Clinical and pathological observations on Jamaican neuropathy: a report on 206 cases

The clinical features of 206 cases of a neuropathic syndrome in Jamaica are presented. The dominant feature is a spastic paraplegia. Approximately half of the cases have evidence of associated posterior column damage. In a minority optic atrophy, nerve deafness of selective anterior horn cell damage is found. The patients have been divided arbitrarily into two categories: 25 cases presenting predominantly with sensory ataxia, and in whom there is a high incidence of optic atrophy and eighth nerve deafness, with slight evidence of pyramidal tract damage. This group has a background of poor nutrition. (b) 181 cases presenting predominantly as a spastic myelopathy, and with a relative low incidence of optic atrophy and eight nerve deafness. The findings in ten necropsies from the spastic group are presented with their cases record. The histopathology is that of a chronic meningo-myelitis, with damage to the long tracts as the major lesion. Involvement of the brain, they grey matter of the cord and spinal nerve roots occurs to a lesser extent. An eleventh case is described with similar pathology, which clinically was thought to be an example of neurosyphilis. The relationship of this syndrome to other neuropathies is considered. The aetiology is discussed, with special reference to the possible role of syphilis and yaws in the spastic group. The majority of these cases have positive treponemal tests in the blood, but only 6 per cent have positive tests in the spinal fluid. The pathology has much in common with that of neurosyphilis, but lacks some of the features generally accepted as typical. The incidence is extremely high relative to other known forms of neurosyphilis in Jamaica. Other factors such as ingested toxins and vitamin deficiences may add to, or modify, the metabolic impairment. It is possible to envisage a spectrum of disease varying from a picture of non-inflammatory long tract degeneration to the active meningo-vascular reaction of florid adhesive arachnoiditis-a spectrum in which common causal factors could operate with variable degrees of intensity. This study emphasizes that our understanding of the pathogenesis and pathology of neurosyphilis in its many forms is far from complete (AU)

Observations on the cyanide content and toxicity of tropical pulses

The content of cyanogenetic glucosides in West Indian and other pulses was estimated by the release of hydrocyanic acid on hydrolysis. In the varieties of phaseolus lunatus (lima bean) examined, the CN content was under 20mg. per cent, and not of the order previously known to cause acute poisoning. Trace amounts were found in 5 other species, and a high content was found in a variety of vicia sativa seeds (common vetch). A comparison of hydrolytic procedures in ph. lunatus showed that whereas the cyanogenetic glucoside is stable on cooking the intact bean, neither human saliva nor dilute hydrochloric acid at 37oC was effective in releasing free HCN from beans crushed after cooking. Animal feeding tests of crushed uncooked beans showed that the toxicity of these varieties was unrelated to their CN content. Severe 'toxicity' of ph. vulgaris (kidney bean, red pea) in rats and guinea pigs was mainly, if not entirely, due to unpalatability, causing starvation. Palatability was much improved by cooking. Absorption and utilisation of other species were good when fed to rats, even at 50 per cent level. There was some evidence of pancreatic hypertrophy and of impaired absorption or utilization of vigna sp. (black-eye pea) and cajanus cajan (gungo pea) in guinea pigs. No neurological lesions were detected in rats in feeding tests of up to 6 month's duration (AU)

The relation of oedema to serum protein and psudocholinesterase levels in the malnourished infant

The extent of clinical oedema in 200 Jamaican cases of infantile protein malnutrition has been compared with the levels of serum proteins and serum pseudocholinesterase activity and with the degree of hepatomegaly. Two separate analyses were made, oedema being assessed clinically and by the amount of initial weight loss during recovery. In both analyses there was significant correlation between the degree of oedema and the values of serum albumin and total proteins. Cases with severe oedema and the values of serum albumin and total protein. Cases with severe oedema consistently had low serum proteins, but only about half of the cases with very low proteins had severe oedema. Both hepatomegaly and low serum pseudocholinesterase activity were significantly related to severe oedema. On the other hand there was no evidence of correlation between liver dysfunction and liver size except in the largest livers. Mortality was highest in cases with severe oedema and very low serum proteins. Liver enlargement and very low serum pseudocholinesterase levels were more constant features of fatal cases, with or without oedema. The possible mechanisms of oedema in kwashiorkor, and the significance of the fatty liver, are briefly discussed(SUMMARY)

