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April2011 Vol.48 Issue:      2 Table of Contents
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Spinal Cord Schistosomiasis: Fifteen years experience

Hatem Badr1, Ashraf Shaker1, Mohamed Mansour1, Mohamed Kasem1,

Ahmad Zaher1, Hassan Salama2, Mohamed Safwat1

 

Departments of Neurosurgery1, Neurology2, Mansoura University; Egypt

 



ABSTRACT

Background: After malaria, Schistosomiasis is the second most prevalent tropical disease. The prevalence of oviposition in the CNS of infected persons varies from 0.3 to 30%. The conus medullaris is a primary site of Schistosomiasis either granulomatous or acute necrotizing myelitis. Objective: To report the clinical, radiological and laboratory results of spinal cord Schistosomiasis (SCS) and to design proper therapeutic regimens. Methods: Seventeen patients, 13 males and 4 females, with SCS were enrolled between 1994 and 2009 at Mansoura University Hospitals. Their median age at diagnosis was 19 years (13 to 30 years). Independent neurological, radiological and laboratory assessments were performed for both groups apart from pathological confirmation that was done early in eight patients (group 1). In group 2, 9 patients, indirect hemagglutination test (IHA) for bilharziasis in blood and CSF was performed. Higher positive titer in CSF than serum indicated SCS plus induction of antibilharzial and corticosteroids for 12 months with 3-year follow up. Results: Rate of neurological symptoms of granulomatous intramedullary cord lesion was assessed independently in 16 cases and acute paraparesis in one case. All patients had positive IHA against S. mansoni with median CSF and serum ranges 1/640 and 1/320 respectively. Seven patients (41.18%) had complete recovery, eight patients (47.06%) showed partial recovery and no response was reported in two patients (11.76%) (p=0.005). No recorded mortality in the current registry. Conclusion: Rapid diagnosis of SCS with early medical therapies for 12 months is a crucial tool to complete recovery. [Egypt J Neurol Psychiat Neurosurg.  2011; 48(2): 151-155]

 

Key Words: Spinal cord Schistosomiasis, Schistosoma mansoni

 

Correspondence to Hassan Salama, Department of Neurology, Mansoura University, 35516 El-Jomhoria st. Mansoura; Egypt. Tel.: +20105067491. Email: hassansalama@yahoo.com




 

INTRODUCTION

 

Schistosoma, especially S. mansoni, spinal cord (SCS) lesion is considered as a primary cause of spinal cord parasitic invasion in Egypt. The worldwide prevalence of CNS oviposition has a wide variability (0.3%- 30%)1,2 but in more endemic area with scientific epidemiological studies, SSC is a frequent causes of non-traumatic myelopathies (6%).3

In 1970, El‑Banhawy and Zidan4 mentioned 9 spinal cord bilharzial cases that were verified histopathologically and they discussed briefly the clinical presentation and their management. Then, few cases with bilharzial spinal cord lesions from Egypt, South Africa and Brazil were reported.5

SCS is commonly assault the lower thoracic/upper lumbar regions and is characterized by cauda and lower cord neurologic symptoms as well as radiological, serologic and pathological confirmations.6 According to diverse studies, there are no clear guidelines for proper medical therapies and their duration as antibilharzial and corticosteroids, or superiority to surgery.7-11 Nevertheless, there is a concept for all parasitic diseases including SCS that early treatment provides superior prognosis and diminishes high morbidity and mortality.9,12

This prospective long term study aimed to design proper therapeutic regimens and to describe the characteristics clinical, radiological and laboratory findings of 17 patients with Schistosomal radiculomyelopathy.

 

MATERIALS AND METHODS

 

This prospective study, a collaborative effort by neurosurgery and neurology departments, Mansoura University in Egypt, was conducted from January 1994 to December 2009. Seventeen patients were enrolled with a highly suspicious history of spinal cord Schistosomiasis. They were divided into two groups: Group 1 included 8 patients who had histopathological confirmation and received medical therapy for 12 months and 3 years follow-up; group 2 included 9 patients who received the same therapies but after CSF and serological tests confirmation. Indirect Hemagglutination (Schistosomiasis Fumouze, France) in both serum and CSF against S. mansoni is diagnostic of active parasitic infection with titer ≥ 1⁄160.

