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Paralytic Diseases of Childhood in the Tropics by NOEL GUILLOZET, M.D., F.A.A.P. Associate Professor of Pediatrics, The University Centrefor the Health Sciences, University of Cameroon, Yaounde, Cameroon Lecturer on Pediatrics, Harvard Medical School, Lecturer on Child Health, School of Public Health, Harvard University Director, The Harvard University Project, U.C.H.S.

Abstract Paralytic diseases of childhood in the western world are increasingly limited to those emanating from birth defects, trauma and familial disease. In tropical Africa these are minor causes of handicap in the face of severe and endemic poliomyelitis, bacterial meningitis and measles. The neurological complications of sickle cell disease, parasitic infestation, dietary toxins and exotic acute illnesses rarely seen in the United States produce many incapacitating handicaps for which both adequate care and rehabilitative facilities are usually lacking. Introduction The frequency of childhood paralytic disease in tropical Africa is distressingly high. The difficulty in obtaining reliable statistics in the developing world is well known but the observant eye notes children and adults with severe neurological and locomotor stigmata in villages and cities throughout the subSaharan nations. A 1975 study in Ghana disclosed that 7.2 of 1,000 school children examined showed evidence of paralytic polio,1 a contrast with the United States with 14 reported cases of paralytic poliomyelitis for the entire year of 1973.2 The social consequences of survival from paralytic diseases in developing nations are often catastrophic. The ability to compete for an education and position are greatly compromised if indeed they remain options at all. In nations where rapid population growth and urbanization already pose great competition for jobs, the handicapped stand little chance. Begging in urban areas is the usual occupational solution for those who are intelligent and strong enough to be outside the home. Rehabilitation facilities when available at all are rarely able to cope with the numbers of seekers of service. Further, the lack of a tradition of medical and social rehabilitation may render the limited successes attainable insufficient to attract parents to sustain the often difficult and expensive task. In a village world where two strong legs are a necessity for transport as well as work, the man or woman who cannot walk is less than a complete being. This communication addresses the diseases and factors provoking severe locomotor handicaps of 294

children in tropical Africa and which generally typify other tropical areas of heavy rainfall, low environmental sanitation, and limited resources. Poliomyelitis Poliomyelitis dominates the paralytic diseases of childhood in tropical Western Africa. The observant eye notes the myriad clinical variations in sequelae on the streets of major cities among the adults and children who pass. Poliomyelitis in Cameroon is a disease of the very young and diagnosis of the acute illness is rarely made in other than infants and young children. More than 90% of children surveyed in Yaounde, Cameroon, demonstrated antibodies against types I, II and III at four years of age.3 This corresponds with clinical observations at Central Hospital, Yaounde that most entrants with acute poliomyelitis are between 8 and 20 months of age. Children of European origin who have been brought to Cameroon occasionally contract the disease at comparatively advanced ages, reflecting the experience in United States in the 30's and 46's when older children and adults were not infrequent victims of severe paralytic disease. Poliomyelitis in sub-Saharan Africa is an endemic disease which, in Cameroon has a distribution favoring the wetter, forested areas of the nation but which is also seen in the dryer sub-Saharan parts of the North. Studies performed in Cameroon by Boche, et al., showed a seasonal pattern of increased number of paralytic polio from January to June, months with rainfall greater in the area of study than during the drier periods of the year. Their study of 524 ill children followed in a local maternal and child health program demonstrated the presence of enteroviruses in 30-60% of children cultured. From 4 to 15.5% were polioviruses. A parallel in the number of paralytic polio cases with the peak of poliovirus carriage was observed.3 It is evident that the suboptimal level of domestic sanitation favours the rapid and early dispersion of poliovirus and other enteroviruses among children, paralleled by the high incidence of gastroenteritis and diarrheal disease in babies and young children. In urban Cameroon the infant ill enough to be hospitalized will usually have been injected three or more times in one or another buttock during the Environmental Child Health, December 1976

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TABLE I Localization of paralysis for 190 referred consecutive cases of polio— 1973-1975 seen at the Center for the Rehabilitation of the Handicapped, Yaounde, Cameroon

No. cases Single lesion

94

1

2

1

54

36

Multiple lesion

96

43

20

27

91

88

12

Total cases

190

44

22

28

145

124

12

(23.2%)

(11.6%)

(14.7%)

(76.3%)

(65.3%)

(6.3%)

