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What is the primary pathophysiological hallmark of multiple sclerosis?
A. Muscle degeneration
B. Immune-mediated demyelination
C. Synaptic overactivity
D. Neuronal regeneration
Answer: B
Rationale: MS is characterized by immune-mediated destruction of myelin in the CNS.
Which of the following is an initial manifestation of myasthenia gravis?
A. Limb paralysis
B. Ptosis and diplopia
C. Incontinence
D. Hyperreflexia
Answer: B
Rationale: Ocular symptoms like ptosis and diplopia are usually the first signs of MG.
Guillain-Barré syndrome is most commonly triggered by:
A. Seizure activity
B. Prolonged immobility
C. Viral or bacterial infections
D. Thyroid dysfunction
Answer: C
Rationale: Infections, particularly by Campylobacter jejuni, often precede GBS onset.
Which medication is commonly used to manage spasticity in patients with MS?
A. Amantadine
B. Pyridostigmine
C. Baclofen
D. Azathioprine
Answer: C
Rationale: Baclofen is the drug of choice for MS-related spasticity.
What is the diagnostic test most specific for confirming MG?
A. CT scan
B. Ice pack test
C. Acetylcholinesterase inhibitor test
D. CSF analysis
Answer: C
Rationale: The edrophonium (Tensilon) test confirms MG by transiently reversing muscle weakness.
Which medication provides symptomatic relief in MG by increasing acetylcholine availability?
A. Diazepam
B. Pyridostigmine
C. Methylprednisolone
D. Gabapentin
Answer: B
Rationale: Pyridostigmine is an anticholinesterase that boosts acetylcholine levels.
What is a common complication of GBS that requires ICU-level care?
A. Loss of vision
B. Renal failure
C. Respiratory failure
D. Cognitive decline
Answer: C
Rationale: Respiratory muscle weakness can rapidly progress in GBS.
Which diagnostic test shows oligoclonal banding in MS?
A. MRI
B. EEG
C. CSF electrophoresis
D. PET scan
Answer: C
Rationale: Oligoclonal bands in CSF support the diagnosis of MS.
Which medication is used to reduce relapse rates in relapsing-remitting MS?
A. Prednisone
B. Interferon beta-1a
C. Diazepam
D. Atropine
Answer: B
Rationale: Interferon beta-1a is a disease-modifying therapy.
What test is used to assess respiratory compromise in GBS?
A. MRI
B. Negative inspiratory force
C. Tensilon test
D. Eye movement tracking
Answer: B
Rationale: Respiratory function tests assess for neuromuscular compromise in GBS.
Name the autoimmune disorder that affects the neuromuscular junction and leads to fluctuating muscle weakness.
Answer: Myasthenia Gravis
Rationale: MG targets acetylcholine receptors, impairing muscle activation.
What autoimmune condition causes ascending paralysis starting from the lower limbs?
Answer: Guillain-Barré Syndrome
Rationale: GBS typically begins with leg weakness that ascends.
What medication is the antidote to edrophonium used during the Tensilon test?
Answer: Atropine
Rationale: Atropine reverses the effects of excessive cholinergic stimulation.
Which imaging test identifies CNS plaques in MS?
Answer: MRI
Rationale: MRI reveals demyelinating plaques in MS patients
Name the therapy involving pooled human immunoglobulin used in MG and GBS.
Answer: IVIG (Intravenous Immune Globulin)
Rationale: IVIG modulates the immune response during exacerbations.
Answer: IVIG (Intravenous Immune Globulin)
Rationale: IVIG modulates the immune response during exacerbations.
Answer: Decreased vital capacity
Rationale: Diminished VC precedes respiratory failure in GBS.
Which organ is surgically removed in MG to improve symptoms?
Answer: Thymus
Rationale: Thymectomy can induce remission in MG.
What kind of reflex change is typical in GBS?
Answer: Hyporeflexia
Rationale: GBS affects peripheral nerves, leading to reduced reflexes.
What medication is used to treat fatigue in MS?
Answer: Amantadine
Rationale: Amantadine helps manage MS-related fatigue.
What nursing intervention helps with MS-related bowel incontinence?
Answer: Bowel training program
Rationale: Structured timing and diet promote regularity.
True or False: Myasthenia Gravis affects coordination and sensation.
Answer: False
Rationale: MG is a purely motor disorder.
True or False: GBS commonly affects cognitive function.
Answer: False
Rationale: GBS spares cognition and consciousness.
