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Congenital heart disease consists of cardiac anomalies that originated before or during the truncus arteriosus stage. The central nervous system, serving all organs, is the first to develop. It is the only organ possessing a third circulation. Immediately after the neural tube closes at the fourth week, its lining of immature ependymal cells secretes a proteinaceous neural tube fluid (NTF) at a pressure higher than the amniotic pressure. The resulting distention helps to shape not only the embryonic brain and spinal cord but also the bordering mesodermal cells that later will form vertebrae. The choroid plexus does not begin to secrete true cerebrospinal fluid until two weeks later. Should hypersecretion occur during this critical two week interval, the neural tube will overdistend and allow NTF to infiltrate into mesoderm (Fig. 1). Here, this fluid with its extraneous protein, may damage cells that are destined to form the anlagen of mesodermal organs such as the heart. It may also damage the primitive gut resulting in pulmonary, gastrointestinal and genitourinary anomalies. The most convincing evidence that the neural tube had been overdistended is the combination of anterior and posterior spina bifida that constitutes bilateral hemivertebrae. Vertebral anomalies are present in congenital heart disease though scarcely recognizable on the chest film of the newborn.

THE two chief manifestations of Graves' disease are hyperplastic goiter with hyperthyroidism and exophthalmos, the onsets of which usually are simultaneous. However, exophthalmos sometimes is the only manifestation of the disease. We consider Graves' disease to be the result of thyroidpituitary dysfunction. Exophthalmos that occurs in patients having Graves' disease is characterized not only by proptosis but also by various associated changes-These changes include retraction of the eyelids and manifestations resulting from increase in bulk of periocular tissues. This increase in bulk is caused by increases in the amount of fat and of mucopolysaccharides, and by an edema that has been proved not to be associated with an excess of water in the tissues (1, 2). The extraocular muscles become enlarged and infiltrated with fat and lymphocytes; eventually they become paralyzed and fibrosed. The proptosis may be extreme, with slight swelling of the lids and conjunctiva. Troublesome symptoms usually include a burn...

Difficulties encountered during operations upon the head or upper spine are frequently caused by: (1) awkward positioning of the patient; (2) increased venous pressure; and (S) embarrassed respirations. In many neurosurgical procedures these factors can be minimized by placing the patient in a sitting or semireclining position in a suitable operating chair. In 19351 we described a neurosurgical chair and the advantages of the sitting position particularly for operations upon the cerebellum and cervical spine. Disadvantages of this position are the occasional occurrence of postural hypotension and the rare development of air embolus. The basic requirements of a neurosurgical chair are as follows: (1) It should maintain the body in a position that will minimize the tendency to postural hypotension and air embolism. (2) Should hypotcnsion develop, it must be possible to place the head lower than the feet. (S) The chair should hold the patient's body in position without the use of restraining straps. (4) Its shape should be such that it is impossible for the patient to slump or to struggle out of position during the course of the operation. (5) It should have a readily adjustable headrest which will hold the head firmly, will permit the surgeon easy access to any portion of the head and upper spine, and at the same time afford the anesthetist free access to the air passages. (6) It should be comfortable for the conscious patient. The chair described below fulfills these requirements. The prototype of this chair originated 15 years ago and it has gradually evolved to its present form as the result of operating experience. Instead of the usual rlght-angled seat, this chair has a seat that is sharply flexed on the back rest, while the leg rest is extended. For the sake of stability, these angles are fixed. This shape prevents forward slumping and at the same time combats the tendency to venous pooling which contributes to postural hypotension and air embolism. To prevent sideways slumping, the back rest, seat, and leg rest are trough-shaped, which shape increases the area of contact with the patient's body and eliminates the need for padding. This in turn affords increased stability of the patient's body. Should the patient struggle at any time during operation, the force of gravity returns him to his original posture as soon as muscular contractions cease. During the 15 years that this type of chair has been used, with no padding other than a cotton blanket, no patient has had any pressure areas develop on the skin.

Some years ago, it came to my attention that a small tumor in the subarachnoid space may so elevate the protein content of spinal fluid that its mechanical interference with fluid removal will increase intracranial pressure. Leakage ofplasma from vessels in the tumor causes the high protein content and represents a breakdown of the blood-cerebrospinal fluid barrier (i). Tumors buried within the brain or cord are less apt to increase cerebrospinal fluid protein but do tend to become cystic. I suspected that this retained protein-rich fluid did not result from liquefaction necrosis but from leakage of blood plasma, i.e., a breakdown ofthe blood-brain barrier. Preliminary studies showed that the Tiselius pattern of cyst fluid resembled that of the patient's serum, offering support to the leakage theory (2). This investigation ofcyst fluids initiated a review ofthe subject of the blood-brain barrier, which proved so interesting that it has sidetracked the original problem.

