Focal epilepsies associated with glioneuronal tumors: review article.

Glioneuronal tumors (GNTs) are an increasingly recognized cause of focal epilepsies, particularly in children and young adults. GNTs consist of a mixture of glial and neuronal elements and most commonly arise in the temporal lobe, particularly in the temporo-anterior-basal mesial site. They are often associated with cortical dysplasia or other neuronal migration abnormalities. Epilepsy associated with GNT is poorly controlled by antiepileptic drugs in many cases; but, it is extremely responsive to surgical treatment. However, the best management strategy of tumor-related focal epilepsies remains controversial and still remain one of the contemporary issues in epilepsy surgery. Temporo-mesial GNT are associated with a widespread epileptic network, defining, therefore, a distinct anatomo-clinico-pathological group with complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy GNT associated with focal epilepsies may have an excellent seizure outcome and, therefore, surgical treatment can be offered early to avoid both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of tumor growth or malignant transformation.

MRI findings in low grade tumours associated with focal cortical dysplasia.

Magnetic resonance imaging (MRI) is mandatory to identify the epileptogenic zone in refractory temporal lobe epilepsy (TLE). The correct identification of lesions is essential to obtain good post-surgery seizure control. Low grade tumours (LGT) and focal cortical dysplasia (FCD) are common findings in symptomatic TLE, and frequently coexist. The aim of this study was to identify the MRI characteristics in the diagnosis of FCD associated with LGT. We analyzed 24 subjects with TLE who underwent tailored surgery. They all had LGTs. Two expert neuroradiologists analyzed the imaging data and compared them with histological results, hypothesizing the causes of diagnostic errors in the identification of FCD. We selected three exemplary cases to report the most important causes of errors. In the diagnosis of FCD we reported false positives and false negatives due to different causes. An incomplete MRI protocol, the large dimensions of the tumour, infiltration and related oedema were the most important factors limiting MRI accuracy. MRI can be limited by an incomplete protocol. In addition, the presence of an LGT may limit the neuroradiological diagnosis of FCD in the temporal lobe. Advanced MRI techniques could help reveal subtle lesions that eluded a previous imaging inspection.

Type a niemann-pick disease. Description of three cases with delayed myelination.

We describe three patients with type A Niemann-Pick disease (NPD-A). NPD-A is an autosomal recessive neuronal storage disease classified among the sphingolipidoses, characterized by accumulation of sphingomyelin in various tissues and in the brain. Magnetic Resonance imaging (MRI) of our three patients showed a marked delay of myelination with frontal atrophy. Few descriptions of this MRI pattern of delayed myelination have been published to date.

Trigeminal perineural spread of head and neck tumors.

Perineural tumor spread (PNS) of head and neck malignancies is a well-known form of metastatic disease in which a lesion can migrate away from the primary site along the endoneurium or perineurium. MR imaging is considered the primary method for evaluating patients with symptoms related to the trigeminal nerve in most clinical settings. Both CT and MR imaging can detect perineural spread, but MRI is the modality of choice because of its capability to detect direct signs (nerve enlargement and enhancement) and indirect signs (neuropathic muscular atrophy, obliteration of fat planes). In addition, MRI is more sensitive because of its superior soft-tissue contrast, its multiplanar capability and decreased artifacts from dental hardware. Fat suppression images after contrast injection are mandatory to better detect nerve enhancement. CT is useful in detecting foraminal enlargement or more destructive bone patterns. Nerve function can be perserved until later in the course of the disease: patients with perineural spread demonstrated at radiologic or pathologic examination may have normal or nonspecific nerve function at clinical examination (patients are misdiagnosed with Bell's palsy or trigeminal neuralgia). Hence MRI assessment of perineural tumor location and extension is important.

Thalamic changes in mesial temporal sclerosis: a limbic system pathology. A case report.

Hippocampal abnormalities correlated with mesial temporal sclerosis (MTS) are well documented. MTS may be associated with extrahippocampal anomalies involving limbic structures along a known neuroanatomic pathway (Papez circuit). We report a patient with MTS and thalamic changes. Seizure-related thalamic damage could have been caused by abnormal electric discharges from the mamillary body to the anterior thalamus through the mamillothalamic tract. This suggests that MTS is not limited to the temporal lobe but could represent a limbic system pathology.

Childhood spinal glioblastoma multiforme with intracranial dissemination. A case report.

Primary spinal cord tumors in children are uncommon and account for approximately 5% of all pediatric central nervous system tumors. Intracranial metastases from primary spinal cord tumors have rarely been reported. Spinal cord glioblastoma in children mainly involves the thoracic region, whereas involvement of the medullaris conus with intracranial dissemination has been described in six cases. This paper describes a pediatric case of thoracic glioblastoma multiforme with intracranial dissemination in the early stage of the clinical course and discuss the clinical and neuroradiological manifestations, the possible patterns of dissemination and finally to consider the therapeutic implications.

Ectopic suprasellar pituitary adenoma. A case report.

The occurrence of a pituitary adenoma located entirely outside the sella turcica, so-called ectopic adenoma, is extremely rare. We report a case of a non secreting-pituitary adenoma located above the diaphragma sellae, with no invasion into the sella turcica, confirmed at surgery. The tumor was initially treated unsuccessfully by operations via the transphenoidal route. After initial negative exploration by the transphenoidal route, the patient was successfully treated by an endoscopic endonasal transphenoidal approach extended to the tuberculum sellae and the posterior planum sphenoidale to access the suprasellar supraglandular region. A brief review of ectopic adenomas and a discussion of the preoperative diagnosis are presented.