Why do children with holoprosencephaly have spasticity and how is it treated?

Spasticity seen in various forms of HPE comes from underlying motor control dysfunction. This leads to imbalances in the control of voluntary muscles movements used in such activities as walking. There are several oral medications that can reduce spasticity.

These include diazepam (Valium) and baclofen. For certain forms of tightness such as dystonia, medications such as (trihexyphenidyl (Artane) can be useful. Botulinum toxin (Botox®) injection is one way of dealing with the muscle imbalances by weakening selective muscles and changing the balance.

There are other ways to approach spasticity. All of these medications have side effects and need careful consideration and monitoring. The field of treating spastcity is evolving quickly and such new approaches as surgery (dorsal rhizotomy) and intrathecal (into the spinal fluid) administration of the antispasticity medication baclofen is now being used in children.

A good lay review of the management of spasticity can be found in ... more.

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