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Moebius syndrome

Mobius syndrome (also spelled Moebius) is an extremely rare neurological disorder. more...

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Clinical features

Mobius syndrome is caused by abnormal development of the cranial nerves. This rare disorder has a number of causes. Most often affected are the cranial nerves VI and VII. Occasionally the cranial nerves V and VIII are affected.

If the cranial nerve VI is affected, the patient suffers from loss of lateral gaze. If cranial nerve VII is affected, the patient suffers from bilateral facial palsy — mask-like expressionless face with mouth constantly held open. If cranial VIII is affected the patient suffers from hearing loss.

Although its rarity often leads to late diagnosis, Infants with this disorder can be identified at birth: by a "mask-like" expression detectable during crying or laughing due to paralysis (palsy) of the sixth and seventh cranial nerves. Other characteristics include:

  • abnormalities in the limbs — their fingers may be webbed, shorter than usual or they may have more than 5 fingers on their hand
  • impaired sucking ability
  • inability to follow objects with the eye—instead the child turns his or her head to follow
  • crossed eyes
  • inability to smile
  • limitation of tongue movement

Later on, the child may develop speech difficulties, crossed eyes, abnormally small eyes, and fluid building up in the lungs, causing bronchopneumonia.

Treatment

There is no specific course of treatment for Mobius syndrome. Treatment is supportive and in accordance with symptoms. Infants may require feeding tubes or special bottles, such as the Haberman Feeder, to maintain sufficient nutrition. Surgery may correct crossed eyes and improve limb and jaw deformities. Physical and speech therapy often improves motor skills and coordination, and leads to better control of speaking and eating abilities. Plastic reconstructive surgery may be beneficial in some individuals. Nerve and muscle transfers to the corners of the mouth have been performed to provide limited ability to smile.

Pathological picture

The causes of Mobius syndrome are poorly understood. Many cases have no obvious cause. Others may be genetic.

Some cases are associated with reciprocal translocation between chromosomes or maternal illness. Some maternal trauma may result in impaired or interrupted blood flow (Ischemia) or lack of oxygen (Hypoxia) to a developing fetus. The use of drugs and a traumatic pregnancy may also be linked to the development of Mobius syndrome. The use of the drugs Misoprostol or Thalidomide by women during pregnancy has been linked to the development of Mobius syndrome in some cases.

Some researchers have suggested that the underlying problem of this disorder could be congenital hypoplasia or agenesis of the cranial nerve nuclei. Certain symptoms associated with Mobius 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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Students gain valuable skills at CAN Learn
From Spokesman Review, The (Spokane), 7/25/02 by Kristen Kromer Staff writer

Paige Robinson was a rage queen.

"If I got in trouble, I'd scream, hit, bite, punch and cuss," said Robinson, 11.

Her sister, Morgan Robinson, 16, used to be so confused by math that she'd get frustrated and give up.

And Tanya Iwan, 11, had trouble seeing, was getting F's and had trouble remembering any type of list.

But since they started coming to CAN Learn, they say things have changed.

Paige has learned how to work through anger so it doesn't spew out in a tantrum. Morgan has found ways to help her brain focus, and as Tanya's vision has improved, she's seen her grades follow suit.

"It's awesome the difference that's been made since Toni started working with them," said Ramona Iwan, Tanya's mother.

In December, neurodevelopmental specialist Toni Hager opened Children's Academy for Neurodevelopment and Learning at 918 S. Monroe, next to Huckleberry's. She works with children of all ages who have developmental or learning problems.

They are the ones who have been labeled as having attention deficit disorder, auditory processing disorder or fetal alcohol syndrome, being developmentally delayed, learning disabled or dyslexic. They are the ones who, for a range of reasons, just don't get it.

Hager, who has degrees in applied and developmental psychology from Eastern Washington University, explains that these types of children have missed a stage of development. To help them, she starts from the beginning, working on mastery of basic birth reflexes, and builds from there.

"We work on reorganizing the central nervous system and training it to do what it missed," she said.

Hager currently sees 15 children at CAN Learn. She said she also works with families in Minnesota, Virginia, Montana and Oregon.

Carli Robinson raised three birth children, then adopted six others who all attend CAN Learn. All came from less-than-ideal situations: They were abused, neglected or had mothers who were drunk or high during pregnancy.

She and others who come to CAN Learn feel they have found an oasis.

"You have to search for people like Toni because the medical profession doesn't have answers for these kids," said Robinson, who also helps out at the center and homeschools her children.

Hager's interest in neurodevelopment started with her own children. Both of her daughters have Moebius syndrome, a disorder in which several cranial nerves are underdeveloped or missing, often resulting in facial paralysis.

Frustrating years of speech, occupational and physical therapy brought few results. It wasn't until she met a neurodevelopment professional that Hager received an explanation for how her daughters' bodies were working and what she could do to help them.

At CAN Learn, children work in groups, or individually if they need more personal attention. Parents are welcome to stay and watch, too. Sessions generally last about an hour and a half. The cost is $400 a month, and students come five days a week.

They do exercises that focus on basic motor skills and reflexes: They lay on a mat and lift their head, roll, march and touch their right hand to their left foot and left hand to right foot. They crawl down a "creep track," keeping opposite arms and legs out straight. They work on hand-eye coordination on the monkey bars and bounce on giant rubber balls. Talking-pen activities have them trace shapes with a pen that beeps when they go out of the lines.

"It's therapy, but we try to make it fun," Hager said.

The exercises are done again and again and again. For that reason, it's not always the children's favorite place to come.

"I don't like it because I'm forced to come," said Tom Robinson, 14, "but I think it helps quite a bit. I had a stuttering problem, and it used to take me a long time to learn how to do things."

He says this without a trace of a stutter.

Moms say they've seen their kids take some big steps - like getting rid of rages - and smaller steps too.

"Now he just kicks walls, instead of putting holes in them," Robinson said of one of her children.

But every bit of progress is celebrated.

"This center has sped up their movement toward progress," Robinson said. "We want to stop the cycles they've come from and give them the opportunity to be successful."

Copyright 2002 Cowles Publishing Company
Provided by ProQuest Information and Learning Company. All rights Reserved.

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