Summary 1.The literature on peptic ulcer in infancy and childhood is reviewed. 2.Particular emphasis is placed upon the association of peptic ulcer with central nervous system lesions, not only in infants and children but also in adults. 3.A case of perforating and bleeding duodenal ulcer associated with medulloblastoma of the cerebellar vermis is presented.

THIS COMMUNICATION concerns phenomena resulting from the activation of inactive nerve fibers by the action current traveling in active fibers. This electrical transmission of a nerve impulse at a false synapse (ephapse) is readily demonstrated in the laboratory. When responsible for clinical symptoms, such misdirection does not readily lend itself to proof. However, Sjoerdsma and Gaynor1described a striking example of nerve stimulation which they attributed to muscle action currents. Their patient exhibited vigorous abdominal pulsations suggesting the presence of a large abdominal aneurysm. Fluoroscopical study showed the pulsations were caused by contractions of the left leaf of the diaphragm occurring synchronously with each cardiac systole. The patient proved to have alkalosis with latent tetany, and the phenomenon ceased when electrolyte balance was restored. These authors uncovered reports of three similar cases. They pointed out the proximity of the left phrenic nerve to the pericardium and postulated that

Traumatic subdural hematoma is a definite clinical entity that has been given considerable attention in the medical literature since Virchow's classic description1of "hematomas of the dura mater" in 1857. The lesion consists of an encysted collection of blood, situated between the dura and the arachnoid membranes, usually over the convexity of the cerebral hemisphere. It is my aim to sketch briefly the clinical and pathologic aspects of this interesting lesion and to dwell particularly on the reason for its delayed clinical manifestations. The majority of published reports of cases of traumatic subdural hematoma are similar in one respect, that is, in the occurrence of a latent interval between the reception of trauma and the onset of pressure symptoms. The cranial trauma that was responsible may have been severe or so trivial as to be readily forgotten. In many instances a history of trauma that had been denied previously

IT HAS BEEN ESTABLISHED that severing the hypophysial stalk in monkeys, without injury to the median eminence of the tuber cinereum, causes no evident modification in the water intake or urine output (i, 2, 3). But if the resection is so high that only half or less of the median eminence is left intact, severe diabetes insipidus invariably follows, as is also the case when the supraopticc hypophysial nerve tract is cut bilaterally in the hypothalamus (4). Since there are species differences, it is not only desirablebut in many cases necessary to check animal experiments by comparable clinical cases before application can be made to man, although there are indications that about the same results should be expected in human beings in this instance (5, 6). The present case is also of special interest because elevated blood pressure in certain cases has been regarded by some as an expression of hyperactivity of the hypophysis.

In this presentation an explanation is sought for the syndrome of increased intracranial pressure which occasionally accompanies an attack of otitis media and which is not due to meningitis, encephalitis or abscess of the brain. Quincke1in 1896 described a syndrome in which the signs of increased intracranial pressure were associated with the finding of clear cerebrospinal fluid on lumbar puncture. He stated the belief that in such cases an excess of cerebrospinal fluid results from hypersecretion of the choroid plexus. He referred to the condition as "serous meningitis" and described otitis as one of the predisposing factors. Various terms have since been coined to describe and label conditions of this type, such as "cerebral pseudoabscess," "meningismus," "serous meningitis," "serous ependymitis," "arachnoiditis," "hypertensive meningeal hydrops," "toxic hydrocephalus," and "otitic hydrocephalus." Recent articles by Symonds,2Frazier,3Dandy,4Davidoff and Dyke,5McAlpine,6Williams,7and Ersner and Myers6described and authors' experiences with

Excision of aneurysms of the hepatic artery involving or distal to the gastroduodenal artery requires restoration of blood flow to the distal hepatic arteries. Successful excision of such an aneurysm utilizing a splenic artery to distal hepatic artery anastomosis is reported.

