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Ossama Al-Mefty, Gary Marano, Srinivasan Rajaraman, G. Robert Nugent, and Nathaniel Rodman

✓ The authors report 22 cases of transient ischemic attacks (TIA's) manifested by amaurosis fugax or hemiparesis or paresthesia of less than 24 hours' duration. None of the patients demonstrated 1) evidence of atherosclerotic cerebral vascular disease on angiography, 2) evidence of intracranial lesion on brain scan, 3) cardiac source of emboli, 4) arteritis or collagen disease, or 5) history of migraine. The only abnormalities found to explain the TIA's were abnormally increased platelet adhesiveness and/or aggregation. All of these patients were followed from 1 to 5 years, and had repeated coagulation studies. Treatment with antiplatelet drugs showed an excellent clinical response with associated decrease in platelet adhesiveness and aggregation. Discontinuance of the antiplatelet drug resulted in a recurrence of the TIA's which coincided with an increase in aggregation and adhesiveness. In two cases the platelet morphology was studied by transmission and scanning electron microscopy. It appears that there is a specific group of patients with TIA's in whom the sole cause of the attack is an abnormality of platelet function. For these people there is a specific therapy and a method of monitoring the treatment.

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G. Robert Nugent, Ossama Al-Mefty, and Sam Chou

✓ Eleven cases of presumed aqueductal stenosis with onset of symptoms after the first decade were reviewed. Ten patients had complete occlusion and one a high-grade stenosis. In 10, the dilated lateral ventricles caused a marked inferior displacement of the third ventricle. Postshunting diagnostic studies on six of these patients revealed ascent of the third ventricle, and in three of these the aqueduct was shown to be patent. It appears that in some cases of advanced communicating hydrocephalus the descending third ventricle kinks or pinches shut the aqueduct, adding an obstructive component which accelerates the clinical picture. The mechanism and clinical features of this process are discussed.

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Ossama Al-Mefty, John R. Jinkins, and John L. Fox

✓ A child with an extensive dural arteriovenous malformation (AVM) of the sigmoid sinus and bilateral occlusion of the transverse sinus is reported. His intracranial venous flow had become rerouted through the right cavernous sinus and then into the right ophthalmic veins. The AVM was associated with severe hydrocephalus and a progressive devastating neurological deficit. The hemodynamic and intracranial pressure changes as well as the mechanism of sinus thrombosis are discussed. The role of aggressive surgical treatment is emphasized.

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Ossama Al-Mefty, John L. Fox, Nayef Al-Rodhan, and James H. Dew

✓ Osteopetrosis is a rare disorder characterized by generalized increased skeletal density with abnormalities of bone modeling. The skull base is usually involved. Loss of vision with optic nerve atrophy is the most common neurological finding and is traditionally believed to be the result of optic nerve compression within the compromised optic canal. However, retinal degeneration has recently been described and is hypothesized to be the etiology of the visual loss (thus challenging the value of surgical decompression).

The authors report their experience with six children suffering from osteopetrosis and severe visual loss. All six patients underwent bilateral microsurgical optic nerve decompression through a supraorbital craniotomy. Improvement in visual acuity occurred postoperatively in five patients, and none had complications. Two technical points are emphasized: 1) optic nerve decompression should be wide and include not only unroofing of the canal but also drilling along both sides of the optic nerve, and 2) the thick, irregular, and highly domed orbital roof should be smoothed down by high-speed drilling to facilitate surgical exposure with minimal retraction of the frontal lobe.

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Ossama Al-Mefty, Louis H. Harkey, Troy H. Middleton, Robert R. Smith, and John L. Fox

✓ Eighteen cases are presented in which magnetic resonance (MR) imaging demonstrated two types of lesions in patients with cervical spondylotic myelopathy. In the first type, localized spinal cord changes at the level of compression, consistent with myelomalacia, were revealed best with T2-weighted images as high-intensity spinal cord signals. In the second type, lesions consistent with either cystic necrosis or secondary syrinx were noted locally, and/or extending longitudinally up, and/or down inside the spinal cord. These latter lesions were best revealed as low-intensity signals on T1-weighted MR images and as a signal-void sign (moving fluid) on proton-density or T2-weighted MR images.

