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A familial syndrome is described which, to our knowledge, has not been previously reported. The entity is transmitted by the dominant mode of inheritance. It is characterized by an annular distribution of pigment, which predominates in the nasal and inferonasal retinal quadrants, and chronic edema of the optic nerve, progressing to optic atrophy. Edema of the optic nerve in any variant of retinitis pigmentosa (RP) has not been reported. We believe that the incidence of five cases in this family makes a unique syndrome. Because two of the members have been inadvertently studied for evidence of intracranial pathology, it was deemed necessary to report this family in the ophthalmic literature.

Doppler ultrasound recordings were made from the anterior cerebral arteries of 40 normal full-term babies and 14 mature babies with intracranial pathology. The maximum velocity waveforms were extracted using spectral analysis and further analyzed using the Pourcelot resistance index method and the principal component method. Principal component analysis was found to be superior to the currently widely used resistance index method and may significantly enhance the value of Doppler ultrasound for detecting abnormal cerebral haemodynamics in neonates.

Intracranial pressure (ICP) has the characteristic of a dynamic physiological phenomenon, and demonstrates many kinds of variation in a day. These ICP variations accompany such physiological phenomenon as respiration, pulse, etc., while ICP rises in cases where there is some intracranial pathology. It therefore is necessary to record ICP continually in order to clarify the mechanism of ICP variation due to intracranial pathology. Sleep is one of the physiological phenomena in diurnal rhythm influencing ICP. It has been observed that ICP increases during REM sleep (SREM).

Forty-seven patients with acute mastoiditis were treated in our hospital over a 2 and a half year period. Seven patients were symptomatic for a short period only. The other 40 gave a history of chronic ear disease, 22 of them with cholesteatoma. Post auricular swelling is an important sign of acute mastoiditis but was found in only 64% of the patients. Forty-five percent of our patients were found to have infection spread beyond the mastoid. Meningitis was the most common complication (13 patients). Most of the patients who presented without post auricular swelling were diagnosed as a result of having one or more complications. We believe that in cases of intracranial pathology or septic fever associated with ear disease, the diagnosis of mastoiditis is most likely. Early operation combined with appropriate antibiotic treatment can prevent further complications and fatal outcome.

Earlier recognition and superior definition of intracranial pathology by computerized tomography (CT) have demanded the introduction of CT imaging into the operating room itself1. A CT Scanner dedicated to improved surgical diagnosis and therapy has been combined with a compatible stereotaxic device to promote exploration, and removal or treatment of a wide spectrum of brain lesions.

The CT head scan is a valuable new device for the diagnosis of intracranial pathology in children. It is especially useful in detection of cerebral tumors, hydrocephalus, blood-fluid collections, and congenital anomalies. Because of its expense, it cannot be recommended for use in screening of children with seizures, headaches, or nonprogressive focal neurologic signs. Other new ultrasonic and tomographic techniques are being developed, making it advisable to use the radiologist as a clinical consultant in selection of appropriate neuroradiologic procedures in children.

Increase in intracranial pressure (ICP) is one of the most common complications in patients with intracranial pathology. In neuroanesthesia the necessity of avoiding drugs which have the potential to increase ICP is well known. This study was designed to evaluate the effects of Propofol (P) on ICP in patients, in comparison with Thiopentone (TPS), in order to assess the suitability of this new intravenous anesthetic agent in neuroanesthesia.

Patients were referred to the Department of Nuclear Medicine for brain scintigraphy to be screened for possible intracranial pathology. These referrals were made in order to reduce the heavy load on the transmission computerized tomography (TCT) facilities. Great clinical importance, therefore, has been attached to scintigraphic findings; this emphasizes the need for an accurate assessment of the predictive value of this procedure.