This hypothesis is offered by a neurological surgeon interested in anomalies of the central nervous system. It is based on accumulating evidence indicating that some neural tube defects result not from failure of the tube to close but from its rupture after closure. The central nervous system, serving all organs, is the first to develop and its maldevelopment may cause damage to other emerging structures. The neural tube closes during the fourth week and is immediately distended by a proteinaceous neural tube fluid (NTF) secreted by its lining cells at a pressure four to five times that of the surrounding amniotic fluid. This NTF has been miscalled “cerebrospinal fluid.” The choroid plexus does not begin to secrete true cerebrospinal fluid (CSF) until 2 weeks later. If oversecretion of NTF should occur during this 2-week interval, the resulting overexpansion of the neural tube may spread apart the developing somites, eventuating in a combination of anterior and posterior spina bifida that constitutes bilateral hemivertebrae. If the distending neural tube ruptures beneath intact cutaneous ectoderm, the escaping NTF will infiltrate mesoderm. The resulting dislocation of cells and their possible injury by the extraneous protein may damage the as yet unidentifiable anlagen of mesodermal organs. If neural tube overdistention splits the underlying notochord and damages primitive gut, anomalies of entodermal organs may result. The neuroenteric cyst is one such anomaly that the neurosurgeon is called upon to treat. He finds it accompanied by hemivertebrae and hydromyelia. A preliminary report on this hypothesis has been published (Gardner and Breuer, '80).

AFTER evacuation of a subdural hematoma the compressed hemisphere may fail to expand spontaneously. This may be a grave prognostic sign. Trotter,1 Coleman,2 Coblentz3 and Voris4 have discussed this aspect of subdural hematoma. We agree with Coleman and Coblentz that failure of the compressed hemisphere to expand is more likely than cerebral edema to cause serious symptoms. Coleman found that the compressed hemisphere may not expand for several days after evacuation of the clot. It has been shown that the hemisphere may still be in a compressed state at autopsy even though the hematoma has been adequately evacuated.5 We have . . .

Congenital anomalies are often multiple and tend to occur in certain combinations. Polycystic kidney disease is frequently found with anomalies involving other organs of the body, such as cysts of the liver or pancreas. An association reported a number of times, but not given much clinical emphasis, is that of polycystic kidney disease and aneurysm of the circle of Willis. The reports of this combination have appeared mainly as necropsy findings,1the patients dying either of subarachnoid hemorrhage from rupture of the aneurysm or of renal failure from polycystic kidney disease. Recognition of the combination prior to death has been unusual, but, with present roentgenologic techniques, the diagnosis can be established and definitive treatment instituted after the appearance of symptoms. During the past two years, three patients have been seen at the Cleveland Clinic with congenital aneurysm of a cerebral artery and polycystic kidney disease. All three were relatively

It is not an uncommon occurrence for an intracranial aneurysm to rupture while it is being exposed at craniotomy. When this occurs, the resulting furious bleeding seriously hampers the surgeon's attempts to ligate or clip the neck of the aneurysm. Bleeding from the ruptured aneurysm can be stopped by temporary occlusion of both common and both vertebral arteries, affording the surgeon a much better opportunity of bringing the lesion under control. With the patient under hypothermia, arrest of the cerebral circulation has been maintained in this way for periods up to 14 minutes without evidence of brain damage. The occlusion may be achieved by closing arterial clamps previously placed on the exposed arteries in the neck or by exerting traction on a loop of tape encircling each artery and threaded through a catheter. Both methods are effective, but they have the disadvantage of requiring a separate manipulation to close or

LITTLE has been added by a century's research to the clinical description of Parkinson's syndrome as given by James Parkinson in his essay on "Shaking Palsy," written in 1817. The pathology of paralysis agitans and postencephalitic and arteriosclerotic Parkinson's disease has been fairly well clarified. The physiology of these disorders remains rather obscure. The treatment of Parkinson's syndrome is still essentially that of medical management. Browder 1 recently reported his results in sectioning the fibers of the anterior limb of the internal capsule in cases of parkinsonism and stated the belief that this procedure should be further investigated. The interruption of the sympathetic nerve supply to the cerebral blood vessels and the extremities as a method of treatment for various disorders of the nervous system is not new. In 1896 Jonnesco, 2 and in 1899 Alexander, 3 performed cervical sympathectomy in the treatment of epilepsy. Mixter and White 4 performed

