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Hydranencephaly of Postnatal Origin

Case Report

Martin H. Weiss, Harold F. Young, and Dee E. McFarland

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Ultrastructural morphology of the olfactory pathway for cerebrospinal fluid drainage in the rabbit

Stephanie S. Erlich, J. Gordon McComb, Shigeyo Hyman, and Martin H. Weiss

✓ Previous physiological studies indicate that the olfactory region serves as a major pathway for cerebrospinal fluid (CSF) drainage into the lymphatic system. The present study was undertaken to determine the ultrastructural characteristics of this egress route. New Zealand White rabbits received a single bolus injection of the tracer ferritin (MW 400,000) into both lateral ventricles in such a manner as not to raise the intraventricular pressure above the normal level. The animals were sacrificed via intracardiac perfusion of fixative between less than 12 minutes and 4 hours following injection. The cribriform region was removed en bloc, decalcified, sectioned coronally, and prepared for light and electron microscopic examination.

The arachnoid, dura, and periosteum surrounding the fila olfactoria passing through the cribriform plate merge together and form the perineurium, which consists of multiple layers of loosely overlapping cells with widely separated junctions and few vesicles. The perineurium surrounding the olfactory filaments at the superficial submucosal level is only one cell thick. The subarachnoid space freely communicates with the perineural space surrounding each filament. No morphological barrier between the perineural space and the loose submucosal connective tissue was identified. Whether or not the perineurium was multi- or singlelayered, ferritin was noted in abundance between the loosely overlapping perineural cells and in the submucosal connective tissue. The distribution of ferritin at 12 minutes was similar to that at 4 hours; however, the quantity of ferritin was increased at 4 hours. These results indicate that no significant barrier to CSF drainage is present at the rabbit cribriform region and that CSF reaches the submucosal region rapidly via open pathways.

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Cerebrospinal fluid drainage as influenced by ventricular pressure in the rabbit

J. Gordon McComb, Hugh Davson, Shigeyo Hyman, and Martin H. Weiss

✓ Artificial cerebrospinal fluid (CSF) containing radioisotope iodinated (125I) serum albumin (RISA) and either blue dextran or indigo carmine was given to white New Zealand rabbits over 4 hours. In one group it was given by ventriculocisternal perfusion, in one by ventricular infusion, and in one by cisterna magna infusion. Blood was sampled continuously from the superior sagittal sinus (SSS) or intermittently from the systemic arterial circulation. Removal of CSF from the cisterna magna during the ventriculocisternal perfusion kept the intracranial pressure (ICP) at 0 to 5 torr, whereas ventricular or cisterna magna infusion raised the ICP to 20 to 30 torr and 15 to 20 torr, respectively. In the two groups with raised ICP, an increased concentration of RISA was present in the optic nerves, olfactory bulbs, episcleral tissue, and deep cervical lymph nodes; but this was not found in the group with normal ICP. In all three groups, the concentration of RISA in the SSS blood was the same as in the systemic arterial blood. The concentration gradient of RISA across the cerebral cortex was similar in both the ventriculocisternal perfusion and the ventricular infusion groups. With cisterna magna infusion, the concentration of RISA was the same on the cortical surface and less in the ventricles compared with the ventricular infusion. It is concluded that, with elevated ICP, CSF drained via pathways that are less evident under normal pressure. Drainage of CSF was similar irrespective of whether the infusion site was the ventricles or cisterna magna. It did not appear that acute dilatation of the ventricles during ventricular infusion compromised the subarachnoid space over the surface of the hemisphere, as the concentration of RISA on the convexities and in the SSS blood did not significantly differ between the groups. Transcortical bulk transfer of CSF was not evident with raised ICP.

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Pituitary gland metastasis from adenocarcinoma of the prostate

Case report

William T. Couldwell, Parakrama T. Chandrasoma, and Martin H. Weiss

✓ A case of prostatic carcinoma metastasis to the pituitary gland is reported. The presentation and rarity of such a lesion is addressed. The literature review yielded only isolated case reports of symptomatic brain metastases unassociated with bone disease from adenocarcinoma of the prostate. The management options of such a lesion are discussed.

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Ultrastructure of the orbital pathway for cerebrospinal fluid drainage in rabbits

Stephanie S. Erlich, J. Gordon McComb, Shigeyo Hyman, and Martin H. Weiss

✓ An increasing number of physiological and morphological studies indicate that cerebrospinal fluid (CSF) drains via nonarachnoidal pathways in several mammalian species. Ultrastructural tracer studies were undertaken to examine the orbital route for CSF absorption in the rabbit. At the termination of the optic nerve subarachnoid space, an area of connective tissue containing numerous small tortuous channels is present. Ferritin (molecular weight 400,000) infused into the ventricles at normal and increased intraventricular pressure was present in these channels by 15 minutes postinfusion, and subsequently reached the intraorbital connective tissue. Elevating the intraventricular pressure did not noticeably alter the morphological appearance of this region or change the gross distribution pattern of the ferritin. Ferritin did not penetrate the scleral barrier to reach the choriocapillaris, nor did it breach the arachnoid barrier layer proximal to the transitional zone at the optic subarachnoid space to reach the dura mater. These results are very similar to those described for the hamster orbital region and the rabbit cribriform region. These experiments support the concept that macromolecules exit the subarachnoid space at the termination of the optic nerve via open channels, and that no significant barrier to drainage of macromolecules in CSF is present at this location.

