172 Postgrad Med J: first published as 10.1136/pgmj.26.293.172 on 1 March 1950. Downloaded from SPASTIC PARAPLEGIA IN MIDDLE AGE By COLIN EDWARDS, M.R.C.P. West End Hospitalfor Nervous Diseases Before launching into this discussion one must of early onset surviving into middle age will cer- pause to define middle age. Obviously it is not tainly have had a remission and there may well the age period which would have been indicated have been subsequent exacerbations and re- 50 years ago, nor is it the actual middle of the missions. Transient visual disturbances-dim- average span of life. Its limits are set by con- ness due to a retrobulbar neuritis or diplopia due vention and they are not clear-cut, but it does not to external ocular palsies-are common in the begin before 40 years of age and nowadays one initial attack and less so in exacerbations. And can hardly say that it ends before 65. some patients are aware of their nystagmus which Paraplegia, with its serious restriction on may often be transient also. Bladder disturbances mobility, is more dramatic and striking than -precipitancy or frequency-are also common paralysis of the arms, and is apt to give its name to early symptoms. Later comes retention and diseases which are more than paraplegic in their usually, last of all, incontinence. effects. Hence a number of the conditions men- The motor symptoms of disseminated sclerosis, tioned will be essentially quadriplegic and more. however, are the commonest, and particularlyProtected by copyright. Spasticity, too, although a term which connotes those involving the lower limbs. Heaviness or essentially pyramidal involvement, with its clasp- weakness of the legs after exertion is the most knife type of muscular hypertonia, can occasionally usual and it is nearly always more marked on one bear superficial resemblances to extra pyramidal side. Where there is associated cerebellar dis- rigidity and so some extra pyrarpidal diseases will turbance and the disease has not begun until the have to be noted. fifth, sixth or early part of the seventh decade, one So much for the terms of reference. has to consider the possibility of one of the cere- Consideration of a clinico-pathological state can bellar degenerations of middle life-notably de- be approached with one's own idea of disease layed cortical cerebellar atrophy, and olivo-ponto- classification as a background pattern. This will cerebellar atrophy, both of which show bilateral ensure that one is comprehensive and is the safest pyramidal involvement. In them, however, the method to follow if under pressure for time in an curve of development of the disease is continuous examination. Viewing one's middle-aged spastic and progressive, nor are ocular manifestations http://pmj.bmj.com/ patient as a problem in diagnosis, however, one common-except nystagmus. would inevitably tend to consider the diagnosis to Rarely Friedrich's ataxia may show a pre- some extent in terms of probability, and the com- dominance of lateral column degeneration over the moner causes would come first to mind. That is posterior, and the legs may be spastic despite how I propose to discuss paraplegia now. absence of tendon reflexes. The familial incidence, As a final prefatory remark one must stress the the foot and spinal deformities, and the lengthy primary importance in neurology of history taking. history without remissions in spite of a varying The clinical signs of neurological disease are so rate of deterioration should differentiate these on September 26, 2021 by guest. spectacular that one can understand why students occasional cases from disseminated sclerosis. are unduly impressed by them-but in no branch Their early onset will serve to distinguish them of medicine is history taking more important, and from the cerebellar atrophies mentioned above. it is certainly the major part of the task of diagnosis No laboratory confirmation of the diagnosis of in our middle-aged paraplegic. disseminated sclerosis is possible, but about half Disseminated sclerosis must rank as one of the of the cases on whose cerebrospinal fluid a sen- commonest causes of spastic paraplegia and sitive Lange test is done show a curve of ' paretic ' although four-fifths of the cases begin before shape or tending towards it. This is combined in middle age, it is some of these four-fifths, as well about one-quarter to one-third of all cases with a as the cases beginning in the fifth and sixth de- slight increase in globulin in the fluid and a cades, that make up many of the ranks of the raised cell count. This combination goes a long middle-aged spastics. The history will usually way towards establishing the diagnosis. point fairly definitely to the diagnosis. Any case Of other paraplegias of inflammatory origin at March 1950 EDWARDS: Spastic Paraplegia in Middle Age Postgrad Med J: first published as 10.1136/pgmj.26.293.172 on 1 March 1950. Downloaded from this time of life those due to syphilis have