- Email: [email protected]
Two cases of schistosomiasis
Two cases of schistosomiasis S. Bissessur*
and J.M. Minderhoud*
*
Introduction
Summary
Schistosomiasis or bilharziasis is the state of the human body being infected with bilharzia ova, with or without reactive tissue, the immature worms (schistosomula) or the mature worms (schistosoma). If faeces or urine infected with this parasite gets into tropical water, the miracidium (embryo with cilia) leaves the shell. If the miracidium reaches the intermediate host, the fresh water snail, within twenty-four hours, one miracidium multiplies into thousands of cercariae. After the loss of their forked tails, the cercariae can penetrate the intact human skin and be transported by the blood stream to the hepatic portal venules. They are then termed schistosomula. After a maturing period of three to four weeks, the schistosoma migrate in pairs to the venous plexus of the large intestine and the mesenterium. Production of ova begins six weeks after infection. Ova may gain entrance to the lumen of intestine or bladder and be excreted with faeces or urine. The cycle is then complete. Ova may also remain lodged in branches of the portal artery and the pulmonary artery. At a later stage this can result in portal or pulmonary hypertension. The literature on bilharzia lists
* Formerly Department of Neurology and Neurophysiology, Neurology, University Hospital Groningen, the Netherlannds.
Two patients with neuroschistosomiasis are reported. In both patients diagnostic problems were encountered. The first case began with an aspecific allergic reaction of facial oedema and abnormal behaviour. The symptoms were followed by generalized convulsive seizures, dysphasia and hemiparesis. The disease paraclinical findings and characteristics, remarkable improvement on antischistosomal drug therapy strongly suggested cerebral schistosomiasis. The second case history starts with a cauda syndrome while in the end the diagnosis of transverse myelitis was confirmed. Key words: diseases.
neuroschistosomiasis,
tropical
the following organs as being most often habitat of this parasite: the liver, the oesophagus, the large intestine, the urinary tract, the portal, mesenteric and vesical veins. Ectopic forms of schistosomiasis are described in many other locations, the chest, skin, eye and the central nervous system. Concerning the nervous system, primary and secundary lesions are mentioned in the literature. Even a simultaneous occurrence of spinal and cutaneous lesions caused by schistosomiasis are described’. Strik-
University Hospital Parimaribo,
Address for correspondence and reprint requests: J. M. Minderhoud, 59, 9713 EZ Groningen, the Netherlands.
Surinam.
* * Department
of
Department of Neurology, University Hospital, Oostersingel
Accepted 21.3.85 Clin Neurol Neurosurg 1985. Vol. 87-3
213
ing secondary lesions as vocal cord paralysis’, polyneuropathy3,“.” and neurological signs caused by subluxation of the cervical spine after an epileptic fit due to cerebral schistosomiasis have been recordedh. In primary lesions of the central nervous system the spinal cord as well as the brain can be involved. Complications as for example subarachnoid hemorrhage due to S.mansoni, or a masslesion in the posterior fossa with the characteristic necrotic granulomas as found by neurosurgical exploration are reported also7.x. Schistosomiasis of the spinal cord are described in the Netherlands before9J0. Although serologic reactions and the examination of faeces, togethers with positive results of treatment are important diagnostic criteria, these can be missing. Goasgrien” described a 21-yearold man with generalized epileptic seizures, who showed negative findings on clinical or biochemical examinations, except blood eosinophilia, and negative specific serology. One year later the CT scan showed a poorly defined hyperdense occipital lesion. The conformation that the lesion was due ro S.mansoni came from the examination of the tissue obtained by operation. Similar difficulties regarding the presenting signs and symptoms and the affirmation of the diagnosis were encountered in two patients we want to present. Both patients were examined in Surinam. Another important reason to describe these neurological diseases is the possibility that, because of the more intensive contact there will be between inhabitants of tropical areas and for example Europe, the prevalence of schistosomiasis of the S .mansoni-type will increase. Case reports Case 1.