True or False: MS affects more women than men.
Answer: True
Rationale: MS incidence is higher in females.
True or False: Spasticity in MS can be managed with benzodiazepines.
Answer: True
Rationale: Diazepam and related drugs are used for this purpose.
True or False: The ice pack test helps diagnose myasthenia gravis in cardiac patients.
Answer: True
Rationale: It avoids the cardiovascular risks of Tensilon.
True or False: Interferon beta is used in both relapsing-remitting and primary progressive MS.
Answer: False
Rationale: It is not effective in primary progressive MS.
True or False: GBS symptoms peak within 2 to 4 weeks.
Answer: True
Rationale: Most cases reach maximum severity by this time.
True or False: IVIG and TPE are used in both MG and GBS.
Answer: True
Rationale: These immune-modulating treatments are common to both.
True or False: MS causes progressive loss of coordination and cognitive function.
Answer: True
Rationale: MS can lead to both motor and cognitive decline.
True or False: In MG crisis, a sudden increase in anticholinesterase drugs improves breathing.
Answer: False
Rationale: Overdose can worsen symptoms—this could indicate cholinergic crisis.
What is the most common cause of syringomyelia?
A. Brain tumor
B. Chiari Malformation
C. Meningitis
D. Trauma
Answer: B
Rationale: Chiari malformation causes cerebellar protrusion that obstructs CSF flow, leading to syrinx formation.
Which of the following is a cardinal sign of Parkinson’s disease?
A. Facial flushing
B. Resting tremor
C. Blurred vision
D. Projectile vomiting
Answer: B
Rationale: A unilateral resting tremor is one of the hallmark signs of PD.
What is the purpose of combining levodopa with carbidopa?
A. To reduce blood pressure
B. To increase liver metabolism
C. To prevent premature breakdown of levodopa
D. To enhance digestion
Answer: C
Rationale: Carbidopa prevents peripheral metabolism of levodopa before reaching the brain.
Which symptom is a classic indicator of syringobulbia?
A. Inability to swallow
B. Leg swelling
C. Seizures
D. Diplopia
Answer: A
Rationale: Syringobulbia affects the brainstem and may cause bulbar symptoms like dysphagia.
What surgical intervention drains a syrinx into the subarachnoid space?
A. Craniectomy
B. Syringotomy
C. Ventriculostomy
D. Myelotomy
Answer: B
Rationale: Syringotomy is a decompressive procedure to relieve fluid pressure in a syrinx.
Which of the following best describes bradykinesia?
A. Complete loss of movement
B. Random jerking movements
C. Slowed voluntary movement
D. Rapid repetitive speech
Answer: C
Rationale: Bradykinesia is characterized by delayed movement and difficulty initiating motion.
Which test is most accurate in showing soft tissue changes in syringomyelia?
A. X-ray
B. CT Scan
C. Myelogram
D. MRI
Answer: D
Rationale: MRI is the best imaging modality for soft tissue detail and identifying syrinxes.
Which class of drugs is used to counteract tremors in Parkinson's disease?
A. Dopamine agonists
B. Anticholinergic agents
C. COMT inhibitors
D. MAO-B inhibitors
Answer: B
Rationale: Anticholinergics reduce acetylcholine's excitatory effects, controlling tremors.
Which medication is a non-ergot dopamine agonist?
A. Pramipexole
B. Selegiline
C. Tolcapone
D. Trihexyphenidyl
Answer: A
Rationale: Pramipexole is a non-ergot derivative used in early PD.
Which procedure stimulates dopamine release by implanting electrodes?
A. Thalamotomy
B. Pallidotomy
C. Deep Brain Stimulation
D. Corticectomy
Answer: C
Rationale: DBS uses electrodes in brain regions to modify dopamine and acetylcholine balance.
What term describes a progressive loss of neuron function leading to death of nerve cells?
Answer: Neurodegeneration
Rationale: Neurodegenerative diseases involve irreversible loss of neuronal structure and function.
Which imaging technique may reveal bony spinal abnormalities associated with syringomyelia?
Answer: CT Scan
Rationale: CT is better for visualizing bone structures than soft tissues.
Name one common autonomic manifestation in Parkinson’s disease.
Answer: Orthostatic hypotension
Rationale: PD frequently affects autonomic function, causing blood pressure instability.
Which drug inhibits dopamine breakdown and can be used in early PD with fewer side effects?
