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Questions and Answers
Which of the following is NOT considered a secondary cause of sustained muscle contractions?
Which of the following is NOT considered a secondary cause of sustained muscle contractions?
The Kayser-Fleischer ring is associated with an excessive deposition of copper in the kidneys.
The Kayser-Fleischer ring is associated with an excessive deposition of copper in the kidneys.
False (B)
What is the most sensitive screening test for Wilson's disease?
What is the most sensitive screening test for Wilson's disease?
Increased 24 hour urine copper
A complication of a Salter-Harris Type III fracture is __________.
A complication of a Salter-Harris Type III fracture is __________.
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Match the following clinical features of Wilson's disease with their corresponding letter in ABCD:
Match the following clinical features of Wilson's disease with their corresponding letter in ABCD:
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Which symptom is NOT commonly associated with Brown-Sequard syndrome?
Which symptom is NOT commonly associated with Brown-Sequard syndrome?
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Wilson's disease is an autosomal dominant disorder.
Wilson's disease is an autosomal dominant disorder.
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What type of fracture classification includes injuries through the growth plate and epiphysis?
What type of fracture classification includes injuries through the growth plate and epiphysis?
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What class of myasthenia gravis (MG) is characterized by generalized weakness with mild symptoms?
What class of myasthenia gravis (MG) is characterized by generalized weakness with mild symptoms?
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AChR antibodies are primarily IgM type and are produced in the spleen.
AChR antibodies are primarily IgM type and are produced in the spleen.
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What is the average time between the onset of symptoms and diagnosis of myasthenia gravis?
What is the average time between the onset of symptoms and diagnosis of myasthenia gravis?
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The reaction caused by AChR antibodies is classified as a type ___ hypersensitivity reaction.
The reaction caused by AChR antibodies is classified as a type ___ hypersensitivity reaction.
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Match the following classes of myasthenia gravis with their descriptions:
Match the following classes of myasthenia gravis with their descriptions:
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What effect do AChR antibodies have on the acetylcholine receptors?
What effect do AChR antibodies have on the acetylcholine receptors?
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MuSK antibodies primarily belong to the IgG1 subtype.
MuSK antibodies primarily belong to the IgG1 subtype.
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What common physical symptoms do individuals affected by ALS report?
What common physical symptoms do individuals affected by ALS report?
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Which symptom is most commonly associated with progressive weakening of the muscles of mastication and swallowing?
Which symptom is most commonly associated with progressive weakening of the muscles of mastication and swallowing?
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What type of weakness primarily characterizes AChR-positive MG?
What type of weakness primarily characterizes AChR-positive MG?
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Frontotemporal dementia is characterized by early behavioral abnormalities and personality changes.
Frontotemporal dementia is characterized by early behavioral abnormalities and personality changes.
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Congenital myasthenic syndrome is a condition that occurs due to transplacental passage of maternal antibodies.
Congenital myasthenic syndrome is a condition that occurs due to transplacental passage of maternal antibodies.
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What is the most commonly used oral acetylcholinesterase inhibitor for myasthenia gravis?
What is the most commonly used oral acetylcholinesterase inhibitor for myasthenia gravis?
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What is the term for the absence of AChR, MuSK, or LRP4 antibodies in the presence of clinical signs or symptoms?
What is the term for the absence of AChR, MuSK, or LRP4 antibodies in the presence of clinical signs or symptoms?
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Weakness followed by muscle __________ is a progression characteristic of the disease.
Weakness followed by muscle __________ is a progression characteristic of the disease.
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Match the symptoms with their associated conditions:
Match the symptoms with their associated conditions:
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The presence of maternal antibodies that attack the neuromuscular junction is characteristic of __________.
The presence of maternal antibodies that attack the neuromuscular junction is characteristic of __________.
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Which of the following antibodies is commonly found in patients with thymoma and AChR-positive MG?
Which of the following antibodies is commonly found in patients with thymoma and AChR-positive MG?
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Which of the following is an early sign of muscle weakness?
Which of the following is an early sign of muscle weakness?
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Match the myasthenia gravis subtype to its primary characteristic:
Match the myasthenia gravis subtype to its primary characteristic:
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Acetylcholinesterase inhibitors are used to decrease the availability of acetylcholine in patients with myasthenia gravis.
Acetylcholinesterase inhibitors are used to decrease the availability of acetylcholine in patients with myasthenia gravis.
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The chance of remission in AChR-positive MG is higher than in MuSK-positive MG.
The chance of remission in AChR-positive MG is higher than in MuSK-positive MG.
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List one indication for hospitalization in a patient with myasthenia gravis.
List one indication for hospitalization in a patient with myasthenia gravis.
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What percentage of MG cases are attributed to AChR-positive MG?
What percentage of MG cases are attributed to AChR-positive MG?
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What is the normal cycle length range considered standard?
