The Sturge-Weber syndrome belongs to the relatively uncommon group of ailments known as heredodegenerative disorders. In 1879 Sturge described a case of epilepsy in a young girl who exhibited a congenital glaucoma and a facial angioma or naevus flammeus. Sturge speculated that the patient's convulsions were caused by a similar naevus formation in the brain. Kalischer (1897, 1901) discovered angiomatotic changes in the meninges, and later the typical calcium deposits corresponding to these angio-mata were found in X-ray examinations by Weber (1922), Dimitri (1923), and Krabbe (1934). The appellation of the Sturge-Weber syndrome varies, owing to the number of its investigators. Levison (1953) calls attention to this question of nomenclature.

It has been suggested (Mednick, 1958) that high drive acting “upon remote response tendencies is a major root of the disordered thinking of schizophrenics”. Thought disorder would be a product of a high level of drive, increasing generalization and reducing discrimination. The relevant drive in this context is anxiety.

Introduction

“I can't concentrate, doctor” is a common complaint among psychiatric patients. The phrase is usually applied in a general sense to describe a state in which the patient has ceased to read the newspapers or watch the television and can no longer cope adequately with his work. In clinical practice the performance of the “serial sevens” test has been thought to give an indication of impairment of this function, whatever it may be (Mayer-Gross, Slater and Roth, 1960). As the symptom is so common, a measure of its severity may be of some value. This paper describes an attempt to develop such a measure and discusses the possible implications of its failure.

There is no doubt that electro-convulsive treatment is a safe treatment, perhaps very safe, yet from time to time reports of a fatality appear in the literature. When death occurs, most authorities agree that it is the cardiovascular system that fails. When there has been no suggestion of respiratory mismanagement, and death has been unrelated to the actual fit, vagal over-activity has received attention as a cause of death. It has been said that almost the only physical contraindication to treatment is a recent coronary thrombosis. Whatever the truth of this may be, uncertainty exists as to the “buffetings” an ischaemic myocardium or a diseased arterial system will tolerate.

Since the initial observations of a relationship between depression and an increase in urinary adrenal glucocorticoids in psychiatric patients by Reiss (1953) and Rizzo et al. (1954), there have been a number of studies confirming this relationship using various chemical techniques (Board et al., 1957; Bunney et al., 1964 and 1965; Gibbons, Gibbons, Maxwell and Wilcox, 1960; Gibbons and McHugh, 1962; Gibbons, 1964; Kurland, 1964a and 1964b; Pryce 1964). One of the questions that immediately comes to mind in reviewing this work has to do with the specificity of this relationship. Perhaps the rise of urine corticoids in depression is a non-specific response to disturbance, as has been demonstrated in various forms of psychological stress (Hamburg, 1962) and in severe psychiatric disturbances such as acute schizophrenic episodes (Sachar et al., 1963). On the other hand it is tempting to hope that there may be a more specific relationship demonstrable between the complex psychobiological phenomenon of depression and some kind of neuroendocrine activation analagous to a syndrome like “periodic hypothalamic discharge” (Wolff et al., 1964). This syndrome is manifested by periodic episodes (lasting several days) of withdrawal and depression associated with marked elevations in 17-hydroxycorticoid excretion. Mandell et al. (1963) have recently demonstrated that subthreshold stimulation of limbic sites intimately related to hypothalamic modulation produces marked changes in plasma 17-hydroxycorticoids in man. Limbic areas have been related to affectual phenomena on a speculative basis since the classical papers of Papez (1937).

Sleep is essential for physical and mental health. In the last 15 years there has grown up the concept of the brain stem reticular activating system. Electroencephalographic studies have shown two qualitatively different and alternating kinds of sleep, the orthodox (“slow wave”, or “forebrain“) and the paradoxical (”hind-brain“, “rapid eye movement”, “activated“, or “dreaming”) phases (Akert et al., 1965). It may be predicted that in the next decade attention will turn increasingly to the chemical basis of sleep. If a man is deprived of sleep for 100 hours, it is extremely difficult to keep him awake and one may suppose that an abnormal biochemical state exists within his central nervous system.

In previous studies of somatosensory cerebral evoked responses in psychiatric patients, we found that two characteristics of these responses discriminated between patients with psychotic depressions and healthy or psychoneurotic subjects. The amplitude of the initial negative-positive component was larger and its recovery, as determined by administering paired “conditioning” and “test” stimuli, was retarded (11, 12, 13). Furthermore, after successful therapy, the recovery measure changed in the direction of “normal” (11).

Saxondale Hospital was one of the first in the Sheffield Region to start Industrial Therapy. Since June 1960 the unit has grown, more patients are employed, and we hope for further expansion in the near future. From our experience it seemed that Industrial Therapy was useful in the management of the chronic psychiatric patient. However, we felt that a more objective assessment could be obtained if we surveyed eight comparable hospitals. A personal survey has certain advantages over postal questionnaires, and we decided to conduct a complete survey of one region. All visits were made between November 1964 and January 1965.