An eight-year-old girl was examined in the emergency department of the University Hospital in Paramaribo, on December 15, 1981, because of generalized epileptic seizures. The epileptic seizures ceased after administration of diazepam i.v. and i.m. and clonazepam i.m. Approximately 24 hours later, the neurological examination showed a grade 3 paraparesis of the legs, increased tendon-reflexes on the right side, absence of abdominal reflexes and bilateral extensor responses. 214
It appeared that the child had been iI1 for the past three weeks. In the beginning her fact had become puffy, her abdomen distended and her behaviour became abnormal. She seemed to he absent-minded and her movements were sluggish. Her speech was difficult to understand. A few days after the onset of these symptoms the first epileptic seizures had occurred. Because of her abnormal. aggressive behaviour she was at first referred to a pediatric-psychiatrist. Except for her father, who was an alcoholic, the family of the child was in good health. The family lived in a schistosoma endemic area. In the ten days after admission the following neurologic syndrome emerged: motor and sensory dysphasia, right-sided hemiparesis with increased tendon-reflexes and a reduced sensitivity to pain on the right side of the body. The abdominal reflexes were absent and the plantar reflexes were extensor bilaterally. Blood morphology and biochemical screening did not show any abnormality, except an increased percentage of eosinophilic granulocytes in the periferal blood (8%). Lumbar puncture revealed normal pressures and a normal Queckenstedt’s test. The CSF contained 213 cells, a normal proteine-content (0.15 g/l) and normal glucose. Cultures of blood and CSF were sterile. Urine-analysis was normal and no schistosoma ova were found in the stools. X-rays of the skull and spinal column did not On the chest-X-ray show abnormalities. peribronchial infiltrations were found. Focal abnormalities in the left high-frontal and right temporal regions were found on the electro-encephalogram. Four-vessel angiography was normal and excluded space-occupying lesions. The serological analysis for schistosoma showed a positive titre (l/64). The IFA schistosoma in the CSF was negative. Treatment with antiepileptic drugs was started. When the clinical condition deteriorated with periods of clouded consciousness and an irregular respiration pattern she was treated with dexamethasone i.m. during nine days (32-2 mg per days). Nevertheless the clinical condition did not improve. Also treatment with the antischistosomal drug hycanthone did not induce improvement at the first dose, but shortly after the second dose
was given a fortnight later she improved rapidly. Except for a slight paressis of the right leg the child improved completely. One month later no abnormalities could be found on the electroencephalogram. Case 2 An l&year-old man was examined on 29th June 1981 in the outpatient clinic of the University Hospital in Paramaribo, because of low back pain. This pain had been present during the preceding days, sometimes accompagnied by pain irradiating along the backside of both legs to the dorsolateral side of the feet. Furthermore, during the preceding 2 or 3 months he had suffered from erectile impotence and during the preceding two days he had experienced difficulty in voiding urine and faeces. The patient had been in good health before, as was found also on medical examination one year previously. Regarding the possibility of schistosomiasis it became evident that he had been swimming in rivers in the schistosoma area. On neurological examination no abnormalities were found. He only indicated local paravertebral pain on pressure in lower lumbar region. He was admitted for further examination three days later. By that time there was a total loss of the ability to erect, as well as the possibility to void urine or faeces. Very recently he had noticed a loss of strength in both legs. Moreover he had become extremely depressed and had expressed suicidal tendencies. On repeated neurological examination normal cerebral functions were found. No abnormalities were present on the cranial nerves and the upper extremities. However, there was a predominant hypertonic paraplegia, grade 4, together with decreased perception of pain and vibration sensivity below the level of L2. The tendon reflexes of the legs were increased, and the plantar reflexes were extensor. The cremaster and anal reflexes were absent. X-ray examination did not show abnormalities. Lumbar puncture revealed turbid CSF under a somewhat increased pressure. Queckenstedt’s test was normal. In the CSF 107213 leucocytes were found, of which 90.5% were monocytes and 9.5% lymphocytes. Total