Percentage of total cases displaying lesions

Upper Extremity R L

Lower Extremity R L

Thoracic Respiratory Musculature

Truncal Musculature

Courtesy of Dr. Bernard Guyer, OCEAC, Yaounde, Cameroon.

previous week and if one limb becomes weak, the injection will inyariably be credited as the cause. The apparently tropic effect of a site chosen for injection of other than polio vaccines for the subsequently paralyzed limb has been both anecdotal and confirmed, but inadequately elucidated. Single lower limb paralysis is the most common single observation and upper limb paralysis the least common single lesion. The incidence of multiple sequelae is impressive in the occasional institution receiving large numbers of referred patients, such as the Center for the Rehabilitation of the Handicapped in Cameroon. The high incidence of truncal muscle involvement seriously complicates rehabilitative efforts for many victims. (Table I.) Pharyngeai and respiratory paralysis are not uncommon. A febrile illness with respiratory symptoms and frequently diarrhea is complicated by

1. Poliomye.lds: severe trunca, weakness may precluce rehabiitadon for ambiJuUcn and limit the cm'ld ;o a cnair-bound life. Environmental Child Health, December 1976

limb pain and weakness. Respiration may be hindered, the cough inadequate and pneumonia may be noted. Prolonged artificial respiratory support is usually impossible for severe paralysis and death ensues. A fairly common post acute clinical presentation is the infant with generalized weakness of all extremities, lower limb, weakness dominating, and truncal weakness. Primary myopathies may be suspected, but rapid onset and non-progression distinguish them from poliomyelitis. Lower limb paralysis is occasionally accompanied by sphincter paralysis of the bladder requiring catheterization, a complication that invariably remits after a few days.4 Persistence of sphincteric malfunction should prompt consideration of a diagnosis other than poliomyelitis.

Rehabilitation Dilemmas in Tropical Poliomyelitis Huckstep has estimated the presence of over one million children with untreated severe residual paralysis in Africa alone.3 Since as a general observation 90% or more of all acute cases occur under the age of five years, the need for early rehabilitation is evident. There is usually little or no tradition of such care. When it can be given, great efforts must be made to assure that the often enormous task of straightening a twisted child not be followed by neglect or abandonment by non-comprehending or disinterested parents. Rehabilitation for this enormously frequent and devastating disease poses no feasible alternate to aggressive TOPV vaccination program in areas of endemic poliomyelitis. Barriers must be overcome to their effective organization in areas of endemicity in tropical Africa and elsewhere. 295

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Meningitis It would be impossible to over-estimate the importance of bacterial meningitis as a cause of paralysis and neurologic handicap in sub-Saharan Africa. Its incidence is enormous, and in children the frequency of the pneumococcus as the causative organism, one noted for its high incidence of posttreatment complications, prejudices the outcome. Epidemic or endemic meningoccocal meningitis in the Sahelien and drier areas of Africa has a higher incidence of early death. In Central Hospital of Yaounde, Cameroon, there were 240 admissions between January 1974 and June 1975 for bacterial meningitis. Of these 80% were in children under two years of age, although the dispersal of cases covered all ages. Too frequently, fever, headache and general unwellness are ascribed to malaria in this highly malarious zone, and the use of quinine, chloroquine and the omnipresent penicillin often delay proper diagnosis or alter the clinical course sufficiently to allow bacterial infection to smoulder for weeks. Faced frequently with a stuporous, comatose

