Mechanism of in vitro photohemiolysis in erythropoietic protoporphyria (EPP).

Four photosensitive patients with EPP showed markedly elevated RBC protoporphyrin levels. In vitro exposure of. a monolayer of their RBC in buffered saline to 3 x 10(6) ergs/mm2 of 4000 Å irradiation (= 45 minutes under a bank of fluorescent black lights) produced complete hemolysis within 24 hours; normal RBC showed negligible hemolysis after similar exposure. All of the cellular potassium was released from the patients' cells before the onset of significant hemolysis. Hemolysis was largely prevented by suspending the patients' cells in a 1:1 mixture of isotonic buffered NaCl and isotonic sucrose. These findings suggest that the initial photochemical lesion is of a "small hole" nature, creating channels through which small ions (but not sucrose) can diffuse. Since the effect of the intracellular colloid is offset by addition of sucrose to the external medium, the lesions appear to be less than 10 Å in diameter.

An ultrasound window to perform scanned, focused ultrasound hyperthermia treatments of brain tumors.

A series of calculations and tests were performed on polyethylene, polystyrene, acrylic, and a commercially available polymethyl methacrylate cranioplastic to determine which material would best serve as an acoustical window in the skull. The purpose of the window is to transmit focused ultrasound to treat brain tumors with hyperthermia. Each material was evaluated based on its ability to transmit power and to protect the brain. The results revealed that, of the four materials tested, polyethylene transmitted the largest percentage of incident power and is the toughest and best suited material to protect the brain. Further physical tests showed that a polyethylene plate does not significantly distort the sound field. Finally, acute and chronic dog tests (supported by theoretical calculations) using the polyethylene as an acoustical window through the skull showed that it will not reach temperatures greater than the target hyperthermia temperature (42 degrees C) at the required intensity levels unless the tumor is near the skull and the skin surface is not cooled. Since polyethylene effectively transmits power without distorting the sound field of overheating in dog tests, it may now be used in Phase 1 clinical ultrasound hyperthermia tests on human patients. However, the chronic animal studies indicated that the collagen which composes the dura thickens under the cranioplasty and absorbs increasingly greater amounts of power with time. A thick layer of organized fibrous tissue also formed on the external surface of the cranioplasty, filling in the cavity formed by the exterior surface of the cranioplasty and by the excision of the temporalis muscle.(ABSTRACT TRUNCATED AT 250 WORDS)

Cutaneous implantation metastasis complicating a superficial temporal-middle cerebral artery anastomosis.

A 52-year-old patient who underwent a superficial temporal-middle cerebral artery anastomosis subsequently developed an unusual squamous cell carcinoma in the skin overlying the anastomosis. The pathological picture and course of events suggest that this tumor was implanted into deeper tissues at the time of operation. Systematic microscopically controlled operation (Mohs' chemosurgical technique) was required to preserve the anastomosis while completely extirpating the tumor.

Posterior fossa subdural hematoma in a hemophiliac.

A successfully managed case of posterior fossa subdural hematoma occurring in a hemophiliac is reported, and the rarity of this lesion is mentioned. The management of the hemophiliac who is a victim of craniocerebral trauma is discussed, with emphasis on the use of computerized tomographic scanning to obtain rapid diagnosis. The importance of prophylactic Factor VIII replacement is emphasized, and appropriate methods of Factor VIII administration are outlined.

Intraventricular fiberscopic observations in adult hydrocephalic patients.

A flexible ventriculofiberscope was used to diagnose intraventricular pathological conditions in eight adult hydrocephalic patients. In each case the entire procedure was recorded on videotape for documentation, review, and teaching. Four representative cases are presented to illustrate the use and value of this technique.

Cerebral vasospasm following aneurysm rupture. A protocol for therapy and prophylaxis.

A retrospective study was made of 195 patients who had ruptured intracranial aneurysms without significant intracerebral hematomas and who recovered to at least Grade III by Hunt and Hess' classification. The first 121 patients underwent aneurysm surgery 10 days to 2 weeks after subarachnoid hemorrhage (SAH) without repeat preoperative angiography and without special attention to volume replacement or avoidance of hypotension. Vasospasm resulted in cerebral ischemia in 15% of this group, more than half of these postoperatively, and was treated successfully in half the patients with a combination of aminophylline and isoproterenol. The later 74 patients were managed with aggressive maintenance of normal circulating blood volume and preoperative angiography at 2 weeks following SAH. If significant vasospasm persisted on angiography, surgery was delayed an additional week and, if spasm was still present then, aminophylline and isoproterenol was added prophylactically to aggressive volume replacement before surgery. In this second group of patients, the incidence of clinical vasospasm was essentially unchanged; however, it was almost completely limited to the preoperative period, and was more effectively treated with aminophylline and isoproterenol. Postoperative vasospasm was almost completely eliminated from the second group of patients.

Dual cerebral and meningeal supply to giant arteriovenous malformations of the posterior cerebral hemisphere.

