Sphenoidal pneumosinus dilatans due to anterior skull base meningiomas - CT and MRI aspects: Report of two new cases and literature review.

We report on two patients disclosing a pneumosinus dilatans (PSD) and an anterior skull base meningioma. Our findings, together with those from the pertinent literature, support the thought that this infrequent anomaly of paranasal sinuses is a useful clue to suspect a concomitant meningioma. Moreover, hypotheses concerning the pathophysiology of PSD are discussed.

Extracranial internal carotid artery: anatomical variations in asymptomatic patients.

The anatomical variations of internal carotid artery (ICA) are mostly asymptomatic, thus being disregarded and only incidentally diagnosed, with very few symptomatic patients. The awareness of these anomalies is crucial to solve the differential with other neck lesions, preventing accidental injuries during neck surgery. Therefore, the aim of our study is to estimate the prevalence of ICA anomalies, using magnetic resonance angiography (MRA) and computed tomography angiography (CTA). 253 patients underwent head and neck MRA and CTA examinations, with multiplanar and volumetric reformations. For each set of images, the following items were investigated: origin, development, course, persistence of embryonic vessels and anomalous origin of collateral branches. In our series, ICA arose from the bifurcation of the common carotid artery at the level of: C4 in 303 hemi-necks (59.9 %); C3 in 98 hemi-necks (19.3 %); C5 in 57 hemi-necks (11.3 %); C2 in 48 hemi-necks (9.5 %). ICA kinking and/or coiling was found in 105 hemi-necks, and location variation of ICA (reversed-type) in two hemi-necks. In just one case the origin of the ascending pharyngeal artery was from ICA (0.2 %), while an anomalous persistence of the proatlantal artery was noticed in three cases (0.6 %). CTA and MRA showed similar accuracy in detecting ICA anomalies.

Cavernous malformations isolated from cranial nerves: Unexpected diagnosis?

OBJECTIVES: Cranial nerves (CN) cavernous malformations (CMs) are lesions that are isolated from the CNs. The authors present three cases of CN CMs, for which MR was demonstrated to be critical for management, and surgical resection produced good outcomes for the patients. Surgical removal is the recommended course of action to restore or preserve neurological function and to eliminate the risk of future haemorrhage. However, the anatomical location and the complexity of nearby neural structures can make these lesions difficult to access and remove. In this study, the authors review the literature of reported cases of CN CMs to analyse the clinical and radiographic presentations, surgical approaches and neurological outcomes. PATIENTS AND METHODS: A MEDLINE/Pub Med search was performed and revealed 86 cases of CN CMs. The authors report three additional cases in this study for a total of 89 cases. CMs affecting the optic nerve (CN II), oculomotor nerve (CN III), facial/vestibule-cochlear nerves (CN VII, CN VIII) have been described. The records of three patients were reviewed with respect to the lesion locations, symptoms, surgical approaches and therapeutic considerations. Clinical and radiological follow-up results are reported. Three patients (2 females, 1 male; age range 21-37 year) presented with three CN lesions. One lesion involved CN III, one lesion involved CN VII-CN VIII, and one involved CN II. The patient with the CN III lesion had a one-month history of mild right ptosis and diplopia. The patient with the CN VII-CN VIII lesion exhibited acute hearing loss and on the left and left facial paresis. The patient with the opticchiasmatic lesion presented with acute visual deterioration on the right and a left temporal field deficit in the left eye. Pterional and orbitozygomatic craniotomies were performed for the CN III lesion and the CN II lesion, and retrosigmoid craniotomy was performed for the cerebello-pontine angle lesion. RESULTS: All patients experienced symptom improvement after surgery. On MR follow-up, recurrence was excluded in all patients. CONCLUSIONS: CN CMs present with specific symptoms and require complex surgical techniques for resection. These lesions are frequently symptomatic, because of the complexity of the origin tissue. Symptomatic CN CMs should be resected microsurgically and completely when possible to prevent further losses of nerve function, improve function, avoid recurrence, and to eliminate the risk of future haemorrhages. The authors discuss the therapeutic options and the radiological features of these infrequent localisation of CMs. Specifically, the authors focus on the role of magnetic resonance imaging in the identification of these rare lesions.

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa-Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature.