per- It produces upgoing toes and increased tendon re- haps been the commonest, although they are flexes but spasticity and paralysis do not become already ceasing to be so. They most commonly marked. Sensory disturbance is slight but the make their appearance late in the first half of life, bladder may sometimes be involved, and there are and if they do appear for the first time in middle occasionally lightning pains. Argyll Robertson life they will be seen in its early part and not pupils are occasionally found. towards its end. Syphilis causes paraplegia most It is difficult clinically to separate Erb's syphilitic commonly by spinal meningo-vascular inflamma- spinal paralysis from syphilitic lateral sclerosis, tion, but also occasionally by setting up a par- and indeed they may well be the same condition, enchymatous degeneration. The lesion in but at times signs of lateral sclerosis with its spastic meningo-vascilar cases is usually a meningo- paresis are associated with those of posterior mvelitis. The meningitis gives rise to pain of root column involvement (loss of some, at least, of the origin. It occurs most commonly in the dorsal tendon reflexes, ataxia, and loss of deep sensation). region and it may occasionally irritate the anterior The condition is then named syphilitic combined roots too, causing muscular cramps or spasm. or postero-lateral sclerosis and is essentially a The meningitis spreads along the pial septa into parenchymatous degeneration. With it may some- the cord and its progress and resolution produce times be associated muscular atrophy due to tract involvement. This process may take from a anterior horn cell degeneration. few days up to many months. Bladder dis- Gumma of the cord is not common. It belongs turbance is frequent and early. Spastic paralysis to the meningo-vascular group of spinal syphilitic is often the result but quite frequently is not disorders and is often associated with some actual markedly bilateral. A bedside indication of the meningitis (both lepto and pachymeningitis), and syphilitic nature of the spinal lesion is the tendency with arteritis. It produces signs and symptoms of to patchy, cutaneous, sensory impairment, par- an intramedullary tumour but the spasticity re- Protected by copyright. ticularly of pain sensation as shown by pin prick. ported has been most usually unilateral with So it is that the victim of syphilitic meningo- contralateral sensory disturbance-the Brown- myelitis will sometimes show bedsores of sur- Sequard syndrome. prising size in an area where tactile sensation is Finally, and rarely, the lower limbs may share almost normal. Where a severe transverse lesion in the general rigidity of a Parkinsonian picture of of the cord supervenes suddenly in a case with syphilitic origin, due to a parenchymatous de- other neurosyphilitic features (e.g., root pains, im- generation in the striatal area, the subthalamic and paired deep and pain sensibility and possibly tegmental mesencephalic region. These rare cases pupillary changes and diminished tendon re- usually show associated tabes dorsalis, general flexes) the cause is usually the thrombosing of a paresis, or tabo-paresis. spinal artery due to endarteritis. More rare types Whenever neurosyphilis is suspected as a cause of syphilitic spastic paraplegia are those resulting of spastic paraplegia cerebrospinal fluid analysis from hypertrophic pachymeningitis, Erb's syphilitic with Wassermann tests, and also a blood Wasser- http://pmj.bmj.com/ spinal paralysis, gumma of the cord and syphilitic mann reaction, must always be done. lateral and combined sclerosis. In meningo-vascular cases the fluid most often Syphilitic hypertrophic pachymeningitis is com- shows a high cell count, usually of 30 to 200, monest in the lower cervical region. The great principally lymphocytic, although in gumma it may dural thickening causes initially signs and symp- be low, and in pachymeningitis it may be almost toms due to root compression-namely pain, normal. paraesthesiae, muscular atrophy and impaired The cell count in spinal parenchymatous neuro- cutaneous sensation of nerve root origin. As syphilis is usually only slightly raised. The on September 26, 2021 by guest. hypertrophy goes on the cord becomes compressed Wassermann reaction may be negative in the fluid with the production first of signs of pyramidal in meningo-vascular cases but is positive in the compression (extensor plantar response and in- majority. It is more frequently positive still in creased tendon reflexes) and later of actual slowly parenchymatous cases. It should be remembered increasing spastic paraplegia and increasing sen- that a negative blood Wassermann reaction in sory loss up to the level of the lesion. association with a positive Wassermann Reaction Erb's syphilitic spinal paralysis has never re- in the cerebrospinal fluid has been estimated to ceived widespread acceptance, at least as a patho- occur in as many as 30 per cent.