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|SPECIAL NI FEATURE: PEARLS FROM PAST - “SOUTH INDIAN PARAPLEGIA” FIRST PUBLISHED IN NEUROLOGY INDIA VOL 14 (1), JANUARY-MARCH, 1966
|Year : 2020 | Volume
| Issue : 2 | Page : 264-267
A Spastic Paraplegic Syndrome of Obscure Etiology
KS Mani1, BD Punekar1, KT Jagannatha Rao2, D Sreedharan Nair3
1 All India Institute of Mental Health, Bangalore, India
2 Mental Hospital, Bangalore, India
3 Tutor in Psychiatry, Medical College, Kottayam, Kerala, India
|Date of Web Publication||15-May-2020|
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mani K S, Punekar B D, Jagannatha Rao K T, Nair D S. A Spastic Paraplegic Syndrome of Obscure Etiology. Neurol India 2020;68:264-7
Following the publication of a series of papers on' Jamaican Neuropathy' by Cruickshank,, spastic paraplegia of obscure etiology, as seen in South India, has aroused our interest. During a 21-month-period, from March 1963 to November 1964, 80 cases of spastic paraplegia have been examined in the Department of Neurology, All India Institute of Mental Health, Bangalore. [Table 1] shows the various diagnostic categories. It can be seen that 18 cases, designated provisionally as' South Indian Paraplegia' by us, form the largest group, accounting for 22.5 per cent of all cases, followed next by obstructive arachnoiditis, which, incidentally, was the commonest cause of cord compression (36.1%) in this series. An attempt is made in this paper to describe the clinical features of South Indian Paraplegia. Similar cases have previously been reported from General Hospital, Madras, by Minchin and Gopalan.
|Table 1: Analysis of 80 cases of paraplegia seen from march 1963 to november 1964 - (21/12 period)|
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This illness, the cause of which is yet unknown, occurs in both sexes and more frequently in young adults, though children and elderly people are not entirely immune. In [Table 2], [Table 3], [Table 4], [Table 5] are given data regarding sex and age distribution, age at onset, and duration of symptoms.
The past histories of these patients have not been significant. An assessment of their economic status showed that 9 belonged to the poor class, 6 to the lower middle class, 2 to the middle class and 1 to the upper middle class. The socio-economic status did not differ from that of a control, group of paraplegics. Out of 18 cases in this group 9 were strict vegetarians, as against 15 out of 37 amongst the control paraplegias. Not a single patient in this series had consumed Lathyrus sativus.
The patients presented with difficulty in walking, often associated with numbness, tingling and/or burning sensations involving, particularly, the feet and/or hands. Many patients complained of pulling pain in both lower limbs, while jerky movements of lower limbs, particularly worse at nights, were often a distressing feature. There was some disturbance of bladder function, either precipitancy or hesitancy, in about three-quarter of the cases. One patient exhibited disturbed behaviour and another had impaired visual acuity. A history of remissions and relapses was conspicuous by its absence.
General examination was not remarkable in any of these cases. An interesting feature, as mentioned by Gopalan, was the presence of a dry and mosaic type of dermatoses over the anterolateral aspect of the legs in 4 out of these 18 cases, but this was also seen, not infrequently, in patients with other neurological disorders. The usual manifestations of marked dietary deficiency were conspicuous by their absence.
Neurological signs [Table 7]
All patients had varying grades of pyramidal deficit involving the lower limbs. A similar but milder disturbance, was seen in the upper limbs in about two-thirds of the cases. Only two patients showed a doubtful segmental level of sensory deficit affecting the modalities of pain and temperature. A mild to moderate degree of stocking and/or glove type of hypaesthesia, hypalgesia and thermo-hypaesthesia, with or without tenderness of the calf muscles, was obtainable in two-thirds of the patients. Half the series showed a mild to moderate distal hypotonia, wasting of the muscles or diminution of the deep reflexes. There was a diminution, but seldom loss, of proprioception, predominantly distal, in the lower limbs in 8 (44.4%) subjects and upper limbs in 3 (16.6%) subjects. A similar deficit in vibration sense, of similar distribution, was noted in the lower limbs in 9 (50%) and upper limbs in 5 (27.8%) cases. Recording of residual urine indicated, in just less than half the cases, objective evidence of bladder involvement; it was seldom a large, atonic bladder. One patient showed a mild but definite optic atrophy of primary type, while another was depressed and negativistic. It is worth emphasizing that 15 (83.3%) patients were still able to walk, though slowly and with support. 2 patients could just manage to stand with support and it was one of these two who was depressed and negativistic. Only one patient was completely bed-ridden and he had a fairly severe associated peripheral neuropathy.
