Cauda equina syndrome as the initial presenting clinical feature of medulloblastoma: a case report
© Al Otaibi et al.; licensee BioMed Central Ltd. 2012
Received: 22 October 2011
Accepted: 23 May 2012
Published: 23 May 2012
Medulloblastoma is one of the most common pediatric brain malignancies. The usual presenting clinical features are related to posterior fossa syndrome or/and hydrocephalus. Cauda equina syndrome is a very rare presentation for this disease.
We describe the case of a three-year-old boy with cauda equina syndrome as the initial presenting clinical feature for medulloblastoma. He was initially diagnosed as having a spinal tumor by magnetic resonance imaging scan. Subsequently, a cranial magnetic resonance imaging scan revealed a posterior fossa tumor with features of dissemination. He had substantial improvement after treatment. This case report is complemented by a literature review related to this unusual presentation.
Medulloblastoma primarily presenting with cauda equina syndrome is very rare. However, spinal drop metastasis should be considered in the pediatric age group to avoid suboptimal management.
KeywordsCauda equina syndrome Drop metastasis Medulloblastoma Paraparesis
Medulloblastoma is the second most common posterior fossa tumor in children. It accounts for 40% of posterior fossa tumors in the pediatric age group . The clinical presentation of this malignant tumor is usually related to the posterior fossa tumor mass effect and/or obstructive hydrocephalus. Cerebrospinal fluid (CSF) dissemination to the cranio-spinal axis occurs in 30% to 40% of cases . However, medulloblastoma primarily presenting with symptoms related to spinal metastasis is extremely rare [2, 3]. To date, there are only a limited number of cases that have been reported in the literature. Here, we report the case of a child with medulloblastoma who presented with progressive paraparesis caused by cauda equina drop metastasis without symptoms related to the primary intracranial tumor.
Our patient was a three-year-old Arabic boy who presented to a peripheral medical center with a two-month history of lower back pain followed by progressive paraparesis. He had no symptomatology related to posterior fossa syndrome or increased intracranial pressure. Six weeks after the start of his symptoms, he developed urinary and bowel incontinence. During the physical examination, the boy was fully awake and cooperative. All cranial nerves were intact. His pupils were 2 mm, bilaterally equal and normally reacting to light. He had full extra-ocular movement with no nystagmus. There was no papilloedema. An examination of the upper extremities was normal. He had symmetrical lower limb weakness with muscle group power grade 1/5 distally and 3/5 proximally associated with hypotonia, absent reflexes and diminished sensations up to the L1 dermatome. His anal tone was reduced. Cauda equina syndrome was suspected, for which he underwent a magnetic resonance imaging (MRI) scan of the spine that revealed spinal canal filling and lesions in the thoracic and lumbar regions. At that stage, he was referred to our institution as a patient with a spinal tumor.
During his treatment course he received rehabilitation therapy. Three months after his initial treatment, he had substantial improvement in his overall neurological functions. He started to walk without assistance and was able to control his bladder and bowel functions. A neuroaxis follow-up MRI after one year showed regression of his cranial and spinal disease (Figures 1C, D and 2B). At the time of writing this report, he has retained good neurological function.
Selected reported cases of cauda equine syndrome as the main initial presenting clinical features
Author (year) and reference
Progressive lower limb weakness
Alla  (article in French)
Radiculitis features followed by cauda equina syndrome features
Cauda equina syndrome features (lower limb weakness and urinary retention)
Present case report
Cauda equina syndrome features
However, spinal intramedullary metastasis of medulloblastoma rarely occurs. To date, there have been a limited number of cases with spinal intramedullary metastasis reported in the literature [12–14]. Zumpano, in 1978, reported on a patient with intramedullary metastatic medulloblastoma several weeks after resecting the posterior fossa tumor . Subsequently, a similar case was described by Stanley et al. in 1986 . Recently, Inoue and colleagues reported on a child who had progressive weakness as the initial presenting features for intramedullary spinal metastasis . In general, medulloblastoma spinal metastasis varied from nodular lesions to complete spinal block . Our patient had extramedullary nodules, leptomeningeal enhancement and spinal block at the lumbar region. The cranial MRI findings were of sufficient importance to alter the management of our patient and to pave the way for different treatment strategies.
