Möbius syndrome: is rare congenital neurological disorder
There are four group: Simple hypoplasia, Primary lesions in CNs, Focal necrosis in brainstem nuclei and Primary myopathy.
Signs and symptoms: Limb abnormalities, Chest-wall abnormalities, Crossed eyes and Difficulty in breathing and/or in swallowing.
It results from a vascular disruption, The use of drugs and a traumatic pregnancy associated with development of Möbius syndrome.
There is no single course of medical treatment for Möbius syndrome.
2. OBJECTIVES:
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To define Mobius syndrome and It’s
classification.
To define Signs and symptoms.
To define Pathogenesis.
To explain Diagnosis and Treatment.
To explain Medication, Epidemiology.
3. DEFINITION:
Möbius syndrome is an extremely rare congenital
neurological disorder which is characterized by facial
paralysis and the inability to move the eyes from side
to side.
It is caused due to the loss of function of motor
cranial nerves (CNs).
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4. CLASSIFICATION OF MÖBIUS
SYNDROME
1-Group(I) Simple hypoplasia or atrophy of CN
nuclei.
2-Group(II) Primary lesions in peripheral CNs.
3-Group(III) Focal necrosis in brainstem nuclei.
4-Group(IV) Primary myopathy with no central
nervous system (CNS) or CN lesion.
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5. SIGNS AND SYMPTOMS:
If cranial nerves V and VIII are affected, the person
experiences hearing loss and the upper lip is retracted
due to muscle shrinkage.
Other symptoms are as follow:
• Limb abnormalities (clubbed feet, missing fingers)
• Chest-wall abnormalities
• Crossed eyes
• Difficulty in breathing and/or in swallowing
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6. Children with Möbius syndrome may have delayed
speech because of paralysis of muscles that move the
lips, expressionless faces and frequent drooling.
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7. PATHOGENESIS:
The causes of Möbius syndrome are poorly
understood. Möbius syndrome is thought to result
from a vascular disruption (temporary loss of blood
flow) in the brain during prenatal development.
The use of drugs and a traumatic pregnancy may
also be linked to the development of Möbius
syndrome.
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8. Certain symptoms associated with Möbius syndrome
may be caused by incomplete development of facial
nerves, other cranial nerves and other parts of the
central nervous system.
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9. DIAGNOSIS:
include the following:
Diagnostic Considerations:
Because birth trauma can cause a syndrome with a
presentation similar to that of Möbius syndrome,
birth trauma should be excluded as soon as possible
after birth.
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10. CT Scanning, MRI, and Ultrasonography:
CT scanning or MRI of the brain may demonstrate
bilateral calcifications in the regions of the CN VI
nuclei. However, these calcifications are not specific
for the disease.
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Brain MRI may help in determining whether other,
perhaps genetically determined, cerebral
malformations are present.
Although prenatal ultrasonography has been used to
depict basal ganglia and brainstem calcifications in
the brains of developing infants.
12. TREATMENT:
There is no single course of medical treatment for
Möbius syndrome. Treatment is supportive and in
accordance with symptoms.
• infants may require feeding tubes or special
bottles to maintain sufficient nutrition.
• Physical, occupational, and speech therapy can
improve motor skills and coordination and can
lead to better control of speaking and eating
abilities.
• Surgery can correct crossed eyes.
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13. Surgical Therapy:
The most recent procedure, called “the smile
operation”, involves microvascular transfer of a
muscle from the thigh (gracillis) to the face and
connecting the nerves that normally supply the
masseter muscle.
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14. MEDICATION:
With few exceptions, pharmacologic intervention in
patients with Möbius syndrome is used only for
symptomatic treatment. Patients with Möbius syndrome
may be predisposed to infections, such as pulmonary
infections resulting from aspiration pneumonia.
Antibiotics used in the treatment of infectious
complications include:
1. Amoxicillin.
2. Trimethoprim and sulfamethoxazole.
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15. EPIDEMIOLOGY:
It is estimated that there are, on average, 2 to 20
cases of Möbius syndrome per million births.
In 2007, the Möbius Syndrome Foundation
estimated that 2000 individuals worldwide have the
condition.
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16. CONCLUSIONS:
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• Möbius syndrome: is rare congenital neurological disorder
• There are four group: Simple hypoplasia, Primary lesions in
CNs, Focal necrosis in brainstem nuclei and Primary myopathy.
• Signs and symptoms: Limb abnormalities, Chest-wall
abnormalities, Crossed eyes and Difficulty in breathing and/or
in swallowing.
• It results from a vascular disruption, The use of drugs and a
traumatic pregnancy associated with development of Möbius
syndrome.
• There is no single course of medical treatment for Möbius
syndrome.