13 research outputs found

    Preliminary Results of a Prospective Study on Methods of Cranial Reconstruction

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    Given its biological and anatomic features, autologous bone is the first choice for cranioplasty after bone decompression. When autologous bone is not available or must be replaced, surgeons can choose among various materials to create an alloplastic cranioplasty. The Italian Society for Neurosurgery promoted a prospective study conducted at 4 Italian neurosurgical units to compare different methods of cranioplasty and to assess the clinical results and incidence of complications

    Endoscopic endonasal approach for craniovertebral junction pathology: A review of the literature

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    Object: The transoral approach is the gold standard for ventral decompression of the brainstem caused by craniovertebral junction (CVJ) pathology. This approach is often associated with significant morbidity, related to swallowing and respiratory complications. The endoscopic endonasal approach was introduced to reduce the rate of these complications. However, the exact role of this approach in the treatment of CVJ pathology is not well defined. Methods: A comprehensive literature search was performed to identify series of patients with pathology of the CVJ treated via the endoscopic endonasal approach. Data on patient characteristics, indications for treatment, complications, and outcome were obtained and analyzed. Results: Twelve studies involving 72 patients were included. The most common indications for treatment were rheumatoid pannus (38.9%) and basilar invagination (29.2%). Cerebrospinal fluid leak was found in 18% of cases intraoperatively and 4.2% of cases postoperatively. One case of meningitis complicated by sepsis and death represents the procedure-related mortality of 1.4%. Of the patients without preoperative swallowing impairment, 95% returned to oral feeding on the 3rd postoperative day. Ninety-three percent of patients experienced improvement in neurological symptoms after the procedure. Conclusions: The endonasal endoscopic approach is effective for the treatment of neural compression caused by CVJ pathology. It offers advantages such as lower rates of postoperative dysphagia and respiratory complications when compared with the more traditional transoral approach. However, these 2 approaches should be seen as complementary rather than alternatives. Patient-related factors as well as the surgeon's expertise must be considered when making treatment decisions

    Oxygen-Ozone Therapy for Herniated Lumbar Disc in Patients with Subacute Partial Motor Weakness Due to Nerve Root Compression: the First 13 Cases

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    none8Intradiscal oxygen-ozone (O2-O3) chemonucleolysis is a well-known effective treatment for pain caused by protruding disc disease and nerve root compression due to bulging or herniated disc. The most widely used therapeutic combination is intradiscal injection of an O2-O3 mixture (chemonucleolysis), followed by periradicular injection of O2-O3, steroid and local anaesthetic to enhance the anti-inflammatory and analgesic effect. The treatment is designed to resolve pain and is administered to patients without motor weakness, whereas patients with acute paralysis caused by nerve root compression undergo surgery 24- 48h after the onset of neurological deficit. This paper reports on the efficacy of O2-O3 chemonucleolysis associated with anti-inflammatory foraminal injection in 13 patients with low back pain and cruralgia, low back pain and sciatica and subacute partial motor weakness caused by nerve root compression unresponsive to medical treatment. All patients were managed in conjunction with our colleagues in the Neurosurgery Unit of Bellaria Hospital and the IRCCS Institute of Neurological Sciences, Bologna. The outcomes obtained are promising: 100% patients had a resolution of motor weakness, while 84.6% had complete pain relief. Our results demonstrate that O2-O3 therapy can be considered a valid treatment option for this category of patients.noneMassimo Dall'Olio;Ciro Princiotta;Luigi Cirillo;Caterina Budai;Fabio de Santis;Stefano Bartolini;Elena Serchi;Marco LeonardiMassimo Dall'Olio;Ciro Princiotta;Luigi Cirillo;Caterina Budai;Fabio de Santis;Stefano Bartolini;Elena Serchi;Marco Leonard

    Anti-Saccades in Cerebellar Ataxias Reveal a Contribution of the Cerebellum in Executive Functions

