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Atypical presentations of conus medullaris and filum terminale myxopapillary ependymomas
Mohammed Sa'adah1, Kassem Al Shunnar, Loai Saadah
1Neurology Department, Rashid Hospital, Dubai, United Arab Emirates. mohdsaadah@hotmail.com
Abstract:
Out of 27 cases of spinal ependymomas seen during a 21 year period (1978-1999), we observed three out of 12 myxopapillary tumours of the conus medullaris and filum terminale with atypical presentations. All the three cases presented with subarachnoid haemorrhage and intracranial hypertension. In addition, the second patient developed persistently low CSF sugar, while the third patient developed hydrocephalus, acute autonomic crisis and SIADH. Although subarachnoid haemorrhage (SAH), intracranial hypertension and hydrocephalus were previously scarcely reported in the literature, the low CSF sugar, acute autonomic crisis and SIADH were never reported singly or in combination. Up to our best knowledge, this is the first report that clearly outlines all these atypical manifestations in this particularly interesting neoplasm. Hence, the above challenging clinical presentations should be borne in mind with cryptic presentations of lower spinal cord and filum terminale ependymomas. A proposal of the mechanism of their production is suggested.
Insights
Atypical spinal ependymomas can present with rare symptoms like subarachnoid hemorrhage and intracranial hypertension. This study highlights unusual neurological complications, including low CSF sugar and autonomic crisis, in these rare tumors.
Area of Science:
- Neuro-oncology
- Spinal Cord Neoplasms
- Neurological Manifestations
Background:
- Spinal ependymomas, particularly myxopapillary types in the conus medullaris and filum terminale, are rare.
- Atypical presentations of these tumors are not well-documented in existing literature.
Purpose of the Study:
- To report and analyze rare and atypical clinical presentations of myxopapillary ependymomas.
- To highlight previously unreported neurological complications associated with these spinal tumors.
Main Methods:
- Retrospective review of 27 spinal ependymoma cases over 21 years (1978-1999).
- Detailed analysis of three cases with unusual presentations of myxopapillary tumors.
Main Results:
- Three out of 12 myxopapillary tumors exhibited atypical features.
- Common atypical findings included subarachnoid hemorrhage and intracranial hypertension.
- Unique complications observed were low cerebrospinal fluid (CSF) sugar, hydrocephalus, acute autonomic crisis, and syndrome of inappropriate antidiuretic hormone secretion (SIADH).
Conclusions:
- Myxopapillary ependymomas can present with a spectrum of rare and severe neurological complications.
- Clinicians should consider these atypical manifestations in the differential diagnosis of lower spinal cord and filum terminale tumors.
- Further research into the pathogenic mechanisms of these presentations is warranted.