What is the normal cycle length range considered standard?
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A regular cycle has a variation in cycle length of greater than 9 days.
A regular cycle has a variation in cycle length of greater than 9 days.
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What phase of the ovarian cycle represents the time during which the follicle and its oocyte develop?
What phase of the ovarian cycle represents the time during which the follicle and its oocyte develop?
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The ____ phase spans from ovulation to the onset of the next menses.
The ____ phase spans from ovulation to the onset of the next menses.
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Which hormone, released by the anterior pituitary, stimulates follicular development?
Which hormone, released by the anterior pituitary, stimulates follicular development?
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Luteinizing hormone (LH) is responsible for initiating the menstrual cycle.
Luteinizing hormone (LH) is responsible for initiating the menstrual cycle.
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Match the following menstrual cycle phases with their descriptions:
Match the following menstrual cycle phases with their descriptions:
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The hypothalamus releases ____ to stimulate the anterior pituitary.
The hypothalamus releases ____ to stimulate the anterior pituitary.
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Study Notes
Muscle Contractions and Movement Disorders
- Sustained muscle contractions can lead to abnormal postures and repetitive twitching.
- Conditions can be focal (e.g., blepharospasm, spasmodic dysphonia, torticollis) or segmental and generalized.
- Idiopathic primary causes include torsion dystonia, inherited in an autosomal dominant manner.
- Secondary causes may involve Wilson's disease, Huntington's disease, Parkinson's disease, anoxia, stroke, or drug-induced conditions.
Wilson's Disease
- An autosomal recessive disorder impacting copper metabolism.
- Excessive serum copper accumulation leads to deposition in liver, kidneys, cornea, and CNS, specifically in the basal ganglia.
- Key sign is Kayser-Fleischer ring, a golden brown-green deposit in the iris.
- Clinical features include hyperkinetic movement, tremor, parkinsonism, dysarthria, and cognitive disturbances.
- Diagnosis through increased serum copper, decreased ceruloplasmin, and increased 24-hour urine copper; liver biopsy is definitive.
- Management includes chelation therapy with penicillamine, trientine, and zinc.
Brown-Sequard Syndrome
- Caused by hemisection of the spinal cord, often due to trauma.
- Symptoms include ipsilateral weakness and joint position sense loss, with contralateral pain and temperature loss.
- Rarely involves bowel and bladder dysfunction.
Salter-Harris Type III Fracture Complications
- Salter-Harris classification details fractures involving the growth plate in pediatric bones.
- Type III: Fracture through the growth plate and epiphysis, leading to potential premature growth plate fusion and osteoarthritis.
- Type IV: Involves the growth plate, epiphysis, and metaphysis, similar complications to Type III.
Myasthenia Gravis (MG)
- Neonatal MG arises from maternal antibody transplacental transmission, resolving within two months.
- Congenital myasthenic syndrome is a hereditary form present from birth.
- AChR-positive MG constitutes 80% of cases, primarily affecting proximal limb muscles, with facial and respiratory weakness.
- Three antibody subtypes: binding (most common), modulating (found in thymoma), and blocking (less than 1% of cases).
- MuSK-positive MG involves mainly facial and bulbar weakness, less limb weakness, and is common in younger females.
- Classification of MG severity ranges from ocular MG (Class I) to generalized MG requiring intubation (Class V).
- Pathogenesis involves AChR antibodies causing postsynaptic membrane damage and muscle contraction failure.
Clinical Manifestations of Amyotrophic Lateral Sclerosis (ALS)
- Initial signs include asymmetrical limb weakness, cramping, gait instability, and fatigue.
- Symptoms progress to symmetrical weakness affecting multiple muscle groups.
- Speech and swallowing difficulties (dysarthria and dysphagia) are common.
- Exaggerated emotional expressions, pseudobulbar affect, and frontotemporal dementia may develop.
- Muscle atrophy, spasticity, and fasciculations indicate advanced disease.
- Typical complications include weight loss and poor executive functioning.
Treatment of Myasthenia Gravis
- Indications for hospitalization include severe bulbar symptoms, low vital capacity, and respiratory distress.
- Outpatient management is suitable for mild cases.
- First-line pharmacotherapy includes acetylcholinesterase inhibitors like pyridostigmine.
Menstrual Cycle and Hormonal Regulation
- Regular menstrual cycles last 24–38 days, with perimenopause causing shorter, more frequent cycles.
- Follicular phase: Begins on day 1 of menses and lasts until LH surge, facilitating follicle development.
- Luteal phase: Follows ovulation, supporting potential pregnancy for about 14 days.
- Endometrial cycle phases include desquamation, proliferation, and secretion in preparation for pregnancy.
- Hormonal regulation involves the hypothalamic-pituitary-ovarian axis, with GnRH stimulating FSH and LH release, which in turn stimulates ovarian functions and ovulation.
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