In infants suspected of intracranial pathology it is often desirable to know the level of the intracranial pressure (ICP). Whilst invasive procedures are seldom indicated, the open fontanelle affords the possibility of non-invasive pressure recordings. Since palpation of the fontanelle is considered unreliable more sophisticated techniques have been applied in recent years. Results applying a fiber-optic device to the open fontanelle have been reported by Gaab et al. (1980), Hill and Volpe (1981) and Vidyasagar and Raju (1977). Problems in interpreting results are due to the externally exerted pressure when applying the transducer to the fontanelle.

Two hundred and seventy-three ultrasound examinations in 140 premature infants were performed with a transportable real-time scanner. Evidence for intracranial pathology was found in 86 (61%) of infants. In 66 infants (47%) a hemorrhage and in 79 infants (56%) ventricular dilatation was present. Choroid plexus larger than 12 mm was suggestive for either a bleeding into or onto the plexus. Cavum septi pellucidi was present in 72% of examined infants.

A technique has been developed for obtaining an index of human CBF using antecubital administration of a bolus of nondiffusible radioactive indicator (99mTc-labeled human serum albumin). The CBF index is obtained from separate estimates of cerebral blood volume (CBV) and cerebral mean transit time (CMTT) in the region of cerebral tissue. Mean results found at two levels of arterial \( {P_{CO_2}} \) obtained in seven subjects with no intracranial pathology are presented in this paper.

It has been pointed out that an absolute value of ICP alone, although important, does not provide enough information about the on-going critical changes of intracranial hemodynamics. Using transcranial Doppler it is possible to record blood flow velocities (FV) of basal arteries of the brain allowing us to monitor the hemodynamic changes instantaneously (Aaslid et al. 1982). We assessed the correlation between ICP and FV to investigate a noninvasive measure of intracranial pathology.

A case of undifferentiated astrocytoma with rapid increase in size of the tumor as documented by sequential computer assisted tomography (CAT) of the head is reported. The case also demonstrates the sensitivity of computer assisted tomography in the detection of intracranial pathology, in the presence of fals localizing neurological findings.

The existence of an actively expanding cyst represents one of the most common complicating factors of many intraaxial lesions: in all these lesions the complete surgical removal is theoretically the most suitable therapeutic approach. Unfortunately, several topographic localizations as well as many anatomo-clinical reasons make the realization of this optimal solution difficult. More precisely, two different kinds of intracranial pathology offer evident examples of such difficulties: the cystic component of craniopharyngiomas (as it is largely referred to) (Miles 1977; Mori et al. 1980) and the (pseudo) cysts of the low grade gliomas (Szikla and Peragut 1975).

Over the past 2–3 years major advances in nuclear magnetic resonance (NMR) imaging have been made. This new non-invasive imaging technique has been developed to the extent that images of the human body can be made which are comparable in appearance to early X-ray CT scans (Moore et al. 1980; Doyle et al. 1981; Smith 1981). The images may be displayed as axial sections in a similar way to X-ray CT, but here the similarity ends. NMR images are composed of either different proton density or proton spin-lattice relaxation time (T1) measurements of the water in different body tissues, whereas X-ray CT images are composed of different X-ray coefficients of absorption. By studying the different proton compositions of different tissues it is possible to produce anatomically accurate images of the human body and to differentiate the different tissues therein. The normal grey and white matter of the brain have been clearly demonstrated (Moore et al. 1980) as have intracerebral tumours (Doyle et al. 1981) and other intracranial pathology (Smith to be published). In the abdomen it is possible to demonstrate the liver, spleen, kidneys and large blood vessels on the T1 images and to demonstrate the vertebral bodies and pelvic bones using the proton density images.

Electroencephalograms of 95 patients with an acute confusional state, referred over three years, were studied to determine their usefulness. The majority were abnormal, and those from patients with intracranial pathology often showed asymmetry of delta activity and paroxysmal discharges. These, and other features, suggested that the EEG was a helpful investigation.

Orbital phlebography has gained increasing importance in the evaluation of otologic and intracranial pathology. The practical use of this method in vascular and neoplastic lesions in the middle cranial fossa, as well as its probable value in cerebello-pontine angle tumours is reported.

