Questions and Answers
What is the most common type of spasmodic dysphonia?
Which type of spasmodic dysphonia is characterized by breathy voice breaks?
Which statement about singer's laryngeal dystonia is true?
How is adductor breathing dystonia characterized?
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Which physiological response has been noted to temporarily improve symptoms in all types of laryngeal dystonia?
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What distinguishes mixed laryngeal dystonia from other forms?
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Which of the following conditions can be associated with spasmodic dysphonia?
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Which of the following is NOT a known sensory trick reported by patients with laryngeal dystonia?
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What is the most common age range for the onset of dystonia?
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Which type of dystonia involves only one isolated body region?
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What percentage of patients with primary laryngeal dystonia may experience dissemination of the disease to another body part?
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Which of the following is NOT a characteristic of idiopathic dystonia diagnosis?
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What is the estimated prevalence of idiopathic dystonia in the United States?
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What does segmental dystonia involve?
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Which clinical findings are typically normal in idiopathic dystonia?
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At what age range has dystonia been recorded to begin at the latest?
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Study Notes
Overview of Dystonia
- Dystonia is characterized by sustained muscle contractions, resulting in twisting, repetitive movements, or abnormal postures.
- Often misdiagnosed due to its rarity and unusual manifestations; it's one of the most commonly misdiagnosed neurologic conditions.
- Estimated prevalence of idiopathic dystonia in the U.S. ranges from 200,000 to 300,000 cases.
Classification of Dystonia
- Classification aids in prognosis and management.
- Onset can occur at any age, with initial signs reported as early as 9 months and as late as 85 years.
- Bimodal distribution of onset: peaks at ages 8 and 42 years.
- Types of dystonia:
- Focal: one isolated body region.
- Segmental: two or more contiguous regions.
- Multifocal: two or more noncontiguous regions.
- Hemidystonia: half the body affected.
- Generalized: widespread, including the trunk and two other sites.
Diagnosis of Idiopathic Dystonia
- Diagnosis is primarily one of exclusion, necessitating:
- Normal perinatal and early developmental history.
- No previous neurological illness or exposure to drugs inducing acquired dystonia (e.g., phenothiazines).
- Normal findings on intellectual, pyramidal, cerebellar, and sensory examinations.
- Normal results from diagnostic studies.
Laryngeal Dystonia
- Up to 16% of patients with primary laryngeal dystonia may experience disease spread to other body parts.
- About 10% of patients with primary laryngeal dystonia report a family history of dystonia.
- Spasmodic dysphonia is an idiopathic focal dystonia affecting the larynx.
- Historically described in 1871 and later linked to other forms of dystonia.
Types of Laryngeal Dystonia
- Adductor Spasmodic Dysphonia (ADSD): 80% of cases; causes strained, strangled voice due to spasmodic adduction of the vocal folds during speech.
- Abductor Spasmodic Dysphonia (ABSD): 20% of cases; leads to breathy voice breaks or a whispering voice due to intermittent or sustained opening of the larynx during speech.
- Mixed Laryngeal Dystonia: Combination of adductor and abductor symptoms.
- Rare forms include:
- Adductor Breathing Dystonia: Vocal cords adduct during inspiration leading to temporary stridor and dyspnea.
- Singer’s Laryngeal Dystonia: Symptoms occur only during singing, often evolving to speech involvement.
Generalized Dystonia and Vocal Dysfunction
- Vocal dysfunction in generalized dystonia can resemble idiopathic spasmodic dysphonia.
- Meige Syndrome: Regional dystonia affecting the head and neck, often observed with blepharospasm oromandibular dystonia, torticollis, or spasmodic dysphonia.
Patient Experiences and Treatment Insights
- Patients report momentary symptom improvement with certain sensory tricks, such as:
- Pinching the nares or pressing hands against various body parts.
- Improved speech noted after yawning, sneezing, singing, or yelling.
- Sensory tricks are common among patients with other cranial dystonia types.
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Description
Explore the complexities of dystonia, a rare neurological syndrome characterized by sustained muscle contractions and abnormal postures. This quiz delves into the diagnosis challenges and prevalence of idiopathic dystonia, aiming to increase awareness and understanding of this condition.
