AIM • FINAL YEAR MBBS • KMU CURRICULUM
KMU Past Paper Practice
Topic 10. Acquired & Hereditary Neuropathies
Neuroscience Module • 20 A-Type Single Best Answer MCQs • Original KMU-style practice (not verified historical questions)
MCQ 1
Question: A 32-year-old man has developed two episodes of wrist drop after sleeping with his arm compressed and one episode of foot drop after prolonged leg crossing. His symptoms improved between episodes, and several relatives report similar attacks. Which disorder best explains the repeated focal palsies?
Options:
Charcot–Marie–Tooth disease type 2
Hereditary neuropathy with liability to pressure palsies
Chronic inflammatory demyelinating polyradiculoneuropathy
Diabetic distal symmetric polyneuropathy
Guillain–Barré syndrome
Correct Answer: Hereditary neuropathy with liability to pressure palsies
Explanation: Inherited susceptibility of peripheral nerves to minor compression causes recurrent focal palsies in different nerve territories.
MCQ 2
Question: An 8-year-old child has repeated painless burns and ulcers on the feet. Examination reveals markedly impaired pain and temperature sensation, while strength is relatively preserved. Similar problems occurred in an older sibling. Which hereditary neuropathy category is most appropriate?
Options:
Predominantly motor axonal neuropathy
Primary inflammatory demyelinating neuropathy
Isolated motor nerve entrapment
Hereditary sensory and autonomic neuropathy
Acute postinfectious polyradiculoneuropathy
Correct Answer: Hereditary sensory and autonomic neuropathy
Explanation: Inherited sensory and autonomic fibre dysfunction reduces protective pain perception, allowing repeated unnoticed injuries.
MCQ 3
Question: A 24-year-old woman has burning discomfort in both soles and reduced pinprick sensation, but vibration sense, muscle power and tendon reflexes are preserved. Routine nerve conduction studies are normal. What is the most appropriate interpretation?
Options:
Pure small-fibre neuropathy remains possible
Peripheral neuropathy has been completely excluded
A large-fibre demyelinating neuropathy is confirmed
The pattern establishes corticospinal tract disease
The findings confirm a primary muscle disorder
Correct Answer: Pure small-fibre neuropathy remains possible
Explanation: Routine conduction tests assess larger fibres and may be normal when damage mainly involves small pain and temperature fibres.
MCQ 4
Question: A 29-year-old farmer presents with a pale skin patch that has reduced sensation, together with enlargement of a nearby peripheral nerve. He also reports weakness of intrinsic hand muscles. Which acquired cause should be included prominently in the assessment?
Options:
Vitamin B12 deficiency
Uremic neuropathy
Leprosy-related peripheral neuropathy
Chemotherapy-induced neuropathy
Hypothyroidism-related neuropathy
Correct Answer: Leprosy-related peripheral neuropathy
Explanation: A hypoesthetic skin lesion with nerve thickening is a characteristic clue to leprosy-related peripheral nerve disease.
MCQ 5
Question: A 41-year-old man develops acute ascending weakness and generalized areflexia after an infection. Lumbar puncture performed on the second day shows normal CSF protein and no significant pleocytosis. Which conclusion is best?
Options:
A normal early CSF result rules out Guillain–Barré syndrome
The result confirms a hereditary neuropathy
The result establishes chronic inflammatory neuropathy
The patient can safely be discharged because CSF is normal
Guillain–Barré syndrome remains possible despite normal early CSF
Correct Answer: Guillain–Barré syndrome remains possible despite normal early CSF
Explanation: Albuminocytologic dissociation may take time to develop, so early normal CSF protein does not exclude GBS.
MCQ 6
Question: A hospitalized patient has progressive symmetric weakness and areflexia consistent with Guillain–Barré syndrome. The team discusses disease-directed treatment. Which regimen should not be used as the sole specific therapy for typical GBS?
Options:
Intravenous immunoglobulin
Oral corticosteroids alone
Plasma exchange
Supportive respiratory monitoring
Swallowing and autonomic surveillance
Correct Answer: Oral corticosteroids alone
Explanation: Corticosteroids alone have not shown benefit in typical GBS, whereas IVIG or plasma exchange can modify the disease course.
