Lesions of the Corpus Striatum in Childhood, With Report of Clinical Cases Illustrating Various Syndromes

Abstract

Every physician who deals with any considerable number of children with paralysis of cerebral origin is impressed by the fact that the motor disturbances vary enormously in type. Most common, perhaps, is the conventional spastic type, either hemiplegic or paraplegic, with scissors gait, contractures and increased reflexes. Others show bizarre abnormalities of motion, speech defect and changes of muscle tone, without great variations in reflexes. Mental defectiveness of any degree may accompany the motor manifestations.Pathologically, a wide variety of lesions have been described, and the searcher in the literature is left with a confused idea that so many causes may produce cerebral palsy, that it is useless to try to localize lesions accurately.As a result children with cerebral palsies have, to a large extent, been grouped as essentially hopeless, except as tenotomies or nerve section might relieve disabling spasticity or contracture. Classification has been distinctly unsatisfactory, and a considerable array of names, mostly descriptive of symptoms, has added to the confusion. This paper is written to put before pediatricians certain facts, recently established by workers in several countries, and to show that lesions of the corpus striatum can logically account for the symptoms of a group of children with motor disabilities, who show disturbance of associated movements, variations in muscle tone and defects of speech. I believe that accurate prognosis and logical therapeutic efforts will be assisted by the separation of these cases from other cerebral palsies.Six cases are presented to show some of the different syndromes resulting from lesions of the corpus striatum in childhood. Of course, it is obvious enough that, without necropsies, such cases are not con¬ clusive, but a study of the literature, and frequent consultation with others, convinces me that there are sound reasons for classifying the cases definitely.

Early Controversies over Athetosis: I. Clinical Features, Differentiation from other Movement Disorders, Associated Conditions, and Pathology

“…Although Cécile Vogt had delineated status marmoratus as a specific pathological entity of presumed prenatal onset, in 1921 Boston child neurologist Bronson Crothers (1884–1959)97 and later authors instead stressed the likelihood that perinatal factors, including birth injury, were responsible for cases of double athetosis 98–100. The association of athetosis with neonatal jaundice in low birthweight babies was established in the 1960s;99–100 subsequently, aggressive treatment of perinatal hyperbilirubinemia produced a decline in chronic bilirubin encephalopathy so that kernicterus is now a rare cause of dyskinetic cerebral palsy 101…”

The Neurology of Cerebral Palsy

“…Similarly the finding of rhesus incompatibility between mother and patient, with a history suggestive of kernikterus and clinical findings of congenital choreoathetosis with defective upward movement of the eyes, high-tone deafness, and enamel defects of the teeth (Fig. 3), allows a diagnosis to be made of dyskinetic cerebral palsy attributable to rhesus incompatibility (Crothers, 1921;Byers, 1942;Evans and Polani, 1950;Forrester and Miller, 1955;Plum, 1965). The clinical picture of more or less symmetrical paresis of the limbs, more marked in the lower limbs than in the upper, and usually associated with rigidity or spasticity, which is found in a high proportion of babies of small birth weight, is also characteristic, though it is much more difficult to identify the 'cause' in this example (Drillien, 1964;Russell, 1960a).…”

Early Controversies over Athetosis: I. Clinical Features, Differentiation from other Movement Disorders, Associated Conditions, and Pathology

“…… The globus pallidus was atrophic, as was also the nucleus caudatus [caudate nucleus], but neither showed the peculiar tissue seen in the putamen. 82 Although Cécile Vogt had delineated status marmoratus as a specific pathological entity of presumed prenatal onset, in 1921 Boston child neurologist Bronson Crothers (1884-1959) 97 and later authors instead stressed the likelihood that perinatal factors, including birth injury, were responsible for cases of double athetosis. [98][99][100] The association of athetosis with neonatal jaundice in low birthweight babies was established in the 1960s; 99-100 subsequently, aggressive treatment of perinatal hyperbilirubinemia produced a decline in chronic bilirubin encephalopathy so that kernicterus is now a rare cause of dyskinetic cerebral palsy.…”