Tue, Mar 26, 2019 - Page 4 News List

Doctor warns over NF1 symptoms

By Tsai Shu-yuan  /  Staff reporter

Chou I-ching, director of the neurology division at China Medical University Children’s Hospital, conducts a news conference in Taichung on Feb. 27.

Photo: Tsai Shu-yuan, Taipei Times

Handedness at a young age and unusual birth marks might be an indication of the rare condition neurofibromatosis type 1 (NF1), a doctor said.

Chou I-ching (周宜卿), director of the neurology division at China Medical University Children’s Hospital, said that a one-year-old girl was diagnosed with NF1 last month after her parents observed right-handedness when she was 11 months old.

The parents saw that she had a tendency to use her right hand most of the time, Chou said. Despite her left hand seeming to have normal strength, she rarely used it and did not spread her fingers or grip things with it.

The parents observed that their daughter almost always held her milk bottle in her right hand, and climbed or reached exclusively with it, she said.

They took her to a doctor and she was referred to a medical center for further examination, which showed that the baby had abnormal marks and a tumor on the basal ganglia on the right side of her brain, Chou said.

The marks were smaller than 5mm and not easy to detect, but were abnormal, she said.

An ultrasonography and magnetic resonance imaging scan revealed a hamartoma on her brain and NF1 was confirmed after a blood test, she said.

The right basal ganglia is associated with voluntary motor movements, language learning and other nerve functions, so the tumor might affect movement in the left hand, Chou said.

However, surgery to remove the tumor is not possible, she said.

Handedness usually starts to develop from age two, as infants’ brains do not begin separating tasks between the two cerebral hemispheres until then, Chou said.

Parents should look for consistent handedness in children under the age of two, because it might be a sign of disease, injury, malnutrition, brain structure anomalies, brain malformation or other problems that might respond to early treatment, she said.

As NF1 is a genetic neurological disorder, multiple tumors might develop in other parts of the body, she said, adding that early treatment might improve movement in the child’s left hand, while her learning and cognitive development would be tracked to prevent developmental delay.

Approximately one in 20,000 people are affected by NF1, which is caused by a gene mutation, Chou said.

People with the condition are likely to develop multiple light-brown marks on their skin as they grow, she said.

They also have higher risk of developing fibromata — benign tumors composed of fibrous or connective tissue — and learning disabilities, she said, adding that NF1 is an autosomal dominant disorder, so although the parents did not have the disease, any children they might have face a 50 percent chance of inheriting the condition.

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