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    Objective: Increasing evidence suggests a cerebellar contribution to modulate cognitive aspects of motor behavior and executive functions. Supporting findings come from studies on patients with neurodegenerative diseases, in which however, given the extent of the disease, the specific role of the cerebellum, could not be clearly isolated. Anti-saccades are considered a sensitive tool to test executive functions. The anti-saccade underlying neural network, consisting of different cortical areas and their downstream connections including the lateral cerebellum, has been largely clarified. To separate the role of the cerebellum with respect to other cortical structures in executive control, we compared the anti-saccade performances in two distinct cohorts of patients with cerebellar disorders (with and without cerebral cortical involvement). Methods: Eye movements during the execution of anti-saccades were recorded in 12 patients with spinocerebellar ataxia type 2 (a cortical-subcortical neurodegenerative disease), 10 patients with late onset cerebellar ataxia (an isolated cerebellar atrophy), and 34 matched controls. Results: In the anti-saccade task, besides dynamic changes already demonstrated in the pro-saccades of these patients, we found in both groups of cerebellar patients prolonged latency with larger variability than normal and increased directional error rate. Errors, however, were corrected by cerebellar patients as frequently as normal. No significant differences were found in patients with and without cortical involvement. Conclusion: Our results indicate, in a large cohort of cerebellar patients, that the cerebellum plays a critical role in the regulation of executive motor control not only, as well known, by controlling the end of a movement, but also modulating its initiation and reducing reflexive responses that would perturb voluntary actions

    Vertebral artery dissection caused by atlantoaxial dislocation in a patient with Marfan syndrome

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    A small number of case reports have documented a link between atlantoaxial dislocation (AAD) and vertebral artery dissection (VAD), but this association has never been described in patients with hereditary connective tissue disorders. We present a case of an 18-year-old female patient, diagnosed with Marfan syndrome since the age of one, who underwent brain MRA for intracranial aneurysm screening revealing tortuosity of the internal carotid and vertebral arteries as well as atlantoaxial dislocation. Since the patient was asymptomatic, a wait-and-see approach was chosen, but a follow-up MRA after 18 months showed the appearance of a dissecting pseudoaneurysm of the V3 segment of the left vertebral artery. Despite the patient being still asymptomatic, it was decided to proceed with C1-C2 stabilization to prevent further vascular complications. Follow-up imaging showed realignment of the atlantoaxial joint and reduction of the dissecting pseudoaneurysm of the left vertebral artery. In our patient, screening MRA has led to the discovery of asymptomatic arterial and skeletal abnormalities which, if left untreated, might have led to severe cerebrovascular complications. Therefore, AAD correction or close monitoring with MRA should be provided to MFS patients with this craniovertebral junction anomaly, even if asymptomatic

    Genotype-phenotype and OCT correlations in Autosomal Dominant Optic Atrophy related to OPA1 gene mutations: Report of 13 Italian families

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    Mutations in OPA1 are responsible of 32â\u80\u9389% cases of Autosomal Dominant Optic Atrophy (ADOA). OPA1 ADOA usually presents in childhood with bilateral, progressive visual loss due to retinal ganglion cells neurodegeneration, but environmental factors are supposed to influence onset and phenotype. Sixty Italian OPA1 mutations carriers (fifty-two symptomatic), belonging to thirteen families, underwent neuro-ophthalmologic evaluation. Visual acuity (n = 60) and Optical Coherence Tomography (OCT) (n = 12) were compared in missense mutations (OPA-M) versus haploinsufficiency-inducing mutations (OPA-H) and correlated with age. Presence of plus phenotypes was investigated. We found four known mutations, the most common being missense c.1034G > A, and a new missense mutation, c1193A > C, the latter in a 54-yrs old female with late-onset phenotype. Visual acuity, colour sensitivity, and optic disc atrophy were sensitive indicators of disease. OCT RNFL thickness was reduced in OPA1 compared to controls. OPA-M showed worst visual acuity than OPA-H, but not more frequent plus-phenotype, observed only in four OPA-H patients. In both groups, visual acuity worsened with age. Our data confirm worst vision in OPA-M, but not increased plus-phenotype. Since most patients belonged to nine families from south-eastern Sicily (a famous region for the cult of St. Lucy, patron of the blinds) local genetic and environmental factors might have accounted for the low occurrence of plus-phenotypes

    Anti-Saccades in Cerebellar Ataxias Reveal a Contribution of the Cerebellum in Executive Functions