2. Post-bacterial meningitis survival is no longer a rarity nor its paralytic sequelae. 296

or convulsing patient with a thick and purulent spinal fluid, the managing physician most often contend with inadequate supplies of drugs, or nursing care inadequate to assure their proper administration. Meningitis in tropical Africa remains a serious cause of mortality but a more serious and rising cause of morbidity and chronic disability. Hydrocephalus following post-meningitic adhesions is common. Thrombophlebitis of the cortical vessels often leads to long term monoplegia, hemiplegia and cranial nerve palsies. Of the less severe sequelae, ocular paresis is extremely common and deafness is also frequently observed'.6 Foot drop and mild spasticity are the most common mild locomotor sequelae. The incidence of surviving but severely damaged children with hydrocephalus, severe mental retardation and diffuse paralysis is rising rapidly in Cameroon. This apparently results from the increasing number of medical evacuations being made from outlying areas to a pediatric service in which the increasing availability of antibiotics and medical personnel is rapidly improving survival. The survival of patients for several years with post-meningitis paralysis is no longer a rarity in Cameroon. Infection-Associated Paralysis — Spinal Epidnral Abscess Spinal epidural abscess is an uncommon disease in North America and Europe but occurs frequently in children as well as adults in tropical Africa. It usually presents with fever, severe back pain and local tenderness. The onset of neurologic symptoms in spinal epidural abscess is usually noted within a week or two if adequate treatment is not begun. Urologic symptoms and paraplegia may occur in the first or second week of the untreated disease.7 Failure to establish a diagnosis and to institute early treatment can culminate in death or permanent paralysis in this acute illness which frequently has its origin in a skin or other infection. It is also associated in children with malnutrition, in which the origin may well be hematogenous from the often present pneumonias, furuncles and chronic, deep pyodermas in children in areas of low environmental sanitation. An important iatrogenic cause is the contaminated lumbar puncture. The frequency of diagnostic lumbar puncture in the "meningitis belt" of sub-Saharan Africa far exceeds that in western countries. Unfortunately, the level of training of personnel and the paucity of sterile material and aseptic techniques leads too frequently to this operative cause of epidural abscess and meningitis. Irrespective of cause, rapid surgical drainage and treatment with appropriate antibiotics can often reverse the course of this grave disease with a high incidence of paralytic sequelae in survivors. Unfortunately, the difficulties in obtaining skilled surgical intervention prejudice outcome in all but a few facilities. Environmental Child Health, December 1976

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Paralytic Post-Measles Encephalomyelltis and the Meningoeneepfaalides In unvaccinated populations, apparent measles will occur in excess of 95% of children during the first decade. The widespread usage in more developed nations of attenuated live vaccine has greatly reduced the incidence of measles and the expected neurological complications, estimated by Miller et al. as less than one in 1,000 cases.8 In Yaounde, Cameroon, which in many ways typifies tropical Western Africa, measles was responsible in 1975 for approximately one fourth of all admissions to the Central Hospital pediatrics service and for one half of hospitalized child deaths. Mild neurological symptoms, particularly meningeal irritability, are frequently seen in hospitalized children and are often attributable to the hyperpyrexia and electrolyte imbalance accompanying severe diarrheas and dehydration often seen in tropical measles. In surviving children, there seem to be surprisingly few neurological sequelae.9 Children, particularly infants in the first year or two of life who constitute the majority of measles cases, may present prolonged convulsions or stupor, which are grim prognostic signs for complete recovery. These patients may progress to hemiplegia or an acute ascending spinal paralysis producing paraplegia. Hemiplegia or ataxia may be permanent if the child survives the acute illness, distinguishing the outcome from that usually seen in acute transverse myelopathy in childhood measles. Frequently these children will ultimately suffer mental retardation, personality changes and a seizure disorder as well. The anatomical lesions have been shown to be those of other demyelinating diseases of the nervous system, perivenous infiltration by mono-nuclear cells of the brain and spinal cord and demyelination with the most intense changes noted in the cerebral white matter and lumbar and upper sacral spinal cord.8 10 ll The occurrence of subacute sclerosing panencephalitis as a late complication of measles has been suspected but usually remains unproven through aversion to autopsies and difficulties in obtaining pathological sections. Post-measles paralysis is a dramatic and infrequent complication in a disease of enormous economic and medical consequence in tropical Africa. Its prevention must become a major priority in each endemic area that can be characterized by an old Arabic saying, "count your children after the measles has passed". The Meningoencephalides Arboviruses abound in sub-Saharan Africa and can provoke neurological as well as enteric symptoms. Some arboviruses are known to cause meningeai irritation, with headache, vomiting, nuchal rigidity and often mental disturbances.12 They may be ushered in with convulsions, tremors, facial and bulbar paralysis as well as cerebullar symptoms. The cerebrospinal fluid often shows elevated pressure, a monocytic Environmental Child Health, December 1976

