404

dilatation of the cerebral ventricles and widening of cortical sulci. These features were visible as early as five days after onset of neurological symptoms (patient 2), became marked over the next few months and then remained stationary.

In two cases areas of white-matter hypodensity were present around the frontal and occipital horns from onset, and persisted. although less marked, to the last examinations.

The initial CT scans for patients 2 and 3 showed mild hypodensity in both lenticular nuclei, which was not visible on later examinations (Fig. I). On all scans, except the initial one for patient 2, a small rounded area of increased density was visible, a fer enhancement, in the posterior half of the left thalamus.

CT scan images in glutaric aciduria are drferent from the punched-out areas of hypodensity seen in acute bilateral striatal necrosis. This is in agreement with pathological reports which indicate neuronal loss and gliosis, without cavitation2.

J. AICARDI F. GOUTIERES

J. M. SAUDUBRAY H. OGIER

Hdpital Necker-Enfants Malades, D epartem ent de PPdiatrie, 149 Rue de Stvres, 75743 Paris Cedex 15.

Recording Motor Defects of Children with Cerebral Palsy

SIR-It is well recognised that terms such as diplegia, hemiplegia and quadriplegia, although useful in clinical discussions, are of limited value in epidemiological studies since different doctors use them to mean dijjierent things‘. The problem of differentiating between some cases of diplegia and quadriplegia is such that some researchers have opted to abandon the use of the word diplegia‘. This may resolve some inconsistencies, but it prevents further investigation of the association of diplegia with low birthweigh t.

method of recording motor defects which We present here for discussion a

Fig. 1. 1. Goodman, S. I., Norenberg, M. D. (1983)

‘Glutaric acidemia as a cause of striatal necrosis in childhood.’ Annals of Neurology, 13,

2. Goodman, S. I.,Norenberg, M. D.,Shikes, R. H., Breslich, D. J., Moe, P. G. (1977) ‘Glutaric aciduria: biochemical and morphologic con- siderations.’ Journal of Pediatrics. 90, 746-750.

3. Goutitres, F., Aicardi, J. (1982) ‘Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children.’ Annals of Neurology, 12, 328-332.

4. Leibel, R. I., Shih, V. E., Goodman, S. I., Bauman, M. L., McCabe, E. R. B., Zwerdling, R. G., Bergman, I., Costello, C. (1980) ‘Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis.’ Neurology

582-583.

(N.Y.), 30, 1163-1168.

has been tested in a small pilot study involving three clinical centres. It has been found to be a useful method of recording information, the most notable exception being the case of a child with truncal ataxia, whose defects had to be described using the section for comments. We would be grateful for any comments on. its use.

m e form used in the pilot study also allowed the recording of mobility, manual dexterity, mental and sensory defects, problems with communications, seizures, congenital and acquired malformations. and genetic and other disorders. At this stage we recommend that at least the presence or absence of the above defects be recorded routinely, where possible giving the results of generally accepted

TYPE OF MOTOR INVOLVEMENT

SPASTICITY Disease involving pyramidal tracts and manifested by an exaggerated stretch reflex, increased tendon jerks and persistently increased tone in some muscle groups.

Cerebral palsy of cerebellar type.

This is a difficult group to describe, but broadly falls into two types, both of which may be found in the same patient.

ATHETOID Inco-ordinate, generalised, purposeless movements (including chore-at hetoid). DYSTONIC ‘Tonus-changing’ syndrome, with patterned postures.

ATAXIA

DYSKINESIA

HYPOTONIA Usually generalised hypotonia.

Coding: O = This type of neuromotor defect is not present.

I = Minor neuromotor signs without functional significance. (This category should be used sparingly: if there is any functional significance use next category.)

2 = Functionally significant but NOT the dominant neuromotor defect in the limb concerned.

3 = Functionally significant and the DOMINANT defect in that limb (if two neuromotor defects are considered equally dominant, code both as 3).

DISTRIBUTION AND SEVERITY OF MOTOR INVOLVEMENT

Coding: 0 = Normal limb: no disability; no neuromotor defect.

1 = Minor impairment: no noticeable disability, but evidence of neuromotor involvement, e.g. increased tendon reflexes in leg but no limp; posturing of arm but no significant functional impairment.

noticeable disability, but only minor loss of funcrion. e.g. abnormal gait or limp.

some purposeful voluntary movement possible.

prevents all or virtually all purposeful voluntary movement.

2 = Mild disability:

3 = Moderate disability:

4 = Severe disability:

HEAD: O= Normal head control

1 = Adequate but abnormal head control

2 = Poor (disabling) or absent head control

Fig. 1. Codes for recording motor defects.

tests (rather than ‘mild, ‘moderate’ or ‘severe’ impairments).

the motor defects on the form compares favourably with that normally taken to record clinical findings in similar detail. nerefore we consider that it is suitable

for use in some ordinary clinical consultations (for example for a periodic review of status) and need not be restricted to research projects. It will also provide a useful method of conveying information when referring patients to other doctors, therapists or special schools.

It appears that the time taken to record

the collection of data. However, it is hoped that eventually agreement can be reached on a common core of data to be recorded in a comparable manner, to allow more reliable pooling of data from different clinicians, and fruitful comparisons between studies.

We gratefully acknowledge the assistance of Dr. A. D. M. Jackson, London Hospital, Dr. D. M. Challis, Thorpe Coombe Children’s Centre, Walthamstow. and Dr. S. Richmond, Princess Mary Maternity Hospital, Newcastle. who participated in the pilot study. We are also grateful to David

d

We realise that different clinicians and researchers will prefer other emphases in

Scrutton, Superintendent Physiotherapist, Newcomen Centre, Guy’s Hospital, for his 405

406

?.KITOR INVOLVF?IENT IN CEREBRAL PALSY

FOK this section please use codes as specified. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . * * * * *

* * * * *

* * *

*

* * *

* * * *

n/pe of rator Involvement

S PAST ICI’rY

ATAXIA

ATIIETOID

DYSTONIC

DYSKINESI

HY POTON I A

L leg

El El r;l El

Distribution and Severity of pator Involvenent

R a m R leo L l s o L arm Head

* * * * * t

* * * * * t

* * * *

* * * * * *

* * *

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

- Pseudobulbar Palsy please tick one hox

Nil signs 0 0 Fl

- Mild dysarthria/dribbling/chewing-swallowinq problems

arked (disabling) dysarthria/dribbling/chewing-swallowing problems

Present clinical picture does not allow definite respnse (uncertain)

CCWIENTS :

Fig. 2. Proposed form, completed for child with athetosis and hypotonia.

helpful comments on the form at various References stages in its evolution, and the Spastics Society f o r support.

PAMELA M. EVANS, M.B., B.S., Research Fellow

EVA ALBERMAN, M.D., Professor of Clinical Epidemiology

l l e London Hospital Medical College, London E l IBB.

1. Alberrnan, E. (1984) ‘Describing the cerebral palsies: methods of classifying and counting.’ and

2. Jarvis, S., Hey, E. (1984) ‘Measuring disability and handicap due to cerebral palsy.’

In Stanley, F., Alberrnan, E. (Eds.) The Epidemiology of the Cerebral Palsies, Clinics in Developmental Medicine No. 87. London: S.I.M.P. with Blackwell Scientific; Philadelphia: Lippincott.