Tucker Syndrome

Tucker Syndrome

Article
Ear, Nose, & Throat (ENT)
Brain & Nerve
+4
Contributed byMaulik P. Purohit MD MPHMay 27, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Congenital Bilateral Recurrent Nerve Paralysis and Ptosis
  • Ptosis Vocal Cord Paralysis

What is Tucker Syndrome? (Definition/Background Information)

  • Tucker Syndrome is an inherited disorder that is characterized by vocal cord paralysis and drooping of upper eyelids. In addition, short stature and speech disorders may be present as well
  • The exact gene(s) involved in the development of this disorder is not known. Since the condition has been reported in only 2 individuals, it is not possible to determine the mode of inheritance
  • Bilateral vocal cord paralysis can be life-threatening and surgical correction is necessary for   good outcome

Who gets Tucker Syndrome? (Age and Sex Distribution)

  • Tucker Syndrome is a rare congenital disorder. The presentation of symptoms may occur at birth
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Tucker Syndrome? (Predisposing Factors)

  • A positive family history may be an important risk factor, since Tucker Syndrome can be inherited
  • Currently, no other risk factors have been clearly identified for the syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Tucker Syndrome? (Etiology)

  • The gene mutation that causes Tucker Syndrome is not known
  • The mode of inheritance of Tucker Syndrome is not clearly known, owing to the limited number of cases in the medical literature

What are the Signs and Symptoms of Tucker Syndrome?

The signs and symptoms of Tucker Syndrome may include:

  • Bilateral ptosis
  • Vocal cord paralysis

In addition, the following signs and symptoms may be present:

Very frequently present symptoms in 80-99% of the cases:

  • Hemiplegia
  • Laryngomalacia
  • Nasal speech
  • Ptosis

Frequently present symptoms in 30-79% of the cases: Premature birth

Occasionally present symptoms in 5-29% of the cases: Severe short stature

(Source: Tucker Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Tucker Syndrome Diagnosed?

Tucker Syndrome is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Tucker Syndrome?

The complications of Tucker Syndrome may include:

  • Breathing difficulty due to bilateral vocal cord paralysis, which can be life-threatening
  • Problems with speech due to paralysis of vocal cords
  • Problems with proper vision, due to drooping of upper eyelids

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Tucker Syndrome Treated?

  • There is no cure for Tucker Syndrome, since it is a genetic condition. The treatment is usually given to manage the signs and symptoms and any complication that develops
  • Corrective surgery may be required for ptosis
  • Surgical correction may be necessary for restoration of airway and may involve the following:
    • Intubation
    • Temporary tracheotomy
    • Re-innervation of larynx
    • Extubation

How can Tucker Syndrome be Prevented?

Tucker Syndrome may not be preventable, since it is a genetic disorder.

  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Tucker Syndrome? (Outcomes/Resolutions)

  • The prognosis of Tucker Syndrome is favorable, with a timely diagnosis and prompt surgical treatment
  • Without proper treatment, bilateral vocal cord paralysis may lead to breathing problems and can be life threatening

Additional and Relevant Useful Information for Tucker Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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