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    ObjectiveIncreasing evidence suggests a cerebellar contribution to modulate cognitive aspects of motor behavior and executive functions. Supporting findings come from studies on patients with neurodegenerative diseases, in which however, given the extent of the disease, the specific role of the cerebellum, could not be clearly isolated. Anti-saccades are considered a sensitive tool to test executive functions. The anti-saccade underlying neural network, consisting of different cortical areas and their downstream connections including the lateral cerebellum, has been largely clarified. To separate the role of the cerebellum with respect to other cortical structures in executive control, we compared the anti-saccade performances in two distinct cohorts of patients with cerebellar disorders (with and without cerebral cortical involvement).MethodsEye movements during the execution of anti-saccades were recorded in 12 patients with spinocerebellar ataxia type 2 (a cortical-subcortical neurodegenerative disease), 10 patients with late onset cerebellar ataxia (an isolated cerebellar atrophy), and 34 matched controls.ResultsIn the anti-saccade task, besides dynamic changes already demonstrated in the pro-saccades of these patients, we found in both groups of cerebellar patients prolonged latency with larger variability than normal and increased directional error rate. Errors, however, were corrected by cerebellar patients as frequently as normal. No significant differences were found in patients with and without cortical involvement.ConclusionOur results indicate, in a large cohort of cerebellar patients, that the cerebellum plays a critical role in the regulation of executive motor control not only, as well known, by controlling the end of a movement, but also modulating its initiation and reducing reflexive responses that would perturb voluntary actions

    The role of dentate nuclei in human oculomotor control: Insights from cerebrotendinous xanthomatosis

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    Key points: A cerebellar dentate nuclei (DN) contribution to volitional oculomotor control has recently been hypothesized but not fully understood. Cerebrotendinous xanthomatosis (CTX) is a rare neurometabolic disease typically characterized by DN damage. In this study, we compared the ocular movement characteristics of two sets of CTX patients, with and without brain MRI evidence of DN involvement, with a set of healthy subjects. Our results suggest that DN participate in voluntary behaviour, such as the execution of antisaccades, and moreover are involved in controlling the precision of the ocular movement. The saccadic abnormalities related to DN involvement were independent of global and regional brain atrophy. Our study confirms the relevant role of DN in voluntary aspects of oculomotion and delineates specific saccadic abnormalities that could be used to detect the involvement of DN in other cerebellar disorders. It is well known that the medial cerebellum controls saccadic speed and accuracy. In contrast, the role of the lateral cerebellum (cerebellar hemispheres and dentate nuclei, DN) is less well understood. Cerebrotendinous xanthomatosis (CTX) is a lipid storage disorder due to mutations in CYP27A1, typically characterized by DN damage. CTX thus provides a unique opportunity to study DN in human oculomotor control. We analysed horizontal and vertical visually guided saccades and horizontal antisaccades of 19 CTX patients. Results were related to the presence/absence of DN involvement and compared with those of healthy subjects. To evaluate the contribution of other areas, abnormal saccadic parameters were compared with global and regional brain volumes. CTX patients executed normally accurate saccades with normal main sequence relationships, indicating that the brainstem and medial cerebellar structures were functionally spared. Patients with CTX executed more frequent multistep saccades and directional errors during the antisaccade task than controls. CTX patients with DN damage showed less precise saccades with longer latencies, and more frequent directional errors, usually not followed by corrections, than either controls or patients without DN involvement. These saccadic abnormalities related to DN involvement but were independent of global and regional brain atrophy. We hypothesize that two different cerebellar networks contribute to the metrics of a movement: the medial cerebellar structures determine accuracy, whereas the lateral cerebellar structures control precision. The lateral cerebellum (hemispheres and DN) also participates in modulating goal directed gaze behaviour, by prioritizing volitional over reflexive movements. Journal compilatio

    Ganglioneurocytoma of the spinal cord: report of a case and review of literature

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    A case of ganglioneurocytoma of the spinal cord in a 51-year-old man is reported. Patient presented with a progressively worsening back pain and hyposthenia of lower limbs. Magnetic resonance imaging of the spinal cord revealed an intradural lesion in the T10–11 spinal segments, hyperintense on T1-weighted sequences. Complete resection was achieved. The patient did not show evidence of recurrence 12 months after surgery. On histology, the lesion was composed of round to oval cells with fine granular, slightly eosinophilic to clear cytoplasm suggesting a neurocytic differentiation. Some ganglion cells were observed. Neither significant cytologic atypia nor mitoses were present. The neoplastic cells were strongly positive with synaptophysin and negative with glial fibrillary acid protein. Neurocytomas are uncommon tumors typically arising within the lateral ventricles near the foramen of Monro. Extraventricular neurocytomas have been reported at various sites with rare examples in spinal cord. In the present paper, a case of ganglioneurocytoma of the spinal cord is reported with review of literature and discussion of differences compared to neurocytomas of other sites
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