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Introduction: In contrast to intracerebral meningiomas spinal manifestations are relatively rare, accounting for approximately 1% of all meningiomas. Mostly, these are slow growing tumours becoming symptomatic due to spinal cord compression. Initially, local pain is one of the leading symptoms; mostly they remain undiagnosed until neurological deficits arise. The incidence increases with age and shows a remarkable female predominance. We here present an unusual case of a craniospinal meningioma with an uncommon clinical picture.
Patient: A 43-year old man complained about recurrent rippling and quivering movements of the tongue for one year. These episodes last for seconds and occur several times a day. He described swelling in his tongue which caused speech difficulties. In addition he was disturbed by strong, prickly neck pain. Needle electromyography revealed myokymic discharges of the tongue, which are suggestive of a hypoglossal nerve lesion. A MRI scan of the brain disclosed a space occupying meningioma in the area of the hypoglossal canal with accompanying displacement of the medulla oblongata. A partial tumour resection with decompression of the medulla and the hypoglossal nerve was performed. Histologically, it was classified as a fibrous meningioma (WHO grade I) with a low proliferation index (MIB-1 3%). Because of a subtotal resection and the eloquent region focal, fractionated irradiation was applied postoperatively (cumulative dose 50.4 Gray) with an ongoing stable disease for 3.5 years.
Conclusion: Meningiomas are mostly slow growing benign tumours with an excellent prognosis compared to other brain tumours. However, they can cause significant morbidity in anatomically distinct regions like the skull base, the cerebrospinal junction or the spine. In case of partial resection postoperative radiotherapy is likely to achieve long term tumour control. In rare cases isolated affection of the cranial nerves can be caused by a meningioma and has to be taken into account like in our male patient with tongue myokymia caused by a meningioma of the craniospinal junction.