Urinary radio-iodine excretion in the malnourished infant

Radio-iodine urinary excretion tests were carried out in 12 infants suffering from protein malnutrition. A technique of iodide precipitation was used, involving a dosage of 0.01 æc. I131/kg. Changes in basal metabolic rate were observed in the same infants. In the initial phase of hospital admission radio-iodine excretion was essentially normal. During recovery some cases showed a decrease in excretion. This decrease, however, was unrelated to dramatic increases in oxygen uptake and body weight and tended to lag behind them. It is concluded that thyroid action may facilitate, but in no way initiates, the accelerated respiratory activity during recovery (AU)

Neurological manifestations of magnesium deficiency in infantile gastro-enteritis and malnutrition

Five cases are described of infantile gastroenteritis in which neurological disturbances including tremor, muscle rigidity, twitches and convulsions were associated with low levels of serum magnesium. Three of the cases were frankly malnourished. Serum calcium levels were normal in all, and parental calcium was without therapeutic effect in three cases, whereas magnesium administration in all five cases induced remission of the neurological disorder (AU)

Changes in the basal metabolic rate of the malnourished infant and their relation to body composition

1. The basal metabolic rate (BMR) was studied by a closed-circuit system in 36 infants suffering from and recovering from severe protein malnutrition. In nine cases, concomitant studies were made of total body water by tritium diultion. 2. In most cases, the initial oxygen consumption tended to be subnormal in relation to calculated surface area, but was approximately normal in terms of body weight and body of solid mass. However, owing to an abnormal preponderance of the most metabolically active organs, notably the brain, this finding suggests a true depression of respiratory activity in the individual organs. In a few marasmic cases, the BMR was initially increased both in terms of body weight and surface area. 3. Serial tests showed a dramatic rise in total oxygen consumption, often more than twofold, during the early weeks of recovery. This rise was followed by a plateau level which was maintained regardless of variable gains in weight. Total oxygen consumption at this plateau level approached the normal for a healthy child of the same age. 4. Body weight did not increase unless the caloric intake consistently exceeded the BMR by 60 to 85 calories per kg per day. The greater the weight deficit, the higher was the BMR during recovery and the higher the caloric requirement for weight gain. 5. It is suggested that the limit of oxygen uptake in recovery may be determined partly through restoration of normal activity in the organs which are least reduced in relation to age and partly through the factor of the mean "metabolic potential" of the individual cell. Cell number per unit of tissue mass is increased in the active tissues of the malnourished growing animal. The mean respiratory activity per cell is therefore much reduced initially, but may approach the normal during recovery (SUMMARY)

Multiple sclerosis in Jamaica

Four cases of clinical multiple scelerosis are described in indegenous Jamaicans, three of whom had never left the Island. Three were in the higher social grades and two were Negro. The course of the disease resembled that in temperate countries. A survey of other neurological cases among 10,000 in-patients and 1,500 autopsies reveal no evidence of syndromes which could be attributed to multiple sclerosis in an unusual form. It is concluded that multiple sclerosis in indigenous Jamaicans is extremely rare, but shows no unusual features

Magnesium balance studies in marasmic kwashiorkor

Studies of magnesium balance were made on 3 Jamaican infants during recovery from marasmic kwashiorkor. Analysis of muscle biopsy specimens indicated a marked deficit of magnesium as well as potassium in all cases. The serum magnesium level was slightly low in one case. A positive magnesium balance was maintained for several weeks in recovery and was accompanied by low urinary excretion. In one case there was considerable intestinal absorption of magnesium, even in the presence of diarrhea. Efficient absorption of a high oral suppliment tended to overcome renal conservation. Previous analysis has suggested that the deficiency of intracellular electrolytes in wet muscle samples is due partly to the reduced intracellular volume associated with protein loss and partly to a true electrolyte deficit. The positive magnesium balance in recovery is therefore attributable both to intracellular repletion and to tissue growth; the latter tends to be masked clinically by loss of excess body water. Little is known of the additional factor of the repletion of magnesium in bone, but the evidence from animal experiments is that the deficit in bone may be very substantial (AU)

The cause of death in tetanus

Clinical observation of the mode of death in 25 fatal cases of tetanus offers evidence of brain-stem intoxication, manifest particularly in the features of hyperprexia, recurrent paralytic apnoea and sudden cardiac arrest. These features bear no direct relation to spinal reflex hyperexcitability. Recovery from apnoea is possible, and two examples are cited. Histological evidence of central intoxication is furnished by the finding of neutral lesions in the dorsal vagal nuclei in two neonatal cases with severe and repeated respiratory failure. Detailed examination of the central nervous system in 9 cases revealed no other specific changes. Chest complications in this series were of secondary significance, and this was attributed in part to a policy of light sedation. Incidential autopsy findings were examples of acute muscle degeneration. Some aspects of the pathology and cause of death in tetanus are discussed in the light of these observations. (AU)