Cauda equina syndrome as the presenting clinical feature for medulloblastoma is rare. Similar clinical presentations in the pediatric age group should raise the suspicion of cranial neoplastic pathology. Cranial neuroimaging is important to rule out drop metastasis and to optimize patient management.
Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Allen JC: Childhood brain tumors: current status of clinical trials in newly diagnosed and recurrent disease. Pediatr Clin North Am. 1985, 32: 633-651.PubMedGoogle Scholar
- Park TS, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE: Medulloblastoma: clinical presentation and management. Experience at the hospital for sick children, Toronto, 1950-1980. J Neurosurg. 1983, 58: 543-552. 10.3171/jns.1983.58.4.0543.View ArticlePubMedGoogle Scholar
- Laurent JP: Brain tumors in children. J Pediatr Neurosci. 1985, 1: 15-32.View ArticleGoogle Scholar
- Meyers SP, Wildenhain SL, Chang JK, Bourekas EC, Beattie PF, Korones DN, Davis D, Pollack IF, Zimmerman RA: Postoperative evaluation for disseminated medulloblastoma involving the spine: contrast-enhanced MR findings, CSF cytologic analysis, timing of disease occurrence, and patient outcomes. AJNR Am J Neuroradiol. 2000, 21: 1757-1765.PubMedGoogle Scholar
- Stanley P, Suminski N: The incidence and distribution of spinal metastases in children with posterior fossa medulloblastomas. Am J Pediatr Hematol Oncol. 1988, 10: 283-287. 10.1097/00043426-198824000-00002.View ArticlePubMedGoogle Scholar
- Pezeshkpour GH, Henry JM, Armbrustmacher VW: Spinal metastases: a rare mode of presentation of brain tumors. Cancer. 1984, 54: 353-356. 10.1002/1097-0142(19840715)54:2<353::AID-CNCR2820540229>3.0.CO;2-R.View ArticlePubMedGoogle Scholar
- Wang AM, Jolesz F, Rumbaugh CL, Zamani AA, Haykal HA: Thoracic spinal metastasis from unexpected cerebellar medulloblastoma: the value of MRI. Comput Radiol. 1986, 10: 161-166. 10.1016/0730-4862(86)90100-9.View ArticlePubMedGoogle Scholar
- Lee LW, Hsu SW, Shih TY, Lee RJ, Kuo YL, Weng HH, Lui CC: Cerebellar medulloblastoma with intraspinal metastasis presenting with acute paraplegia: a case report. Chin J Radiol. 2000, 25: 255-258.Google Scholar
- Khafaga Y, Kandil AE, Jamshed A, Hassounah M, DeVol E, Gray AJ: Treatment results for 149 medulloblastoma patients from one institution. Int J Radiat Oncol Biol Phys. 1996, 35: 501-506. 10.1016/S0360-3016(96)80012-5.View ArticlePubMedGoogle Scholar
- Barreiro F, Torres MSP: The cauda equina syndrome in an adult, caused by a medulloblastoma. Arch Neurobiol (Madr). 1974, 37: 393-400.Google Scholar
- Alla P, de Jaureguiberry JP, Carloz E, Gisserot O, Valance J, Jaubert D: Meningoradiculitis revealing an occult medulloblastoma-PNET in an adult. Rev Neurol (Paris). 1998, 154: 542-545.Google Scholar
- Zumpano BJ: Spinal intramedullary metastatic medulloblastoma. Case report. J Neurosurg. 1978, 48: 632-635. 10.3171/jns.1978.48.4.0632.View ArticlePubMedGoogle Scholar
- Barnwell SL, Edwards MS: Spinal intramedullary spread of medulloblastoma. Case report. J Neurosurg. 1986, 65: 253-255. 10.3171/jns.1986.65.2.0253.View ArticlePubMedGoogle Scholar
- Inoue T, Kumabe T, Takahashi T, Nakajima T, Watanabe M, Tominaga T: Spinal intramedullary metastasis of medulloblastoma at initial diagnosis. Childs Nerv Syst. 2007, 23: 113-116.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.