Seven cases of giant posterior hemisphere arteriovenous malformations are described. The significance of meningeal feeding vessels from the external carotid artery in addition to the primary cerebral supply through the internal carotid and vertebral arteries to these malformations is discussed. The necessity of bilateral selective external carotid arteriography is stressed, and the value of preoperative embolization is questioned.

Use of free latissimus dorsi musculocutaneous flaps in various neurosurgical disorders.

Musculocutaneous (skin-muscle) flaps have been used predominantly by plastic surgeons for a variety of reconstructive purposes. With the advent of microvascular techniques, the area to be reconstructed is no longer limited to the arc of rotation of the vascular pedicle of the muscle. Instead, the muscle and overlying skin that is best suited for the reconstructive procedure may be dissected out as a free flap, with microvascular anastomosis of an arterial supply and venous drainage to locally existing vessels. This report demonstrates the usefulness of musculocutaneous free flaps in the repair of large defects of the scalp, cranium, and dura after trauma or resection of invasive neoplasms. The authors describe the use of a latissimus dorsi free musculocutaneous flap in eight patients who would have required multiple operations to provide coverage by other techniques. There have been no major complications, and adequate repair has been accomplished, even in cases involving exposed brain.

Neurological manifestations of primary rhabdomyosarcoma of the head and neck in children.

The authors describe four of their own cases of primary rhabdomyosarcoma (RMS) of the head and neck and summarize an additional 25 cases collected from the world literature, each manifesting neurological involvement. The need for early diagnosis and radical surgery is stressed.

Complications of detachable balloon catheter technique in the treatment of traumatic intracranial arteriovenous fistulas.

As carotid-cavernous sinus fistulas (CCSF) are not life-threatening, therapeutic attempts should carry a very low morbidity and mortality. The authors believe that utilization of detachable balloons is the treatment of choice for CCSF, although this procedure is not without risk. Seven patients have been treated for high-flow traumatic intracranial fistulas over the past 2 years with detachable balloons using a coaxial catheter system. This paper presents several complications that have been encountered during this experience, and advances suggestions regarding treatment and avoidance of potential complications.

Effects of intravenous nitroglycerin on the intracranial pressure and volume pressure response.

Ventricular fluid pressure (VFP) and volume-pressure response were measured during nitroglycerin (NTG) infusion in nine patients anesthetized with N2O and fentanyl. The patients' ventilation was controlled, and PaCO2 was kept at 32 +/- 4 mm Hg. When an infusion of 0.01% NTG was given intravenously to decrease the mean blood pressure to 95.1%, 84.7%, and 78.2% of control, the VFP increased from control levels of 9.94 +/- 2.14 mm Hg to 12.89 +/- 2.25, 15.6 +/- 2.85, and 14.43 +/- 3.45 mm Hg, respectively. The volume-pressure response showed a significant increase when blood pressure decreased to 84.7% and 78.2% of control. These results suggest that intravenous NTG caused an increase in the intracranial pressure and a decrease in the intracranial compliance.

Concentration of 3',5' cyclic adenosine monophosphate in ventricular CSF of patients following severe head trauma.

Previous studies have demonstrated that cerebrospinal fluid (CSF) from the lateral ventricle of patients without disturbance of sensorium or intracranial pressure, contains 15 to 30 nM 3',5' cyclic adenosine monophosphate (cAMP). The concentration of this cyclic nucleotide was measured by radioimmunoassay in 133 samples of CSF from the lateral ventricle of 26 patients who were comatose following acute head trauma for periods up to 40 days. Concentration of CSF cAMP in diminishing coma Grades V, IV, III, II, and I was 1.5 +/- 0.1 nM; 1.24 +/- 0.34 nM; 3.14 +/- 0.7 NM; 10.06 +/- 3.47 nM; and 13.36 +/- 1.38 nM, respectively. After the sensorium cleared (coma Grade 0), cAMP was 22.0 +/- 1.7 nM. The correlation between the grade of coma and cAMP concentration was -0.80 (p greater than 0.01). These results imply that alteration in the level of consciousness following head trauma is associated with a disturbance of cAMP metabolism within the central nervous system. Possible mechanisms explaining this observation as well as therapeutic implications are discussed.

Hypothalamic hypothyroidism and hypogonadism in prolonged traumatic coma.

