Spinal and cranio-cervical mycetoma: A difficult surgery, with poor prognosis.

BACKGROUND: Few central nervous systems (CNS) cases of actinomycetoma have been recorded in the literature, and most were reported in tropical and subtropical regions. The management of this invasive infection is difficult, especially when it affects the spine and the cranio-cervical regions. CASE: We report an unusual case of a cranio-cervical junction actinomycetoma, in a patient presenting a cerebellar syndrome from brainstem compression. The CT scan showed a compressive solid osteolytic lesion in the cranio-cervical junction. The patient underwent cranio-cervical decompression and lesion resection. The diagnosis of actinomycetoma was confirmed on immune-histochemistry and molecular analysis. At 4 months' follow-up, the patient presented a fatal recurrence disseminating within the cerebellum and the spine. CONCLUSION: The surgical treatment of CNS actinomycetoma presented poor prognosis and a disseminating recurrence. We believe that clinicians and surgeons must be informed about these "new" infectious pathologies that are so difficult to treat, especially with the arrival of migrant patients from endemic countries in conflict.

Intracranial nociception.

Understanding intracranial nociceptive innervation is essential to understand the pathophysiology of headaches. Our knowledge about human intracranial nociception comes from sparse observations during neurosurgical procedures performed in awake patients, from human anatomical studies and from experimental studies in animals. In this article we review the anatomical and functional organization underlying nociceptive innervation. Intracranial nociception is mainly mediated by the trigeminal system, except in the posterior cranial fossa that is innervated by the first cervical roots. For decades, the dura mater, its vessels and major cerebral blood vessels were considered as the only intracranial pain-sensitive structures. Recent animal and human studies have suggested that smaller brain arteries and potentially pia mater might also be pain sensitive. Nociceptive neurons innervating intracranial blood vessels project via the ophthalmic division (V1) to the trigeminal ganglion and store several neurotransmitters including glutamate, substance P and calcitonin gene-related peptide (CGRP). The trigeminal ganglion, root and brainstem nuclei have a specific topographic and functional somatotopy. Progressive transition between the trigeminal spinal nucleus and the dorsal horn of the cervical spinal cord, and convergence of nociceptive inputs from the face, intracranial structures and the occipital area on the so-called "trigemino-cervical complex" may explain some headache features, relations between facial and occipital pain, and efficacy of occipital nerve stimulation in headache. The specific anatomic organization of the trigeminal system, from the primary-order neuron in the trigeminal ganglion, to the second-order neuron is the trigeminal nuclei, may explain a part of the various characteristics of headaches.

Unilateral vagus nerve lesion revealing clival meningocele.

INTRODUCTION: Cranial nerve lesions can be secondary to a space-occupying lesion of the skull base compressing adjacent nerves. CASE REPORT: We report the case of an 84-year-old man, who presented with rapid and concomitant onset of dysphagia and ipsilateral recurrent laryngeal nerve paralysis, suggesting an isolated lesion of the vagus nerve. MRI revealed a diagnosis of previously unknown clival meningocele. DISCUSSION: Unilateral vagus nerve paralysis constitutes an exceptional mode of presentation of meningocele. Only a few isolated cases of clival meningocele have been reported to date, with no cranial nerve repercussions. The symptomatic management adopted in this case allowed rapid improvement of the patient's disorders.

Long term surgical results of 154 petroclival meningiomas: A retrospective multicenter study.

BACKGROUND: Outcomes of petroclival meningiomas (PCM) (morbidity, permanent cranial nerves deficit, tumor removal and recurrence) are inconsistent in the literature, making it a challenge to predict surgical morbidity. METHODS: A multicenter study of patients with PCMs larger than 2.5cm between 1984 and 2017 was conducted. The authors retrospectively reviewed the patients' medical records, imaging studies and pathology reports to analyze presentation, surgical approach, neurological outcomes, complications, recurrence rates and predictive factors. RESULTS: There were 154 patients. The follow-up was 76.8 months on average (range 8-380 months). Gross total resection (GTR) was achieved in 40 (26.0%) patients, subtotal resection (STR) in 101 (65.6%), and partial resection in 13 (8.3%). Six (2.6%) perioperative deaths occurred. The 5-year, 10-year and 15-year progression-free survival (PFS) of GTR and STR with radiation therapy (RT) was similar (100%, 90% and 75%). PFS of STR without adjuvant radiation was associated with progression in 71%, 51% and 31%, respectively. Anterior petrosectomy and combined petrosectomy were associated with higher postoperative CN V and CN VI deficits compared to the retrosigmoid approach. The latter had a significantly higher risk of CN VII, CN VIII and LCN deficit. Temporal lobe dysfunction (seizure and aphasia) were significantly associated with the anterior petrosectomy approach. CONCLUSIONS: Our study shows that optimal subtotal resection of PCMs associated with postoperative RT or stereotactic radiosurgery results in long-term tumor control to equivalent radical surgery. Case selection and appropriate intraoperative judgement are required to reduce the morbidity.

Cerebellopontine angle gangliogliomas: Report of two cases.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA). POPULATION AND METHODS: The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification. The postoperative course was uneventful without any adjuvant treatment and the 5-year imaging follow-up indicated a stable remnant tumor. The second patient was a 35-year-old male who presented with acute vertigo and imbalance associated with recent prominent headaches; MR imaging showed a large heterogeneous and post-contrast enhanced tumor mass located in the CPA cistern with a mass effect on the brain. An optimal subtotal surgical resection was performed. The pathologists concluded a WHO grade III ganglioglioma. In spite of adjuvant radiotherapy and chemotherapy, the evolution proved unfavorable and patient died from cancer complications within a 2-year period. In both cases, the precise origin of the tumor could not be clearly identified even if the major component was present in the cisternal space. CONCLUSION: Gangliogliomas growing into the cisternal spaces are exceedingly rare particularly in the CPA. Due to its infiltrating behavior and major difficulties to identify the tumor margins, total resection is not routinely feasible. The histological grading is the most important predictor for oncological prognosis.

[Multiple meningiomas]. / Méningiomes multiples.

Multiple meningiomas (MMs) or meningiomatosis are defined by the presence of at least 2 lesions that appear simultaneously or not, at different intracranial locations, without the association of neurofibromatosis. They present 1-9 % of meningiomas with a female predominance. The occurrence of multiple meningiomas is not clear. There are 2 main hypotheses for their development, one that supports the independent evolution of these tumors and the other, completely opposite, that suggests the propagation of tumor cells of a unique clone transformation, through cerebrospinal fluid. NF2 gene mutation is an important intrinsic risk factor in the etiology of multiple meningiomas and some exogenous risk factors have been suspected but only ionizing radiation exposure has been proven. These tumors can grow anywhere in the skull but they are more frequently observed in supratentorial locations. Their histologic types are similar to unique meningiomas of psammomatous, fibroblastic, meningothelial or transitional type and in most cases are benign tumors. The prognosis of these tumors is eventually good and does not differ from the unique tumors except for the cases of radiation-induced multiple meningiomas, in the context of NF2 or when diagnosed in children where the outcome is less favorable. Each meningioma lesion should be dealt with individually and their multiple character should not justify their resection at all costs.