Hemifacial spasm can be the presenting symptom of a fourth ventricle tumour. A short case-illustrated review and pathogenetic considerations

Department of Neuroscience, Padua University Hospital, Padova, Italy.
Acta Neurochirurgica (Impact Factor: 1.77). 09/2011; 153(12):2383-7. DOI: 10.1007/s00701-011-1137-8
Source: PubMed


In this short case-illustrated review we aimed to analyse the possible nuances of hemifacial spasm (HFS) as the presenting symptom of a tumour of the fourth ventricle. The issue is remarkable since HFS can be secondary to a fourth ventricle tumour, even when no other neurological signs are reported. In addition, the possible presentation with only upper facial muscle involvement, as in the presented case, can be deceitful because this is characteristic of the benign and much more frequent "typical" form. Based on our intra-operative data and on the previously reported cases, we think that pathogenesis could be referable to the facial nerve nucleus involvement and that clinical nuances could be related to the specific somatotropy of the nucleus under the fourth ventricle floor that, as in our case, can be infiltrated by tumour. Resolution of the disorder can usually be obtained after the complete resection of the tumour that in the reported case resulted a subependymoma (WHO grade I), so far never described in literature associated with HFS.

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    ABSTRACT: Hemifacial spasm (HFS) is generally caused by a neurovascular conflict (NC) at the root exit zone (REZ) of the facial nerve at the brainstem. Although a direct compression to the seventh cranial nerve (CN) by the anterior inferior cerebellar artery (AICA) is generally the most frequent cause, secondary HFS may be related to other pathological conditions. HFS due to an intracranial mass lesion is exceptionally rare and it has been reported in very few cases. The online database was searched for English-language articles reporting cases of HFS due to brainstem mass lesions and the possible pathophysiological mechanisms involved in its genesis. A 47-year-old man affected by an anaplastic astrocytoma of the brainstem at the level of the ponto-medullary junction developed right HFS. He underwent a subtotal surgical removal of the tumor with complete resolution of the HFS. This is the ninth reported case of HFS caused by an intrinsic brainstem tumor. The exceptional rarity of the relationship between intra-axial tumors and peripheral HFS was analyzed.
    No preview · Article · Nov 2014 · Neurosurgical Review