RECENT ADVANCES IN THE MANAGEMENT OF CEREBRAL PALSY SPASTICITY & DYSTONIA
Dr. A. K. Purohit*
Prof. & Head, Department of Neurosurgery Nizam's Institute of Medical Sciences, Hyderabad Email: akpcpcp@gmail.com*
Spasticity is the most common impairment in cerebral palsy. There are various grades of this impairment and there are different combinations of the parts affected along with other impairments like somatic, special somatic and cognitive deficiencies. All these factors adversely affect the prognosis. However, the development of complications of spasticity also play an important role in the prognosis. Above all meager awareness about cerebral palsy and the lack of early intervention has shown poor development of these children. All these factors have made management of spasticity and the development of child with cerebral palsy a challenging task.

There are many modalities of management of cerebral palsy like therapeutic exercises, antispasticity drugs, orthopedic interventions, neurosurgical interventions and additional (complimentary) therapies. All of them have somewhere some role in these cases. However, that depends on the experience of the clinician to choose correct method for a particular problem keeping the child has a whole in mind. But, mostly they are used at a wrong time or for a wrong indication. Not only that professionals who know one particular modality of treatment prefer to treat with that modality only. Because of this there is bias and the family gets confused. In nutshell we can say that:

A. The professionals are biased with what they know and
B. The families are confused as to what is the best for their child

As such child with cerebral palsy does not only havespasticity as a sole impairment, but can have many other motor deficiencies related to muscular tone, power and balance (posture and movement). They can have associated disorders like mental sub-normality, seizure, speech and hearing impairment, vision defects, etc. Therefore, a team of specialists is essential to look after the child as a whole and not to look only towards impairment like spasticity and treat it.

The treatment of the spasticity and for that matter any patient should be from non-invasive techniques through less invasive to invasive techniques. Therefore, we advocate following steps of spasticity managements.

Non-invasive:
  1. Physiotherapy, occupational therapy
  2. Use of external appliances

Less invasive:
  1. Oral antispasticity drugs
  2. Injections of medications like Baclofen (ITB-Intrathecal Baclofen-Pump), Phenol, Botulinum Toxin
  3. Spinal cord stimulation

  4. Invasive:
    1. Neurosurgical procedures
      • SPR (Selective Posterior Rhizotomy
      • SMF (Selective Motor Fasciculotomy)
    1. Orthopedic procedures
      • STR (Soft Tissue Release) surgeries
      • Tendon transfer Another important method of classification of interventions can be based on distribution of harmful spasticity. It is divided into following two types.
      • Focal spasticity
      • Diffuse spasticity

    The interventions for these two topographical kinds of spasticity are classified as mentioned below. However, there are cases where strictly these suggestions cannot be followed because of the presence of other variables like control, contractures, etc.

    Focal spasticity:
    1. Physical methods - i) Therapeutic exercises, Physiotherapy ii) other physical methods
      (like ice therapy etc. iii) Complimentary therapies
    2. Medicated injections - i) Botulinum Toxin ii) Phenol /alcohol iii) ITB pump
    3. Neurosurgical procedures - SMF
    4. Orthopedic procedures - i) STR ii) Tendon transfer

    Diffuse spasticity:
    1. Physical methods
    2. Neurosurgical procedures, Non-ablative neurosurgical procedures, i) Pumps/stimulators Intrathecal baclofen (ITB) pump, Spinal cord stimulation ii) Ablative neurosurgical procedures
    3. Orthopedic procedures - i) Multiple STR ii) Multiple tendon transfers

    It is generally understood that the causative factor of muscle shortening that is spasticity should be managed be relieving spasticity and that is best possible by stimulating or interrupting one of the following mechanism:
    1. Stimulation of supra-segmental control: Pyramidal and para-reticular formation are responsible for supra-segmental control. They synapse with anterior horn cells and inhibit their natural ability to generate impulses. Thereby they balance the muscle tone.
      In cerebral palsy, as it is well known that the damage to the motor areas of the brain results in lesser transmission of impulses through the motor tracts. Therefore, the spinal cord stimulation can help in transmission of impulses through poorly functioning pyramidal tracts.
      Ideally speaking regeneration or transplantation of the damaged neural tissue should be considered as the main modality of treatment. However presently this methodology is in experimental stage and hopefully would come in vogue in coming decades.

    2. Inhibition of segmental control:It is known that by nature that the anterior horn cells (AHC) continuously discharge impulses, which are carried by anterior roots through peripheral nerves to the myoneural junctions of the muscles. However from the tendons through peripheral nerves and posterior roots, the stretch sense reaches the grey matter of the spinal cord, which in turn synapses with the AHC and this circuit makes the spinal reflex arch. By nature it can be called as facilitatory reflex arch. If it is not inhibited by suprasegmental fibres (pyramidal tracts), then it would lead to development of spasticity. Exactly this thing happens in cerebral palsy. Scientists for the past more than a century are working on this spinal reflex arch to reduce spasticity. Presently following procedures are performed on this arch:
      1. Spinal Roots: 1. Rhizotomy SPR
      2. Peripheral nerves and myoneural junction: 2. SMF 3. Peripheral nerve blocks Phenol, Alcohol 4. Myo-neural junction block Botulinum toxin
      3. Muscles: 5. Lengthening of muscle to reduce golgi tendon stretch reflex contribution to spinal reflexes arch. However, these methods are best suited when organic i.e. permanent shortening of muscle get settled.
      4. Spinal Cord: 6. ITB

    There are a few methods, which may be good for the individual patient from medical point of view. However, affordability may become a problem in the children from developing and under developed country. Therefore, social medicine comes into importance while managing the case. Say for example rhizotomy (SPR) and SMF fasciculotomy cost around Rs. 10,000 to 20,000 where as botulinum toxin and ITB cost around Rs. 50,000 to 3,00,000/-, There is recurring expenditure also in the later two methods. However, in some cases these non ablative procedures may be the best suitable procedures from medical point of view. The other methods, which are ablative, may not be best suitable. However, if the family cannot afford then the child cannot be left for non-development and to get into complications of spasticity. A judgment by the physician as to what is best for the family is very much expected and that would only motivate the family to sustain the longterm habilitation of the child.

    Dystonia in Cerebral Palsy
    There are many drugs to control dystonia. However, they are not effective in all the cases. There fore, in selected cases of diffuse variety, especially if associated with diffuse spasticity, ITB can be recommended. Presently, such selected cases have shown some facial effects. In our series some functional improvement was noticed.
    In hemidystonic cases and in a few diffuse cases, stereotactic thalamic-basal ganglionic stimulatory or ablative surgery can be considered. The nanotechnology in future is likely to be quite promising in this group of cases.
    In a few segmental and focal dystonic cases botulinum toxin and or selective motor fasciculotomy (SMF) might help. In these cases drugs is less preferred because systemic medication for life long is associated with other drug related complications. The orthopedic surgery is invariably associated with recurrence. The use of BT with recurring expenditure is extremely expensive. Therefore, some help can be extended by SMF. In author's series some control on dystonia could be achieved and in a few cases better use of the hand in bimanual activities was noticed.
    To some up it is very important for clinicians to know evaluation of the child with cerebral palsy and to correctly decide the best helpful procedures for that child and the family. It is also essential to acquire knowledge and skill in as many procedures as possible. So that bias for what they know is eliminated.
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