High-intensity reverberation artifacts frequently occur within the fetal cranium during obstetric ultrasound examinations. One hundred ninety-six static ultrasonograms obtained from 100 consecutive obstetric patients were reviewed to determine the incidence, shape, and location of high-intensity reverberation artifacts within the fetal head. Such artifacts occurred in one-third of the fetuses examined. The most common appearance was an arc-shaped artifact that occurred most frequently in the fetal hemicranium farthest from the maternal skin surface. Such an artifact can mimic epidural fluid collections and cause confusion for examiners not familiar with this finding. A discussion of this "pseudoepidural" artifact, its cause, and ways of excluding underlying intracranial pathology is presented.

The value of Gadolinium DTPA (Gd-DTPA) in Magnetic Resonance Imaging (MRI) of intracranial pathology is well established (Stack et al. 1985). The usefulness of Gd-DTPA in MRI of the spine is the subject of recent studies (Baleriaux et al. 1987; Bydder et al. 1985; Slasky et al. 1987; Sze, Abramson et al. 1988; Sze, Krol et al. 1988). This report concerns our experience of spinal tumours encountered in one hundred and four patients with spinal disorders examined by MRI and Gd-DTPA.

To the Editor.— The introduction of the computerized tomographic (CT) scan in neurologic practice has added an important and valuable diagnostic tool to our armamentarium. Numerous papers are appearing in its praise; indeed a new journal entirely devoted to this new procedure is being published. No one doubts that the CT will revolutionize our diagnostic approach to intracranial pathology. In all this enthusiasm, very few have called attention to the very serious threat posed by the uncontrolled utilization of this procedure to the education and training aspects of neurology, as well as to the health and welfare of patients. How frequently does the family practitioner get a sense of false security about his headache or seizure patient when told that the CT scan is normal? How often is the CT substituted for a history and neurologic examination? How many patients are told that the CT scan will save them the

45.8% of the patients suffering from an acute haemorrhagic stroke presented a high serum bilirubin concentration in the first 5 days after the stroke. A parallel increase of direct bilirubin was observed. Normalisation of the bilirubin concentration was seen in the follow-up examination between the 6th and 15th day after the acute stroke. The parallel rise in the plasma of direct and total bilirubin in the haemorrhagic stroke patient suggests the possibility that intracranial pathology and increased intracranial pressure act together to cause changes in hepatic cell polarity.

A series of 440 patients studied by echoencephalography is reported. Rather than dividing the patients in the classic two groups of less than 3 mm and more than 3 mm deviation of the midline, the patients in this study were divided into three groups, correlating the degree of midline shift to the presence of intracranial pathology. Group I, showing 0 to 2.5 mm shift proves to be normal with a "miss" rate of only 10%. Group II, showing 2.5 to 3.5 mm shift is regarded as a borderline group with an incidence of significant intracranial pathology of 46%, clearly indicating the need for further evaluation. Group III, demonstrating greater than 3.5 mm shift from the midline, is designated with confidence as abnormal, with a yield of significant intracranial pathology of 100% in this study.Os autores estudaram por meio do ecoencefalógrafo 440 pacientes os quais foram divididos em três grupos, de acordo com a medida do desvio das estruturas centrais do cérebro (ao contrário dos clássicos dois grupos — com menos de 3 mm e mais de 3 mm de desvio). Subseqüentemente cada um dos três grupos foi analisado com relação ao diagnóstico final de cada paciente. Os pacientes do grupo I, com desvio máximo de 2,5 mm e considerados normais com relação ao ecoencefalograma, apresentaram aproximadamente 10% de patologia intracraniana que não foi denunciada pelo eco. O grupo II, considerado questionável e com desvio de 2,5 até 3,5 mm, mostrou uma incidência de patologia intracraniana de 46% e, porisso mesmo, claramente necessita outros meios diagnósticos. No grupo III, com mais de 3,5 mm de desvio e considerado anormal, quase todos os doentes apresentaram patologia intracraniana séria, principalmente de ordem tumoral.

