Muscular Dystrophy Overview

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Questions and Answers

What is the primary benefit of using a rubber dam in dental procedures?

  • It prevents water from splashing.
  • It improves the efficiency of dental tools.
  • It reduces inhalation of foreign substances. (correct)
  • It enhances visibility during treatment.

In patients with facioscapulohumeral muscular dystrophy, what activity is most likely to be impacted?

  • The ability to lift objects like a toothbrush. (correct)
  • Hand-eye coordination tasks.
  • Visual acuity for reading.
  • Walking or running.

Why should dental anesthetics be used cautiously in patients with neuromuscular disorders?

  • They may cause allergic reactions.
  • They can exacerbate existing respiratory issues. (correct)
  • They lead to prolonged recovery times.
  • They do not affect muscular control.

Referral to a registered dietitian for patients with muscular dystrophy is considered for which reason?

<p>To assess and recommend on nutritional status and feeding abilities. (B)</p> Signup and view all the answers

What specific dental treatment should be introduced to children with Duchenne muscular dystrophy at the earliest age possible?

<p>Preventive dental treatment. (B)</p> Signup and view all the answers

Intraoral stretching devices are used in patients with DMD primarily to preserve what?

<p>Temporomandibular range of motion. (A)</p> Signup and view all the answers

What oral manifestation is commonly found in various forms of muscular dystrophy?

<p>Facial muscle weakness. (C)</p> Signup and view all the answers

Which muscle group is particularly affected in patients with facioscapulohumeral muscular dystrophy?

<p>Masticatory muscles. (C)</p> Signup and view all the answers

What is a common characteristic of myotonic dystrophy?

<p>Difficulty in relaxing contracted muscles (D)</p> Signup and view all the answers

Which muscular dystrophy typically manifests between 30 and 50 years of age?

<p>Oculopharyngeal muscular dystrophy (D)</p> Signup and view all the answers

What is a significant risk associated with weakened bones in muscular dystrophy patients?

<p>Elevated risk of fractures (B)</p> Signup and view all the answers

Which of the following describes a common sign or symptom of DMD or BMD as patients age?

<p>Shortness of breath (D)</p> Signup and view all the answers

In which population is the prevalence of myotonic dystrophy particularly high?

<p>Saguenay-Lac St. Jean area of Quebec (B)</p> Signup and view all the answers

What connection is there between Duchenne and Becker muscular dystrophies and the heart?

<p>They may result in cardiomyopathy (C)</p> Signup and view all the answers

What symptom is connected to respiratory issues in DMD or BMD patients?

<p>Mechanical breathing assistance may be needed (D)</p> Signup and view all the answers

Which of the following complications is NOT typically associated with muscular dystrophy?

<p>Renal failure (D)</p> Signup and view all the answers

Which of the following factors is commonly associated with open bites?

<p>Lip incompetence (C)</p> Signup and view all the answers

What is the primary cause of muscular dystrophies?

<p>Genetic mutations (B)</p> Signup and view all the answers

Which type of muscular dystrophy is most common in males?

<p>Duchenne muscular dystrophy (D)</p> Signup and view all the answers

What characterizes the incidence of Duchenne muscular dystrophy?

<p>1 in 3,500 births (B)</p> Signup and view all the answers

Which condition is a common complication for tube-fed patients?

<p>Rapid caries accumulation (C)</p> Signup and view all the answers

Which of the following statements is true about autosomal dominant forms of muscular dystrophy?

<p>Males and females are equally affected (A)</p> Signup and view all the answers

What is the most common feeding tube used for patients with neuromuscular disorders?

<p>Percutaneous endoscopic gastrostomy (PEG) tube (D)</p> Signup and view all the answers

What is a typical management strategy for childhood-onset muscular dystrophy?

<p>Early intervention services (C)</p> Signup and view all the answers

What type of muscular dystrophies are Duchenne MD and Becker MD classified as?