All patients had routine blood counts with no significant findings. The blood V.D.R.L. (Venereal Diseases Research Laboratory) test for syphilis was negative in all cases. Tubeless gastric analysis with Diagnex Blue© was performed on all patients. Four had achlorhydria of which 2 were histamine fast, but these were statistically not different when compared to the findings in an age-sex matched control group. Spinal fluid examination showed a mild pleocytosis in 2 cases (maximum 100 cells per cubic milliliter), while in 11 instances there was a slight to moderate increase in the CSF proteins (maximum 150 mg. per 100 ml.). The serological test for syphilis was negative in the spinal fluid in all cases. The spinal fluid sediment was examined for fungi in every case, but with negative results. Radiological studies of the spine, including myelography, were performed in all instances with negative findings.
We have estimated the serum proteins, vitamin C. nicotinic acid and Vitamin Bl2 levels in the blood, along with two sets of controls, one from normal individuals and a second consisting of a heterogeneous collection of paraplegia due to definitive but varicd etiologies [Table 8] and [Table 9]. Results to date have not shown any statistically significant difference between the three groups, except that the serum vitamin B12 levels were significantly lower in BOTH groups of paraplegia compared to normal controls [vide [Table 9].
In the absence of autopsy studies it may be speculated that there is a secondary demyelination in the lateral columns with, possibly, a minimal involvement of the posterior columns. The presence of peripheral neuropathy may really be a manifestation of affection of either the posterior root ganglia or the root entry zones in the spinal cord.
Treatment and follow-up
Of the 18 cases, 14 (77.8%) have been available for follow-up so far. Among them none have recovered, but so far there has been no death, thereby precluding any possibility of further studies. There was slight to moderate improvement in 2 patients, very slight improvement in 7, no change in 7 and 2 were slightly worse than before. 16 cases were treated with betamethasone by mouth, while 14 patients had vitamin B Complex by mouth and vitamin B1 and B12, parenterally. While under betamethasone, patients tended to show a slight initial improvement, in that, spasticity became less, but at the end of the therapy, this regressed. With vitamin therapy, 3 patients showed a slight improvement, but in 9 there was no change.
| » Discussion|| |
The etiology of this condition remains obscure. On available clinical evidence multiple sclerosis is unlikely, with the exception of one case out of these 80 patients. Pathological studies may show a primary demyelinating disease, but this appears to be unlikely. The clinical picture of a fairly common occurrence in young adults, women as well as men, an insidious onset, the presence of peripheral neuropathy and absence of dietetic history would appear to exclude lathyrism, the features of which have been well described by Shourie, Ganapathy and Dwivedi, Gopalan, Saha, et al. and Chaudhri, et al. None of our patients gave a history of having consumed Lathyrus sativiıs. There have been reports from time to time in India of what has been referred to as non-lathyrus lathyrism (Shah), particularly in association with the contamination of foods, with the black vetch Vicia sativa. This occurs in sharply localized epidemics, but its relationship to classical lathyrism, or the syndrome under discussion, is not clear. It is interesting that Minchin and Gopalan considered that their cases from Madras were really examples of non-lathyrus lathyrism. The role of other toxic factors in the diet requires a very careful study. So far clinical and ancillary findings do not suggest treponematosis as in the case of 'Jamaican Neuropathy. Further studies are clearly indicated to rule out a luetic etiology and also to determine the relationship of this syndrome to “Jamaican Neuropathy”.
It is tempting to ascribe this syndrome to nutritional deficiency. But there has been no evidence so far — clinical or biochemical — that these patients suffer from any malnutrition. Moreover, a predominant involvement of the pyramidal tracts is very rare in nutritional neuropathies.,, The lowered levels of serum B12 occurred in both groups of paraplegia and may be an effect rather than a cause of the paraplegia. However, this requires a more careful study in a larger series of cases.
