In pediatric practice, delay in starting to walk and difficulty in walking are common problems. These conditions may indicate a systemic disease (such as rickets), mental retardation, or, more commonly, a muscular or neurologic process. Congenital cerebral disease because of periventricular leukomalacia accounts for a majority of cases of infantile diplegia (weakness predominantly of the legs, with minimal weakness of the arms). Present at birth, it becomes manifest in the first months of life and may appear to progress, but actually the progression is only apparent, being exposed as the motor system develops; later there may seem to be slow improvement as a result of the normal maturation processes of childhood. These disorders fall under the heading of cerebral palsy, as discussed in Chap. 38. Congenital malformation or birth injuries of the spinal cord are other possibilities. Friedreich ataxia and familial paraplegia, muscular dystrophy, tumor, and the chronic varieties of polyneuropathy tend to appear later, during childhood and adolescence, and are slowly progressive causes of leg weakness and walking disorder. Transverse (usually demyelinative) myelitis is another cause of acute paraplegia in childhood.
All that has been said about the spinal causes of paraplegia applies to quadriplegia, the lesion being in the cervical rather than the thoracic or lumbar segments of the spinal cord. If the lesion is situated in the low cervical segments and involves the anterior half of the spinal cord, as typified by the syndrome resulting from occlusion of the anterior spinal artery, there is a level on the trunk, below which pinprick and thermal sense is lost but there is retained vibration, deep sensation, and joint position sense (anterior spinal artery syndrome). In all these processes, the paralysis of the arms may be flaccid and areflexic in type and that of the legs, spastic. If there is pain, it is usually in the neck and shoulders and there is numbness of the hands; elements of ataxia from posterior column lesions may accompany the paraparesis. Compression of the C1 and C2 spinal cord segments is caused by dislocation of the odontoid process. Rheumatoid arthritis and Morquio disease are other causes of compression of the upper cervical cord of special note; in the latter, there is pronounced dural thickening.
A progressive syndrome of monoparesis, biparesis, usually of the arms, and then triparesis involving the leg on the side of the last affected arm (“around the clock” pattern) is caused by tumors and a variety of other compressive lesions in the region of the foramen magnum and high cervical cord. This is putatively explained by the pattern of corticospinal fiber decussation at the cervico-medullary junction. Bilateral infarction of the medullary pyramids from occlusion of the vertebral arteries or their anterior spinal branches is a rare cause of quadriplegia. Repeated strokes affecting both hemispheres may lead to bilateral hemiplegia, usually accompanied by pseudobulbar palsy (see Chap. 22 on spastic dysarthria and
Chap. 24 on pseudobulbar laughing and crying). In infants and young children, aside from developmental abnormalities and anoxia of birth, certain metabolic cerebral diseases (metachromatic and other forms of leukoencephalopathy, lipid storage disease) may be responsible for a quadriparesis or quadriplegia, but always with psychomotor compromise.
A rare pattern that may be considered a fragment of quadriplegia occurs in instances of infarction of the motor cortex in the vascular border zones between the middle and anterior cerebral arteries. The syndrome is one of paralysis of the proximal arms and sometimes, legs, colorfully called “man in the barrel.”