David W. Beck and Arnold H. Menezes
✓ A series of 12 patients with mass lesions arising from Meckel's cave is presented. Patients' age on presentation ranged from 13 months to 71 years. Nine of the 12 patients had symptoms referable to the fifth cranial nerve, but only three complained of facial pain. The 12 patients presented eight different pathological entities, including meningioma, lipoma, schwannoma, malignant melanotic schwannoma, arachnoid cyst, neurofibroma, epidermoid tumor, and chordoma. Computerized tomography and magnetic resonance imaging were most useful in localizing the lesion to Meckel's cave. All 12 patients underwent a subtemporal approach to the lesion, and gross total removal was achieved in 11. Postoperative results were excellent with no increased neurological deficits seen 3 months postoperatively. Most patients had resolution of the cranial nerve deficits except for fifth nerve function, which was impaired in nine patients postoperatively. This series demonstrates that lesions in Meckel's cave can have a varied and unusual presentation, as well as an assortment of pathology. Total removal of lesions in this area resulted in relief of symptoms in most patients, with minimum morbidity.
David W. Beck, Jeffrey J. Olson and Edward J. Urig
✓ The treatment of trigeminal neuralgia by percutaneous retrogasserian glycerol rhizotomy was assessed in a series of 58 patients with a follow-up period ranging from 2 to 40 months postoperatively. All patients were considered medical failures prior to the procedure. Idiopathic trigeminal neuralgia was the diagnosis in 54 patients, and four patients had trigeminal neuralgia associated with multiple sclerosis. Forty-two patients (72%) reported complete relief from the procedure and are taking no medications. Four patients (7%) are much improved and require only minimal drug therapy. Twelve patients (21%) were considered treatment failures. The recurrence rate after initial relief of symptoms was 11%. Ten patients (17%) noticed a mild decrease in facial sensation following the procedure, and one additional patient had a profound sensory loss including loss of corneal reflex. The authors conclude that, while percutaneous retrogasserian glycerol rhizotomy may be useful in the treatment of trigeminal neuralgia, more clinical series and documentation of recurrence rate and complications are needed before any firm conclusions can be reached as to the efficacy of this therapy.
David W. Beck, Neal F. Kassell and Charles G. Drake
✓ The authors report a case of glomus jugulare tumor presenting with papilledema and visual loss. The tumor was extremely vascular with significant shunting of arterial blood into venous sinuses. There was no intracranial extension of tumor, and papilledema resolved after removal of the lesion.
Report of three cases
Carl J. Graf, Cordell E. Gross and David W. Beck
✓ Continuous cerebrospinal fluid (CSF) drainage may be used in the treatment of CSF fistula. The procedure, however, is not without risk. Marked gradients between the intracranial and intraspinal CSF pressures and intravasation of air through an unsealed fistula may produce serious neurological problems. The use of continuous CSF drainage requires an alert, informed nursing staff to avert catastrophe.
Jeffrey J. Olson, David W. Beck, Janet A. Schlechte and Pao-Min Loh
✓ Meningiomas have been shown to have steroid-binding proteins. In vitro, estradiol, progesterone, and the antiestrogen tamoxifen stimulate tumor growth. However, incubation of tumor cells with an antiprogesterone agent results in tumor inhibition. In this investigation, a human meningioma was implanted subcutaneously in athymic nude mice. Two treatment groups were established, one receiving the antiprogesterone agent RU-38486 (10 mg/kg/day in suspension) and the other receiving only vehicle. After 3 months, the tumor growth index (defined as the tumor volume at 3 months divided by the initial tumor volume) was 0.25 ± 0.46 (mean ± standard deviation) in the group receiving antiprogesterone and was 1.54 ± 0.58 in the control group (p = 0.041). Further investigation of the effect of antiprogestational agents on the growth and hormone-binding proteins of other meningiomas will better define the mechanism of their effects.
Jeffrey J. Olson, David W. Beck, Janet Schlechte and Pao-Min Loh
✓ Speculation that meningiomas are subject to endocrine influence is supported by their higher incidence in women, reports of exacerbation of symptoms during pregnancy, and the discovery that these tumors harbor progesterone- and estrogen-binding proteins. To evaluate if these properties could be exploited therapeutically, specimens from three convexity meningiomas were used for estrogen- and progesterone-binding protein assays and establishment of tissue cultures. Each tumor (designated A, B, and C, respectively) was grown in experimental media containing 7.5 × 10−5 to 10−12 M 17β-estradiol, 2.5 × 10−4 to 10−12 M progesterone, 10−7 to 10−9 M tamoxifen (an estrogen antagonist), and 10−6 to 10−10 M RU486 (a progesterone antagonist). After incubation, cell growth was compared to control preparations by counting the meningioma cells present in each medium.
