Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis
J. L. Gastaut, B. Michel, D. Figarella-Branger and H. Somma-Mauvais
Service de Neurologie, Hopital Ste Marguerite, Marseilles, France.
Although it was first described over a century ago (by Charcot in 1865; by
Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not
universally accepted. Despite this skepticism, several well-documented
cases of isolated degeneration with varying degrees of involvement of
corticospinal pyramidal pathways have been reported in the literature. The
clinical manifestations in these cases can take one of two forms, ie,
isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic
tetraplegia associated with a pseudobulbar syndrome featuring severe
spastic dysarthria (chronic progressive bilateral spinobulbar spasticity)
on the other hand. Obviously, without firm pathologic data, PLS is a
hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for
bilateral spinobulbar spasticity, it would appear to be the only diagnosis
possible once investigate findings have eliminated the other possibilities,
such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form
of multiple sclerosis. To underscore this point, in this report, five cases
of chronic progressive bilateral spinobulbar spasticity developed over 5,
10, 12, 10, and 28 years, respectively, for which the only possible
diagnosis was PLS. It was concluded that there are three forms of
degenerative diseases of the principal motor pathways: one involving both
central and peripheral neurons, ie, amyotrophic lateral sclerosis; one
involving only peripheral neurons, ie, spinal amyotrophy; and one involving
only central motor neurons, ie, PLS.