<p>X-linked (A)</p> Signup and view all the answers

At what age does muscle weakness typically begin for individuals with Duchenne MD?

<p>Before 5 years of age (B)</p> Signup and view all the answers

Which therapeutic modality is specifically designed to maintain muscle strength in Duchenne MD patients?

<p>Corticosteroid treatment (C)</p> Signup and view all the answers

What is the main reason females with an X-linked gene mutation may not exhibit symptoms of muscular dystrophy?

<p>They have two X chromosomes with one normal gene. (C)</p> Signup and view all the answers

Which of the following describes Becker MD in relation to Duchenne MD?

<p>It is milder and less progressive. (A)</p> Signup and view all the answers

What is a common early motor development delay seen in children with DMD?

<p>Walking (C)</p> Signup and view all the answers

Which of the following regions is the first to typically show muscle weakness in both Duchenne MD and Becker MD?

<p>Pelvis and femoral regions (A)</p> Signup and view all the answers

What role do medications like eteplirsen and golodirsen play in the treatment of DMD?

<p>They target specific genetic mutations. (A)</p> Signup and view all the answers

What is a common oral cavity issue observed in Duchenne muscular dystrophy (DMD)?

<p>Hypertrophy of the masseter muscles (D)</p> Signup and view all the answers

Which condition is characterized by difficulties in swallowing?

<p>Oculopharyngeal muscular dystrophy (OPMD) (B)</p> Signup and view all the answers

Which of the following conditions is commonly associated with reduced salivary secretion?

<p>Myotonic dystrophy type 1 (B)</p> Signup and view all the answers

What oral condition is frequently observed in individuals with myotonic dystrophy?

<p>Open bite malocclusion (D)</p> Signup and view all the answers

What is a characteristic of the tongue in individuals with DMD?

<p>Enlarged hypotonic tongue (A)</p> Signup and view all the answers

What anatomical change occurs with aging in DMD patients?

<p>Increased angle between the maxilla and mandible (A)</p> Signup and view all the answers

Which condition may lead to drooling in patients with muscular dystrophy?

<p>Difficulty swallowing liquids (C)</p> Signup and view all the answers

Which symptom is associated with temporomandibular joint (TMJ) disorder in myotonic dystrophy?

<p>Reduced TMJ range of motion (D)</p> Signup and view all the answers

What is a common dental healthcare program component for patients with Duchenne muscular dystrophy?

<p>Oral hygiene instruction (D)</p> Signup and view all the answers

Which condition is discussed regarding its prevalence in the United States?

<p>Duchenne muscular dystrophy (B)</p> Signup and view all the answers

What is a dental characteristic of patients with Duchenne muscular dystrophy?

<p>Difficulty in oral hygiene (D)</p> Signup and view all the answers

What benefit is associated with improving temporomandibular range of motion in patients with Duchenne muscular dystrophy?

<p>Easier tooth brushing (B)</p> Signup and view all the answers

What procedure is considered invasive dental hygiene for patients with Duchenne muscular dystrophy?

<p>Scaling teeth (C)</p> Signup and view all the answers

Which organization provides information on muscular dystrophy including dental care?

<p>Muscular Dystrophy Canada (D)</p> Signup and view all the answers

What is an essential component of a dental healthcare program for patients with Duchenne muscular dystrophy?

<p>Taking dental impressions (D)</p> Signup and view all the answers

Which procedure is NOT typically part of dental care for patients with Duchenne muscular dystrophy?

<p>Providing orthodontic care (B)</p> Signup and view all the answers

Flashcards

Rubber dam use in dental procedures for MD patients

A rubber dam prevents foreign substances from entering the patient's airway during dental procedures.

Hand and wrist weakness in MD patients

Muscle weakness in the hands and wrists can make brushing and flossing difficult for those with MD.

Shoulder weakness in facioscapulohumeral MD

Shoulder muscle weakness in facioscapulohumeral MD hinders lifting objects like toothbrushes, requiring assistance for oral hygiene.

