesterase inhibitor** and an **allosteric potentiator of nicotinic acetylcholine receptors**. - It enhances cholinergic neurotransmission and is used in the treatment of mild to moderate Alzheimer's disease.
Question 10: A 40-year-old man is brought to the doctor by his family for a rapid intellectual decline. Examination reveals fast, semi-purposive movements in his hands. His family history is significant for similar illnesses. What is the most likely diagnosis?
- A. Pre-senile Dementia
- B. Lewy body dementia
- C. Corticobasal degeneration
- D. Huntington's disease (Correct Answer)
Explanation: ***Huntington's disease*** - The combination of **rapid intellectual decline**, **chorea** (fast, semi-purposive movements), and a **family history** of similar illness strongly points to Huntington's disease [3]. - It is an **autosomal dominant** neurodegenerative disorder characterized by progressive motor, cognitive, and psychiatric symptoms. *Pre-senile Dementia* - This is a general term for dementia occurring before age 65, and while the patient has intellectual decline, it doesn't specify a cause or specific motor symptoms. - It does not account for the characteristic **chorea** and strong familial pattern seen in this case. *Lewy body dementia* - Characterized by **fluctuating cognition**, **visual hallucinations**, and **parkinsonism**, which differ from the movements described [1]. - While it involves cognitive decline, the prominent motor feature is usually rigidity and bradykinesia, not chorea [2]. *Corticobasal degeneration* - This condition typically presents with asymmetric rigidity, apraxia, and alien limb phenomenon, not with the generalized choreiform movements. - While it causes cognitive decline, the specific motor symptoms do not align with the patient's presentation.
Want unlimited practice?
Get full access to all questions, explanations, and performance tracking.
Start For Free