protein was increased (1.3 g/l), and sugar was normal. The cultures of blood, urine and CSF were sterile. On myelography a swelling of the conus medullaris was the only finding. Specific examinations of stools and urine were negative for schistosoma ova. The serological reactions on schistosomiasis were negative. A fortnight after admission the clinical condition had improved somewhat. Strength had increased in his legs, micturation had improved as well as defaecation, although he was incontinent for faeces sometimes. He again experienced erection in the morning. No clear diagnosis was made when he left hospital about six weeks after admission. On follow-up he was found to become depressed and again showed suicidal tendencies, particularly since his prospective marriage was endangered by his impaired potency. He was admitted again five months later. Neurologic examination now showed: brisk tendon reflexes on both legs with indifferent plantar responses. The cremaster and anal reflexes were slightly positive. Lumbar puncture showed clear CSF under normal pressure. The CSF contained 10/3 leucocytes of which half were monocytes and half lymphocytes. The proteinecontent was slightly increased (0.53 g/l) and glucose was normal. This time the internist was asked to perform rectoscopy. This revealed a large quantity of schistosoma granuloma in the rectum. As also the serologic testing on schistosomiasis was positive (titre l/64) and examination of faeces showed schistosoma ova, the diagnosis schistosoma mansoni was evident. Urological examination did not show abnormalities. The patient was treated with 500 mg oxamquine twice a day. Nevertheless, there was no further improvement. A third and final evaluation was made in Leiden (the Netherlands) a half year later, that is about 14 months after the first admission and one and a half year after the first symptoms. In the CSF slight abnormalities were still present: 28/3 cell of which the major part were mononuclear cells. Total protein was increased (0.76%). The Mancini was elevated with a normal IgG-index. While the serologic reaction to schistosomiasis was positive in serum, this was negative in CSF. Also the examination of stools 215
was negative three times. There was a clearly increased IgG content in the serum. No further examination of the spinal cord was done. Discussion Bilharziasis is called after the German physician Theodore Bilharz, who, while performing an autopsy in 1851 discovered ova with lateral and terminal protrusions. Schistosomiasis is synonymous to bilharziasis. In his discription, Jonckheere12 gave a detailed historical review of the origin of the earliest findings of schistosomiasis in Egypt, where till hitherto the illness has been endemic. In his work ‘Une maladie Egyptienne’ he reported about haematurie caused by lesions of the kidneys and urinary tracts produced by the schistosoma haematobium in the ancient Egypt. The epidemiological importance of schistosomiasis finds expression in the fact that schistosomiasis listed among the six most important diseases of the W.H.O.‘“. The clinical picture of neurobilharzia is influenced by the degree of immunity of the patient to this disease, the region of contamination and the stage of infection. The patient may be a non-immune newcomer (European) or an immune inhabitant of a bilharzia-infected region. Reviews in the literature show geografic preferences in the localization of schistosoma types. S.Mansoni is found in South America (Surinam, Brazil, Venezuela and some of the Caribbean islands), Africa and the middle and far East. S.Haematobium is found in Africa and the Middle East; S. japonicum shows a preference for the Far East, Japan, China, the Philippines S.mekongi and while and Indonesia, S.Japonicum are encountered in Mekong and Malaya. The stage of infection is closely reflected in the progression of the symptoms, and confirms to the syndrome of the biharzia type and the organ of location. The following categorization into syndromes, based on the stages of infection after penetration of the cercariae and further development of the parasites, appears to be the most appropriate: - dermatitis: Immediate and delayed allergic skin reaction (for example swimmer’s itch), 216