Bisphosphonate-related osteonecrosis of the jaw

Dental hygienists need to be aware of potential side effects of bisphosphonate treatment, such as osteonecrosis of the jaw.

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Anaesthesia considerations for MD patients

Anaesthetic agents should be used cautiously in MD patients, considering their pre-existing neuromuscular conditions and potential cardiac complications.

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Dietary considerations for MD patients

Due to chewing difficulties and dysphagia, MD patients often need nutritional assessments and advice from a registered dietitian.

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Preventive dental care for DMD children

Early preventive dental care is crucial for children with DMD, including regular prophylaxis, fluoride application, and oral hygiene instruction.

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Oral hygiene modifications for MD patients

Modifications to tooth brushing and other hygiene activities may be needed in MD patients, depending on the affected muscle groups.

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Masseter muscle hypertrophy in DMD

A condition where the jaw muscles become larger due to a buildup of fat and connective tissue, even though the actual muscle tissue is shrinking.

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Dysphagia in muscular dystrophies

Difficulty swallowing, often caused by muscle weakness in the throat and mouth.

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Enlarged tongue in DMD

A condition where the tongue becomes enlarged and weak, often seen in Duchenne muscular dystrophy (DMD).

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Malocclusion in myotonic dystrophy

A condition where the jaw doesn't close properly, leading to issues with chewing and turning the head, often observed in myotonic dystrophy.

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Swan neck deformity in myotonic dystrophy

An abnormal forward curve in the neck, often noticed in myotonic dystrophy due to weakness of the neck muscles.

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Reduced jaw joint movement in DMD

A condition where the jaw joint has reduced movement, often seen in DMD.

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Drooling in muscular dystrophies

Excessive drooling due to difficulty swallowing liquids, often seen in oculopharyngeal muscular dystrophy and other forms of MD.

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Xerostomia in muscular dystrophies

Dry mouth, often occurring in myotonic dystrophy type 1, caused by decreased saliva production.

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Open bite

A condition where the upper front teeth don't meet the lower front teeth, often due to lip incompetence, mouth breathing, enlarged tongue, or tongue pushing against the teeth.

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Muscular dystrophies (MD)

A group of genetic conditions that cause progressive muscle weakness and degeneration.

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Duchenne muscular dystrophy

The most severe form of MD, primarily affecting boys and causing progressive muscle weakness.

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Becker muscular dystrophy

A less severe form of MD, also primarily affecting boys, causing milder muscle weakness and later onset.

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Percutaneous endoscopic gastrostomy (PEG) tube

A feeding tube placed directly into the stomach, allowing for direct delivery of nutritional formulas.

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Hyperbolic dental arches

A condition where the back teeth are pushed out, usually due to an imbalance between the tongue and cheek muscles.

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Overjet

A condition where the upper front teeth are positioned forward, over the lower front teeth.

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Underjet

A condition where the lower front teeth are positioned forward, over the upper front teeth.

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What is Muscular Dystrophy (MD)?

A group of genetic disorders that cause progressive muscle weakness and degeneration.

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How is MD typically inherited?

The defective gene is located on the X chromosome, meaning males are more likely to be affected than females.

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What is Duchenne Muscular Dystrophy (DMD)?

A form of MD where muscle weakness begins typically before age 5, characterized by progressive muscle degeneration.

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What is Becker Muscular Dystrophy (BMD)?

A milder form of MD compared to DMD, with a later onset typically between 7 and 12 years old.

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What is the genetic cause of DMD and BMD?

DMD and BMD are both caused by mutations in the dystrophin gene, which leads to a deficiency of the dystrophin protein.

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What are some common symptoms of DMD and BMD?

Individuals with DMD or BMD may experience difficulties with activities that require muscle strength, like walking, running, and climbing stairs.

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What is a common treatment approach for MD?

Corticosteroids are often prescribed to maintain muscle strength for as long as possible, especially in DMD.

