[MIM 270 600]

(infant spastic diplegia)

Spastic paresis of the lower limbs due to perinatal cerebral injury (anoxia, ischemia) or severe prematurity (periventricular leukomalacia) that causes damage to upper motor neurones. The same term is also used for a rare autosomal recessive transmitted disorder that associates spastic diplegia and severe mental retardation.

Upper motor neuron lesion causes chronic suffering at the level of the involved muscles.

Treatment: antispastic agents (baclofen, dantrolene, tizanidine), orthoses,  orthopedic surgery, botulinum toxin injections, intrathecal baclofen  pump

Anesthetic implications:

management for a child with motor impairment and often mental retardation or epilepsy. If planning to use BIS in a child who is mentally retarded, place the BIS before induction for obtain baseline (usually lower) values while  awake. Relative resistance to non-depolarizing muscle relaxants. The use of succinylcholine does not carry a risk of hyperkalemia, although more extrajunctional muscarinic receptors  are present on the muscle membrane. Difficult neuraxial block in case of scoliosis. Provide preventive treatment of muscle spasms in addition to the analgesic treatment. Do not abruptly stop the administration of intrathecal baclofen !

References : 

-        Nolan J, Chalkiadis GA, Low J, et al. 
Anaesthesia and pain management in cerebral palsy. 
Anaesthesia 2000; 55:32-41.

-        Frei FJ, Haemmerle MH, Brunner R, et al. 
Minimum alveolar concentration for halothane in children with cerebral palsy and severe mental retardation. 
Anaesthesia 1997; 52:1056-60.

-        Valkenburg AJ, de Leeuw TG, Tibboel D, Weber F. 
Lower bispectral index values in children who are intellectually disabled. 
Anesth Analg 2009; 109:1428-33.

-        Theroux M, Akins RE, Barone C. 
Neuromuscular junctions in cerebral palsy: presence of extrajunctional receptors. 
Anesthesiology 2002; 96:330-5.

-        Deon LL, Gaebler-Spira D. 
Assessment and treatment of movement disorders in children with cerebral palsy. 
Orthop Clin N Am 2010; 41: 507-17.

Updated: January 2019