during and after penetration of the skin by cercariae. - Katayama syndrome: This syndrome. characterized by fever and eosinophilia, often causes pulmonary and gastro-intestinal complaints. It makes its appearance in the immature phase of the worms and is considered to be a serum reaction to worm antigen or an immunecomplex illness. Recently a case of a Katayama-syndrome was reported and in connection with this case it was pointed out that the intensive traffic between Surinam and the Netherlands increased the risk of prevelance of this syndrome in the latter country14. - chronic schistosomiasis: In this case there is an organ lesion which is dependent on the localization of the bilharzia worm, the bilharzia ovum and the tissue reaction around the mature worm and/or ovum. In cases of cerebral bilharziasis the schistosoma ovum is flooded with histiocytes, epitheloid cells, multinuclear giant cells and fibroblasts, which are concentrically surrounded by a proliferation of lymphocytes. plasmacells and eosinophilic granulocytes. This phenomenon - the granuloma - causes an oedematous reaction of fibrillar astrocytes. The clinical picture commences in cerebra with the symptomatology of an acute or subacute encephalopathy. This may be accompanied by focal deficits. Spinal bilharziasis may produce the signs and symptoms of a radiculitis, a transverse myelitis or a spinal tumor. Radiculitis is mainly encountered at the level of the cauda equina. A few days or weeks after infection the patients begin to suffer of lowback pain. Later on paraparesis, impairment of micturation and sexual functions may develop. It is possible to make the diagnosis of transverse myelitis a few days or weeks after infection in case of a non-immune patient. but in slightly immune patients signs and symptoms can develop after months or even years. The bilharzia granuloma may give rise to a (sub)total transverse lesion syndrome with the characteristics of a spinal tumor. Recommended investigations: Examinations focussed on schistosoma and other often encountered parasites are: a. Somato-neurological anamnesis paying par-
titular attention to characteristics of the clinical picture of bilharzia. examinations in neurob. supplementary schistosomiasis: faeces and urine analysis can be done by a rapid method, according to the Kate/Katz method and the nucleopore filtration of urine sampless. However, the normal laboratory analysis according to Ridley concentration method faeces analysis is well known. IFA schistosoma assessed in serum and CSF. In order to measure the total and IgG-specific antibodies in the acutely and chronically S.mansoni-infected patients the schistosoma-specific antigen reaction with PSAP (phenol sulfuric test active peak) is mentioned15. According to this reaction patients with acute infection show a high IgM antibody and low IgG levels. Arriving at the differential diagnosis, the laboratory techniques of detecting neurocruzi , cysticercosis , neurotrypanosoma neurotoxoplasmosis , neuroamoebiasis and neuromalaria should be considered. X-ray, myelography, electroencephalography and CT-scan are considered as routine procedures16. In reported cases of schistosomiasis the treatment procedures often indicated are the administration of antischistosomal drugs, corticosteroids and/or surgical intervention. The effective antischistosomal drugs seemed to be for S.mansoni:oxamniquine I (Mansil @), for s.haematobium: metriphonate (Bilarcil @) and for all human schistosoma species: praziquanta1 (Biltricide @). In the case of spinal cord location of schistosoma granuloma and schistosoma structural lesions in cerebra neurosurgical intervention combined with drug therapy may be necessary.
References w. Schistosomiasis of spinal cord and skin. S Afr Med J 1975; 49:75-g. EL-AHBIADY LM. Left vocal cord paralysis in bilharzial pulmonary aneurysm. J Egypt Med Assoc 1974; 57 (56):232-6. RISELEV VB. Secondary lesions of the nervous system in some infections and toxic-allergic conditions in Zambia. Med J Zambia 1975; 9,5:125-7. GHONEIM MA, ASHAMALLAH A, EL-BOLRAINY. Role Of perineuritis in the atomic dilated bilharzial ureter. Int Surg 1976; 61:411-2. MOSTAFAM,HAB~N MA,ABDEL-MONEIM setal. Peripheral polyneuropathy in bilharziasis. J Egypt Med Assoc 1972; 55:44-59. wo WQ, FRITZLEN T. Subluxation of cervical Spine in major epileptic seizure due to cerebral schistosomiasis. Surg Neurol 1976; 5:333-5. POMPEUF,SAMPAIODELACERDAPR. Subarachnoidhemorrhage due to S. mansoni. J Neurology 1979; 221:203-7.
SAXE N, GORDON
BAMBIBRA
EA, ANDRADE
J DE S,
CESARINI I,RODRIGUEZ PA,
tumoral form of schistosomias: Report of a case with cerebellar involvement. Am J Trop Med Hyg 1984; 33:76-9. LUYENDIIK w, LINDEMAN J.Schistosomiasis (bilharziasis) mansoni of the spinal cord simulating an mtramedullary tumor. Surg Neuroll975; 4:457&l. POOL GM. Tropical paraplegia: A case of transverse myelitis, probably caused by schistosomiasis mansoni. Paraplegia 1978-79; 16:113-7. DRUMMOND
C.A.S.A.The
CX3ASGRIENJ,ANTOINEHM,SALIOUP,HERBELLEAUT,PUTZ c. Bilharziose ctrebrale a mansoni. Rev Neurol (Paris) 1984; 140,
DM, IALLON PM, BACQUET
schistosoma 4:293-5.