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Colao A, Di Sarno A, Cappabianca P, et al: Withdrawal of long-term cabergoline therapy for tumoral and nontumoral hyperprolactinemia. N Engl J Med 349:2023–2033, 2003

William T. Couldwell, Martin H. Weiss, and Edward R. Laws Jr

Background

Whether the withdrawal of treatment in patients with nontumoral hyperprolactinemia, microprolactinomas, or macroprolactinomas is safe and effective has been unclear. We performed an observational, prospective study of cabergoline (a dopamine-receptor agonist) withdrawal in such patients.

Methods

The study population included 200 patients—25 patients with nontumoral hyperprolactinemia, 105 with microprolactinomas, and 70 with macroprolactinomas. Withdrawal of cabergoline was considered if prolactin levels were normal, magnetic resonance imaging (MRI) showed no tumor (or tumor reduction of 50 percent or more, with the tumor at a distance of more than 5 mm from the optic chiasm, and no invasion of the cavernous sinuses or other critical areas), and if follow-up after withdrawal could be continued for at least 24 months.

Results

Recurrence rates two to five years after the withdrawal of cabergoline were 24 percent in patients with nontumoral hyperprolactinemia, 31 percent in patients with microprolactinomas, and 36 percent in patients with macroprolactinomas. Renewed tumor growth did not occur in any patient; in 10 female patients (22 percent) and 7 male patients (39 percent) with recurrent hyperprolactinemia, gonadal dysfunction redeveloped. In all diagnostic groups, prolactin levels at the time of recurrence were significantly lower than at diagnosis (p < 0.001). The Kaplan–Meier estimated rate of recurrence at five years was higher among patients with macroprolactinomas and those with microprolactinomas who had small remnant tumors visible on MRI at the time of treatment withdrawal than among patients whose MRI scans showed no evidence of tumor at the time of withdrawal (patients with macroprolactinomas, 78 percent vs. 33 percent, P = 0.001; patients with microprolactinomas, 42 percent vs. 26 percent, P = 0.02).

Conclusions

Cabergoline can be safely withdrawn in patients with normalized prolactin levels and no evidence of tumor. However, because the length of follow-up in our study was insufficient to rule out a delayed increase in the size of the tumor, we suggest that patients be closely monitored, particularly those with macroprolactinomas, in whom renewed growth of the tumor may compromise vision.

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Head Injury Management

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Nelson syndrome: historical perspectives and current concepts

Mark Hornyak, Martin H. Weiss, Don H. Nelson, and William T. Couldwell

✓The appearance of an adrenocorticotropic hormone (ACTH)–producing tumor after bilateral adrenalectomy for Cushing disease was first described by Nelson in 1958. The syndrome that now bears his name was characterized by hyperpigmentation, a sellar mass, and increased plasma ACTH levels. The treatment of Cushing disease has changed drastically since the 1950s, when the choice was adrenalectomy. Thus, the occurrence, diagnosis, and treatment of Nelson syndrome have changed as well. In the modern era of high-resolution neuroimaging, transsphenoidal microneu-rosurgery, and stereotactic radiosurgery, Nelson syndrome has become a rare entity. The authors describe the history of the diagnosis and treatment of Nelson syndrome. In light of the changes described, the authors believe this disease must be reevaluated in the contemporary era and a modern paradigm adopted.

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Subdural empyema of the cervical spine: clinicopathological correlates and magnetic resonance imaging

Report of three cases

Michael L. Levy, Brian H. Wieder, John Schneider, and Martin H. Weiss

✓ A paucity of formally described information is available in the scientific literature regarding spinal subdural empyema. Patients presenting with neurological deterioration associated with subdural empyema are rarely identified, and treatment is often based upon anecdotal cases. The authors contribute three cases of primary cervical spinal subdural empyema and review the seven found in the literature. All patients had clinical evidence of neurological compromise, cervical tenderness, cervical pain, and leukocytosis upon admission. Cervical involvement ranged from C-2 to C-7. All patients underwent laminectomy with durotomy and drainage. The authors recommend prompt surgical decompressive laminectomy, copious irrigation, and drainage, followed with appropriate adjunctive antibiotic therapy for treatment of these patients.

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Editorial: Radiosurgery for pituitary adenomas

Martin H. Weiss