[The neuropathology of childhood cerebral hemiplegia].
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21 cases of children with congenital hemiparesis have been studied by computerized tomography (CT) of the head and somatosensory evoked potentials (SEP). Computerized tomography is correlated with clinical features and may disclose cerebral lesions and their physiopathological mechanisms. Somatosensory evoked potentials allow a more functional approach of the handicap but are not perfectly correlated with the motor deficit. SEP are correlated with the topography of cerebral lesions showed by CT. These two investigations seem to be interesting and complementary for the exploration of congenital hemiparesis.
We describe a series of 13 patients, ten males and three females, with ages ranging between three and fourteen years, who show unilateral polymicrogyria (in the left cerebral hemisphere in four cases and in the right in nine). The first and the main clinical alteration in all 13 cases was hemiparesis that did not change along the years. Ten patients had seizures that were completely controlled in seven. Three cases never presented seizures. Spontaneous threat of abortion or arterial hypertension during the first five months of pregnancy occurred in five cases. Weight less than 3 kg at birth was observed in six cases. The only patient who shows severe mental retardation associated with deafness is a girl who had infantile spasms at 20 days of age and presents duplication of a short arm of the chromosome X. Conventional magnetic resonance (MR) studies, performed in four cases did not disclose polymicrogyria but only an enlarged cortex that was diagnosed as cortical dysplasia. Three-dimensional MR (3DMR) images are very important not only to see the polymicrogyria, but also its extension and severity, especially if, moreover the axial, coronal and sagittal views, oblique, frontal, occipital, basal and superior images of the hemispheric cortical surface are performed.
Selected spastic patients with cerebral palsy can be helped by peripheral reconstructive surgery, of the upper limb. Although surgery cannot make a limb that was functionally poor into a perfect one, it can greatly improve the preoperative condition. Most poor surgical results are caused by incorrect selection of patients or poor execution of surgical procedures. The worst mistake is to perform soft tissue procedures--tendinous release or tendinous transfers--on a patient with pure athetosis. The results in these cases are unpredictable and often fail. The same concept is applied to rigidity, hypotonia, dystonia, and ataxia. The surgical program is organized according to the type and severity of the deformity (clinical groups). The goals are to correct the deformities and to improve the muscular balance of the hand in one surgical stage. It must be remembered that spastic muscle cannot be used for tendon transfer with the same efficiency as in patients with a flaccid paralysis. Results were satisfactory in 92% of cases of groups I and II. If group III is included, the percentage of satisfactory results reduces significantly.
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This study investigated the reliability of the modified Tardieu scale in the assessment of biceps spasticity in the upper limbs of children with hemiplegic cerebral palsy (CP). Ten children, with hemiplegic CP participated in the study: six males (mean age 9 years, SD 4 years) and four females (mean age 12 years, SD 3 years). Blinded, duplicate measures of dynamic elbow extension were performed on the hemiplegic arm at time 0 and 7 days later, using the three angular velocities described in the Tardieu scale (V1, slow; V2, speed of gravity; V3, as fast as possible). The resulting elbow joint angles were defined as R1, the angle of catch following a fast velocity stretch at either V2 or V3; and R2, the passive range of movement achieved following a slow velocity stretch at V1. Both elbow joint angle and movement angular velocity were measured by three-dimensional kinematics. Median error in measured elbow joint angle within one session ranged from 3 to 5 degrees. Between sessions median absolute differences in measured elbow joint angle ranged from 4 to 13 degrees, with measurement errors of up to 25 to 30 degrees in some participants at the fastest velocity (V3). The therapist was able to apply three significantly different angular velocities as required for the Tardieu scale (p<0.001). However, the ranges of the three angular velocities overlapped, with fast velocities for some participants being equivalent to slow velocities for other participants. Three out of 10 participants had an intersessional difference in their R2-R1 score of more than 20 degrees. From this study, we concluded that the R2-R1 value determined from the modified Tardieu scale may be of limited value in assessing biceps spasticity the upper limbs in children with hemiplegic CP.