MCQ 7
Question: A 37-year-old man with rapidly progressing suspected Guillain–Barré syndrome develops a weak cough and choking when drinking water. Which additional neurological involvement is the immediate concern?
Options:
Isolated sensory loss of the toes
Chronic foot deformity
Basal ganglia movement dysfunction
Bulbar muscle weakness compromising airway protection
Slowly developing intrinsic hand wasting
Correct Answer: Bulbar muscle weakness compromising airway protection
Explanation: Difficulty swallowing and an ineffective cough indicate possible bulbar weakness and aspiration or airway risk.
MCQ 8
Question: A 46-year-old typist has tingling in the thumb, index and middle fingers and intermittent nighttime waking. Power is preserved and symptoms are mild. Which initial treatment best addresses the likely local nerve compression?
Options:
Neutral-position wrist splinting
Immediate immune globulin infusion
Long-term systemic immunosuppression
Empirical vitamin B12 injections irrespective of results
Routine lumbar spine surgery
Correct Answer: Neutral-position wrist splinting
Explanation: A neutral wrist splint reduces median nerve compression in mild carpal tunnel syndrome, particularly during sleep.
MCQ 9
Question: A 52-year-old carpenter has progressive thenar muscle wasting and weakness of thumb opposition, along with longstanding numbness in the median nerve distribution. Symptoms persist despite conservative measures. What is the most appropriate next management step?
Options:
Continue observation indefinitely
Start treatment for generalized inherited neuropathy
Seek specialist assessment for nerve decompression
Arrange treatment for acute demyelinating polyradiculoneuropathy
Treat presumptively for spinal cord inflammation
Correct Answer: Seek specialist assessment for nerve decompression
Explanation: Progressive motor loss in an entrapment neuropathy suggests significant nerve compromise and warrants specialist surgical assessment.
MCQ 10
Question: A 49-year-old woman has progressive sensory symptoms. The examination shows reduced pinprick and temperature sensation in both feet but preserved joint position sense. Which clinical finding most strongly indicates that small sensory fibres predominate?
Options:
Absent ankle reflexes with profound distal muscle wasting
Severe loss of vibration at both great toes
Marked sensory ataxia in darkness
Spasticity with extensor plantar responses
Loss of temperature discrimination with preserved proprioception
Correct Answer: Loss of temperature discrimination with preserved proprioception
Explanation: Small fibres transmit pain and temperature, whereas joint position sensation primarily depends on large myelinated sensory fibres.
MCQ 11
Question: A 68-year-old man presents with unexplained progressive sensorimotor polyneuropathy. Common metabolic and nutritional tests are unrevealing. Examination and clinical history raise concern for an underlying abnormal circulating protein. Which further test category is most relevant?
Options:
Electroencephalographic monitoring
Serum protein studies
Routine stool microscopy
Cardiac exercise testing
Isolated plain foot radiography
Correct Answer: Serum protein studies
Explanation: Selected serum protein testing can help identify a paraproteinaemic cause when clinical clues and initial investigation findings warrant it.
MCQ 12
Question: A 44-year-old patient has chronic burning pain attributed to acquired peripheral neuropathy. Paracetamol has provided minimal relief. Which alternative is specifically used to treat neuropathic pain rather than simply ordinary nociceptive pain?
Options:
Amoxicillin
Furosemide
Metformin
Duloxetine
Levothyroxine
Correct Answer: Duloxetine
Explanation: Duloxetine is used for neuropathic pain; conventional simple analgesics often do not adequately control neuropathic burning symptoms.
MCQ 13
Question: A 58-year-old woman with severe distal sensory loss accidentally develops a blister after using a hot-water bottle. She did not feel the heat. Which preventive advice most directly addresses the mechanism of injury?
Options:
Avoid direct heat and inspect skin regularly
Use heat until the skin becomes visibly red
Increase activity only when all sensation returns
Apply prophylactic antibiotics to intact skin daily
Avoid protective footwear to improve awareness
Correct Answer: Avoid direct heat and inspect skin regularly
Explanation: Loss of protective sensation permits unnoticed thermal injury; avoiding excessive heat and examining the feet reduce preventable harm.
