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Samuel F. Ciricillo and Philip R. Weinstein

✓ The authors report a case of progressive foramen magnum syndrome due to deposits of calcium pyrophosphate dihydrate crystals, which caused reactive hypertrophy in the posterior longitudinal ligament at C-1 and in the transverse ligament of the atlas in an 84-year-old woman. This is the first reported case of symptomatic pseudogout in this anatomic location. Rapid neurological recovery followed transoral decompression of the cervicomedullary junction.

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Samuel F. Ciricillo and Mark L. Rosenblum

✓ To explore the potential usefulness of imaging studies in the diagnosis of focal central nervous system (CNS) lesions associated with acquired immunodeficiency syndrome (AIDS), the authors retrospectively examined the radiographic studies of 149 AIDS patients who presented with signs and symptoms of the three most common focal CNS lesions. Of these patients, 74 (50%) had Toxoplasma abscesses, 45 (30%) had primary CNS lymphoma, and 30 patients (20%) had progressive multifocal leukoencephalopathy (PML). Magnetic resonance (MR) imaging was more sensitive than computerized tomography (CT) in detecting lesions, especially in cases of PML. Whereas CT was unable to distinguish mass lesions caused by toxoplasmosis from those caused by lymphoma, 71% of the solitary lesions seen on MR images were lymphomas. These results indicate that empirical treatment for toxoplasmosis, the most common initial treatment for AIDS patients with neurological symptoms stemming from mass lesions, is not likely to be successful for patients with solitary lesions on MR images. Rather, early biopsy is advisable. If the presence of lymphoma is confirmed, the rapid initiation of treatment can allow prolonged high-quality survival.

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Samuel F. Ciricillo, Richard L. Davis and Charles B. Wilson

✓ The authors report the case of a patient harboring a posterior fossa neuroepithelial cyst who presented with positional facial weakness and syncope. The patient recovered rapidly after cyst fenestration and placement of an internal cyst-cisternal shunt. The pathogenesis and principles of diagnosis and management of these rare lesions are reviewed.

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Michael S. B. Edwards, William M. Wara, Samuel F. Ciricillo and A. James Barkovich

✓ Six children with a history of isolated facial nerve dysfunction or dizziness and nausea were treated for brain-stem glioma between 1984 and 1992. Computerized tomography and/or magnetic resonance (MR) imaging showed a focal, uniformly enhancing mass involving the facial nerve nucleus of the pons. All patients underwent biopsy; the histological diagnosis was juvenile pilocytic astrocytoma in five cases. In the remaining case the biopsy was nondiagnostic, although the surgeon believed that the lesion was a glioma. Postoperatively, five patients underwent conventional focal megavoltage radiation therapy (180 to 200 cGy/day) over a period of 5½ weeks to a total dose of approximately 5400 cGy. One child's family refused radiation therapy; she remained well and stable for 4 years, despite persistent facial weakness, and was eventually lost to follow-up review. Four irradiation-treated patients had complete resolution of their tumors on MR images and have had no evidence of neuropsychological or neuroendocrinological deficits during 4½ to 8 years of follow-up evaluation. Patients whose neuroradiological studies show a lesion resembling those in this series should undergo biopsy and, if the histology of a low-grade tumor (in particular, a juvenile pilocytic astrocytoma) is confirmed, should then receive focal radiation therapy with conventional megavoltage dosages.

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Samuel F. Ciricillo, Philip H. Cogen, Griffith R. Harsh and Michael S. B. Edwards