IONCRHEERE F.Une
maladie Egyptienne. Bruxelles: Edition De La Fondation Egyptologique, Reine Elisabeth Part Du Cinquantenaire 1944; La Medicine Egyptienne No. I: 33-45. WEBBE G. The six diseasesof W.H.O. Schistosomiasis: some advances. Brit Med J 1981; 283:1104-6. NIE DE J. Het Katayamasyndroom in Suriname. Ned Tijdschr Geneesk 1983; 127:372-3. MINGUETTI G, FERREIRA MVC. Computed tomography in neurocysticercosis. J Neurol Neurosurg Psychiatry 1983; 46:936-42. NASH TE,GARCIA-COYCO C,RUIZ-TIBEN E,NAZARIO-L6PEZ G, TORRES-BORGES A. Differentiation of acute and chronicschistosomiasis of antibody responses to specific schistosome antigens. Am J Trop Med Hyg 1983; 32:776-84. HA, VAZQUEZ
217
and J.M. Minderhoud*
*
Introduction
Summary
Schistosomiasis or bilharziasis is the state of the human body being infected with bilharzia ova, with or without reactive tissue, the immature worms (schistosomula) or the mature worms (schistosoma). If faeces or urine infected with this parasite gets into tropical water, the miracidium (embryo with cilia) leaves the shell. If the miracidium reaches the intermediate host, the fresh water snail, within twenty-four hours, one miracidium multiplies into thousands of cercariae. After the loss of their forked tails, the cercariae can penetrate the intact human skin and be transported by the blood stream to the hepatic portal venules. They are then termed schistosomula. After a maturing period of three to four weeks, the schistosoma migrate in pairs to the venous plexus of the large intestine and the mesenterium. Production of ova begins six weeks after infection. Ova may gain entrance to the lumen of intestine or bladder and be excreted with faeces or urine. The cycle is then complete. Ova may also remain lodged in branches of the portal artery and the pulmonary artery. At a later stage this can result in portal or pulmonary hypertension. The literature on bilharzia lists
* Formerly Department of Neurology and Neurophysiology, Neurology, University Hospital Groningen, the Netherlannds.
Two patients with neuroschistosomiasis are reported. In both patients diagnostic problems were encountered. The first case began with an aspecific allergic reaction of facial oedema and abnormal behaviour. The symptoms were followed by generalized convulsive seizures, dysphasia and hemiparesis. The disease paraclinical findings and characteristics, remarkable improvement on antischistosomal drug therapy strongly suggested cerebral schistosomiasis. The second case history starts with a cauda syndrome while in the end the diagnosis of transverse myelitis was confirmed. Key words: diseases.
neuroschistosomiasis,
tropical
the following organs as being most often habitat of this parasite: the liver, the oesophagus, the large intestine, the urinary tract, the portal, mesenteric and vesical veins. Ectopic forms of schistosomiasis are described in many other locations, the chest, skin, eye and the central nervous system. Concerning the nervous system, primary and secundary lesions are mentioned in the literature. Even a simultaneous occurrence of spinal and cutaneous lesions caused by schistosomiasis are described’. Strik-
University Hospital Parimaribo,
Address for correspondence and reprint requests: J. M. Minderhoud, 59, 9713 EZ Groningen, the Netherlands.
Surinam.
* * Department
of
Department of Neurology, University Hospital, Oostersingel
Accepted 21.3.85 Clin Neurol Neurosurg 1985. Vol. 87-3
213
ing secondary lesions as vocal cord paralysis’, polyneuropathy3,“.” and neurological signs caused by subluxation of the cervical spine after an epileptic fit due to cerebral schistosomiasis have been recordedh. In primary lesions of the central nervous system the spinal cord as well as the brain can be involved. Complications as for example subarachnoid hemorrhage due to S.mansoni, or a masslesion in the posterior fossa with the characteristic necrotic granulomas as found by neurosurgical exploration are reported also7.x. Schistosomiasis of the spinal cord are described in the Netherlands before9J0. Although serologic reactions and the examination of faeces, togethers with positive results of treatment are important diagnostic criteria, these can be missing. Goasgrien” described a 21-yearold man with generalized epileptic seizures, who showed negative findings on clinical or biochemical examinations, except blood eosinophilia, and negative specific serology. One year later the CT scan showed a poorly defined hyperdense occipital lesion. The conformation that the lesion was due ro S.mansoni came from the examination of the tissue obtained by operation. Similar difficulties regarding the presenting signs and symptoms and the affirmation of the diagnosis were encountered in two patients we want to present. Both patients were examined in Surinam. Another important reason to describe these neurological diseases is the possibility that, because of the more intensive contact there will be between inhabitants of tropical areas and for example Europe, the prevalence of schistosomiasis of the S .mansoni-type will increase. Case reports Case 1.