Answer: Selegiline
Rationale: Selegiline is a monoamine oxidase-B inhibitor that preserves dopamine.
What is the typical age of onset for idiopathic Parkinson’s disease?
Answer: 60s
Rationale: Most PD cases begin around the 6th decade of life
Which test is used when MRI cannot be performed in diagnosing syringomyelia?
Answer: Myelogram
Rationale: Myelograms visualize spinal cord structure when MRI is contraindicated.
What is the surgical removal of part of the vertebrae to relieve spinal cord pressure called?
Answer: Laminectomy
Rationale: This decompresses the spinal canal by removing vertebral bon
Which pharmacologic agent may be used to relieve muscle spasms in syringomyelia?
Answer: Methocarbamol
Rationale: It's a muscle relaxant used for symptomatic management.
What antiviral agent may increase dopamine release in Parkinson’s disease?
Answer: Amantadine
Rationale: Amantadine enhances dopamine availability in the CNS.
Which procedure diverts CSF from a syrinx to another body cavity?
Answer: Shunting
Rationale: Shunts are used to drain fluid and relieve pressure from the syrinx.
True or False: Syringomyelia always presents with symptoms.
Answer: False
Rationale: Some patients are asymptomatic and only require monitoring.
True or False: A diagnosis of Parkinson’s disease requires at least 2 of the 4 cardinal signs.
Answer: True
Rationale: Diagnosis is clinical and based on signs like tremor, rigidity, and bradykinesia.
True or False: PET and SPECT scans are definitive tests for Parkinson’s disease.
Answer: False
Rationale: These support diagnosis but are not definitive.
True or False: Levodopa should be started early to prevent disease progression.
Answer: False
Rationale: Levodopa is often delayed to reduce long-term complications.
True or False: Parkinson’s disease affects both motor and non-motor functions.
Answer: True
Rationale: PD includes psychiatric and autonomic symptoms.
True or False: Syringomyelia can result from post-infectious arachnoiditis.
Answer: True
Rationale: Inflammation post-infection may obstruct CSF flow.
True or False: Entacapone requires liver function monitoring due to hepatotoxicity risk.
Answer: False
Rationale: Tolcapone carries the hepatotoxicity risk, not entacapone
True or False: Anticholinergics are the most effective drug for bradykinesia.
Answer: False
Rationale: These help tremor more than bradykinesia.
True or False: A mask-like face is a common finding in Parkinson’s disease.
Answer: True
Rationale: Facial muscle rigidity causes this appearance.
True or False: Dopamine agonists are typically used only in the advanced stages of PD.
Answer: False
Rationale: They are used in early stages to delay levodopa initiation.
Which gene mutation is responsible for Huntington’s Disease?
A. ApoE4
B. HTT
C. SOD1
D. MAPT
Answer: B
Rationale: Huntington’s Disease is caused by a mutation in the HTT gene, leading to the formation of abnormal Huntingtin protein.
What triad of symptoms characterizes Huntington’s Disease?
A. Memory loss, hallucinations, fever
B. Rigidity, hyperreflexia, dysphagia
C. Motor dysfunction, cognitive impairment, behavioral changes
D. Sensory loss, spasticity, ataxia
Answer: C
Rationale: The hallmark triad includes choreiform movements (motor), dementia (cognitive), and psychiatric symptoms (behavioral).
Which neurotransmitter is involved in the neurodegeneration seen in ALS?
A. Acetylcholine
B. Glutamate
C. Dopamine
D. Serotonin
Answer: B
Rationale: Glutamate excitotoxicity is believed to contribute to neuronal injury in ALS.
A patient presents with progressive speech slurring and palatal weakness. Which neurodegenerative disorder is most likely?
A. Huntington’s Disease
B. Alzheimer’s Disease
C. ALS with bulbar involvement
D. Parkinson’s Disease
Answer: C
Rationale: Bulbar symptoms in ALS include dysarthria, palatal weakness, and dysphagia.
What cardinal brain structure shows atrophy in Alzheimer’s Disease on MRI?
A. Thalamus
B. Pons
C. Hippocampus
D. Cerebellum
Answer: C
Rationale: The hippocampus, crucial for memory, shows early and progressive atrophy in AD.
What is the inheritance pattern of Huntington’s Disease?
A. Autosomal recessive
B. X-linked
C. Mitochondrial
D. Autosomal dominant
Answer: D
Rationale: A child of an affected parent has a 50% chance of inheriting the gene.
