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At a glance

A very rare syndrome combining mental retardation and early spastic diplegia. Same term also used for permanent damage to upper motor neuron secondary to cerebral anoxia and periventricular leukomalacia (prematurity).


Little Syndrome.


The incidence and prevalence remain unknown.

Genetic inheritance

Autosomal recessive inheritance.

Clinical aspects

Affected patient presents with spastic diplegia in early infancy. Motor development is slow and there is generalized muscular hypotonia and hyperreflexia. Ataxia is usually absent but gait is often waddling with inward-turning feet. There is mild to moderate mental retardation with slow speech development. Longterm follow-up has not been reported.

Anesthetic considerations

Assess gait and motor function. Obtain history of previous anesthetics and muscle biopsy result if available. Biochemistry including potassium and creatine phosphokinase level. Careful intraoperative positioning is needed. Prophylactic treatment of muscle spasms. Bispectral index (BIS) values may be low before induction of anesthesia.

Pharmacological implications

Relative resistance to nondepolarizing muscle relaxants. Succinylcholine safe to use despite increase of extra junctional muscarinic receptors.


Gustavson  K, Modrzewska  K, Erikson  A: Hereditary spastic diplegia with mental retardation in two young siblings. Clin Genet 36:439, 1989.  [PubMed: 2591069]
Valkenburg  AJ, de Leeuw  TG,  et al: Lower bispectral index values in children who are intellectually disabled. Anesth Analg 109:1428, 2009.  [PubMed: 19843782]

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