An eight-year-old girl was examined in the emergency department of the University Hospital in Paramaribo, on December 15, 1981, because of generalized epileptic seizures. The epileptic seizures ceased after administration of diazepam i.v. and i.m. and clonazepam i.m. Approximately 24 hours later, the neurological examination showed a grade 3 paraparesis of the legs, increased tendon-reflexes on the right side, absence of abdominal reflexes and bilateral extensor responses. 214
It appeared that the child had been iI1 for the past three weeks. In the beginning her fact had become puffy, her abdomen distended and her behaviour became abnormal. She seemed to he absent-minded and her movements were sluggish. Her speech was difficult to understand. A few days after the onset of these symptoms the first epileptic seizures had occurred. Because of her abnormal. aggressive behaviour she was at first referred to a pediatric-psychiatrist. Except for her father, who was an alcoholic, the family of the child was in good health. The family lived in a schistosoma endemic area. In the ten days after admission the following neurologic syndrome emerged: motor and sensory dysphasia, right-sided hemiparesis with increased tendon-reflexes and a reduced sensitivity to pain on the right side of the body. The abdominal reflexes were absent and the plantar reflexes were extensor bilaterally. Blood morphology and biochemical screening did not show any abnormality, except an increased percentage of eosinophilic granulocytes in the periferal blood (8%). Lumbar puncture revealed normal pressures and a normal Queckenstedt’s test. The CSF contained 213 cells, a normal proteine-content (0.15 g/l) and normal glucose. Cultures of blood and CSF were sterile. Urine-analysis was normal and no schistosoma ova were found in the stools. X-rays of the skull and spinal column did not On the chest-X-ray show abnormalities. peribronchial infiltrations were found. Focal abnormalities in the left high-frontal and right temporal regions were found on the electro-encephalogram. Four-vessel angiography was normal and excluded space-occupying lesions. The serological analysis for schistosoma showed a positive titre (l/64). The IFA schistosoma in the CSF was negative. Treatment with antiepileptic drugs was started. When the clinical condition deteriorated with periods of clouded consciousness and an irregular respiration pattern she was treated with dexamethasone i.m. during nine days (32-2 mg per days). Nevertheless the clinical condition did not improve. Also treatment with the antischistosomal drug hycanthone did not induce improvement at the first dose, but shortly after the second dose
was given a fortnight later she improved rapidly. Except for a slight paressis of the right leg the child improved completely. One month later no abnormalities could be found on the electroencephalogram. Case 2 An l&year-old man was examined on 29th June 1981 in the outpatient clinic of the University Hospital in Paramaribo, because of low back pain. This pain had been present during the preceding days, sometimes accompagnied by pain irradiating along the backside of both legs to the dorsolateral side of the feet. Furthermore, during the preceding 2 or 3 months he had suffered from erectile impotence and during the preceding two days he had experienced difficulty in voiding urine and faeces. The patient had been in good health before, as was found also on medical examination one year previously. Regarding the possibility of schistosomiasis it became evident that he had been swimming in rivers in the schistosoma area. On neurological examination no abnormalities were found. He only indicated local paravertebral pain on pressure in lower lumbar region. He was admitted for further examination three days later. By that time there was a total loss of the ability to erect, as well as the possibility to void urine or faeces. Very recently he had noticed a loss of strength in both legs. Moreover he had become extremely depressed and had expressed suicidal tendencies. On repeated neurological examination normal cerebral functions were found. No abnormalities were present on the cranial nerves and the upper extremities. However, there was a predominant hypertonic paraplegia, grade 4, together with decreased perception of pain and vibration sensivity below the level of L2. The tendon reflexes of the legs were increased, and the plantar reflexes were extensor. The cremaster and anal reflexes were absent. X-ray examination did not show abnormalities. Lumbar puncture revealed turbid CSF under a somewhat increased pressure. Queckenstedt’s test was normal. In the CSF 107213 leucocytes were found, of which 90.5% were monocytes and 9.5% lymphocytes. Total