3. Post-measles paralysis: convulsions, and encephalitis occasionally terminate in persisting rigidity and incapacity.

response of 50-400 cells and increased protein levels.13 In tropical zones the history of preceding fever and vague illness with gastrointestinal symptoms resembles that of many other childhood illness and may be of only indirect value for diagnosis. Viral meningoencephalitis is acute in onset and usually followed by remission after a ten- to fourteenday course. Occasionally, there may be an associated, persisting paralysis and impaired mental function. Acute Transverse Myelopathy The syndrome of acute transverse myelopathy is commonly seen in children and adults. It usually presents with back pain which may be generalized or radicular in character. Weakness, which may be severe, numbness, and urinary retention may follow. Rapidity of onset is characteristic with paralysis ensuing in less than three days, usually within twentyfour hours and occasionally in hours after the onset of presenting neurologic symptoms. Transient spinal shock is usually accompanied by posterior column 297

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sparing although a sensory level is evident. Deep tendon reflexes are initially abolished or depressed but hyperreflexia and plantar extensor responses often appear after a day or two. The spinal luid may be normal or show mild elevation of protein and a pleocytosis in approximately half of the cases.14 15 The etiology of acute transverse myelopathy is obscure and the evolution in childhood usually so much more favourable than in adults as to suggest that the causes are dissimilar in the two groups.16 There have been well-documented associations with a variety of infectious diseases of childhood of which measles is dominant followed by mumps, rubella and the usage of live vaccines. As a sequelae of measles, an endemic and costly problem in sub-Saharan Africa, it is one of the occasional findings among many acute neurological symptoms. The association of viral diseases with the syndrome seems valid but it is rarely possible to document this with the laboratory facilities in most developing nations. Clinically, the frequency with which hospitalizations in tropical Africa are preceded by a febrile disease with gastro-intestinal system makes this history of limited value in acute transverse myelopathy. An occasional herald of collagen vascular disease or multiple sclerosis and other demyelinating disorders in adults in northern latitudes, acute transverse myelopathy is virtually never followed by multiple sclerosis in the equatorial latitudes, where it is virtually unknown.10 17 Another possible explanation of the acute transverse myelopathy syndrome occurring in children with sickle cell SS disease is discussed elsewhere in this communication. Eventual complete or nearly complete recovery of function is the usual course in children. The majority of adults improve, but in all age groups there may be persisting neurological handicaps.

culminating in lower limb paralysis.20 This seems most unusual in the absence of severe dehydration and sludging of cells. Three cases of flaccid lower extremity and sphincter of sudden onset with associated loss of sensation and clinical course suggestive of irreversibility in children with SS genotype have been recently described in Cameroon.21 There was no mental clouding or increased CSF pressure or dehydration. All were children between 5 and 14 years of age. Acute anterior poliomyelitis, very rarely seen in that country after age three, was excluded. There was no recent antecedent trauma or history of injection. Guillain-Barre polyneuritis, tumor, congenital anomaly and epidural abscess were All presented a considered unlikely.7 22 23 neurological picture suggestive of segmentai necrosis of the spinal cord with profound and enduring motor and sensory deficits which were ascribed to vascular disruption associated with sickle cell disease.

Sickle Cell Disease and Associated Paralyses Strokes due to cerebral occlusion are well known in sickle cell disease and if not immediately fatal may leave residual hemiplegia,18 19 an occasional observation in children of black populations in subSaharan Africa. Far more frequent are the vasoocclusive episodes which provoke the common complaints of pain in the extremities and joints. The periosteal reaction, bony destruction and tendency to develop osteomyelitis are well known and may, in a patient severely weakened by malnutrition, present a pseudo-paralysis in which the patient avoids all motion to minimize pain. Notwithstanding the oft-quoted but unproven claim that SS carriers enjoy special resistance to malaria, in Cameroon it is clear that malaria compounds the problem of anemias aggravated by frequent hemolytic crises, inadequate diet and intestinal parasitoses. Wolman and Hardy have described spinal cord infarction associated with sickle cell trait and 298

4. Sickle Cell SS disease: fatal stroke and hemiplegia are well known; bilateral foot drop and a slapping gait developed abruptly in this homozygous boy under longterm medical surveillance. Environmental Child Health, December 1976