The size and shape of the sella turcica can be significantly influenced by prolonged elevation in the cerebrospinal fluid pressure. Mechanisms whereby such alterations of the sellar architecture may be produced have been postulated in recent years (du Boulay and El Gammal, 1966). Thirteen patients with intracranial pathology and intrasellar extension of the subarachnoid recess are reported. Eleven of these patients had alteration in the architecture of the sella turcica. These cases suggest that the intrasellar extension of the subarachnoid recess may play an important role in the production of certain types of sellar changes (Categories I, III and IV). They also support the hypothesis previously advanced, explaining mechanisms by which some sellar changes occur in association with increased intracranial pressure (du Boulay and El Gammal, 1966).

The brain scan is now widely employed for the evaluation of patients with suspected intracranial lesions. It is a simple, safe, relatively accurate diagnostic procedure and may be used both to screen patients and to complement cerebral arteriography and air studies in the identification of intracranial lesions. Although not as accurate as the latter two procedures in the detection of intracranial pathology (1–3), the brain scan has the decided advantages of lower patient morbidity, better demonstration of the extent of the lesion, availability on an out-patient basis, and a simplicity that makes its repeated use practical in following the course of a disease. In spite of its value, the brain scan has two major disadvantages: (a) it fails to localize all intracranial lesions, particularly those related to occlusive vascular disease, and (b) a positive scan alone is not sufficient to accurately identify the etiology of the lesion (tumor vs. infarction, etc.). The introduction of the scintillation camera has...

This paper proposes a review of intracranial lesions in the dog as observed at the School of Veterinary Medicine, University of Pennsylvania.

Background The management of minimal, mild and moderate head injuries is still controversial. In 2000, the Scandinavian Neurotrauma Committee (SNC) presented evidence-based guidelines for initial management of these injuries. Since then, considerable new evidence has emerged. Methods General methodology according to the Appraisal of Guidelines for Research and Evaluation (AGREE) II framework and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. Systematic evidence-based review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology, based upon relevant clinical questions with respect to patient-important outcomes, including Quality Assessment of Diagnostic Accuracy Studies (QUADAS) and Centre of Evidence Based Medicine (CEBM) quality ratings. Based upon the results, GRADE recommendations, guidelines and discharge instructions were drafted. A modified Delphi approach was used for consensus and relevant clinical stakeholders were consulted. Conclusions We present the updated SNC guidelines for initial management of minimal, mild and moderate head injury in adults including criteria for computed tomography (CT) scan selection, admission and discharge with suggestions for monitoring routines and discharge advice for patients. The guidelines are designed to primarily detect neurosurgical intervention with traumatic CT findings as a secondary goal. For elements lacking good evidence, such as in-hospital monitoring, routines were largely based on consensus. We suggest external validation of the guidelines before widespread clinical use is recommended.

Real time (RT) ultrasonic sector scanning of the head was performed through the anterior fontanelle in 130 neonates for the following indications: birth weight

Bilateral acoustic tumors, often felt to represent a central form of von Recklinghausen's disease, pose a particularly challenging problem to the otologist. This retrospective study reviews the evaluation and surgical results in 15 patients with bilateral acoustic tumors from whom 23 tumors were removed. Early diagnosis can be achieved by clinical awareness of the disease, thorough radiologic evaluation, and appropriate family screening. Total tumor removal with preservation of hearing is usually possible if the lesion is not larger than 1.0 cm. Total removal with hearing preservation is usually not possible if the lesion is larger than 2.0 cm. Hydrocephalus and additional tumors can complicate the overall clinical picture. Guidelines for appropriate management of bilateral acoustic tumors are based on tumor size, hearing sensitivity, and associated intracranial pathology.