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The chickenpox virus (varicella zoster virus; VZV) is known to cause large and small vessel central nervous system vasculopathies that may be associated with strokes in both adults and children. We present the case of a female aged 2 years 6 months who developed a chronic progressive small-vessel vasculopathy with radiological features of moyamoya disease as a manifestation of congenital varicella syndrome. Clinically, the condition was characterized by recurrent ischaemic strokes, which were brought under control using intravenous acyclovir. The case is unique in that it is the first report of congenital varicella syndrome to occur after a maternal herpes zoster infection. Furthermore, it is the first case of symptomatic VZV infection in a child to occur after a maternal infection occurring in the third trimester of pregnancy.
The electromyographic (EMG) responses to tendon taps at the ankle and ensuing muscular twitch forces and temporal parameters were studied at varying angles across the joint range in 18 children, aged 3 to 14.9 years, with congenital hemiparetic cerebral palsy and 22 healthy (control) children, aged 3 to 13.6 years. Those subjects with hemiparesis were community ambulators without assistance. In all subjects, passive muscle stretch caused a waxing of the reflex EMG and twitch force near neutral (with the sole of the foot at right angles to the tibia) and a diminution of these with further dorsiflexion. Twitch times increased with each dorsiflexing increment, being slowest at maximum dorsiflexion and fastest at the resting plantarflexion angle. Heterogeneity of the hemiparetic-limb data is evident when compared with data of non-paretic and unaffected limbs, with clear differences in the clonic (fast twitch) as opposed to non-clonic (slow twitch) muscles. In four cases with clinical clonus, clonus frequency was reduced by passive dorsiflexion. Plaster immobilization for 1 month produced clonus which was previously absent in one subject, and caused a fast-twitch phenotype to emerge in two subjects. Follow-up after heel-cord lengthening in one subject showed that clonus frequency diminished from 9 to 3 Hz with slowing and strengthening of muscle-twitch phenotype. Short- and long-term peripheral manipulations appear to regulate neuromuscular excitability according to whether muscles are loaded or unloaded. Although damage to the nervous system provides the setting for reflex excitability, the data suggest that the muscle length (which specifies the joint angle) and the muscle-twitch phenotype of any given limb for any given case appear to dictate the actual speed and strength of reflex muscle-twitch and clonus profiles. This study illustrates how peripheral manipulations of muscles and tendons may alter the expression of what have hitherto been considered as exclusively central phenomena.
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The segmented EMG activity in flexor carpi radialis to imposed wrist movements was studied in 18 hemiplegic subjects with vascular lesions of the sensorimotor cortex or internal capsule and compared to that in age-matched normal subjects. The segmented EMG activity in the stretched muscle was normalized against the maximum M response elicited by electrical stimulation of its peripheral nerve in hemiplegic and normal subjects. This M response was used to estimate the maximal activity of the motoneuron pool. Twelve of the hemiplegic patients showed a stereotyped abnormal segmentation pattern of the EMG activity characterized by: an increased M1 segment of prolonged duration with both an increased sensitivity to low initial velocities of displacement and an increased slope of the input-output relationship for the range of velocities tested; absent or diminished activity during the interval of the normal M2-3 segment; and a late component not evident in normal subjects. The increased magnitude of the EMG activity during the M1 interval, markedly exceeded the normal range of control values. A method to demonstrate the response distribution of the interrelationship of the magnitude of the M1 segment using two variables (background EMG activity and initial velocity) for individual responses rather than average responses is presented. The results of the study indicate that: the increased excitability, reflected in the enhanced EMG over the M1 segment does not result from increased levels of resting alpha motoneuron activity and may result from disruption of polysynaptic internuncial influences on alpha motoneurons; the absent or diminished activity over the interval of the normal M2-3 segment may partially result from the interruption of a transcortical reflex by the vascular lesions.
In this paper different models of anosognosia are confronted and data concerning denial behaviors are presented that were collected on a selected population of right brain-damaged patients affected by motor and neglect disorders. Anosognosia for motor impairment and anosognosia for cognitive impairments were found to be dissociated, as well as anosognosia for the upper and lower limb motor impairments. These findings are then discussed in an attempt to choose the more suitable theoretical framework for interpreting the various disorders related to denial of illness.