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Acute Infantile Hemiplegia Paralysis and hemiparesis associated with dehydration In tropical agricultural nations, low standards of sanitation and contaminated water supplies dispose to frequent gastro-enteritis. With severe gastro-enteritis, or the severe diarrheas and reduced fluid intake in tropical measles, dehydration is common and facilities for rehydration by parenteral routes are often unavailable or distant, not surprisingly, the complications of vascular collapse and electrolyte imbalance contribute to heavy mortality rates. A frequent complication seen is hemiplegia from cerebral thrombosis following late or inadequate rehydration. Gangrene, usually of a lower extremity, is also seen occasionally in rural hospitals receiving severely dehydrated children for whom treatment was postponed too long. If elicited, a history of severe diarrhea with hospitalization and institutional onset of symptoms is frequently found in children presenting with spastic hemiplegia and pyramidal tract-dominant gait. Superimposed dehydration with cyanotic congenital heart disease and attendent polycythemia is another frequent and important cause of hemiplegia secondary to apparent thrombosis. Acute hemiplegia without dehydration, polycythemia or cardiac disease Acute hemiplegia ushered in with a convulsion, frequently Jacksonian in type, fever and coma but unaccompanied by dehydration is occasionally seen. The cerebrospinal fluid is invariably normal at the onset but may show modest elevation in the number of leukocytes on subsequent examination. The state is usually seen in children under two years of age, is generally non-progressive after the acute incident and is frequently followed by fixed hemiparesis, mental retardation, focal convulsions and aphasia if the paralysis was on the right side. The cause is unknown but thrombosis or embolism are suspect. It has been seen as a complication of measles by the author. Carotid arteritis following severe and chronic throat infections and cervical lymphadenitis has also been suggested.24 Non-Hematologic Vascular Causes of Paralysis Vascular causes of paralysis in childhood are difficult to demonstrate in most tropical nations because radiological and neurosurgical diagnostic potential are usually lacking. In general, vascular anomolies of the spinal cord may cause sudden spinal cord dysfunction as rupture and hemorrhage into the spinal cord subarachnoid or epidural space occurs.25 .The best documented arterial ischemic syndrome is anterior spinal artery dysfunction. It is characterized by a usually abrupt onset of pain, often radicular in character, with paresthesias along the upper levels of the lesions and paralysis developing within hours or Environmental Child Health, December 1976

days.15 The anatomical arrangement of the blood supply of the spinal cord has as its significant features an arterial supply from one anterior and two posterior longitudinal trunks which are filled by afferent radicular arteries of multiple origin entering along the nerve roots. Within the considerable anatomical variation in individuals, there are approximately eight significant anterior radicular arteries, each unilateral. There are fifteen or more posterior radicular arteries. Although considerable longitudinal and circumferential anastomoses exist outside the spinal cord, the many end vessels within the cord render it vulnerable to compromise.26 The principal branch of the anterior spinal artery is the artery of the central sulcus which passes up the central sulcus and enters the gray matter of one side or of the other. The anterior spinal artery syndrome, or loss of perfusion, is usually held to involve the pyramidal tracts and lateral and ventral spinothalamic tracts and to spare the posterior columns. With occlusion of the anterior spinal artery of several segments, central sulcal arteries would be affected producing a bilateral cord lesion.15 Paralysis is usually bilateral and often accompanied by spinal shock, and inevitably by sphincter dysfunction. Vibratory and position senses are unimpaired and pain and temperature sensations are abolished. Etiologic causes for spinal cord infarction are embolism, thrombosis, and collagen vascular disease in the childhood age range with diffuse vascular disease occurring at later ages.25 27

Toxic and Nutritional Neuropathies After the exclusion of numerous infectious, traumatic, parasitic, familial degenerative diseases and trauma provoking paralysis in children in tropical Africa, there remain many other causes of handicapping neurological disease that are less well explained. Many are presumed to be nutritional in origin. Dietary variables in tropical and subptropical Africa are almost infinite and often seasonal. In areas paralleling the Sahara, shortages of foodstuffs periodically force parents and children to eat nonusual plants and seeds which may be toxic. In nations which are predominantly rural populations, poor and captive rural living situations with severely limited dietary variety and quantity are frequently teamed with the stresses of infectious and parasitic diseases and the increased needs of childhood growth, pregnancy and lactation.28 Under such stress, various non-specific clinical signs of malnutrition may be manifest. Frequently associated with neuropathies in nutritionally deprived children patients are glossitis, cheilosis and angular stomatitis and follicular hyperkeratosis. Amblyopia and optic atrophy and scrotal dermatitis are other less common signs of malnutrition seen occasionally in adults 2 ' and infrequently in children. 299