protein was increased (1.3 g/l), and sugar was normal. The cultures of blood, urine and CSF were sterile. On myelography a swelling of the conus medullaris was the only finding. Specific examinations of stools and urine were negative for schistosoma ova. The serological reactions on schistosomiasis were negative. A fortnight after admission the clinical condition had improved somewhat. Strength had increased in his legs, micturation had improved as well as defaecation, although he was incontinent for faeces sometimes. He again experienced erection in the morning. No clear diagnosis was made when he left hospital about six weeks after admission. On follow-up he was found to become depressed and again showed suicidal tendencies, particularly since his prospective marriage was endangered by his impaired potency. He was admitted again five months later. Neurologic examination now showed: brisk tendon reflexes on both legs with indifferent plantar responses. The cremaster and anal reflexes were slightly positive. Lumbar puncture showed clear CSF under normal pressure. The CSF contained 10/3 leucocytes of which half were monocytes and half lymphocytes. The proteinecontent was slightly increased (0.53 g/l) and glucose was normal. This time the internist was asked to perform rectoscopy. This revealed a large quantity of schistosoma granuloma in the rectum. As also the serologic testing on schistosomiasis was positive (titre l/64) and examination of faeces showed schistosoma ova, the diagnosis schistosoma mansoni was evident. Urological examination did not show abnormalities. The patient was treated with 500 mg oxamquine twice a day. Nevertheless, there was no further improvement. A third and final evaluation was made in Leiden (the Netherlands) a half year later, that is about 14 months after the first admission and one and a half year after the first symptoms. In the CSF slight abnormalities were still present: 28/3 cell of which the major part were mononuclear cells. Total protein was increased (0.76%). The Mancini was elevated with a normal IgG-index. While the serologic reaction to schistosomiasis was positive in serum, this was negative in CSF. Also the examination of stools 215
was negative three times. There was a clearly increased IgG content in the serum. No further examination of the spinal cord was done. Discussion Bilharziasis is called after the German physician Theodore Bilharz, who, while performing an autopsy in 1851 discovered ova with lateral and terminal protrusions. Schistosomiasis is synonymous to bilharziasis. In his discription, Jonckheere12 gave a detailed historical review of the origin of the earliest findings of schistosomiasis in Egypt, where till hitherto the illness has been endemic. In his work ‘Une maladie Egyptienne’ he reported about haematurie caused by lesions of the kidneys and urinary tracts produced by the schistosoma haematobium in the ancient Egypt. The epidemiological importance of schistosomiasis finds expression in the fact that schistosomiasis listed among the six most important diseases of the W.H.O.‘“. The clinical picture of neurobilharzia is influenced by the degree of immunity of the patient to this disease, the region of contamination and the stage of infection. The patient may be a non-immune newcomer (European) or an immune inhabitant of a bilharzia-infected region. Reviews in the literature show geografic preferences in the localization of schistosoma types. S.Mansoni is found in South America (Surinam, Brazil, Venezuela and some of the Caribbean islands), Africa and the middle and far East. S.Haematobium is found in Africa and the Middle East; S. japonicum shows a preference for the Far East, Japan, China, the Philippines S.mekongi and while and Indonesia, S.Japonicum are encountered in Mekong and Malaya. The stage of infection is closely reflected in the progression of the symptoms, and confirms to the syndrome of the biharzia type and the organ of location. The following categorization into syndromes, based on the stages of infection after penetration of the cercariae and further development of the parasites, appears to be the most appropriate: - dermatitis: Immediate and delayed allergic skin reaction (for example swimmer’s itch), 216
during and after penetration of the skin by cercariae. - Katayama syndrome: This syndrome. characterized by fever and eosinophilia, often causes pulmonary and gastro-intestinal complaints. It makes its appearance in the immature phase of the worms and is considered to be a serum reaction to worm antigen or an immunecomplex illness. Recently a case of a Katayama-syndrome was reported and in connection with this case it was pointed out that the intensive traffic between Surinam and the Netherlands increased the risk of prevelance of this syndrome in the latter country14. - chronic schistosomiasis: In this case there is an organ lesion which is dependent on the localization of the bilharzia worm, the bilharzia ovum and the tissue reaction around the mature worm and/or ovum. In cases of cerebral bilharziasis the schistosoma ovum is flooded with histiocytes, epitheloid cells, multinuclear