What medication is used to manage chorea in Huntington’s Disease?
A. Levodopa
B. Tetrabenazine
C. Donepezil
D. Riluzole
Answer: B
Rationale: Tetrabenazine reduces involuntary movements in HD.
Which medication is FDA-approved for ALS and is thought to extend life by a few months?
A. Donepezil
B. Baclofen
C. Tetrabenazine
D. Riluzole
Answer: D
Rationale: Riluzole is a glutamate antagonist with modest life-extending benefits in ALS
Which symptom is seen in the early stage of Alzheimer’s Disease?
A. Complete loss of speech
B. Inability to recognize family
C. Memory loss and difficulty completing familiar tasks
D. Total dependence for ADLs
Answer: C
Rationale: Early AD is marked by memory problems and mild confusion
What class of drugs is used in mild to moderate Alzheimer’s Disease to prevent acetylcholine breakdown?
A. SSRIs
B. NMDA antagonists
C. Cholinesterase inhibitors
D. Antipsychotics
Answer: C
Rationale: Donepezil, rivastigmine, and galantamine are cholinesterase inhibitors used to maintain cholinergic function
Name the disorder characterized by progressive choreiform movements, dementia, and behavioral changes.
Answer: Huntington’s Disease
Rationale: These are the classic triad of Huntington’s Disease.
What motor symptom in ALS is indicated by muscle twitching visible under the skin?
Answer: Fasciculations
Rationale: Fasciculations are a hallmark lower motor neuron sign in ALS.
What protein accumulates as plaques in the brain of Alzheimer’s patients?
Answer: Beta-amyloid
Rationale: Beta-amyloid plaques are thought to play a key role in AD pathophysiology
What test confirms the genetic mutation for Huntington’s Disease?
Answer: CAG repeat testing in the HTT gene
Rationale: Expanded CAG trinucleotide repeats confirm diagnosis.
Which brain area shows early imaging changes in Alzheimer’s Disease?
Answer: Hippocampus
Rationale: The hippocampus is involved in encoding new memories.
Which medication class is used to manage spasticity in ALS?
Answer: Muscle relaxants (e.g., Baclofen, Dantrolene)
Rationale: These reduce muscle rigidity and improve comfort.
Which psychiatric condition is commonly seen in Huntington’s Disease?
Answer: Depression
Rationale: HD is often associated with mood disorders, especially depression and anxiety.
Which neurotransmitter is primarily deficient in Alzheimer’s Disease?
Answer: Acetylcholine
Rationale: Loss of cholinergic neurons leads to memory deficits in AD.
Which NMDA antagonist is used in moderate to severe AD?
Answer: Memantine
Rationale: Memantine blocks excessive calcium influx via NMDA receptors.
Which neurodegenerative disease requires early planning for ventilatory support?
Answer: Amyotrophic Lateral Sclerosis
Rationale: ALS often progresses to respiratory failure due to muscle atrophy.
True or False: ALS affects both upper and lower motor neurons.
Answer: True
Rationale: ALS shows signs of both upper and lower motor neuron dysfunction.
True or False: Alzheimer’s Disease always presents with sudden memory loss.
Answer: False
Rationale: AD has a gradual onset and progression.
True or False: Riluzole is used in Huntington’s Disease.
Answer: False
Rationale: Riluzole is specific for ALS management.
True or False: NMDA antagonists work by increasing calcium influx.
Answer: False
Rationale: They block NMDA receptors to reduce calcium overload.
True or False: In HD, chorea worsens significantly in late-stage disease.
Answer: False
Rationale: Motor symptoms often decrease as cognitive and functional decline progresses.
True or False: A PEG tube may be required in late-stage ALS.
Answer: True
Rationale: Dysphagia necessitates alternative feeding routes.
True or False: CT scan is diagnostic for Alzheimer’s Disease.
Answer: False
Rationale: CT may rule out other causes; diagnosis is largely clinical and supported by MRI and labs.
True or False: Genetic counseling is important for families affected by Huntington’s Disease.
Answer: True
Rationale: HD is autosomal dominant; offspring have a 50% risk.
True or False: Emotional lability can occur in both ALS and HD.
Answer: True
Rationale: Both disorders can involve mood swings and psychiatric symptoms.
True or False: The primary goal of treatment for neurodegenerative diseases is cure
Answer: False
Rationale: There is no cure; management is aimed at symptom relief and quality of life.