Samuel M. Wolpert

The value of neuroradiology in infantile spasms

The results of neuroradiologic studies of 71 children with infantile spasms treated with ACTH were correlated with the developmental outcome at follow-up (mean 62 months, range 14 to 207 months). Fifty-two (73%) patients had an abnormal NRS on initial evaluation; 49% had cerebral atrophy, 18% had congenital anomalies, and 6% had hydrocephalus. Twenty patients were normal on initial clinical evaluation. At follow-up only the eight (40%) with normal NRS were normal. Twelve (60%) who had unexpected abnormalities on NRS were retarded at follow-up. CAT scanning is necessary to predict the developmental outcome in developmentally normal children with infantile spasm. Eight of nine patients with normal NRS in the early treatment group were developmentally normal at presentation and follow-up. Ten patients who were developmentally normal before spasms began, and had normal NRS but were in the late treatment group, were retarded at initial evaluation and follow-up. This finding suggests that early treatment of children who have a normal NRS and normal development at onset of spasm prevents mental retardation.

The value of computed tomography in evaluating patients with prolactinomas.

The use of computed tomography (CT) in 28 patients with surgically proved prolactinomas is described. The authors experience suggests that in evaluating patients with galactorrhea-amenorrhea syndrome, CT in combination with pluridirectional tomography usually provides sufficient information to make further invasive studies unnecessary. In selected cases with low-density areas within the sella as seen on CT, pneumoencephalography is still advisable to distinguish between the empty sella and the necrotic tumor. Angiography is still necessary to exclude vascular causes for suprasellar and parasellar masses such as aneurysms and meningiomas.

Intermittent downbeat nystagmus due to vertebral artery compression

Downbeat nystagmus (DBN) uncommonly occurs as a transient phenomenon, and it rarely occurs in patients with cerebrovascular disease. We observed a patient with intermittent DBN and lightheadedness due to transient obstruction of his dominant vertebral artery when he turned his head to his left side. Surgical removal of an osteophyte at the site of the angiographically demonstrated lesion relieved his symptoms

Cystic lesions of the posterior fossa in infants A comparison of the clinical, radiological, and pathological findings in Dandy‐Walker syndrome and extra‐axial cysts

AN IhlPOHTAiYT PART of the neurological examination of infants is skull transillumination. A specific pattern of abnormal transillumination in the posterior fossa has been described in the Dandy-Walker syndr0me.l-4 Recently, two infants were seen with abnormal posterior fossa traiisillumination unlike that seen with the Dandy-Walker syndrome. Posterior fossa extraaxial cysts (PFEAC) were demonstrated by angiography and air contrast studies and confirmed by surgical exploration. T h e purpose of this paper is to describe and correlate the pattern of skull transillumination, neuroradiological features, gross and microscopic pathology, and certain clinical features of these two patients with PFEAC and to compare these features with similar ones in two children with the Dandy-Walker syndrome.5-7 The similarities and dissimilarities in the pathogenesis of these two conditions also will be discussed.

Cavernous sinus syndrome due to prolactinoma: resolution with bromocriptine.

Cavernous sinus syndrome due to a pituitary tumor is quite rare. Our patient had a large prolacinoma that extended superiorly, causing bitemporal visual field defects, and laterally into the right cavernous sinus, causing tearing, dysesthesia in the distribution of the fifth cranial nerve, a loss of the corneal reflex, and a sixth cranial nerve palsy. Prolactin levels ranged between 800 and 1000 ng/mL. Treatment with 7.5 mg of bromocriptine daily caused a marked reduction in the size of the tumor and resolution of her visual field defects and cranial nerve dysfunction over 6 months. We feel that bromocriptine should be considered as initial therapy for patients with prolactinomas extending into the middle or posterior cranial fossae.

The Radiology of Pituitary Adenomas—An Update

Prior to the utilization of sellar tomography in patients with intrasellar tumors, adenomas less than 10 mm in diameter were seldom detected radio-graphically. The application of pluridirectional and, more recently, computed tomography (CT) of the sella to the workup of these patients has revolutionized their management. Patients with tumors ranging in size from 5 mm in diameter to large supra- and parasellar masses can now be medically or surgically managed entirely on the basis of tomography, CT of the sella, the endocrine findings, and the visual-field examination. Pneumoencephalography (PEG) is now seldom indicated in these patients, though on occasions, particularly when an empty sella is suspected, the definitive diagnosis is achieved by PEG. Even more recently, a water-soluble contrast agent, metrizamide, for subarachnoid space opacification has been introduced. The agent can be maneuvered into the suprasellar cisterns, and the use of metrizamide cisternography with CT may eliminate PEG in the few instances in which it is still presently indicated (see Chapter 10, Fig. 19).

Two Types of Congenital Cysts of the Posterior Fossa: A Comparison of Their Clinical and Pathological Characteristics and Their Embryogenesis

Two types of cystic lesions can occur in the posterior fossa of infants and children. In one type there is a cystic dilatation of the 4th ventricle known as the Dandy-Walker (D-W) syndrome, and in the second type, a cyst or cysts overlie the cerebellum. This is referred to as a posterior fossa extra-axial cyst (PFEAC). These two entries may be differentiated clincally as will be illustrated by presentation of the findings in 2 infants with PFEAC and 2 with D-W syndrome. The pattern of skull transillumination is distinctive for each entity, although the differences are subtle, and can enable the clinician to suspect the correct diagnosis immediately. Arteriographic and air contrast findings show silmilar abnormalities in the supratentorial region in both conditions but distinctive differernces are present in the infratentorial from that in D-W syndrome. Inferences drawn from a comparsion of the arteriographic and histologic findings in these 4 cases with the appearance of certain brain structures at different stages of human embryogenesis indicate that both conditions are congenital abnormalities occuring before the 3rd fetal month but affecting the leptomeninages in one instance (PFEAC) and the cerebellar anlage in the other (D-W syndrome).