It is suggested that segmental lesions at the level of the spondylotic bar represent early proton changes from pressure in and around the same zones that evolve into gray-matter enhancement regions shown as “snake-eyes” on delayed computerized tomography (CT) after myelography. The longitudinal lesions are thought to be the same pencil-shaped zones of cystic necrosis evolving into a secondary syrinx in the late stages (and usually found in the anterior portion of the dorsal columns during delayed CT after myelography). As spinal MR imaging continues to improve, these lesions will be demonstrated more clearly within the cord substance.

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Ossama Al-Mefty, Jane E. Kersh, Anupam Routh, and Robert R. Smith

✓ Radiation therapy plays an integral part in managing intracranial tumors. While the risk:benefit ratio is considered acceptable for treating malignant tumors, risks of long-term complications of radiotherapy need thorough assessment in adults treated for benign tumors. Many previously reported delayed complications of radiotherapy can be attributed to inappropriate treatment or to the sensitivity of a developing child's brain to radiation.

Medical records, radiological studies, autopsy findings, and follow-up information were reviewed for 58 adult patients (31 men and 27 women) treated between 1958 and 1987 with radiotherapy for benign intracranial tumors. Patient ages at the time of irradiation ranged from 21 to 87 years (mean 47.7 years). The pathology included 46 pituitary adenomas, five meningiomas, four glomus jugulare tumors, two pineal area tumors, and one craniopharyngioma. Average radiation dosage was 4984 cGy (range 3100 to 7012 cGy), given in an average of 27.2 fractions (range 15 to 45 fractions), over a period averaging 46.6 days. The follow-up period ranged from 3 to 31 years (mean 8.1 years). Findings related to tumor recurrence or surgery were excluded.

Twenty-two patients had complications considered to be delayed side effects of radiotherapy. Two patients had visual deterioration developing 3 and 6 years after treatment; six had pituitary dysfunction; and 17 had varying degrees of parenchymal changes of the brain, occurring mostly in the temporal lobes and relating to the frequent presentation of pituitary tumors (two of these also had pituitary dysfunction). One clival tumor, with the radiographic appearance of a meningioma, developed 30 years post-irradiation for acromegaly. This study unveils considerable delayed sequelae of radiotherapy in a series of adult patients receiving what is considered “safe” treatment for benign brain tumors.

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Ossama Al-Mefty and Vinod K. Anand

✓ A modification of the preauricular skull-base approach is described. After sectioning and downward displacement of the zygomatic arch, the coronoid process of the mandible is dissected and sectioned at its base. The temporal muscle, with its coronoid insertion, is then retracted upward. This approach provides direct and unobstructed access to the temporal and infratemporal fossae. Adequate vascularity of the temporal muscle is maintained. The exposure encompasses the internal carotid artery in the neck for vascular control. Extensive reconstruction is eliminated. The described technique was used in seven patients with lesions of the skull base. There was no operative mortality, and morbidity consisted of temporary restriction of mandibular opening in two patients.

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Ossama Al-Mefty

✓ Anterior clinoidal meningiomas are frequently grouped with suprasellar or sphenoid ridge meningiomas, masking their notorious association with a high mortality and morbidity rate, failure of total removal, and recurrence. To avoid injury to encased cerebral vessels, most surgeons are content with subtotal removal. Without total removal, however, recurrence is expected. Recent advances in cranial-base exposure and cavernous sinus surgery have facilitated radical total removal.

The author reports 24 cases operated on with vigorous attempts at total removal of the tumor with involved dura and bone. This experience has distinguished three groups (I, II, and III) which influence surgical difficulties, the success of total removal, and outcome. These subgroups relate to the presence of interfacing arachnoid membranes between the tumor and cerebral vessels. The presence or absence of arachnoid membranes depends on the origin of the tumor and its relation to the naked segment of carotid artery lying outside the carotid cistern. Total removal was impossible in the three patients in Group I, with postoperative death occurring in one patient and hemiplegia in another. Total removal was achieved in 18 of the 19 patients in Group II, with one death from pulmonary embolism. In the two patients in Group III, total removal without complications was easily achieved.