✓ The best operative intervention for children with arachnoid cysts remains the subject of controversy. Recent reports stress that craniotomy for cyst fenestration is associated with a low incidence of morbidity and mortality and may leave the child shunt-independent. The cases of 40 pediatric patients with arachnoid cysts treated between 1978 and 1989 are reported. Five children with mild symptoms and small cysts that remained stable on follow-up studies have not required surgical intervention. Of 15 patients with cysts initially treated by fenestration, 10 (67%) showed no clinical or radiographic improvement postoperatively and have undergone cyst-peritoneal (eight patients) or ventriculoperitoneal (VP) shunting (one patient), or revision of a VP shunt placed for hydrocephalus before cyst fenestration (one patient). Two other patients with existing VP shunts required no further procedures. Thus, only three (20%) of 15 patients initially treated by fenestration remain shunt-independent after a median follow-up period of 8 years. The 20 other patients were initially treated by cyst shunting and all improved postoperatively; shunt revision has been necessary in six (30%) of these 20 patients because of cyst recurrence, Cyst location influenced the success of shunt treatment; none of the seven middle cranial fossa cysts treated by shunting have required revision, but results with cysts in other locations were less favorable. In all locations, though, shunting was more successful than fenestration. It is concluded that cyst-peritoneal or cyst-VP shunting is the procedure of choice for arachnoid cysts in most locations, including those in the middle cranial fossa.

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Samuel F. Ciricillo, William P. Dillon, Matthew E. Fink and Michael S. B. Edwards

✓ The case of a young girl with a pericallosal venous malformation associated with multiple cryptic vascular malformations (CVM's) is described. The presenting cryptic malformation, which hemorrhaged, was completely excised, but the venous malformation was not. Routine follow-up magnetic resonance images obtained over the past 9 years have documented the development of multiple new cryptic malformations along the radicles of the venous malformation. Magnetic resonance imaging and cerebral angiography revealed venous outflow obstruction at the junction of the venous malformation with the straight sinus. The association of CVM's with anomalous venous drainage patterns and the role of venous hypertension in the pathogenesis of cryptic malformations are discussed. This case suggests that CVM's associated with a venous malformation may recur and new ones may develop if the venous malformation is not excised, particularly if venous hypertension is also present. The likelihood of a surgical cure in these patients may depend on complete excision of both anomalies, which is rarely feasible because of the potentially devastating results of resecting a venous malformation. Alternative treatments for patients with both types of lesions are discussed.

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Van V. Halbach, Christopher F. Dowd, Randall T. Higashida, Peter A. Balousek, Samuel F. Ciricillo and Michael S. B. Edwards

Object. In this study the authors report on the results of endovascular treatment for mural-type vein of Galen malformations (VGMs) in a group of infants.

Methods. Eight children (six infants and two neonates) who suffered from symptoms caused by a mural-type VGM were treated by means of endovascular therapy. Their age at the time of treatment ranged from 13 days to 19 months (mean 7.6 months). Two neonates and three infants who presented with hydrocephalus and increased head circumference, one of whom was stabilized with a shunt, underwent elective closure of the malformations 3, 4, 6, 6, and 13 months later, respectively. Two patients presented with hemorrhage; one had an intraventricular hemorrhage (IVH) on the 1st day of life and one, a 5-month-old infant, suffered a large parenchymal hemorrhage and an IVH; both patients were immediately cured by means of endovascular techniques. One child presented with a seizure and cortical venous drainage that were treated immediately. Eleven separate treatment sessions were conducted; eight via transarterial femoral access and the remaining three via a transvenous approach. Two patients were treated by using transfemoral transvenous embolization with fibered coils, and one patient required a transtorcular transvenous approach to permit complete closure of the fistula with electrolytically detachable coils. The embolic devices used included silk suture emboli (three patients), electrolytically detachable coils (three patients), and fibered platinum coils (seven patients). In seven patients, complete closure was demonstrated on postembolization arteriographic studies. The eighth patient had stagnant flow in a giant 6-cm varix treated with arterial and venous coils but has not yet undergone follow-up studies. Late follow-up arteriography was performed in four patients at times ranging from 11 to 24 months postprocedure. In one patient, thrombosis of the malformation and shrinkage of the varix were confirmed on follow-up computerized tomography scanning. The remaining three patients have not yet undergone follow-up angiographic examination. Two asymptomatic complications occurred, including separation of the distal catheter, which was removed with a snare device, and a single platinum coil that embolized to the lung, producing no symptoms in 101 months of clinical follow up. The follow-up period ranged from 3 to 105 months, with a mean of 52 months.

Conclusions. Endovascular therapy is the treatment of choice for mural-type VGMs and offers a high rate of cure with low morbidity.