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6. Yaar, I., Herishanu, Y., Lavy, S. (1973) 'Radiation myelopathy.' European Neurology, 10, 83-88. 7. Kristensson, K., Molin, B., Sourander, 0. (1 967) 'Delayed radiation lesions in the human spinal cord.' Acta

8. Godwin-Austen, R. B.. Howell, D. A., Worthington, B. (1975) 'Observations on radiation myelopathy.'

9. Puck, T. T., Morkovin, D., Marcus, P. I., Cieciura, S. J. (1957) 'Action of X-rays on mammalian cells, 11.

10. Zeeman, W. ( 1 966) 'Pathogenesis of radiolesions in the mature central nervous system.' In Proceedings of

1 1. Howell, D. A,, Embree, G . , Tatlow. W. F. T. (1963) 'Observations on strokes and cerebral edema with

12. Palmer. J . J. (1972) 'Radiation myelopathy.' Brain, 95, 109-112. 13. Marty, R., Minkler, D. S. (1973) 'Radiation myelitis simulating a tumor.' Archives of Neurology, 29,

14. Ballweg, G . P., Donnenfeld, H., Chusid, J. G . (1976) 'Subacute radiation myelopathy in a twelve-year-old

15. Hoffman, H. L. (1955) 'Acute necrotic myelopathy.' Brain, 78, 377-393. 16. Ellis. F. (1971) 'Nominal standard dose and the ret.' British Journal ofRadiology, 44, 101-108. 17. Wara, W. M., Philips, T. L., Sheline, G. E., Schwade, J. G . ( I 975) 'Radiation tolerance of the spinal cord.'

18. McFarland, D. R., Honvitz, H., Saenger, E. L., Bahr, G. K. (1969) 'Medulloblastoma-a review of prognosis

19. Zeeman, W., Samorajski, T. (1971) 'Effects of radiation on the nervous system.' I n Berdjis, C. C. (Ed.)

20. Price, R. A., Jamieson, P. A. (1975) 'The central nervous system in childhood leukaemia. 11. Subacute

21. Jones, A. (1964) 'Transient radiation myelopathy.' British Journal of Radiology, 37, 727-744. 22. Freeman, J. E., Johnstone, P. G . B., Voke, J. M. (1 973) 'Somnolence after prophylactic cranial irradiation in

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25. Sundaresan. N., Gutierrez, F. A,, Larsen, M. B. (1978) 'Radiation myelopathy in children.' Annals of

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VESTIBULAR DYSFUNCTION IN AUTISTIC CHILDREN AUTISTIC children are known to enjoy whirling, and it has been observed that they tend not to develop associated dizziness, vertigo, loss of balance, nausea or vomiting'-3. They also have been observed to have abnormalities of posture and b a l a n ~ e ~ . ~ . Such findings have suggested that these children have some form of vestibular dysfunction6-", and have inspired experimental work which has been interpreted as supporting the notion of dysfunction in the vestibular system and its central connections9-I7. On the basis of this work there has been an attempt to explain the symptomatology of autism, in neurophysiological terms, as the result of vestibular dysfunction7.".

These interesting studies are problematic in several ways. Generally they have lacked appropriate controls, particularly for mental agei7. The findings, both clinical and experimental, may apply only to certain sub-groups of autistic children " . I 7 , and may not be specific to autism, having been reported in schizophrenia'" and in non-autistic children with cognitive disordersI6. There are also questions as to the interpretations made of the findings: for instance, are the clinical manifestations used to infer vestibular dysfunction specific signs of disturbance in the vestibular system? Can the major clinical manifestations of autism be explained on the basis of the presumed vestibular defect? Is the notion of vestibular defect particularly suggestive of a given locus of dysfunction, such as brain stem or telencephalic? We shall consider this last question first.

Vestibular dysfunction generally is considered to indicate disturbance in the brain stem, vestibular nerve or labyrinth, but also is compatible with a telencephalic disturbance.

656

ANNOTATIONS

Although the telencephalic projections of vestibular information have not been studied as extensively as those of other forms of sensory input, and their clinical neurological significance is not entirely clear, there is good evidence from human and animal studies that vestibular information reaches neocortical structures and from there projects to the basal ganglia. PENFIELD, studying the results of electrical stimulation of the human cortex, was able to elicit vestibular responses from areas close to the auditory projection in the posterior regions of the first temporal gyrusiy.zo. FREDRICKSON et ulz ’ found that, in the Rhesus monkey, electrical stimulation of the vestibular nerve produces specific cortical evoked potentials in the lower and posterior part of the postcentral gyrus at the base of the intraparietal sulcus. Single neurons in this region also respond to proprioceptive, as well as vestibular, stimulizz, a bimodal convergence similar to that previously found for vestibular and visual information in the cortex of the catz3. All of the above cortical regions are known to project to the basal gangliaz4.zs where further multimodal sensory integration takes place.

The results of animal experimental work by DENNY-BROWN~~, DELMAS-MARSALET et d.,27 and, in particular, M E T T L E R ~ ~ , ~ ’ are pertinent to the findings of diminished post-rotary nystagmus in autistic children 9 - i 1 . 1 4 . 1 5 . MEITLER and METTLER formed bilateral ablations of (a) the frontal region exclusive of the caudate, (b) the frontal region including the head of the caudate, and (c) the labyrinth. Rotation of animals with frontal and frontal-caudate lesions produced effects similar to those seen in normal animals, but with less obvious distress during rotation. The usual post-rotatory symptomatology (forced movements, postures and nystagmus) was present but did not persist. Animals with frontal-caudate lesions differed from animals with frontal lesions only in showing no evidence of subjective discomfort during rotation and more rapid subsidence of post-rotatory symptoms. Animals with vestibular lesions presented a different picture, characterized by a crouching posture and by ataxia accompanied by gross tremors. Removal of the heads of both caudate nuclei some weeks later abolished these symptoms.