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Toxic Causes of Paralysis and Ataxis Lathyrism Vetches of the lathyrus family, lathyrus peas and akta seeds, found in Africa and India are often eaten at times of food shortage or to accompany a chronically meager diet. Their intake is often associated with the insidious onset of spastic paraplegia which may vary from leg stiffness with an awkward gait to a scissors gait or total inability to walk.30 Sensory defects are absent and sphincter function is conserved. The regional distribution and seasonal character of onset are helpful in establishing the diagnosis. There is no disturbance in sensorium. Sinclair and Jelllffe have identified the toxic agent as beta-proprionitrile.31 Heavy Metal and Cyanide Provoked Neuropathy Chronic heavy metal ingestion can be as serious as an environmental hazard to children in village Africa as in an urban ghetto, as for example when a discarded automobile battery casing is employed for water or food storage or preparation. Contaminated food or drink from lead-soldered vessels, and the fumes from batteries being salvaged are frequent causes of acute and chronic toxicity. Ataxia, persisting vomiting, seizures and coma are initially confused with cerebral malaria. Peripheral neuropathy and hemiparesis are occasionally noted in children who are, as a rule, older than those seen for lead intoxication in Europe and the United States.

5. Unproven but suspect environmental hazard: the three days of water soaking required to remove cyanide from peeled manioc is being accomplished in this old battery case, suggesting a risk of lead intake. These urban dwellers have no access to the fast-moving streams traditionally used for manioc preparation.

In Western Nigeria, and other areas of sub-Saharan Africa, both children and adults occasionally present with progressive paraesthesias of the lower extremities followed by unsteadiness of gait. There is gradually progressive loss of vibratory sense which may move proximally toward the waist. Generalized cutaneous 300

sensory deficits follow and deep tendon reflexes may be exaggerated. Optic atrophy and extensive pyramidal tract involvement have been noted in adults.29 " Elevated plasma and urinary thiocyanate levels have been found in many of these patients which some authors associate with chronic dietary intake of cyanide.28 n The dominant foodstuff of much of Western and Central Africa is manioc and related families of tubers which bear cyanide. Those whose diet is almost entirely composed of this tuber group are apparently at risk, although various means of preparation can alter the content of cyanide. The manioc-dominated diet poses an inevitable fundamental nutritional problem in its lack of protein of reasonable quality and protective vitamins which first risk kwashiorkor in young children, and avitaminosis and inadequate growth in the older child.28 31 Further, this tuber group contains a source of hydrocyanic acid which is released from a cyanogenetic glycoside. Subsequent detoxification of HCN to an excretable-SCN radical is accomplished by -SH or sulphydryl groups supplied by the dietary proteins of good nutritional quality. Excretion failure culminates in intoxication, or more frequently in a protein-draining effect through amino acid losses required for the continuous detoxication of small doses of dietary cyanide. It is further suggested that available hydroxycobalmin or B12, a substrate essential for neuronal function, is depleted through its affinity for combination with cyanide.28 Monekosso considers this interference with hydroxycobalmin a possible unitary explanation for a broad range of neurological abnormalities seen in areas where the manioc family dominates the diet. He has suggested that sublethal doses of cyanide depress tissue respiration and aggravate signs of supposed riboflavin deficiency such as angular stomatitis and glossitis.

Neuropathies in Vitamin B Complex Deficiency Pure single vitamin deficiencies are probably infrequent in children posing nutritional neuropathies. Poverty and monotonous diets lacking in sufficient good quality protein in children with the added needs of growth are further influenced by chronic diarrheas and deranged intestinal absorption. B complex deficient clinical neuropathies are infrequently seen in relation to the frequency of angular stomatititis, glossitis and other cutaneous signs of severe deficiency. A significant number of older children received at Central Hospital in Yaounde for gait disturbances and inability to ambulate display their neurological problems as a part of global malnutrition in which cutaneous signs are present. Vitamin Bj or thamine deficiency is responsible for beri-beri. Clinical beri-beri, apart from its cardiac manifestations with right sided failure, ascites and weakness presents an often rapid onset of neurological symptoms in infants and younger children. Younger Environmental Child Health, December 1976