The estimation of magnesium in small biological samples by flame spectrophotometry

Studies of human magnesium in this laboratory called for a simple method of estimating the magnesium comtent of large numbers of small biological specimens (Montgomery, 1960). It has been found that a technique involving the Unicam S.P. 900 flame spectrophtometer fulfils this need. Estimations have been made on serum, red cells, urine, and acid digests of muscles and faeces. A standard instrument was used, burning acetylene and air, with a new standard atomiser containing a metal jet. Readings were made at the atomic line of 285.2mu (AU)

The relation of the treponematoses to idiopathic neuropathies in Jamaica

An approach is made to the aetiology of an idiopathic paraplegic syndrome in Jamaica. The main features comprise upper motor neurone paresis and sensory loss of posterior column type, with optic atrophy, nerve deafness and selective peripheral nerve lesions in a minority. Symptoms arise most commonly in the fourth decade, and show little change after the first few months. Serology is negative in the CSF in all cases and in the blood of the majority. Nevertheless some evidence of past treponemal infection can be found in over 50 percent. Abnormality of the CSF, including a positive colloidal gold reaction, is found in one third of cases; and special study of this group shows that treponemal infection has occurred in 80 percent and also that the history of illness is significantly shorter than in the remainder. Comparison of the two groups suggests that they may be progressive stages of the same syndrome. Autopsy studies reveal meningovascular and degenerative processes which are not typical of syphilis. Analysis of the geography of the condition reveals a concentration in certain inland rural areas - less than 20 percent occurring in inhabitants of Kingston. The detailed distribution closely follows that of yaws 20 years ago. The evidence is discussed that this syndrome - and perhaps some cases of frank spinal arachnoiditis - may be late manifestations of treponemal infection, whether veneral or otherwise, due to a strain variant acting as a poor stimulus to standard tests, but producing atypical meningovascular disease (Summary)

Basal metabolic rate and thyroid activity in the malnourished infant (abstract only)

A closed-circuit method is described for the estimation of B.M.R. in infants. Serial studies were made on 30 severely malnourished infants at intervals during recovery . In some of these cases estimates of total body water were also made by use of tritiated water, and oxygen uptake in contrast to high levels in marasmus, but in no case was the activity subnormal when related to body solids. During recovery, total oxygen uptake increased dramatically, sometimes threefold, reaching a constant plateau level which was maintained regardless of the changes in weight. This level appeared to be determined by the age of the child rather than its height - i.e. it approached the average for a normally built child of the same age. Hormonal influence has been studied by the use of a micro-technique measurement of radioiodine excretion. It is evident that the peak period of growth and of oxygen uptake is associated with thyroid hyperactivity - indicating a state of "anabolic hyperthyroidism". These phenomena pose wide questions in the fields of normal growth and energy requirement, both in the whole body and at cellular level (AU)

Magnesium metabolism in the malnourished infant (abstract only)

Experimental depletion of magnesium in animals has been shown to induce a parallel, and even greater, depletion of potassium in the body tissues. It has been suggested that this might be due to interference with magnesium-dependent enzymes required for the maintenance of normal potassium balance within the cell. These observations have not hitherto had any direct application in natural human states. This paper describes the results of analysis of the electrolyte content of muscle biopsy specimens in 20 infants with severe protein malnutrition before and after recovery, and in 12 fatal cases. Magnesium was estimated by a new technique of flame spectrophotometry. A gross depletion of muscle magnesium was demonstrated, which closely paralleled that of potassium. These changes were not reflected in the magnesium content of serum or red cells. Estimations of seventy 24hr. urine specimens in recovering infants showed an avid magnesium retention prolonged over many weeks, in contrast to the brief conservation of potassium and phosphorous. The contents of both magnesium and potassium in the muscle of clinically recovered infants were still markedly below normal levels. The significance and possible applications of these findings are discussed (AU)

Albers-Schonberg's disease: a changing concept

1. A type of bony sclerosis is described, occurring in nine members of a Jamaican family and resembling the more benign form of Albers-Schonberg's disease. The parents were consanguinous. Three of the patients facial palsy at the same age, and one had bilateral optic atrophy and proptosis. 2. Although radiological changes occurred of all grades of severity, certain features often described in this condition were lacking. In one child the onset of radiological changes was observed at the age of eleven years. 3. Serum studies showed increased alkaline phosphatase activity. 4. These features are discussed in the light of present-day knowledge and theory of the pathology of Albers-Schonberg's disease (SUMMARY)