Introduction Methylprednisolone acetate * has been administered intrathecally in more than 1,000 patients for the treatment of neurologic disorders 1-3 during the last three years. The treatment has proved safe, and the results are encouraging. It has become a standard method of treatment for arachnoiditis resulting from trauma, Pantopaque, or laminectomy. With this new method of administration we thought it expedient to ascertain: first, how long methylprednisolone acetate remains in the cerebrospinal fluid after intrathecal administration; secondly, whether methylprednisolone acetate injected at the lumbar level is dispersed evenly throughout the subarachnoid space, or remains in a higher concentration at the site of injection, by analyzing fluids obtained simultaneously at lumbar and cisternal punctures; thirdly, the duration of action of the steroid as judged by the effect on endogenous secretion of corticosteroids; fourthly, the effect on spinal fluid pressure, protein and cell content of various amounts of methylprednisolone acetate. Methods, Procedures, and

The paraplegic patient often has been dismissed as one of the unfortunate by-products of war or accident and thereby often has been the victim of neglect and ignorance. The large number of persons with paraplegia resulting from World War II injuries necessitated establishment of paraplegia centers where patients were rehabilitated as fully as possible. It soon became evident that the primary problems in these long-term patients were directly related to metabolic changes caused by their condition. Many of these changes had been noted in paraplegic patients in civilian life, but the small opportunity for study of large groups of patients often prevented the development of thought and investigation of these problems. In recent years there has been widespread reemphasis and realization of the importance of metabolism and its changes. It has been found that many of the metabolic changes heretofore apparently limited to the paraplegic person are nonspecific changes that

Excerpt Graves' disease may be considered to be a thyroid-pituitary syndrome. The two chief manifestations of the disorder are exophthalmos and hyperplastic goiter with hyperthyroidism. They usuall...

To the Editor.— A recent letter, "Diphenylhydantoin in the Treatment of Duodenal Ulcer" ( 219 :511, 1972), has encouraged me to express a long held hypothesis that both pernicious anemia and polycythemia are primarily of neurologic origin. In pernicious anemia the gastric mucosa is thin, pale, and atrophic. There is anacidity, low hemoglobin, posterolateral sclerosis, and no peptic ulcer. In polycythemia the gastric mucosa is thick, turgid, and hypertrophic. There is hyperacidity, elevated hemoglobin, no posterolateral sclerosis, and often peptic ulcer. All of which suggest that pernicious anemia may be the end result of sympathicotonia, ie, local sympathetic over-function (as in Raynaud's disease) affecting gastric mucosa. Conversely, its antithesis, polycythemia, may be the expression of the end result of vagotonia, ie, parasympathetic over-function affecting gastric mucosa. If this thesis is correct, both are primary neurologic disorders. Since splanchnicectomy raises gastric acidity and vagotomy lowers it, pernicious anemia should respond to the

In a prior communication 1 we described our attempts to modify herpes simplex following the decompression operation 2 on the fifth cranial nerve by using hydrocortisone ointments and bacterial ointments locally. In 148 patients, approximately one-third developed herpes, and we could not modify the attack rate by the use of the above agents. Since that time, 76 more patients have been operated on for tic douloureux using the same decompression or neurolysis type of procedure. Twenty-eight of these patients were painted with a 3% solution of an iodophor (Wescodyne) consisting of 7.75% polyethoxy polypropoxy ethanol-iodine complex; 3.75% nonylphenylether of propylene glycol-iodine complex; and 0.10% hydrogen chloride before and after operation twice daily. The use of this iodine complex did not modify the attack rate, as 12 of the 28 individuals developed florid herpes simplex. In addition, 4 patients were given poliomyelitis immune globulin (human), Merck Sharpe & Dohme, 10 cc.

Pulsating exophthalmos, or retrobulbar arteriovenous aneurysm, a lesion first reported by Benjamin Travers in 1809, is a relatively rare condition meriting report whenever seen. An average of about five cases is reported each year. Harkness1collected six hundred and twenty-one cases from the literature in his paper published in May, 1930. An exhaustive review of the literature is not attempted here, that angle of the problem having been dealt with ably by de Schweinitz and Holloway,2later by Locke3and still later by Harkness. Diagnosis of the lesion usually may be made from the patient's history and appearance alone. The history is fairly typical: After trauma resulting in a period of unconsciousness, the patient has failure of vision or blindness in one eye, unilateral exophthalmos and a rushing, roaring sound in the head synchronous with the pulse. Palpation of the eye commonly discloses a thrill, and auscultation

