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.

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.

High field functional MRI.

Functional magnetic resonance imaging (fMRI) has become the most widely used approach for studying brain functions in humans. The rapid and widespread diffusion of fMRI has been favoured by the properties this technique presents, and particularly by its sensitivity in analysing brain functional phenomena and by the lack of biological invasiveness, resulting in an unprecedented and unparalleled flexibility of use. These properties of fMRI brought the functional examination of the brain within the reach of the whole neuroscience community and have appreciably stimulated the research on the functional processes of the living brain. Among the main features of fMRI, its spatial and temporal resolution represents clear advantages compared with the other methods of functional neuroimaging. In fact, the high spatial resolution of fMRI permits to produce more precise and better localised information, and its temporal resolution provides the potential of a better understanding of neural dynamics at the level of single functional areas and of the neural constituents of functional patterns. A fundamental possibility of improving spatial and temporal resolution without excessively degrading signal-to-noise ratio consists in the use of high magnetic field intensity fMRI units. Besides, high field units make the use of more demanding fMRI paradigms, like single trial event related studies, much more compatible with the need of a solid statistical evaluation. This has notably promoted the diffusion of high field MRI units for human studies throughout the world, with very high field MRI units, up to 8 T, working in a few research centres, and a larger number of MRI units with field intensity ranging between 3 and 5 T.

Patterns of recovery and change in verbal and nonverbal functions in a case of crossed aphasia: implications for models of functional brain lateralization and localization.

We present a 2-year verbal and nonverbal follow-up of a crossed aphasic patient. The patient had suffered from widespread ischemic damage in the area of right middle cerebral artery, with a parieto-temporal lesion. Three months postonset he showed classical Wernicke's aphasia associated with oral, limb and constructional apraxia and left hemineglect. However, follow-up findings showed a complex, dynamic pattern entirely consistent with cognitive models of language and nonlanguage abilities. Current models of functional brain lateralizations could not satisfactorily account for such longitudinal, fine-grain observations.

How many alien hand syndromes? Follow-up of a case.

We report the follow-up of a patient affected by a persistent alien left nondominant hand. CT and MRI showed an ischemic lesion localized to the mesial frontal region and involving the supplementary motor area and anterior fibers of the corpus callosum. These findings support theories suggesting a critical role of both these lesions in determining "chronic" forms of alien hand of either upper limb.

[MR vs CT: which is the diagnostic advantage in neuroradiology]. / RM vs TC: quale il guadagno diagnostico in neuroradiologia.

The comparative adequacy was studied of the two main neuroradiological imaging methods, MR imaging and CT, through the analysis of 1500 MR examinations with a preliminary CT study. The comparison was made by giving a score to the diagnostic contribution of MR imaging, especially as far as changes in the therapeutical approach were concerned. The results varied according to the various pathologies, to the different anatomical regions explored, and to the site of the masses. The overall score was higher in the spine and the spinal cord than in brain pathology. In brain neoplastic pathology, eg, the diagnostic capabilities of CT and MR imaging were similar in most cases, especially in supratentorial locations, while MR imaging was clearly superior in the spinal cord district. In infratentorial location, the better anatomical details make MR imaging the method of choice. In conclusion, CT confirms its accuracy in the detection of brain pathology, while MR imaging proves more sensitive in white matter diseases, and in particular locations, e.g. the posterior fossa. In the spine and spinal cord districts, the very high average score mainly confirms MR capability to demonstrate spinal canal normal and pathological content. Moreover, the important role of MR imaging is stressed in pediatric pathology, together with CT primary role in emergency cases.

Craniopharyngioma of the cranial base and nasopharynx.

A rare case of craniopharyngioma extending to the cranial base and nasopharynx is reported and eight other cases in the literature are reviewed. The embryology and the clinical features of these tumors are discussed. Tomograms of the skull and computed tomography are the most useful radiological tools of investigation. A nasoseptal or transpalatal approach to surgery, which has been performed in reported cases of cystic nasopharyngeal craniopharyngiomas, was not performed on our patient because of the hard consistency and the diffusely infiltrating aspect of the tumor.

[The spinal vacuum phenomenon. Radiographic and tomodensitometry aspects]. / Il fenomeno "vacuum" spinale. Aspetti radiografici e tomodensitometrici.

The spinal vacuum phenomenon is readily recognized by computed tomography: there fore its observation has become frequent enough. The side of the gaseous collection (intervertebral disk, vertebral body, apophyseal joint) depends on the disease inducing the phenomenon. Radiological and CT aspects of this findings are discussed. The usefulness of vacuum phenomenon in the diagnosis of several pathological conditions is examined.

[Iatrogenic changes in the dural sac. Radiologic semeiology]. / Alterazioni iatrogene del sacco durale. Semeiologia radiologica.

The authors have studied the pathological appearances of follow-up and post-operative radiculography. It has been possible to distinguish between lesions due to arachnoiditis and periduritis.

[Phlebography of the cavernous and intercavernous sinuses; an investigation complementary to CT detecting intrasellar space-occupying lesions]. / La flebografia dei seni cavernosi ed intracavernosi quale indagine complementare alla TC nello studio delle lesioni espansive intrasellari.

The technique of peri-hypophyseal phlebography is briefly described and the theoretical bases that inspire its utilizations and indications are pointed out. On the basis of 20 preliminary cases, the main applications of this investigation are illustrated, with a particular attention concerning the early diagnosis of lesions too small or too dishomogeneous to be detected with certainly on CT (i.e. intrapituitary microadenomas smaller than 5 mm; associated intrasellar lesions as empty sella + pituitary tumor).

CT in the diagnosis of sellar and parasellar lesions.

The Authors review their recent experience in investigating 185 cases of sellar and parasellar lesions, using as preliminary procedure stratigraphy and CT; further examinations were performed only in cases with unclear diagnosis. Their data confirm the reliability of this non-invasive protocol that allowed the detection of pathological changes in nearly 95% of cases and was able to define with certainty the nature of the lesions in over 72% of cases. However, other investigations were performed in 116 cases (63%): in 57 (31%) for diagnostic purpose, in 59 (32%) as complementary pre-operative studies.

[Fogging effect in the ischemic cerebral infarction to computerized tomography (author's transl)]. / L'effetto maschera nell'infarto cerebrale ischemico alla tomografia computerizzata.

We have examined, with systematic serial computerized tomography (CT) studies, 6 patients with persistent ischemic cerebral infarction. The CT of the ischemic cerebral infarction shows, during the third week, an isodense area. We have called this phenomenon "fogging effect" because at this effect and the CT report could be negative. The possibility of a false negative can be avoided by a control study with contrast medium: the infarction has an intensive contrast enhancement.