The above findings lead to the question of the specificity of vestibular signs. As noted in experimental animals, damage to the labyrinth did not produce the sort of manifestations observed in autistic children, but similar manifestations did appear with lesions of the neostriatum. In humans, abnormalities of posture and balance like those described in autism, and, in addition, the absence of position-related nystagmus, have been noted in patients with Parkinsonism, a disease primarily produced by neostriatal dysfunction. MARTIN~O even hypothesized that the basal ganglia might be the ‘higher authority’ in the brain that decides whether or not vestibular stimulation will excite postural reflexes, and cited MUSKENS’ notion3’ that the basal ganglia, particularly the neostratum, are the head ganglia of the vestibular system. We would suggest that although such manifestations are ‘vestibular’ in the broad sense, they are in fact supramodal and related to function of integrative CNS structures. In turn, dysfunction of such supramodal, integrative structures would determine not only ‘vestibular’ signs but a host of other manifestations which are not a consequence of vestibular defects and which include abnormal responses to visual and auditory stimuli, as well as the preference autistic children have for somatosensory ~ t i m u l i ” ~ ~ ~ . The last is in keeping with observations by M E T T L E R ~ ~ which suggest that a striatally deprived organism can come under ‘proprioceptive domination’.

It is remarkable that preliminary data on the relationship of vestibular dysfunction to ratings of autistic behaviour (categorized into disturbances of perception, motility and relating) showed that almost all of the significant correlations were with disturbances of

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DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY. 1979, 21

motilitys. Such motor disturbances are most probably related to dysfunction of the basal ganglia and related structures3’.

In conclusion, the ‘vestibular’ disturbances of childhood autism seem to be neither primary nor specific. It is possible that along with defects in attention, affect and motility, such disturbances signify the presence of some form of dysfunction in basal ganglia and related structures, which may be present in a variety of psychiatric disorders, such as

or schiz~phrenia~~,~’ .

Departments of Psychiatry* and Neurologyt , University of Iowa College of Medicine, Iowa City, Iowa 52242.

RALPH G. MAURER* ANTONIO R. DAMASlOt

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12. Ornitz, E. M., Forsythe, A. B., de la Pena, A. (1973) ‘The effect of vestibular and auditory stimulation on the REMs of REM sleep in autistic children.’ Archives of General Psychiatry, 29, 786-791.

13. Omitz, E. M., Forsythe, A. B., de la Pena, A. (1973) ‘The effect of vestibular and auditory stimulation on the rapid eye movements of REM sleep in normal children.’ Electroencephalography and Clinical Neurophysiology, 34, 379-390.

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28. Mettler, F. A., Mettler, C. C. (1940) ‘Labyrinthine disregard after removal of the caudate.’ Proceedings of the Sociery for Experimental Biolo y and Medicine, 45, 473-475.

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CONTROVERSIES ABOUT DUCHENNE MUSCULAR DYSTROPHY. (2) BRACING FOR AMBULATION

LIFE with Duchenne muscular dystrophy (DMD), for both the affected boy and his parents, is punctuated by a series of crises. After the diagnosis itself the next major crisis to be faced is the moment when he loses the ability to walk, usually between the age of eight and 11 years. Parents fear this moment more than any except death itself. Perhaps it is partly because the wheelchair has become the universal symbol of disability, but certainly the loss of their boy’s ability to walk confirms in a graphic and inescapable way the prognosis they had been given and had been hoping against hope might be wrong.

In 1962 SPENCER and VIGNOS’ described successful prolongation of ambulation in boys with DMD by the combined use of tenotomies and full-length calipers. Since then the introduction of new light-weight materials has greatly improved the calipers, and many other clinics have confirmed the effectiveness of the Others, including the present writer, have been much less enthusiastic about the procedure and only one centre in Britain at the Hammersmith Hospital is at present providing it as a regular service to Duchenne patients.

The technique is based upon observations of stance and gait as the boys approach the point of being unable to walk. The essential limiting factor is muscular weakness, but contracture, itself largely secondary to weakness and abnormal posture, may also play a part. Critical weakness develops first in the quadriceps muscles and in the extensors of the hips. A little unguarded flexion of the knees or hips while the boy is standing or walking results in jack-knifing and a sudden fall. Consequently these joints must remain fully extended whenever they are bearing weight, and at all times the body’s centre of gravity must be carried immediately above them both. From this simple premise appears to stem the typical posture, the wide-based gait on the toes, with the shoulders and head thrust back to induce the characteristic lumbar lordosis. Often the boy will stand squarely on one leg, the other half-astride, balancing on a plantiflexed forefoot. This posture usually results before long in mild but critical contractures of the hip flexors on the side of the ‘balancing’ leg, and especially of the ilio-tibia1 band (tensor fascia lata). Less often, these contractures develop symmetrically. Naturally they restrict full hip-extension (as well as adduction) and VIGNOS and ARCHIBALD’ have pointed out that they may thus be the final limiting factor in preventing the boy from walking. At the same time, equinus contractures develop as a

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