To change Your Site Name go to the 'Page Master' under the 'Design' menu
Soon after the tremor started, Niamh began to experience much larger and far more distressing movements.
These manifested themselves in several ways:
Throwing her head backwards, going completely rigid and arching her back and screamingGoing rigid, clenching her fists and drawing them up to her chest/under her chin, with her legs rigid and raised in a v sit position with head bowed to chest and jaw open and moaning. Twisting her head extremely to the left (almost looking backwards over her shoulder) and with her tongue going blue/purple protruding from her mouth whist she rasped for breath.
Initially, these (especially the blue tongue ones) were terrifying to watch.
We were sure these episodes were seizures as they look remarkable similar, however EEG's concluded that they were not.
They are believed to be caused by the incorrect functioning of part of the brain that is responsible for movement - the basal ganglia. It is believed that this incorrect functioning arises from the imbalance of neurotransmitters.
Continual abnormal postures and relentless muscle spasms cause the child considerable pain and discomfort.
Dystonia can affect virtually any single part of the body (focal) or several different areas at once (multifocal/generalised)
How are they diagnosed?
In a child with an existing seizure disorder, Dystonia can look very like seizures so the first thing to do is to capture a posture or spasm on an EEG to rule out epileptic activity. After seizures have been ruled out, they are principally diagnosed by appearance, although an MRI scan may prove helpful as it would confirm whether there is any damage to the basal ganglia area of the brain which would be consistent with this condition.
An MRI scan Niamh had taken shortly after these began confirmed atrophy to the Basal Ganglia area of her brain - consistent with her diagnosis of Dystonia.
Unlike seizures, it is sometimes possible to bring a person out of a dystonic spasm by distraction. With Niamh we used to gently move her limbs forward and backwards to bring the limb out of spasm, or by jiggling her around, blowing in her face, or clapping her hands together - any kind of movement or distraction really.
This often worked to "break" the immediate pain but some days she would just go straight back into another one - so distraction ended up a full time job.
General muscle relaxant drugs are usually prescribed to relax the whole body's muscles thus making it more difficult for the muscles to spasm. Baclofen is commonly used, but Clonazepam is also often tried as a long term treatment as it can cause muscle relaxation as a side effect.
Sedation with Chloral Hydrate and Buccal Midazolam was the only combination that really helped Niamh when her spasms were bad.
With oral Baclofen, only a very small amount of the medication actually reaches the spinal cavity thus meaning very high doses are
often required to achieve a therapeutic effect.
If the oral dose is not effective (or causes intolerable side effects) then Intrathecal Baclofen or "Baclofen Pump" may be considered.
The Baclofen Pump is a method of administering Baclofen directly into the cavity surrounding the spinal cord (intrathecal space) and
thus removing the need for higher doses of oral medication.
The Baclofen is given directly into the space around the spinal cord via a permanent, surgically implanted pump that is placed in the
abdomen with a catheter (tube) that goes to the spinal cord
Although this treatment was considered for Niamh's Dystonic Spasms, she was simply not heavy/big enough to accommodate the
pump within her small frame. We were told that 10Kg was the absolute minimum weight to be considered but it also depends on the
shape of the child as the pump must be accommodated within their body.
Botox is also used to treat spasm in areas where the specific muscle groups responsible for the spasm can be identified. Botox is injected into the affected muscle group responsible and it temporarily paralyzes that muscle - reducing the chance of spasm.
Depending on the area being treated, paralysing a muscle group may lead to other more serious problems; for example – Niamh’s spasms were particularly problematic in her next and after she had her Botox, she had a short term reduced ability to swallow and cough which led to breathing difficulties for her.
We found the effects of Botox was very good in reducing Niamh’s spasms but that results were very short lived - for her it only lasted for 2 weeks. As the procedure could only be repeated every 12 weeks we were left with a long wait between treatments.
Status Dystonicus refers to a life threatening condition where the body goes into permanent dystonic spasm. It requires hospitalization and immediate heavy sedation/induced coma of the child for their own safety.
Niamh only had one incidence of Status Dystonicus in her life in July 2008. She was placed into an induced coma for one week and heavily sedated for a further four weeks. Although she survived this episode her recovery was not complete and her underlying condition had deteriorated significantly. After this episode she required daily sedation for her dystonic spasms and continual oxygen therapy to assist her breathing.
You are viewing the text version of this site.
To view the full version please install the Adobe Flash Player and ensure your web browser has JavaScript enabled.
Need help? check the requirements page.