In the past few years, decompression of [ill] sensory root of the fifth cranial nerve [ill]Taarnhoj operation) has largely replaced [ill]esection of the nerve root as the surgical treatment for trigeminal neuralgia used at the Cleveland Clinic Hospital. * The advantage of the decompression operation is that the sensation of the face is preserved. Herpes simplex often follows intracranial operative procedures on the fifth cranial nerve. The large number of decompression operations done provides a predictable source of patients with herpes simplex for scientific observation, and the additional incentive to find a preventive for this annoying complication in the patient's postoperative recovery period. Several years ago, the impression gained in informal conversations with dermatologists was that most of them believed that, while the course of the well-developed herpes simplex eruption was not influenced significantly by hydrocortisone, applied topically, the drug might be of some value if used early in the course

The term chordoma refers to a neoplasm arising from embryonic rests of the chorda dorsalis or notochord, which in its development more or less reproduces the structure of the primitive notochord. Chordomas arise in the craniospinal axis in certain definite locations, the selection of which can be explained on an embryologic basis. While they rarely metastasize, the clinical course is characterized by slow expansile enlargement with destruction of adjacent bone and compression of neural structures and by recurrences after operation. EMBRYOLOGY The embryology of the chorda dorsalis has been reviewed by others1and will be given briefly here. The notochord forms the primitive axial skeleton of all vertebrates, and in certain lower forms, such as the Amphioxus, it forms the sole axial skeleton. The structure is formed primarily by the proliferation of cells from the anterior end of the primitive streak. While some writers consider the chorda to be

A kindred originally described 40 years ago now includes 97 individuals with definite or possible bilateral acoustic neuroma. The trait is autosomal dominant with high penetrance. Onset is at about 20 years of age and survival without surgery varies from 2 to 42 years. Severe accidents, including drowning, are common in the family, possibly due to neurological deficits. In screening such individuals, vestibular function is probably most sensitive, but complete audiometric studies, special roentgenologic examinations, and brain scan have proved useful. No single study can be relied upon to diagnose this disease or to exclude the diagnosis. Peripheral signs suggesting neurofibromatosis are rare but other central nervous system tumors, often asymptomatic, are common, which suggests that this trait represents a "central" form of neurofibromatosis. It is distinct from unilateral acoustic neuroma based on natural history and genetic etiology.

The Water-Bed The infant with hydrocephalus is prone to the development of pressure sores on the scalp, particularly during the postoperative period. This tendency can be eliminated by floating the infant on a bag of water, which is made redundant and relaxed by restraining it within an encompassing box. Such a bag may be made from a tube of plastic film with a flat width 2 to 3 times the width of the containing box. A piece of this tubing, twice the length of the box, is tied off at both ends after being filled with water to the desired level. Since the ends may not be rendered watertight, they should not be tucked underneath but should be maintained slightly above the fluid level. Figure 1 illustrates the principle applied to a bassinet. The patient, in fact, floats in water without getting wet. In actual use, a cotton blanket or

The most important single factor in the prevention or treatment of bedsores is frequent movement of the patient, so that pressure at any one point is not permitted to exist too long. Munro 1 stated that the essentials of treatment in cases of spinal cord injury are (1) to move the patient on an hourly time schedule, (2) to prevent the development of any serious exhaustion by such methods as appropriate feeding, transfusions and administration of vitamins, (3) to prevent the occurrence of sepsis and to maintain a constantly dry bed by proper use of a tidal drainage apparatus and (4) to avoid all forms of external artificial splinting or support to the spinal column so long as the patient with the spinal cord injury is bedridden. In certain types of cases, the frequent changes in the patient's position which are necessary to maintain the skin in a healthy state

The skeletal anomalies of the Klippel-Feil syndrome are present also in the adult with syringomyelia, 1 in the adolescent with diastematomyelia, 2 and in the infant with myelomeningocele. 1 These anomalies, believed due to improper segmentation of the mesodermal somites in embryonal life, 2 consist of varying degrees and combinations of dilatation, bifid states, fusion, shortening, and curvature of the craniovertebral column, with associated deformities of the rib cage and extremities. Within the lumen of the distended craniovertebral column, the central nervous system also exhibits varying degrees of distention of its lumen (hydrocephalus and hydromyelia). 1,3 Hypothesis This occurrence of a distended tube of neural tissue within a distended tube of bone suggests cause and effect, namely, that overdistention of the neural tube (hydrocephalus and hydromyelia or "hydrocephalomyelia") in embryonal life has resulted in distortion of the encompassing sclerotomes, which then give rise to distorted bones—that in the individual case

