Peripheral Neuropathy

Question 1

Guillian Barre syndrome etiology?

Answer 1

History of event 1-4 weeks before, like GIT infection, upper UTI, surgery, immunization.

Question 2

Guillian Barre clinical picture?

Answer 2

Primarily motor, paralysis of LMN and ascending upwards

Question 3

Most serious complication of Guillian Barre?

Answer 3

Respiratory complications

Question 4

Investigations for Guillian Barre?

Answer 4

CSF and Nerve conduction

Question 5

Treatment of Guillian Barre?

Answer 5

Plasma exchange for moderate to severe cases, or IVIG

Question 6

Carpal tunnel syndrome affected nerve?

Answer 6

Median nerve

Question 7

Carpal tunnel syndrome diagnostic investigations?

Answer 7

Nerve conduction and EMG

Question 8

Duchenne muscular dystrophy is what?

Answer 8

Absence or severe deficiency of dystrophin protein

Question 9

Clinical picture of Duchenne muscular dystrophy?

Answer 9

Gower’s sign, which is difficulty in walking, frequent falls, difficulty in getting upstairs, and difficulty in getting up from the floor and the child is using his hands to support his knees

Question 10

Duchenne is a progressive course, what is the common cause of death?

Answer 10

repeated chest infections or associated cardiomyopathy

Question 11

Diagnosis of Duchenne?

Answer 11

Elevated CPK, EMG shows myopathic pattern, and muscle biopsy for dystrophin analysis.

Question 12

Treatment for Duchenne?

Answer 12

• Encourage the child to maintain an active life to prevent contractures
• Orthopedic management & physiotherapy
• Long term corticosteroid therapy may ameliorate the course (for a short period)
  Genetic counseling

Question 13

What is Becker’s muscular dystrophy

Answer 13

It is similar to Duchenne MD but has a milder course. It is due to defect in the same gene and same location (Xp21)

Question 14

Myasthenia Gravis is characterized by what

Answer 14

muscle weakness and fatigue affecting extra ocular muscles, bulbar muscles and extremities.
The disease is characterized by fluctuation in the muscle weakness and fatigue

Question 15

Diagnosis of Myasthenia Gravis?

Answer 15

Edrophonium test (tensilon) which is an ACh inhibitor injected IV; if improvement occur it is diagnostic

Question 16

Treatment of Myasthenia Gravis?

Answer 16

Corticosteroids and plasma exchange + high dose IVIG.

Question 17

The diagnosis of myasthenia gravis is confirmed by the presence of autoantibodies against which of the following structures?
A) Voltage-gated calcium channels
B) Acetylcholine receptors (AChR)
C) Muscle-specific kinase (MuSK)
D) Dystrophin protein

Answer 17

Acetylcholine receptors

Question 18

Which of the following treatments provides only symptomatic relief and does not alter the underlying autoimmune process in myasthenia gravis?
A) Thymectomy
B) Intravenous immunoglobulin (IVIG)
C) Corticosteroids
D) Acetylcholinesterase inhibitors

Answer 18

Acetylcholinesterase inhibitors

Question 19

Which of the following is a first-line symptomatic treatment for the neuropathic pain associated with diabetic neuropathy?
A) Beta-blockers
B) Dopamine agonists
C) Antiepileptic drugs like gabapentin
D) Benzodiazepines

Answer 19

Gabapentin

Question 20

A patient diagnosed with Guillain-Barré syndrome is beginning to show a rapid decline in respiratory function. Which of the following is the most appropriate initial immunomodulatory treatment?
A) Oral prednisone
B) Intravenous immunoglobulin (IVIG)
C) Cyclosporine
D) Rituximab

Answer 20

IVIG

Question 21

Nerve conduction study for GB shows what?

Answer 21

(F wave latency) and reduced conduction velocity

Question 22

Duchenne incidence ratio.

Answer 22

3 per 100,000

Question 23

A 32-year-old woman presents with progressive weakness in her hands and feet over the past 3 weeks. Two weeks prior, she had a diarrheal illness. Neurological exam reveals symmetric weakness in all limbs, areflexia, and mild distal paresthesia. Cerebrospinal fluid analysis is most likely to show which of the following?
A. Elevated white blood cell count with normal protein
B. Normal white blood cell count with elevated protein
C. Low glucose with elevated protein
D. Elevated white blood cell count with low glucose

Answer 23

Normal WBC’s with elevated protein

Question 24

A 5-year-old boy is brought to the clinic because of frequent falls and difficulty climbing stairs. His parents note that he uses his hands to “walk up” his own legs when rising from the floor. On examination, he has pseudohypertrophy of his calves and proximal muscle weakness. Which of the following laboratory findings is most expected in this patient?
A. Low serum creatine phosphokinase (CPK)
B. Elevated serum creatine phosphokinase (CPK)
C. Positive acetylcholine receptor antibodies
D. Protein-cell dissociation in cerebrospinal fluid

Answer 24

Elevated CPK

Question 25

A 28-year-old woman presents with painless, fluctuating weakness that worsens with repeated use and improves with rest. The weakness primarily affects her ocular and bulbar muscles. Which of the following diagnostic tests is most specific for confirming the diagnosis? A. Serum creatine phosphokinase (CPK) B. Acetylcholine receptor antibody titer C. Nerve conduction study showing axonal loss D. Muscle biopsy showing inflammatory changes

Answer 25

ACH receptor antibody titer

Question 26

A patient with a predominantly sensory neuropathy is evaluated. Which of the following is the LEAST likely underlying cause? A. Diabetes Mellitus B. Vitamin B12 deficiency C. Guillain-Barré syndrome D. Chronic renal failure

Answer 26

Guillain-Barré syndrome

Question 27

The mother of a 10-year-old boy with Duchenne Muscular Dystrophy asks about the genetic basis of her son's condition. Which of the following is the most accurate explanation? A. It is an autosomal dominant disorder causing myotonia. B. It is an X-linked recessive disorder resulting from a dystrophin deficiency. C. It is an autoimmune disorder affecting the neuromuscular junction. D. It is a mitochondrial disorder with maternal inheritance.

Answer 27

X-linked recessive

Question 28

A patient with a confirmed diagnosis of myasthenia gravis is scheduled for an intravenous edrophonium (Tensilon) test. A positive test is characterized by: A. A marked decrease in serum CPK levels B. A sudden drop in blood pressure C. A transient improvement in muscle strength D. The appearance of fasciculations

Answer 28

Improvement in muscle strength

Question 29

Which of the following is a characteristic feature of the muscle weakness seen in myasthenia gravis? A. Worsens with rest and improves with activity B. Accompanied by muscle wasting and hyporeflexia C. Fluctuates and worsens with repeated use D. Primarily affects distal muscles before proximal ones

Answer 29

Fluctuates and worsens with repeated use