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Questions and Answers

Which of the following is NOT considered a secondary cause of sustained muscle contractions?

  • Wilson's disease
  • Autosomal dominant dystonia (correct)
  • Parkinson's disease
  • Huntington's disease

    • The Kayser-Fleischer ring is associated with an excessive deposition of copper in the kidneys.

    False

    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 __________.

    <p>premature growth plate fusion</p> Signup and view all the answers

    Match the following clinical features of Wilson's disease with their corresponding letter in ABCD:

    <p>A: Asterixis = Movement disorder B: Basal ganglia degeneration symptoms = Parkinsonism C: Cirrhosis = Liver damage D: Dementia = Cognitive decline</p> Signup and view all the answers

    Which symptom is NOT commonly associated with Brown-Sequard syndrome?

    <p>Bowel bladder dysfunction</p> Signup and view all the answers

    Wilson's disease is an autosomal dominant disorder.

    <p>False</p> Signup and view all the answers

    What type of fracture classification includes injuries through the growth plate and epiphysis?

    <p>Salter-Harris Type III or IV</p> Signup and view all the answers

    What class of myasthenia gravis (MG) is characterized by generalized weakness with mild symptoms?

    <p>Class II</p> Signup and view all the answers

    AChR antibodies are primarily IgM type and are produced in the spleen.

    <p>False</p> Signup and view all the answers

    What is the average time between the onset of symptoms and diagnosis of myasthenia gravis?

    <p>1 year</p> Signup and view all the answers

    The reaction caused by AChR antibodies is classified as a type ___ hypersensitivity reaction.

    <p>2</p> Signup and view all the answers

    Match the following classes of myasthenia gravis with their descriptions:

    <p>Class I = Ocular MG Class IV = Generalized MG with severe weakness Class V = Generalized MG requiring intubation Class III = Generalized MG with moderate weakness</p> Signup and view all the answers

    What effect do AChR antibodies have on the acetylcholine receptors?

    <p>Block acetylcholine binding</p> Signup and view all the answers

    MuSK antibodies primarily belong to the IgG1 subtype.

    <p>False</p> Signup and view all the answers

    What common physical symptoms do individuals affected by ALS report?

    <p>Limb weakness, cramping, gait instability, fatigue, stiffness, incoordination</p> Signup and view all the answers

    Which symptom is most commonly associated with progressive weakening of the muscles of mastication and swallowing?

    <p>Dysphagia</p> Signup and view all the answers

    What type of weakness primarily characterizes AChR-positive MG?

    <p>Proximal limb muscle weakness</p> Signup and view all the answers

    Frontotemporal dementia is characterized by early behavioral abnormalities and personality changes.

    <p>True</p> Signup and view all the answers

    Congenital myasthenic syndrome is a condition that occurs due to transplacental passage of maternal antibodies.

    <p>False</p> Signup and view all the answers

    What is the most commonly used oral acetylcholinesterase inhibitor for myasthenia gravis?

    <p>Pyridostigmine</p> Signup and view all the answers

    What is the term for the absence of AChR, MuSK, or LRP4 antibodies in the presence of clinical signs or symptoms?

    <p>Seronegative MG</p> Signup and view all the answers

    Weakness followed by muscle __________ is a progression characteristic of the disease.

    <p>atrophy</p> Signup and view all the answers

    Match the symptoms with their associated conditions:

    <p>Dysphagia = Muscle weakness in swallowing Pseudobulbar affect = Uncontrollable emotional episodes Spasticity = Muscle tightness and stiffness Hyperreflexia = Exaggerated reflex responses</p> Signup and view all the answers

    The presence of maternal antibodies that attack the neuromuscular junction is characteristic of __________.

    <p>neonatal myasthenia gravis</p> Signup and view all the answers

    Which of the following antibodies is commonly found in patients with thymoma and AChR-positive MG?

    <p>Modulating antibodies</p> Signup and view all the answers

    Which of the following is an early sign of muscle weakness?

    <p>Proximal arm weakness</p> Signup and view all the answers

    Match the myasthenia gravis subtype to its primary characteristic:

    <p>AChR-positive MG = Proximal limb muscle weakness MuSK-positive MG = Facial and respiratory weakness Seronegative MG = Absence of AChR, MuSK, or LRP4 antibodies Neonatal MG = Condition present at birth due to maternal antibodies</p> Signup and view all the answers

    Acetylcholinesterase inhibitors are used to decrease the availability of acetylcholine in patients with myasthenia gravis.

    <p>False</p> Signup and view all the answers

    The chance of remission in AChR-positive MG is higher than in MuSK-positive MG.

    <p>True</p> Signup and view all the answers

    List one indication for hospitalization in a patient with myasthenia gravis.

    <p>Significant bulbar symptoms</p> Signup and view all the answers

    What percentage of MG cases are attributed to AChR-positive MG?

    <p>80%</p> Signup and view all the answers

    What is the normal cycle length range considered standard?

    <p>24-38 days</p> Signup and view all the answers

    A regular cycle has a variation in cycle length of greater than 9 days.

    <p>False</p> Signup and view all the answers

    What phase of the ovarian cycle represents the time during which the follicle and its oocyte develop?

    <p>Follicular phase</p> Signup and view all the answers

    The ____ phase spans from ovulation to the onset of the next menses.

    <p>luteal</p> Signup and view all the answers

    Which hormone, released by the anterior pituitary, stimulates follicular development?

    <p>FSH</p> Signup and view all the answers

    Luteinizing hormone (LH) is responsible for initiating the menstrual cycle.

    <p>False</p> Signup and view all the answers

    Match the following menstrual cycle phases with their descriptions:

    <p>Desquamation = Shedding of the endometrial lining Proliferative phase = Endometrial proliferation with straight, tubular glands Secretory phase = Maturation of spiral arteries and endometrial glands Follicular phase = Development of follicle and oocyte until ovulation</p> Signup and view all the answers

    The hypothalamus releases ____ to stimulate the anterior pituitary.

    <p>gonadotropin-releasing hormone (GnRH)</p> Signup and view all the answers

    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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