The effects of a mixture of diazepam and fentanyl on cerebral blood flow (c.b.f.) and cerebral metabolism of oxygen (CMRo2) were studied in eight normal subjects and 13 patients with organic brain disease. The coupling of flow and metabolism and the carbon dioxide responsiveness of the c.b.f. were studied also. In the normal subjects the injection of the mixture resulted in a significant decrease in c.b.f. (34%), and a similar decrease in CMRo2 (34.5%). The vasoreactivity of the brain to carbon dioxide was maintained. C.b.f. decreased in all patients with intracranial pathology.

The marked narrowing of the head which develops in many preterm infants often causes concern to their parents, who may worry that the unusual shape will persist or that it may be associated with intracranial pathology and developmental delay. The biparietal (BP) and anteroposterior (AP) diameters were measured weekly in 203 preterm infants during their stay in NICU, and at regular intervals up to three years of age. The AP/BP ratio was calculated (Baum J D and Searls D, Dev Med and Child Neurol. 1971 13: 576-581). This ratio rose from a mean of 1.36 in the first week to a mean of 1.48 at six weeks and fell to a mean of 1.42 by 13 weeks. Less mature babies and those with lower birth weights showed more flattening but this was not statistically significant. The presence of intracranial pathology detected by ultrasound did not affect head shape. 11% of babies showed marked early flattening (AP/BP ratio 1.55). These were compared with the rest of the group. At three years there was no significant difference in AP/BP ratio or in Griffiths Development Quotient. We conclude that by the age of 3 years the babies showing very marked early head flattening did not differ from the rest of the cohort in the areas examined. (NICU = Neonatal Intensive Care Unit)

In a volvular seizure, the patient walks repetitively in small circles. This may form the whole of the attack or may precede a generalized tonoclonic seizure. It may be due to primary epilepsy or secondary to focal intracranial pathology. The direction of rotation may be ipsiversive or contraversive to the side of the epileptic focus. Although associated with complex partial and adversive seizures, it forms a distinctive entity, which may be due to involvement of the striatum. It has probably been underreported in the past. Four cases are reported herein.

• We used real-time cranial ultrasonography to diagnose intracranial hemorrhage and to follow the development and resolution of posthemorrhagic hydrocephalus in a premature infant. This technique is a bedside procedure that provides detailed imaging of intracranial pathology in newborn infants, without removing them from their incubators or exposing them to ionizing radiation. (Am J Dis Child1981;135:319-321)

OPTOKINETIC nystagmus (OKN) was introduced as a clinical tool by Barany1in 1921, but in spite of intensive study and investigation, knowledge of the mechanisms underlying the phenomenon and the anatomic substrates involved in its production is incomplete. An "optokinetic center" has been assumed to exist mainly on the basis of analysis of optokinetic dysfunction in patients with lesions more or less well localized on clinical grounds. This hypothesis, while consistent with classical anatomic conjecture, has not proved fruitful in understanding the phenomenon. Witness the fact that little agreement has been reached regarding the location of the so-called "center."2-5 Although it is generally agreed that disturbances of optokinetic nystagmus are frequent in patients with intracranial pathology, the clinical value of optokinetic testing has been disputed. While some authors have insisted that a unilateral defect in OKN is virtually diagnostic of a parietal lobe lesion,4,6others have taken

The correct diagnosis of a cerebral aneurysm during the life of the patient has been regarded as somewhat difficult. Earlier writers on the subject were even doubtful as to whether a diagnosis could be made. In 1859, Gull 1 gave expression to this pessimism by writing: "Although we may from the circumstances sometimes suspect the presence of an intracranial aneurysm, we have at the best no symptom upon which to ground more than a probable diagnosis." In 1916, more than half a century later, with all the additional knowledge of the intervening years, Fearnsides 2 considered that this dictum still held true. However, this does not mean that we have ceased to gather knowledge in regard to the clinical features of the disease, especially with the advancement of neurosurgery and the consequent increasing interest in intracranial pathology. Our therapeutic horizon has grown wider, and this has given a tremendous impetus