giant cells and fibroblasts, which are concentrically surrounded by a proliferation of lymphocytes. plasmacells and eosinophilic granulocytes. This phenomenon - the granuloma - causes an oedematous reaction of fibrillar astrocytes. The clinical picture commences in cerebra with the symptomatology of an acute or subacute encephalopathy. This may be accompanied by focal deficits. Spinal bilharziasis may produce the signs and symptoms of a radiculitis, a transverse myelitis or a spinal tumor. Radiculitis is mainly encountered at the level of the cauda equina. A few days or weeks after infection the patients begin to suffer of lowback pain. Later on paraparesis, impairment of micturation and sexual functions may develop. It is possible to make the diagnosis of transverse myelitis a few days or weeks after infection in case of a non-immune patient. but in slightly immune patients signs and symptoms can develop after months or even years. The bilharzia granuloma may give rise to a (sub)total transverse lesion syndrome with the characteristics of a spinal tumor. Recommended investigations: Examinations focussed on schistosoma and other often encountered parasites are: a. Somato-neurological anamnesis paying par-
titular attention to characteristics of the clinical picture of bilharzia. examinations in neurob. supplementary schistosomiasis: faeces and urine analysis can be done by a rapid method, according to the Kate/Katz method and the nucleopore filtration of urine sampless. However, the normal laboratory analysis according to Ridley concentration method faeces analysis is well known. IFA schistosoma assessed in serum and CSF. In order to measure the total and IgG-specific antibodies in the acutely and chronically S.mansoni-infected patients the schistosoma-specific antigen reaction with PSAP (phenol sulfuric test active peak) is mentioned15. According to this reaction patients with acute infection show a high IgM antibody and low IgG levels. Arriving at the differential diagnosis, the laboratory techniques of detecting neurocruzi , cysticercosis , neurotrypanosoma neurotoxoplasmosis , neuroamoebiasis and neuromalaria should be considered. X-ray, myelography, electroencephalography and CT-scan are considered as routine procedures16. In reported cases of schistosomiasis the treatment procedures often indicated are the administration of antischistosomal drugs, corticosteroids and/or surgical intervention. The effective antischistosomal drugs seemed to be for S.mansoni:oxamniquine I (Mansil @), for s.haematobium: metriphonate (Bilarcil @) and for all human schistosoma species: praziquanta1 (Biltricide @). In the case of spinal cord location of schistosoma granuloma and schistosoma structural lesions in cerebra neurosurgical intervention combined with drug therapy may be necessary.
References w. Schistosomiasis of spinal cord and skin. S Afr Med J 1975; 49:75-g. EL-AHBIADY LM. Left vocal cord paralysis in bilharzial pulmonary aneurysm. J Egypt Med Assoc 1974; 57 (56):232-6. RISELEV VB. Secondary lesions of the nervous system in some infections and toxic-allergic conditions in Zambia. Med J Zambia 1975; 9,5:125-7. GHONEIM MA, ASHAMALLAH A, EL-BOLRAINY. Role Of perineuritis in the atomic dilated bilharzial ureter. Int Surg 1976; 61:411-2. MOSTAFAM,HAB~N MA,ABDEL-MONEIM setal. Peripheral polyneuropathy in bilharziasis. J Egypt Med Assoc 1972; 55:44-59. wo WQ, FRITZLEN T. Subluxation of cervical Spine in major epileptic seizure due to cerebral schistosomiasis. Surg Neurol 1976; 5:333-5. POMPEUF,SAMPAIODELACERDAPR. Subarachnoidhemorrhage due to S. mansoni. J Neurology 1979; 221:203-7.
SAXE N, GORDON
BAMBIBRA
EA, ANDRADE
J DE S,
CESARINI I,RODRIGUEZ PA,
tumoral form of schistosomias: Report of a case with cerebellar involvement. Am J Trop Med Hyg 1984; 33:76-9. LUYENDIIK w, LINDEMAN J.Schistosomiasis (bilharziasis) mansoni of the spinal cord simulating an mtramedullary tumor. Surg Neuroll975; 4:457&l. POOL GM. Tropical paraplegia: A case of transverse myelitis, probably caused by schistosomiasis mansoni. Paraplegia 1978-79; 16:113-7. DRUMMOND
C.A.S.A.The
CX3ASGRIENJ,ANTOINEHM,SALIOUP,HERBELLEAUT,PUTZ c. Bilharziose ctrebrale a mansoni. Rev Neurol (Paris) 1984; 140,
DM, IALLON PM, BACQUET
schistosoma 4:293-5.
IONCRHEERE F.Une
maladie Egyptienne. Bruxelles: Edition De La Fondation Egyptologique, Reine Elisabeth Part Du Cinquantenaire 1944; La Medicine Egyptienne No. I: 33-45. WEBBE G. The six diseasesof W.H.O. Schistosomiasis: some advances. Brit Med J 1981; 283:1104-6. NIE DE J. Het Katayamasyndroom in Suriname. Ned Tijdschr Geneesk 1983; 127:372-3. MINGUETTI G, FERREIRA MVC. Computed tomography in neurocysticercosis. J Neurol Neurosurg Psychiatry 1983; 46:936-42. NASH TE,GARCIA-COYCO C,RUIZ-TIBEN E,NAZARIO-L6PEZ G, TORRES-BORGES A. Differentiation of acute and chronicschistosomiasis of antibody responses to specific schistosome antigens. Am J Trop Med Hyg 1983; 32:776-84. HA, VAZQUEZ
217