After detailed history, routine laboratory tests were done; the investigations included complete blood count, blood urea, serum creatinine, fasting blood sugar, serum potassium, serum sodium, serum calcium, serum phosphorous, liver function tests, ESR, and microscopic stool examination for Schistosomal eggs, biochemistry and cytomorphologic examination of the cerebrospinal fluid obtained by lumbar puncture. In addition, all patients had initial MRI of the dorso-lumbar region (T1, T2, and FLAIR weighted images) and after one year of therapy. Patients were scheduled for 3-year follow up.

For the current study, we classified response to treatment as follow: (1) Full recovery, if patient has complete improvement, (2) Partial recovery, if the patient indicated minor neurological deficits; (3) No recovery, if patient has no improvement whatsoever or severe permanent motor or sensory loss.

 

Statistical Analysis

The demographic, clinical, and technical data were collected using a ‘data collection form’ and entered into a computerized database before statistical analysis. Continuous variables were compared using analysis of variance for repeated measures. P-value less than 0.05was considered statistically significant. All data were expressed as mean ± standard deviation (SD) or patient’s number (n) and percentage (%) as appropriate.

 

RESULTS

 

The seventeen patients enrolled in this study were divided into two groups: group 1 included 8 subjects (7 males and 1 female) group 2 included 9 patients (6 males and 3 females) with a mean age of 20.29±5.8 years. The median age for all patients was 19 years (13-30) with male to female ratio (3.25:1).

The common clinical presentations were progressive motor and sensory symptoms with sphincter disorders in the granulomatous type resembling intramedullary cord lesion (16 cases). While, acute weakness of both lower limbs with sensory level and retention of urine in the acute necrotizing type resembling transverse myelitis was reported in one case.

Bladder dysfunction was a constant finding in all enrolled patients (100%). Paraesthesia, low back pain or radicular pain in the lower limbs was the most frequent early sensory manifestation (88.2%) (Table1).

 

Table 1. Major neurologic finding in all patients with SCS.

 

Neurological finding

n=17

%

Bladder dysfunction

17

100

Lower limb muscle weakness up to walk with support

14

82.4

Paraesthesia/ Lumbar /Radicular pain

15

88.2

 

Nine patients (52.9%) yielded obvious S. mansoni eggs in their stool but indirect hemagglutination (IHA) against S. mansoni antibodies was positive in CSF and serum of all patients. The titre was more elevated in the CSF (median titer 1/640) than the serum (median titer 1/320) as well CSF protein raised in all patients (median 70 mg/dl).

MRI scans were correlated with the clinical findings in all patients (Figure 1). One out of 17 (5.9%) patients showed minimal enhancement with myelitic form (Figure 3) but 16 patients revealed enlargement of spinal cord at conus with contrast enhancement (Figures 1 & 2). Therapeutic outcome is shown in Table 2.

 

Table 2. Therapeutic outcome.

 

Outcome

n=17

%

Full recovery

7

41.18

Partial recovery

8

47.06

No recovery

2

11.76


 

  

 

Figure 1. (A) image is sagittal T1 weighted MRI with gadolinium that showed diffuse enlargement of the conus with irregular enhancement. (B) image is contrast axial T1WI; shows diffuse enlargement of conus medullaris with diffuse hypointense signal, pathology specimen in (C) shows multiple bilharzial ova with terminal spine.

 

 

Figure 2. Bilharzial granuloma of the conus medullaris. (A)Sagittal T2WI; shows diffuse enlargement of the conus with diffuse T2 hyperintense signal (B) Sagittal T2WI of the same patient after six months of treatment with anti-bilharzial therapy and corticosteroid. It shows reduction of the conus swelling.