MCQ 14
Question: A 13-year-old boy has slowly progressive pes cavus, hammer toes and difficulty with running. Neither parent has apparent neurological symptoms. Which statement about an inherited neuropathy is most accurate?
Options:
It cannot occur without an affected parent
Normal parental examinations exclude CMT
A negative family history does not exclude hereditary neuropathy
The findings establish a recent bacterial infection
Childhood foot deformity always indicates a spinal cord lesion
Correct Answer: A negative family history does not exclude hereditary neuropathy
Explanation: Recessive inheritance, variable expression and newly arising variants can explain apparently sporadic inherited neuropathy.
MCQ 15
Question: A 10-year-old girl is suspected of having a hereditary motor and sensory neuropathy. Examination confirms distal wasting and foot deformity. Which additional history most directly clarifies the impact of the disease at her age?
Options:
Past adult occupational solvent exposure
History of coronary artery disease in grandparents
Ability to participate in school, play and daily activities
Recent episodes of adult-onset hypertension
Past childhood appendicectomy
Correct Answer: Ability to participate in school, play and daily activities
Explanation: Pediatric assessment should establish how distal weakness affects developmentally relevant mobility, participation and everyday function.
MCQ 16
Question: A 14-year-old boy with a hereditary neuropathy develops difficulty manipulating buttons and pencils because of progressive intrinsic hand muscle weakness. Which supportive service is most closely matched to his new limitation?
Options:
Urgent plasma exchange
Prophylactic broad-spectrum antibiotics
Routine spinal immobilization
Continuous bed rest
Occupational therapy for hand function
Correct Answer: Occupational therapy for hand function
Explanation: Occupational therapy supports fine motor tasks and independence when distal hand weakness impairs daily activities.
MCQ 17
Question: A 12-year-old with inherited neuropathy develops painful rigid foot deformity despite footwear modification and rehabilitation. The deformity increasingly restricts walking. What is the most appropriate next consideration?
Options:
Specialist orthopedic assessment
Immediate treatment with IV immunoglobulin
Long-term oral antibiotics
Complete cessation of walking and exercise
Repeated lumbar punctures
Correct Answer: Specialist orthopedic assessment
Explanation: Painful or progressive function-limiting deformity may need orthopedic evaluation when conservative care is insufficient.
MCQ 18
Question: A 55-year-old man diagnosed with peripheral neuropathy has worsening gait instability and several recent falls. His underlying metabolic condition is already being treated. Which additional intervention best addresses a major source of preventable disability?
Options:
Empirical antiviral therapy
Indefinite restriction to bed
Routine oral corticosteroids
Balance training and individualized rehabilitation
Immediate neurosurgical exploration
Correct Answer: Balance training and individualized rehabilitation
Explanation: Rehabilitation improves mobility, balance and safety while cause-specific treatment addresses the underlying neuropathy.
MCQ 19
Question: A 34-year-old woman developed numbness and weakness after several weeks of progressive nerve symptoms. She has a known hereditary neuropathy but has also recently developed laboratory-confirmed vitamin B12 deficiency. Which clinical principle is most appropriate?
Options:
Ignore the deficiency because the genetic diagnosis explains all symptoms
Evaluate and treat the acquired deficiency as a possible additional contributor
Assume all new symptoms represent irreversible genetic progression
Avoid further assessment once a hereditary label is established
Start plasma exchange for every hereditary neuropathy exacerbation
Correct Answer: Evaluate and treat the acquired deficiency as a possible additional contributor
Explanation: An inherited neuropathy does not prevent a second, treatable acquired neuropathic process from contributing to deterioration.
MCQ 20
Question: Parents of two children with the same inherited neuropathy ask whether both will inevitably lose walking ability at the same age. Which response best reflects appropriate counselling?
Options:
Both children will have an identical course because the diagnosis matches
The subtype guarantees a fixed age of major disability
Rehabilitation determines the genetic inheritance pattern
Future disability can be predicted exactly from nerve conduction velocity
Severity and progression can vary, so follow-up should be individualized
Correct Answer: Severity and progression can vary, so follow-up should be individualized
Explanation: Hereditary neuropathies have variable clinical expression; a diagnosis alone cannot predict an individual child’s functional course.