- Case report
- Open Access
- Open Peer Review
Cauda equina syndrome as the initial presenting clinical feature of medulloblastoma: a case report
https://doi.org/10.1186/1752-1947-6-135
© Al Otaibi et al.; licensee BioMed Central Ltd. 2012
- Received: 22 October 2011
- Accepted: 23 May 2012
- Published: 23 May 2012
Abstract
Introduction
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.
Case presentation
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.
Conclusions
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.
Keywords
- Cauda equina syndrome
- Drop metastasis
- Medulloblastoma
- Paraparesis
Background
Medulloblastoma is the second most common posterior fossa tumor in children. It accounts for 40% of posterior fossa tumors in the pediatric age group [1]. 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 [2]. 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.
Case presentation
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.
(A) Axial magnetic resonance imaging T1-weighted image with gadolinium, showing a posterior fossa tumor. (B) Sagittal magnetic resonance imaging T1-weighted image with gadolinium showing posterior fossa tumor (arrow) and frontobasal tumor (arrowhead), suggestive of cerebrospinal fluid (CSF) metastasis. (C,D) Post-treatment, one-year follow-up axial and sagittal magnetic resonance imaging revealed regression of the disease.
(A) Pre-treatment spinal T1-weighted image with gadolinium demonstrating a nodular extra-medullary enhancement (arrowhead) and spinal block in the lumbar region (arrow). (B) Post-treatment one-year follow-up spinal magnetic resonance imaging revealed regression of the disease.
Morphology of classic medulloblastoma as shown in (A) (hematoxylin and eosin stain; original magnification, 200×) with strong and uniform expression of synaptophysin (B) (Novocastra™, clone 27 G12, 1:100; original magnification, 200×) and BAF47 (C) (BD Biosciences, clone 25, 1:200; original magnification, 200×).
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.
Discussion
Selected reported cases of cauda equine syndrome as the main initial presenting clinical features
Author (year) and reference | Age | Gender | Clinical presentation |
---|---|---|---|
Wang [7] | 28 years | Male | Progressive lower limb weakness |
Alla [11] (article in French) | 64 years | Male | Radiculitis features followed by cauda equina syndrome features |
Lee [8] | 4 years | Female | Cauda equina syndrome features (lower limb weakness and urinary retention) |
Present case report | 3 years | Male | 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 [12]. Subsequently, a similar case was described by Stanley et al. in 1986 [5]. Recently, Inoue and colleagues reported on a child who had progressive weakness as the initial presenting features for intramedullary spinal metastasis [14]. In general, medulloblastoma spinal metastasis varied from nodular lesions to complete spinal block [5]. 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.
Conclusions
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.
Consent
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.
Declarations
Authors’ Affiliations
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