 

 

 

Figure 3. Schistosomal myelitis of the conus medullaris. Contrast sagittal T1WI

shows mild swelling of the conus with scattered granular enhancement.

 

 


DISCUSSION

 

Spinal cord Schistosomiasis is a rare disease even in endemic areas. In 1968, the first case of spinal Schistosomiasis in Egypt was reported.13 The assumed mechanism that explains predilection of lower spinal cord is the existence of valve-free venous plexus (Batson system) that anatomizes the intra-abdominal and spinal veins. This shunt that becomes more active and patent in cases with increase intra-abdominal pressure permits the S. mansoni eggs to migrate via these plexus to spinal cord.14

There is another hypothesis that mentioned elimination of eggs directly inside the vessels due to the anomalous migration of adult worms; this hypothesis is strengthened by the occasional finding of adult worms and eggs in a row inside vertebral vessels.15

Anyhow, the extension of the lesions depends on the degree of parasitic infestation load and the host's immunologic response. As well, the interval between infection and onset of the spinal cord manifestations varies from several days up to 6 years.16

Spinal cord granuloma was confirmed radiologically in 16 out of 17 patients and histopathologically in 7 patients who were enrolled early in this study. In one case devastating myelitis was diagnosed radiologically and histopathologically (Figure 2). The onset of symptoms in this patient was acute that suggests myelitis while the course in the remaining cases was chronic and progressive form.

The granulomatous or myelitis pathological forms could induce spinal cord damage via mass effect, anterior spinal artery occlusion or extensive immune reaction due to high antigenic structure of Schistosoma mansoni ova what is labeled delayed hypersensitivity reactions.6,17-19

The male predominance in current study (3.25:1) could be explained, besides the pelvic anatomy differences, by greater exposure of men to Schistosomal infestation because the agriculture job is mostly restricted to men in Egypt.20

In current study, the myelitic form (one patient) presented acutely and showed no recovery while in granulomatous forms (16 cases), 7 patients showed total recovery, 8 had partial recovery and 1 showed no recovery.

In most published series, the myelitic form outcome is very poor compared to granulomatous form. This study is in harmony with other high powered studies in spite of severe motor and sensory symptoms in current series.12,21,22

According to current study and others, there is a reliable predictor of CNS involvement; if the patient has higher CSF indirect hemagglutination (IHA) antibodies against S. mansoni titer (1/640) than serum level (1/320). This statement made huge changes in our protocol of SCS diagnosis in last 6 years. We instituted high CSF-IHA titer as surrogate to pathological confirmation as a noninvasive and reliable laboratory test as well there is no outcome differences among both groups.2,22 

The sensitivity of single smear is 50% only, so repeated stool examinations for S. mansoni eggs were performed in all patients of current study. They revealed positive results in 9 cases (52.9%) which are consistent with Paz et al results (60%).10,22

Anyhow, fecal smear is a good positive finding but does not exclude SCS diagnosis. For this reason, the rectal biopsy (snips) is recommended in cases with negative stool examinations but unfortunately this procedure was not done in our series because it is invasive technique and culturally harmful.12

Beyond doubt, the definite diagnosis of spinal cord Schistosomiasis necessitates proper histopathological examination of the spinal cord biopsy that was done in group 1 in current study. In the main, high quality MRI that is sensitive more than specific in SCS and reliable surrogate CSF laboratory tests make the biopsy of the spinal cord as a reserved tool for confusing cases because it is an invasive technique.

The therapeutic regimen in current study included Praziquantel drug in four therapeutic points over one year. It is used 40 mg/kg in 3 divided doses over a day for 3 consecutive days and the drug was ingested by the same previous regimen every 4 months for at least one year. The prednisone (no less than 2 months) was used in initial dose 40 mg/day and tapering over few weeks. Praziquantel is a broad-spectrum antibilharzial drug against adult worms so it prevented further deposition of ova and new granulomata formation. The simultaneous use of corticosteroids was aimed to dampen the immune response and reduce proinflammatory cytokines around intramedullary granuloma.21,23

In current series, 3 out of 4 patients who discontinued steroids early (less than 60 days) developed recurrence of the myelopathy symptoms. In addition, we observed in few cases a clinical improvement with the maintenance of steroid therapy for longer therapeutic period.