Neurofibromatosis type 1 (NF1) is a relatively common single-gene disorder, and is caused by heterozygous mutations in the NF1 gene that result in a loss of activity or in a nonfunctional neurofibromin protein. Despite the common association of NF1 with neurocutaneous features, its pathology can extend to numerous tissues not derived from the neural crest. Among the rare cerebrovascular abnormalities in NF1, more than 85% of cases are of purely occlusive or stenotic nature, with intracranial aneurysm being uncommon. Predominantly, the aneurysms are located in the internal carotid arteries (ICAs), being very rare bilateral aneurysms. This report describes a very unusual case of fusiform aneurysms of both ICAs in a Caucasian NF1 patient, with a new pathogenic intragenic heterozygous deletion of the NF1 gene, presenting at age 22 years with Tolosa-Hunt syndrome, because of partial thrombosis of the left giant intracavernous aneurysm. Medical treatment with anticoagulant therapy allowed a good outcome for the patient. In conclusion, early identification of cerebral arteriopathy in NF1 and close follow-up of its progression by neuroimaging may lead to early medical or surgical intervention and prevention of significant neurologic complications.

Transient ischaemic attack: an exceptional presenting syndrome of a superior sagittal sinus thrombosis.

We report a case of a transient ischaemic attack (TIA) as an exceptionally rare form of cerebral vein thrombosis. A 41-year-old man presented with a headache and five episodes of a transient left hemiparesis lasting approximately 10-15 min over the course of 2 weeks. Arterial origins of the disease were ruled out by detailed investigations. However, MR venography revealed a superior sagittal sinus thrombosis with numerous prominent collateral venous channels. No parenchymal lesion was seen. After medical therapy, no further transient neurological deficit occurred. Follow-up MR venography showed sinus recanalisation.

[Acustic neurinoma: correlation between clinic and MRI]. / Neurinoma dell'acustico: correlazione tra clinica e RM.

The purpose of this paper is to study acoustic neuroma diagnostic process, describe tumor's molecular basis and its magnetic resonance imaging characterization, which is considered to be the gold standard diagnostic tool to study this disease.

Solitary lumbar osteochondroma presenting with spinal cord compression.

We report a case of a middle-aged woman with a solitary osteochondroma of the L2 right lamina with intraspinal extension and spinal cord compression. The case is unusual in terms of localisation and age at clinical presentation. In fact, spinal osteochondromas as solitary lesions-especially when affecting the lumbar segment-are rare tumours of a maturing adolescent skeleton, infrequently affecting the neurological structures, because most of the lesions grow out of the spinal canal. Although unusual, they should be considered in the differential diagnosis. Prompt and accurate radiological investigations are important in planning appropriate management. Surgical total excision is the best treatment modality to remove spinal cord and/or nerve root compression, and to avoid the risk of recurrence or malignant transformation.

Giant frontal intraparenchymal schwannoma: unexpected diagnosis.

We report the case of an intraparenchymal frontal schwannoma, surgically resected in a middle-aged, otherwise healthy woman. Magnetic resonance imaging was ineffective for the definitive diagnosis and was instead afforded by histology. This case is unique because the rare lesion presented as a giant mass; to the best of our knowledge, such a finding has not been previously reported.

Isolated transient blindness and isolated oculomotor nerve palsy secondary to single mesencephalic metastases: report of 2 cases.

PURPOSE: Metastatic lesions of midbrain are quite rare, and only unusually they produce isolated cranial nerve palsy. METHODS: We report two cases of 2nd and 3rd cranial nerves impairment, produced by solitary midbrain metastases. RESULTS: In both patients Magnetic Resonance (MR) allowed a correct diagnosis, subsequently confirmed by histology. CONCLUSIONS: The metastatic involvement of midbrain would be included in the differential diagnosis of neuro-ophthalmic conditions to avoid the risk of misdiagnosis and consequently delay in management of this special population. MR is crucial for early detection of this infrequent pathology.

Computed tomography and magnetic resonance angiography in the evaluation of aberrant origin of the external carotid artery branches.

Aim of our study was to evaluate the prevalence of aberrant origin of the branches of the external carotid artery (ECA) in 97 patients by computed tomography (CTA) and magnetic resonance angiography (MRA) and to compare the accuracy of these two techniques in the visualization of the ECA system. All patients underwent CTA and MRA examination of the head and neck. Multiplanar and volumetric reformations were obtained in all cases. For each set of images, the presence of aberrant origin of the branches of the external carotid artery was investigated. MRA and CTA images of each patient were compared to define their information content. Anatomical anomalies were found in 88 heminecks, with a prevalence of 53.3%. In the 61 patients in whom the CTA was performed before the MRA, the latter method showed only 92% of abnormalities detected at the first examination; in the 36 patients in whom MRA was performed first, CTA identified all of the anomalies highlighted by the former, adding 12 new. Knowledge of the anomalies of origin of the ECA branches is essential for the head and neck surgeon; the high prevalence of anomalies found in our series as in the previous studies indicates the opportunity to perform a CTA or a MRA of the head and neck before any surgical or interventional procedure. CTA is the method of choice in the evaluation of anomalies of origin of the branches of the ECA and in the definition of their course.