The removal of the right cerebral hemisphere is a procedure that seldom is indicated. From the surgical standpoint, its use is restricted to those cases of infiltrating glioma involving the midportion of the hemisphere in which it would appear that this procedure and none other could be effectual in removing the entire growth. From the humanitarian standpoint, it should be offered only to those patients desirous of living under the resultant adverse circumstances, which should also be carefully explained to the relatives. Dandy,1in 1928, indicated the feasibility of this procedure and reported five cases. Of this series, the first patient lived three and one-half years but died of recurrence of the growth; the second patient died within three months from recurrence; the third died of hemorrhage forty-eight hours after the operation; the fourth died two weeks after the operation from pneumonia, while the fifth was living two months

The olfactory groove meningioma is a benign tumor which arises from the midline of the floor of the anterior fossa of the skull, and its removal is beset with difficulties for the following reasons: First, the location of the tumor renders it relatively inaccessible; second, it is a large and vascular growth; third, its nutrient vessels come up through the base of the skull so that they are difficult to control until after the tumor is removed. The best method of dealing with these tumors is to perform a right frontal craniotomy and to excise the portion of the right frontal lobe overlying the lateral aspect of the growth. With the electrosurgical unit the tumor is then removed in fragmentary fashion, after which the nutrient vessels in the floor of the skull at its area of attachment are treated by application of the cautery and bone wax. The loss of

Tumors involving the spinal cord or its coverings in the vicinity of the level of the foramen magnum are rare. Cruveilhier1in 1856 reported a neoplasm the size of a pea in the left olive of the medulla in a patient who during life presented no symptoms. Although other isolated cases have been recorded by Balz,2Leyden,3Bostroem4and Gowers and Watzoldt,5these tumors have usually been observed incidentally at necropsy, and in almost every instance the clinical records have been meager. Martin and Greenfield6presented the complete clinical record for three years, with the necropsy report in a case of fibroblastic meningioma, weighing 32.5 Gm., that filled the cisterna magna. Elsberg7described a case of an aneurysm of the right vertebral artery that caused signs of extramedullary compression of the medulla and upper cervical region of the cord, but the patient died

Cysts lined by ependymal epithelium and yet isolated from the normal ependymal cavities of the brain are relatively rare. The case reported here is interesting in that the cyst caused central deafness which disappeared after the cyst was opened into the ventricular system. REPORT OF CASE History. —A girl, aged 16, came to the Cleveland Clinic on April 5, 1932, because of blindness and deafness. Eight months previously she had fallen from a tree and had been stunned for a few minutes. Failure of vision began soon after this. An ophthalmologist refused to fit her with glasses and made a diagnosis of tumor of the brain, which was not credited by the parents. Four months later she began to have recurring bouts of emesis. She staggered when walking, and had moderate frontal headaches which never became severe. Hearing had failed markedly. Before the accident the patient had had no illnesses

A most unusual survival of a gunshot wound of the head was apparently materially favored by an equally unusual treatment aimed at expansion of the area of cerebral defect.

It has been recognized that some relationship exists between meningeal growths and those tumors which arise from the nerve sheaths or their components, but only recently has the nature of this association been given any considerable study. Most of the interest has evolved about that special form of generalized neurofibromatosis in which the central nervous system is predominantly affected. Mainly through the work of Harbitz, 1 Preiser and Davenport 2 and Schaltenbrand, 3 generalized von Recklinghausen's disease has been long recognized as a familial disorder. Only recently, however, has it been shown that central neurofibromatosis is also hereditary in that special form of the disease and may be transmitted as a dominant trait with little or none of the peripheral manifestations of the disorder. 4 A special variety of central neurofibromatosis, bilateral acoustic tumors, has been described by Gardner and Frazier 5 and Gardner 6 and also established on a

In 1930, Gardner and Frazier 1 reported a family in which von Recklinghausen's disease, in the form of bilateral tumors of the acoustic nerve, had been transmitted as a dominant mendelian trait through five generations. At the time of the original report, 38 of the 217 members of the family were affected. Necropsy was performed on 2 members of the family and revealed bilateral acoustic neurofibromas. After the publication of the first report, symptoms of a tumor of the spinal cord developed in 1 of the affected members. This tumor was removed and likewise proved to be a neurofibroma; the case was subsequently reported by Gardner. 2 The present report, the third concerning this family, deals chiefly with the pathologic material obtained from 4 members since the last publication. 3 One of these members (case 4) represents the only case thus far observed in which the disease has been recognized