- Department of Neurosurgery, Centro Hospitalar de Lisboa Central EPE, Lisboa, Portugal
Department of Neurosurgery, Centro Hospitalar de Lisboa Central EPE, Lisboa, Portugal
DOI:10.4103/2152-7806.132235Copyright: © 2014 Pappamikail L This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Pappamikail L, Fernandes P, Casimiro Gonçalves. Medullary schistosomiasis. Surg Neurol Int 09-May-2014;5:66
How to cite this URL: Pappamikail L, Fernandes P, Casimiro Gonçalves. Medullary schistosomiasis. Surg Neurol Int 09-May-2014;5:66. Available from: http://sni.wpengine.com/surgicalint_articles/medullary-schistosomiasis/
Background:Schistosomal infestation of the central nervous system is a rare cause of cord compression, although a predominant one in endemic areas.
Case Description:A 38-year-old male, native of Ivory Coast, with a history of 1 month of progressive paraparesis, neurogenic bladder, diminished deep tendon reflexes of the lower limbs, and sensory level. The magnetic resonance imaging (MRI) showed a medullary lesion at D4-D5 level, suggestive of an intramedullary tumor. Laminotomy of D3 to D5 and excision of a grayish white lesion according to a preliminary histopathologic review suggestive of a high grade glioma. Definitive histopathology review established the diagnosis of medullary schistosomiasis.
Conclusion:Schistosomal myeloradiculopathy should be considered in patients presenting with cord compression or features of transverse myelitis, especially in patients from endemic areas or low social economic settlements.
Keywords: Differential diagnosis, medullary schistosomiasis, outcome
According to the World Health Organization (WHO) about 200 million people are infected with schistosomiasis worldwide.[
We report a case of a 38-year-old male, native of Ivory Coast, living in Portugal for the past 6 years, presenting with a history of 1 month of deterioration of motor function in his lower limbs with symptoms of neurogenic bladder (urinary retention and overflow incontinence). The patient was admitted to our emergency room, with assymetric paraparesis with motor strength grade 3 (right lower limb) and 4 (left lower limb), with diminished deep tendon reflexes of the lower limbs, sensory level at D10, and urinary retention ensuring the need to bladder catheterization, with otherwise unremarkable neurologic examination.
The magnetic resonance imaging (MRI) documented a moderate and diffuse enlargement of medullary cord, in the D4-D5 region, with a central nonhomogeneous T2 hypersignal as well as heterogeneous enhancement after intravenous injection of gadolinium, with a granular pattern (disruption of the blood-brain barrier) [
The postoperative period was uneventful, with slight decrease in lower limb proprioception and suspended sensory level between D10 and D12 in the immediate postoperative period, which progressively disappeared [
The diagnosis of schistosomiasis was based solely on the demonstration of eggs and adult worm in the granuloma supplied for histopathology review [
On histopathological examination, the spinal cord tissue showed edema, demyelination, and a chronic inflammatory cellular infiltrate with some eosinophils. Numerous eggs were observed, some containing miracidia with intact nuclei and a few showed calcifications. Occasional giant cells were seen around the eggs.
As described by Salomão et al.,[
After diagnosis, the patient was treated medically with praziquantel at a dose of 40 mg/kg/day in two doses 6 h apart for two consecutive days and steroids (prednisolone 5 mg/day for 6 weeks).
The postoperative MRI, under steroid and antihelminthic, showed progressive reduction in the edema and enlargement of the cord, with concurrent regression of neurologic deficits, allowing deambulation with support of a clutch at his 2-month follow-up. At 6 months, deambulation was independent, maintaining urinary catheterization, however.
S. mansoni (as well as S. haematobium) is the commonest species in the East African and Middle East Region, it also is present in some parts of South America and the Caribbean.[
The adult worm form resides in the mesenteric or pelvic venules (S. mansoni is more common in the superior mesenteric veins), where the female releases the eggs, which move toward the lumen of the intestines. When in contact with water, the eggs swell due to osmotic pressure and the shell breaks open freeing the motile, ciliated larvae (miracidia), which penetrate the slow-moving snails that serve as intermediate hosts. Here, they transform into sporocysts, which mature into cercaria, which in turn swim and penetrate the skin of human host. The cercaria mature in the bloodstream and eventually set up in the aforementioned venules.[
The most accepted hypothesis of dissemination to the medullary cord is based on Batson's studies,[
The lateral spike of the egg of the Schistosoma mansoni may impede its further progress in embolizing the brain (the lower mass and absence of lateral spike in S. japonicum, make it the species responsible for cerebral infestation).[
Schistosomal myelopathy tend to occur early after infection and is more likely to be symptomatic than cerebral schistosomiasis. It may present as an acute or subacute myelopathy that may be accompanied by polyradiculitis, bowel or bladder disfunction, paraparesis, paraplegia, lumbosacral pain, and sensory disturbances.[
There are two described forms of medullary disease to Schistosoma spp: Transverse myelitis (typically to S. haematobium; microscopic inflammatory response, with necrosis, vacuolization, and atrophy of the nervous tissue) and granulomatous or tumoral form (typically to S. mansoni) due to the inflammatory response surrounding the egg, frequently in the medullary cone. Spinal schistosomiasis can also present as a progressive or acute paraparesis or tetraparesis, albeit a rare form of presentation.[
Treatment with antischistosomal drugs interrupts egg production and fibroblastic stimulating factors by destroying the adult worm, thus limiting the inflammatory reaction of the central nervous system and granuloma formation.[
Schistosomal myeloradiculopathy should be considered in patients presenting with cord compression or features of transverse myelitis, especially in patients from endemic areas or low social economic settlements. The typical presentation, with acute or subacute myelopathy was not present in this case, but instead a delayed presentation, with the late onset of myelopathy of rapid progression of parapresis with sensory disturbances and bladder dysfunction, which were not directly attributable to any specific immunological or individual variation.
It is our assumption that in light of not being previously diagnosed with Schistosomal infestation and therefore not receiving treatment for eradication, this allowed for the continuous production of viable eggs, which eventually disseminated to the medullary cord.[
The imaging pattern in the MRI, although not present in every case, when observed should suggest the diagnosis, which is characterized by linear central enhancement with gadolinium, surrounded by multiple punctiform nodules in a tree-like pattern.[
Diagnosis from CSF studies and stool/urine/rectal biopsy might obviate need for surgery, especially in transverse myelitis. However, in deteriorating neurological conditions, urgent surgical intervention followed by antischistosomal drug therapy remains the treatment of choice, ranging from simple decompressive laminectomy to mass exeresis.[
In this patient, the diagnosis of neuroschistosomiasis was not considered initially, being that there was no history suggesting primary infection, the long period (6 years) between presumed exposure and development of symptoms (with myelopathy occurring early after infection being the norm)[
It is our belief, as is of other authors,[
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