Olfactory delusional syndrome and intracranial meningioma.

We report the case of a 37-year-old female in which the removal of a suprasellar neoplasm was accompanied by the disappearance of a longstanding olfactory delusion syndrome. In primary care the patient condition was exclusively thought to be psychic in origin, neglecting the possible, not infrequent, organic contribution. The delayed diagnosis produced neurological impairment, only partially recovered after surgical therapy. This case might help to improve the patient management via multi-specialist cooperation and to broaden the knowledge about somatic mechanisms of psychic disturbances, are not often taken into account.

Post-traumatic peripheral facial nerve palsy: surgical and neuroradiological consideration in five cases of delayed onset.

The facial nerve is frequently injured after head trauma with or without temporal bone fractures. Computed tomography (CT) is the best procedure for detecting the fracture line at the level of the facial nerve canal and for assessing any associated lesions within the temporal bone. Magnetic resonance (MR) is required if there is a facial nerve paralysis, unexplained by CT findings. We present five cases of delayed post-traumatic facial nerve palsy without evidence of temporal bone fractures on CT, thus studied on MR. MR was essential for diagnosing the nerve impairment. Neuroradiological findings, clinical presentation, and electrodiagnostic tests influenced the management of the patients.

Solitary olfactory groove schwannoma.

Intracranial schwannomas that do not involve major cranial nerves in the posterior fossa are uncommon, especially if they are not associated with neurofibromatosis type II (NF-2). Subfrontal olfactory groove schwannomas are extremely rare. We report a cystic schwannoma arising from the olfactory groove in a 38-year-old Caucasian male who presented with headache, vomiting and visual impairment. We briefly review the pertinent literature, stress the distinctive clinical and neuroradiological findings and discuss the surgical planning and theories about the pathogenesis of schwannomas in this unusual location.

Problem in diagnostic radiology.

The case of a 58-year-old woman is described. She presented with what initially seemed to be a transient ischaemic attack. Clinical imaging, however,revealed an intracranial lipoma of the cisterna magna associated with a defect of the occipital bone and spina bifida occulta of the atlas.

Intramedullary neurenteric cysts of the spine. Report of three cases.

Neurenteric spinal cysts are infrequently occurring dysraphic lesions that are caused by persistent or abnormal communication among neuroectoderm, notochord, and endoderm. They are generally located at the intradural, extramedullary compartment of the low cervical or upper spinal canal. They occur primarily in infants and in young adults in combination with other congenital abnormalities. The authors report on three cases of lower-thoracic and thoracolumbar intramedullary neurenteric cysts that atypically presented in adult patients in whom there was no concomitant malformation. These lesions appear notable for location, clinical presentation, intraoperative findings, and imaging features.

Azathioprine and interferon beta(1a) in relapsing-remitting multiple sclerosis patients: increasing efficacy of combined treatment.

Current treatments of relapsing-remitting multiple sclerosis (RRMS) with immunosuppressive or immunomodulatory drugs have been shown to modify the course of the disease in a significative number of patients. However, in many cases, the response to either interferon beta (IFN-beta) or azathioprine (AZA) treatments was not satisfactory and new therapeutic approaches are needed. We studied clinical and MRI efficacy, safety and tolerance of AZA and IFN-beta(1a) combined therapy in 23 patients with clinically definite RRMS, who had not previously been responsive to either monotherapies. Our cases were divided into three subgroups: 8 previously untreated patients (subgroup A) with at least 2 years of natural course of the disease, 8 patients (subgroup B) previously treated with AZA for 2 years and 7 patients (subgroup C) previously treated with IFN-beta(1a) for 2 years. The baseline Expanded Disability Status Scale (EDSS) ranged from 2 to 4 in all subgroups. All patients completed 2 years of combined treatment with a dose of AZA adjusted to reduce lymphocyte count down to 1,000 +/- 100/microl in association with IFN-beta(1a) at a dose of 6 MIU every other day. The mean number of relapses during the combined treatment period was significantly lower than that observed before combined therapy in all the three subgroups. Also, the mean Delta EDSS score was significantly lower during combined treatment than in monotherapy in subgroups B and C. Moreover, after 2 years of combined treatment, the number of new T(1) hypointense lesions, the number and volume of proton density/T(2) hyperintense lesions and the gadolinium enhancement of T(1) hypointense lesions were significantly lower than before combined treatment. After 2 years of treatment, this combination therapy appears to be safe and well tolerated and no serious side effects were reported. Despite some limitations of our study design, the information regarding efficacy, safety and tolerance of the association of AZA and IFN-beta is most encouraging.