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children often cannot complain of the paresthesias that disturb adults but will manifest weakness and the rather distinctive generalized tenderness of the limbs. Sensory loss and areflexia can rapidly follow and, if treatment is not begun, progressive atrophic paralysis may ensue. Its occurrence in some cases of kwashiorkor is apparent to the author, who assures generous treatment with injectabie thiamine in young children with suggestive signs. Elevated serum pyruvic acid above 0.4 to 1 mg per cent may be noted in some cases.30 Vitamin B, or nicotinic acid deficiency in its pure form is responsible for pellagra with its cutaneous, gastrointestinal and neurological manifestations. Its occurrence is still noted in famine zones of Africa. Commonly seen in urban centers where relocated families may suffer severe privation, severe cases of advanced protein-caloric malnutrition in children in childhood with dysphagia, ataxia, amblyopia or ophthalmoplegia, loss of deep tendon reflexes and stupor are occasionally seen. The presentation is suggestive of advanced pellagra. With severe kwashiorkor is apparent to the author, who assures generous treatment with injectabie thiamine in young gardens then readily develop erythema in intact areas of skin and begin to desquamate. The neurological signs may regress entirely with aggressive nutritional therapy.

6. Pellagrous skin changes among the severely malnourished are occasionally accompanied by polyneuropathies and gross gait disturbances unexplicabie by weakness alone. Snvironmental Child Health, December 1976

Parasitic Causes of Neurologic Disease and Paralysis Echinococcosii Echinococcal disease (echinococcus granulosus) is seen predominantly in those parts of tropical and subtropical Africa where sheep and possibly goats are raised. The more usual presentation is the unilocular cyst which may occur in any part of the body but more frequently in the liver, lungs, omentum and mesentery. Occasionally, twenty years may elapse after initial infection before the cyst comes to the notice of a physician.33 Often asymptomatic in the earlier stages, the increase in size may ultimately produce symptoms of pressure on adjacent organs. The central nervous system is occasionally invaded. When the brain is the site of cyst formation, seizure disorders and blindness can occur.33 34 In northwestern Cameroon and Nigeria echinococcus has been occasional implicated as a cause of symmetrical bilateral paralysis in children and adults. Diagnosis is rendered difficult and is rarely suspected because of the absence of the classic cyst and tumour which suggest the underlying disease. A positive skin test, the Casoni test, performed with an intracutaneous injection of echinococcal antigen or an indirect hemagglutination test may be helpful in establishing the diagnosis.33 After the exclusion of other causes it should be considered in areas of known echinococcal endemicity. After hatching in the duodenum the larvae penetrate its wall and are carried through the body, some occasionally coming to rest in varying proximity to the spinal cord where development of a cyst may provoke a foreign body reaction and meningeal irritation or signs of compression, depending on the size and site of the lesion. Symmetrical paresthesias or paralysis or both may arise at any level imitating a spinal tumour. With intimate proximity of a developing cyst to the spinal cord, an acute and symmetrical process may mimic transverse myelopathy or polyneuritis. Diagnosis may be accomplished by the demonstration of daughter cysts in the cerebrospinal fluid which may, however, show little other abnormality. These have reputedly been discovered at autopsy. Effective treatment has not been established to the author's knowledge, and treatment of non-CNS echinococcal lesions remains an essentially surgical one in the absence of effective drugs.33 Sshistosomiasis Schistosomiasis susceptibility is known at all ages and the peak incidence occurs between seven and fifteen years. Its presentation is highly variable depending on the involved species. S. hematobium is widely disseminated in Africa while S. mansoni and S. intercalatum are found in West, Central and East Africa. It is a widespread and serious cause of chronic morbidity in the African continent. In Egypt alone at least 37% of the population is believed to be infected and in 1965 the World Health Organization estimated 301