Prolonged coma afterhead trauma is associated with depletion of 3', 5' cyclic adenosine monophosphate (cAMP) in the cerebrospinal fluid (CSF). Because cAMP has previously been implicated in neurorendocrine secretion, this study examines the pituitay-hypothalamic function in 15 adult male patients (to exclude the effects of puberty and menses) with traumatic coma lasting longer than 2 weeks. Ventricular CSF cAMP was measured at 2- to 4-day intervals for 10 to 25 days. Simultaneously, plasma hormone concentrations were also determined. In all 15 cases, CSF cAMP and plasma levels of thyroid-stimulating hormone (TSH), thyroxine (T4), free T4, triiodothyronine (T3), luteinzing hormone (LH), follicle-stimulating hormone (FSH), and testerone became subnormal. In 11 patients whose level of consciousness fluctuated, the reduction in plasma T4 and testerone were proportional to both severity of coma ( r greater than 0.81, p less than 0.05) and depletion of CSF cAMP (r greater than 0.81, p less than 0.05). In four patients who remained deeply comatose for 17 to 25 days, the hypothyroidism and hypogonadism persisted. In six patients who regained consciousness, both endocrine defects improved partially or completely. Injection of 1) thyrotrophic-releasing hormone and 2) gonadotrophic-releasing hormone elicited normal or supernormal increases in plasma concentrations of 1) TSH, and 2) LH and FSH, reduced, respectively, suggesting a suprahypophyseal deficiency. These observations demonstrate that suprahypophyseal hypothryoidism and hypogonadism may occur regularly in patients with traumatic coma lasting longer than 2 weeks.

Bilateral giant intracavernous carotid aneurysms treated by bilateral carotid ligation.

Bilateral common carotid ligation was performed successfully in a patient with bilateral giant intracavernous carotid artery aneurysms. The patient presented with complete left ophthalmoplegia and impending right ophthalmoplegia, which was treated with right carotid ligation followed one week later by subtotal left carotid occlusion. Subsequent to this treatment, the patient had a dramatic recovery and remains well one year following operation.

Aminophylline and isoproterenol in the treatment of cerebral vasopasm.

Eighteen patients with cerebral vasospasm following subarachnoid hemorrhage were treated with 21 trials of intravenous aminophylline and isoproterenol. After angiographic demonstration of spasm, all patients were started on a continuous intravenous infusion of aminophylline 125 mg/hr and isoproterenol 125 micrograms/hr, preferably for a period of at least five days before the medications were tapered. Of the 21 treatment trials, there were 11 definite beneficial responses and ten failures. This study emphasizes the importance of instituting this therapy as rapidly as possible following the confirmation of symptomatic cerebral vasospasm. In addition, if a response occurs it will be noted within 24 hours of institution of the medications. If no response is noted following this time period, the medications should be discontinued. The major complications of this therapy include hypotension, tachycardia and arrythmias. These complications may necessitate diminishing the dosage of the medications. However, if a response has been obtained, every effort should be made to avoid discontinuing therapy prematurely since cerebral vasospasm may recur. The use of isoproterenol and aminophylline is based on their demonstrated ability to increase cyclic adenosine monophosphate (cyclic AMP) levels in vascular smooth muscle, thus producing relaxation of vasospasm.

Pseudoaneurysm complicating superficial temporal artery--middle cerebral artery bypass.

A patient with transient ischemic attacks from occlusion of a left common carotid artery was treated with common carotid endarterectomy immediately followed by superficial temporal artery-middle cerebral artery bypass. Subsequent angiography demonstrated a pseudoaneurysm formation at the anastomotic site. To our knowledge, this is the first report of this complication occurring in a human being.

Monitoring intraventricular pressure using an implanted reservoir in head injured patients.

Intracranial pressure monitoring facilitates the management of patients with severe craniocerebral trauma. The advantages of monitoring from the intraventricular space are outweighed by the increased incidence of infections with this technique. A system using an implanted Rickham reservoir attached to an intraventricular catheter with exchangeable external connections has been used successfully for monitoring intracranial pressure in 34 of 35 consecutive head-injured patients, with one minor infection.

Cerebral aneurysms of traumatic origin.

This report reviews 41 cases of traumatic cerebral aneurysms, including four cases of our own which are presented in detail. They may follow penetrating or closed head injury, and are usually associated with significant additional intracranial damage. Almost half of the patients presented with a delayed subarachnoid hemorrhage within three weeks of the initial head injury, defining an important neurological syndrome. Those patients whose post-traumatic aneurysms have been surgically obliterated have an associated mortality which is half that of patients treated by nonsurgical methods.

Continuous monitoring of intracranial pressure in severe closed head injury without mass lesions.

Intracranial pressure (ICP) was monitored continuously for up to two weeks in 40 patients with severe closed head injury with no angiographic evidence of mass lesion. Two-thirds of these patients had normal pressure. The results indicated that there is a lack of correlation between clinical presentation and ICP. Reliable prognostic factors include clinical evidence of brain stem dysfunction and age, but not intracranial pressure. From the data we have concluded that the clinical value of ICP monitoring in this type of head injury is uncertain.

Interventional neuroradiology: a joint effort of the neuroradiologist and neurosurgeon in the treatment of vascular lesions of the head and neck.

Close cooperation between the neuroradiologist and neurosurgeon has made possible the treatment of certain lesions of the head and neck by embolization procedures. The experience at Emory University Hospital with embolizations of arteriovenous malformations, carotid-cavernous fistulae, dural-cavernous sinus fistulae and glomus jugulare tumors is discussed.