Finally the role of heavy metal intoxication requires careful study. They can gain entry into the body from several sources, for example, as part of treatment devised by certain systems of indigenous medicine.
| » Summary|| |
A clinical analysis of 18 cases of spastic paraplegia of obscure origin, termed tentatively 'South Indian Paraplegia', is given. These cases constitute the commonest cause of paraplegia in our series. Generally of insidious onset, and often in young adults of both sexes, this illness tends to cause a moderate pyramidal dysfunction, mainly in the lower limbs and to some extent in the upper also. Bladder involvement is usually present at the time of admission, but is seldom severe. Nearly two-thirds of the cases exhibit a moderate degree of predominantly sensory distal polyneuropathy. Results of investigations to date, including certain blood vitamin estimations, have not been rewarding. No patient has died in the limited period of observation till now. The possible etiological factors have been discussed. More work on a larger number of cases and over an extended period of observation, is clearly necessary.
lt is a pleasure to acknowledge with gratitude the help of Sri T. R. Raghunatha Rao and Mr. Ramaswamy for the haematological, and biochemical studies. We are greatly indebted to Prof. P. S. Sharma and his colleagues, Dr. D. S. R. Sarma and Mr. E. G. Srinath of the Department of Biochemistry, Indian Institute of Science, Bangalore, for the estimation of nicotinic acid and B12 assays. We are grateful to Sri N. Sundararaj for his invaluable help in the statistical analysis of the data and Sri K. H. Radhakrishna for his secretarial assistance. We owe a deep debt of gratitude to Glaxo Laboratories (India) Private Ltd., Bombay, Dr. T. K. R. Menon of Anglo-French Drug Co., Ltd., Bombay and Dr. H. C. Barbhaiya, Assistant Medical Director, Sarabhai Chemicals, Ahmedabad, for their generous supplies of Betametha-sone, Beplex-Forte® and vitamin B1 and B12 injections, and Diagnex Blue® respectively. We wish to place on record our sincere thanks to the numerous physicians and surgeons who referred these cases to us. Finally we are grateful to the Director, All India Institute of Mental Health, Bangalore, for his help in this work and permission to report our findings.
| » References|| |
Idem. A Neuropathic Syndrome of Uncertain Origin. W Indian Med J 1956;5:147.
Idem. Neurological Disorders in the Tropics, in Modern Trends in Neurology, III Series, Ed. Williams D. Butterworths; London 1962, p. 200-226.
Minchin RLH. Primary Lateral Sclerosis of South India, Lathyrism without Lathyrus. Brit Med J 1940;1:253.
Gopalan C. The Lathyrism Syndrome. Trans Roy Soc Trop Med Hygiene 1950;44:333.
Shourie KL. An Outbreak of Lathyrism in Central India. Indian J Med Res 1945;33:239.
Ganapathy KT, Dwived MP. Studies on Clinical Epidemiology of Lathyrism. Report of Indian Council'of Medical Research, Delhi, 1961.
Idem. Lathyrism – A Preventable Paralysis Report of National Research Laboratory. Hyderabad (India), 1964.
Saha TK, Chatterjea SB, Chaudhri RN. Lathyrism in a Rural Area of West Bengal. Bull Calcutta Sch Trop Med 1960;8:98.
Chaudhri RN, Saha TK, Mitra P. Lathyrism: Clinical Features, Bull. Calcutta Sch Trop Med 1962;10:155.
Shah SRA. A Note on Some Cares of Lathyrism in a Punjab Village. Indian Med Gaz 1939;74:385.
Montgomery RD, Cruickshank EK, Robertson WB, McMENEMEY WH. Clinical and Pathological Observations of Jamaican. Neuropathy — A Report on 206 Cases. Byain 1964;87:425.
Clarke CA, Sneddon IB. Nutritional Neuropathy in Prisoners-of-War and Internees From Hong-Kong. Lancet 1946;1:734.
Cruickshank EK. Dietary Neuropathies. Vitamins and Hormones 1952;10:1.
Spillane JD, Scott GI. Obscure Neuropathy in the Middle East, Report on 112 Cases in Prisoners of War. Lancet 1945;2;261.
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8], [Table 9]