Based on current results, SCS is diagnosed in a young patient, from endemic area, has symptoms of lower cord lesion and supported by MRI findings in addition to higher titer of CSF indirect hemagglutination (IHA) test.

In conclusion, although SCS can be managed both medically and surgically, medical regimens are most wise option in clear cut cases and even no role for surgery except in confusing cases and for biopsy that can be considered for avoiding the side effects of surgery and the problems posed by comorbidity.

 

Limitation

We accept that our study is underpowered and limited by the small sample size. We tried to highlight the significance of early diagnosis and management of SCS. So, further adequately powered, multicenter trials are recommended.

 

Acknowledgment

The authors would like to thank all the patients and their families for their patience and cooperation.

 

[Disclosure: Authors report no conflict of interest]

 

REFERENCES

 

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10.      Lambertucci JR. Silva LC. Amaral RS. Guidelines for diagnosis and treatment of schistosomal myeloradiculopathy. Rev Soc Bras Med Trop. 2007; 40(5):544-81.

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13.      El‑Banhawy A. A case of dorsal spinal intramedullary Schistosoma mansoni granuloma. J Egypt Surg Soc. 1969; 4: 130‑3.

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18.      Cohen J, Capildeo R. Schistosomal myelopathy. Br Med J. 1977; 7:1258.

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الملخص العربى

 

خلفيه البحث : يعد مرض البلهارسيا الثاني من حيث الانتشار عالميا بعد مرض الملاريا ويعد الجهاز العصبي وخصوصا نهاية النخاع الشوكي من الأماكن المفضلة لوضع بيض دوده البلهارسيا المعوية (المنسونيه).

الهدف من البحث : هو تقييم الفحص السريري والأشعة بالرنين المغناطيسي والنتائج المختبرية في تشخيص بلهارسيا الجهاز العصبى مع وضع تصور علاجي لمثل هذه الحالات. الطريقة البحثية وخصائص المرض: شمل هذا البحث سبعة عشر مريضا منهم ثلاثة عشر ذكور وأربعة إناث مصابون بمرض بلهارسيا النخاع لشوكي في الفترة من 1994 إلي 2009. متوسط أعمارهم تسعة عشر عاما. تم تشخيص ثماني حالات في مستشفيات جامعة المنصورة  في بداية البحث بالرنين المغناطيسي و الفحص بالمعمل المناعي مع أخذ عينه من مكان الاصابه و لكن باقى الحالات لم يتم أخذ عينات منهم للفحص الباثولوجي ويعزي ذلك أن نسبه الاختبار غير المباشر المناعي في سائل النخاع الشوكي كانت مرتفعة لدرجه تشخيصيه شبه مؤكده. وقد تم إعطاء كل المرضي علاج برازيكوانتيل لمدة عام وعقار الكورتيكوستيرويد لمدة شهرين على الأقل.

 نتائج البحث : ظهرت أعراض إصابة الجهاز العصبي من عدم التحكم في البول والضعف في الساقين و خلافه في حالات حبيبات آفة البلهارسيا في 16 مريض كما أصيبت حاله واحده بخزل حاد بالأطراف السفلية وقد لوحظت استجابة كاملة في سبعة مرضي بينما كانت جزئيه في ثماني مرضي ولم يتم شفاء اثنين من المرضي تماما وفي خلال مدة ثلاث سنوات لم يتم تسجيل أى حالة وفاة أثناء متابعة كل المرضي.

خلاصة البحث : خلصت هذه الدراسة إلي النصح بالتشخيص المبكر مع إعطاء كلا الدوائيين لفترة كافيه.



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