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that 180 to 200 million persons were infected worldwide. Hematuria from S. hematobium is so common in parts of Western Africa that it is virtually disregarded by African children. In hyperendemic areas where its incidence may be nearly universal, boys apparently regard it as a natural phenomena, the counterpart of menstruation in girls.36 Schistosomiasis is primarily a rural disease and its propagation depends on the contamination of fresh water with urine or feces containing schistosome eeggs. The water must be populated by snails capable of infection by schistosomal miracidia and the release of cercariae infective to man. Human contact with the infected water through bathing, wading or consumption completes the circle of infection which can be accomplished in many social or agricultural settings in tropical nations. In the life cycle of all species of the parasite, distribution through the lymphatic system, veins, right heart and lungs is well known. The preferential distribution of different species through various venous plexuses with symptomology referred to the system or region invaded is well known.35 Symptomology may be protean, depending on the intensity of parasitization and duration of infection, and may affect virtually every organ system in the body. In brief, the schistosome egg stimulates first an eosinophilia followed by a granulomatous response of varying severity. Repeated insult may produce accelerated secondary reactions with tissue and morphological destruction.36 Granulomas may be symptomatic through their pressure on adjacent organs. Neurological symptoms are seen most frequently in S. Japonicum, not seen in Africa, in which numerous central nervous system manifestations may occur through the deposition of eggs in the brain. This may provoke diffuse small lesions in the grey or white matter which may give rise depending on location to seizures, spasticity, altered reflexes, coma or other signs. Neurological symptoms are uncommon in areas of light or sporadic infestation with schistosomiasis but are regularly noted by alert clinicians in areas of endemicity of S. mansoni and S. hematobium as well as S. japonicum. Headaches, disorientation, seizures and confusion may occur and be ascribed to other disease processes, but may result from the eggs which pass into the systemuc circulation during the venous phase of any of the schistosoma, and are filtered out in the brain and spinal cord.37 S. mansoni and S. hematobium, while occasionally responsible for brain lesions, more frequently produce spinal symptoms. Invasion of the spinal cord can occur with the formation of a granuloma in which eggs are found. Severe cord compression may be followed by radiculitis of the cauda equina or the signs and symptoms of acute transverse myelopathy. Altered reflexes, spasticity, weakness of the legs, paraplegia and incontinence may occur37 and their occurrence in 302

children or adults in endemic areas should suggest schistosomiasis. Cerebrospinal fluid pressure may be under pressure and may show a positive circumoval In symptomatic precipitin test (COP).36 granulomatous lesions that can be located, surgical removal is recommended and antimony has been suggested for patients with spinal symptoms without surgically amenable lesions. Schistosomiasis, with its protean manifestations and tissue and organ damage, poses great treatment difficulties. Fortunately, most cases of S. hematobium can and do recover without treatment and children under age five, may lose the disease spontaneously. Miracil D, niridazole and antimony have been used for the severely symptomatic and treatment is generally recommended for all known infections.38 The more severe neurological defects which persist despite chemotherapy pose great difficulty since the option of neurosurgical exploration is infrequently open in developing nations. Schistosomiasis remains a largely unresolved public health dilemma for which clinical treatment is imperfect. An added cause of fever and anemia among children who may be poorly nourished and an important cause of chronic illness in adults, schistosomiasis may also pose difficult problems of neurologic diagnosis and severe locomotor handicaps that are difficult to manage successfully with the limited resources of most tropical developing nations.

Acute Trauma: A Major Cause of Childhood Paralysis The world-wide proliferation of vehicles has been accompanied in the medically more developed nations by what could be called the medicine of traumatology. The ability to rapidly intervene with emergency thoracic, orthopedic and neurosurgical procedures and the supportive services of a blood bank and intensive care unit are lacking in most developing nations. In 1976, one sub-Saharan nation of six millions with its own school of medicine and several nursing schools has no trained thoracic or orthopedic surgeon and a single neurosurgeon who does not perform surgery. Many surgeons and generalists are pressed into service for emergencies that regularly surpass their training and experience, but few risk neurosurgical interventions for vertebral column injuries and hemorrhage into the brain or spinal cord. A wealthy and privileged few may secure medical evacuation to Europe, but this is an infrequent option for those with injuries to the central nervous system. With improved support facilities, such as more hospitals and increasing provision for blood banking, fluid therapy and primary care given by generalist physicians and nurses, survival is improving for accident victims. Many are surviving with severe neurological impairment, however, and this includes children. Environmental Child Health, December 1976

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12. 13.

14. 15. 16. 17.

7. Overloaded and unstable vehicles collide frequently, a major cause of accidental death and neurologic handicap in survivors.

Unrestrained in vehicle accidents, often in the arms of their mother in a crowded and unstable vehicle, very young children frequently suffer penetrating injuries to the head and damage to the vertebral column. Children fall while scaling palm trees for nuts or for the collection of sap for palm wine. They occasionally sustain compression fractures of the lumbar spine with compromise of the spinal cord. Inability to secure neurosurgical intervention condemns many children and young adults as well to permanent lower motor paralysis and frequently to later death through renal failure for survivors of the acute insult.

18. 19. 20. 21. 22. 23. 24. 25. 26.

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Paralytic diseases of childhood in the tropics.

N0ELGU1LL0ZET Paralytic Diseases of Childhood in the Tropics by NOEL GUILLOZET, M.D., F.A.A.P. Associate Professor of Pediatrics, The University Cent...
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