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What are some other therapeutic modalities for MD?

Physical therapy, assistive devices, and surgery are also used to manage symptoms and improve quality of life for individuals with MD.

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Facioscapulohumeral Muscular Dystrophy (FSHD)

A type of muscular dystrophy that primarily affects the muscles of the face, shoulders, and upper arms, causing weakness and difficulty with facial expressions, raising arms, and other movements.

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Myotonia

A common symptom of Myotonic Dystrophy, characterized by difficulty relaxing muscles after they contract. Imagine a hand that's squeezed into a fist and remains that way for an extended time.

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Duchenne Muscular Dystrophy (DMD)

An inherited disorder that causes progressive muscle weakness and degeneration, typically affecting boys.

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Becker Muscular Dystrophy (BMD)

A less severe form of muscular dystrophy that resembles DMD but progresses more slowly. It's like a 'milder' version of DMD.

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Facioscapulohumeral Muscular Dystrophy (FSHD)

An inherited disorder that causes progressive muscle weakness and degeneration, primarily affecting the muscles of the face, shoulders, and upper arms.

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Scoliosis

A serious complication of muscular dystrophy, especially DMD, characterized by curvature of the spine. It can affect breathing and mobility.

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Cardiomyopathy

A medical condition characterized by weakened heart muscles. It can lead to heart failure and irregular heartbeats. This condition can develop as a consequence of DMD and BMD.

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Respiratory Compromise

A serious condition that affects the muscles involved in breathing. It can make it difficult and challenging to breathe.

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Dental care for DMD patients

Dental care for people with Duchenne muscular dystrophy (DMD) should include preventative measures, oral hygiene instruction, fitting a mouth guard and taking impressions.

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Dysphagia in DMD

People with DMD often have difficulty swallowing due to muscle weakness in their throat and mouth.

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Drooling in DMD

DMD patients may drool excessively due to the difficulty in swallowing liquids.

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Hand and wrist weakness in DMD

DMD patients may have weak hand and wrist muscles which can make brushing and flossing difficult.

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Rubber dam use for DMD patients

A rubber dam is often used during dental procedures in DMD patients to prevent foreign substances from entering the airway.

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Study Notes

Muscular Dystrophy

  • Muscular dystrophy (MD) is a group of genetic conditions causing progressive muscle weakness and degeneration.
  • Common types include Duchenne MD (DMD) and Becker MD (BMD).
  • DMD typically affects males, and onset is usually before age 5.
  • BMD is a milder form of DMD, and onset is generally between ages 7 and 12.
  • Some types have adult onset.
  • Medical clearance is advised before dental procedures if the patient is receiving immunosuppressant medications, general anesthetic agents are contemplated, or if there are concerns about medication-related osteonecrosis of the jaw (MRONJ).
  • Antibiotic prophylaxis is not usually necessary.
  • Postponing dental procedures might be considered sometimes, but not always. This depends on risk factors and patient conditions.
  • Oral management implications include falls risks, wheelchair use, muscle weakness, swallowing issues, breathing problems, and potential complications during general anesthesia.
  • Oral manifestations include facial muscle weakness, masticatory muscle issues, oral cavity atrophy, dysphagia, tongue weakness, and malocclusion.
  • Specific precautions and modifications for dental hygiene are recommended depending on the type and severity of MD, such as using warm towels and adjusting chair positions.

Contraindications

  • Initiating non-invasive dental hygiene procedures is not contraindicated.
  • Initiating invasive dental hygiene procedures may be, although not typically, contraindicated.
  • A medical consult is needed if the patient is being treated with immunosuppressant medications or if anesthesia is deemed necessary.

Medical Considerations

  • Medical clearance is essential for patients on immunosuppressant medications, those undergoing dental procedures requiring anesthesia, and in situations where MRONJ exists.
  • Antibiotic prophylaxis is generally not required, unless the patient is on prolonged corticosteroid therapy.

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