Tumors A, B, and C contained estrogen-binding proteins of 8.45, 13.6, and 26.9 fmol/mg cytosol protein and progesterone-binding proteins of 210, 130, and 126 fmol/mg cytosol protein, respectively. The media containing 17β-estradiol and progesterone caused 21% to 36% growth stimulation in Tumors A and B. In Tumor A, the addition of tamoxifen stimulated growth by 35%, while it caused only transient stimulation in Tumor B and had no effect on Tumor C. RU486, the progesterone antagonist, caused inhibition of cell growth in all three tumors, ranging from 18% to 36%.
These data suggest that selected meningiomas are subject to hormonal influence in vitro. The inhibition of meningioma growth in vitro by the antiprogesterone, RU486, has not been previously reported, and serves to encourage further development of alternative modes of therapy for recurrent and unresectable meningiomas.
Linda Ansbacher, Nancy Low, David Beck, David Boarini, Charles Jacoby and Pasquale A. Cancilla
✓ Neoplastic angioendotheliosis is a rare disorder characterized by intravascular neoplastic proliferation of endothelial cells within vessels of all caliber in the meninges and neuropil. Ischemic infarcts of brain and spinal cord result from occlusion of the lumina by neoplastic cells or fibrin thrombi. Transition from reactive to neoplastic endothelium can be identified in many vessels. Steroid therapy can be beneficial in reduction of severity of symptoms, but cannot alter the course of disease. Therapeutic intervention must be undertaken promptly after the diagnosis is confirmed by meningeal and cortical biopsy if the inexorable course of the disease is to be altered.
Carter E. Beck, Norman W. Rizk, Lydia T. Kiger, David Spencer, Laureen Hill and John R. Adler
✓ A patient with severe and protracted symptoms from intracranial hypotension is described. The patient's presentation was marked by diffuse encephalopathy and profound depression of consciousness. This case report expands the presently known clinical spectrum of this uncommon and generally benign illness. The clinical and laboratory findings typically observed in the syndrome of intracranial hypotension are outlined. The pathophysiological mechanisms of the phenomenon are briefly discussed. Intracranial hypotension is a potentially severe illness with specific treatments that are distinct from the treatment of most neurological diseases. Three cardinal features—postural headache, pachymeningitis, and descent of midline cerebral structures—should prompt the diagnosis.
David S. Hersh, Julie E. Hoover-Fong, Natalie Beck, Amir H. Dorafshar and Edward S. Ahn
Recent reports have described early endoscopic suturectomy as a treatment option for patients with syndromic craniosynostosis, but such patients often require subsequent calvarial remodeling. The authors describe their experience with this patient population and seek to identify predictors of sufficiency of endoscopic surgery alone.
The medical records of patients with syndromic craniosynostosis who underwent endoscopic repair were retrospectively reviewed. Demographic data, operative details, and follow-up data were collected.
A total of 6 patients with syndromic craniosynostosis underwent endoscopic surgery followed by helmet therapy during the study period. Of these, 3 patients were male. The involved syndromes included Crouzon, Pfeiffer, Jackson-Weiss, Muenke, Saethre-Chotzen, and craniosynostosis-3 (n = 1 each). The patients underwent endoscopic surgery at a median age of 2.1 months (range 0.9–4.1 months). The median estimated blood loss was 30 ml (range 20–100 ml), with 2 patients requiring a transfusion. The median length of stay in the hospital was 1.5 days (range 1–4 days), and the median follow-up was 29.0 months (range 16.8–81.7 months), with 1 patient (16.7%) requiring an open revision. Three patients (50%) were classified as Whitaker Category I at the last follow-up. The patients for whom additional open surgery was performed or recommended (Whitaker Category IV) were the oldest patients in the cohort, ranging from 2.6 to 4.1 months at the time of surgery.
This series demonstrates that endoscopic surgery can be sufficient to treat syndromic craniosynostosis without subsequent open calvarial remodeling over a median follow-up period of at least 2 years. The findings suggest that younger age at the time of endoscopic surgery may be an important factor in determining the sufficiency of this procedure. Even among patients who require subsequent open calvarial remodeling, early endoscopic surgery may allow for growth and development of the brain and skull while delaying the need for open remodeling until the patient is older and can better tolerate the procedure.