The need for the development of shorter and more effective techniques of psychotherapy justifies a careful investigation of any methods which offer this possibility. A number of therapeutic techniques (7, 15, 18, 20) now often subsumed under the heading of Behaviour Therapy (4) and based on various principles of learning theory call for examination, since their exponents claim that these methods can make therapy both shorter and more effective than can other methods, e.g., those based on psychoanalytic theory. Published reports of results with behaviour therapy to date consist largely of the assessment of results made by a therapist on his own patients, e.g., Wolpe (20, 21), Lazarus (9), Rachman (12). Cooper (2) studied results achieved by other therapists on thirty patients treated with various techniques of behaviour therapy. His survey included a comparison, using “blind” independent assessors, of a group of patients treated with behaviour therapy and a matched group of patients treated by other techniques. Cooper concluded that this comparison showed a definite advantage for behaviour therapy in the symptomatic treatment of phobias, but he could find no evidence supporting the claim of a superiority of the methods of behaviour therapy over conventional techniques in producing general changes. Unfortunately Cooper does not report the experience level of the therapists in his study except to say that they were a “variety of student and staff psychologists”. Evidence for a superiority of behaviour therapy must derive from a controlled comparison of matched groups of patients treated with techniques of behaviour therapy and with other techniques by therapists of comparable competence. But, pending judgment from such a controlled study, we believe ample evidence exists that techniques of behaviour therapy do bring comparable improvements more rapidly than do other treatments in some patients. The greater precision and shortening of therapy for these patients would alone constitute a very notable advance in psychotherapy, apart from claims of a wider or general applicability of these techniques. For this reason we believe it important to investigate these various techniques in as much detail as possible. Wolpe describes the most important of them as systematic desensitization (20, 21).

Since the initial review of patients in Glenside Hospital (Cooper and Early, 1961), a number of surveys have been carried out—Gore and Jones (1961), Norton (1961), Hassall and Hellon (1964), and Hassall, Spencer and Cross (1965). These studies are not directly comparable and all contain elements of subjective assessment.

One of the common clinical interests shared by physicians and psychiatrists is the genetically determined syndrome to which Klinefelter has given his name. Over the past few years positive identification has become possible through advances in cytogenetics, and more recently evidence has been accumulating which seems to indicate that a significant correlation may exist between Klinefelter's Syndrome and the development of other diseases, malignant disease in particular. Mental retardation is one of the most common symptoms found in the syndrome, so the psychiatrist is often in the best position to detect it. The case to be described here as well as having a few interesting facets of psychopathology may also provide more evidence of the pathological loading which the basic genetic abnormality seems to carry.

This investigation began as an attempt to measure the anxiety shown by patients while awaiting electro-convulsive therapy. One measure used was an assessment of the degree of palmar digital sweating, using the plastic paint technique described by MacKinnon (1963). This is a method by which the number of active sweat glands in a given area at a given time can be counted. In a preliminary trial, the effect on sweating of waiting for E.C.T. was equivocal, but there seemed to be an increase in sweating in depressed patients as their clinical condition improved. In the main trial, therefore, the emphasis was placed on differences in sweating of depressed patients when ill and recovered, though the reading in the E.C.T. waiting-room was retained.

Reorientation of the mental health services towards community care was one of the main recommendations contained in the report of the Royal Commission on Mental Health Law (1957) and accepted as Government policy. It was implied that there would be a considerable expansion in the services provided by local health and welfare authorities for the benefit of the mentally disordered, and local authorities were asked to draw up a proper order of priorities and plan their activities accordingly (Ministry of Health, 1959). The priorities depended upon an assessment of needs and time had to elapse before these became apparent. Since four complete years have passed since the coming into operation of the Mental Health Act, 1959, detailed assessment has become possible and this has revealed the difficulties experienced in the community by two special groups of patients, namely mentally abnormal offenders and patients of no fixed abode (N.F.A.s).

In a previous survey of hospital in-patients it was observed that among schizophrenics the earlier born were affected to a greater extent (Rao and Sundararaj, 1964). Following a suggestion by Slater (personal communication) the work was extended and further data collected on a larger number of patients to determine the role of birth order in schizophrenics. The present communication reports the findings.

Recent developments in the vexed questions of the relative importance to be attributed to genetic and environmental factors in the aetiology of schizophrenia, and the manner in which they interact to produce a phenotypic psychosis, are highlighted by David Rosenthal's series of critical papers (1959–1962) on twin and family studies and the book edited by him chronicling the lives of a set of monozygotic quadruplets all concordant for schizophrenia (1963). In his thorough and open-minded critique Rosenthal argues that previously reported concordance rates for monozygotic (MZ) twins have been misleadingly high. To this point we shall return in a moment. Rosenthal finally concludes that “the best information we now have with respect to the whole broad question of heredity and environment in schizophrenia, is to be found mainly in the five major studies of twins.” Unlike some who have pointed out weaknesses in the studies, he believes that “our task is to determine their source, extent and implications, not to dismiss them offhandedly because they contain errors” (1962a, p. 132).

In 1963 Ödegård published a catamnestic study of the 5,010 boys and girls who graduated (i.e. left after obtaining the “graduation certificate” by examination) from the high-schools (Gymnasiums) of Norway 1916–20. When this population group was followed until 1950, 117 were found to have been admitted to psychiatric hospitals for a psychosis. This material proved too small for a detailed statistical analysis, and the conclusions, although interesting and suggestive, remained inconclusive.

Patients with idiopathic narcolepsy are seen by both neurologists and psychiatrists. The diagnosis has traditionally been made on the basis of a history of frequently falling asleep against the patient's wishes, attacks of cataplexy or loss of muscle tone upon sudden emotion, episodes of sleep paralysis, and an account of vivid, dream-like experiences during the periods of involuntary sleep.

In the practice of our department at the Middlesex Hospital, the need has been felt for a means of rapid quantification of common symptoms and traits relevant to the conventional diagnostic categories of psychoneurotic illness. Until now, none of the available British scales have fulfilled this requirement. The most widely used is the Maudsley Personality Inventory (Eysenck, 1959) with its subsequent modifications. This, however, although scientifically based, is limited to the assessment of broad categories such as “neuroticism” and “extraversion” which appear to go only a small way towards describing the wide variability of psychoneurotic disturbances. Foulds and his co-workers (Foulds, 1965) have for a number of years developed personality scales in which clinical sophistication and a rigorous methodology are combined. Their Symptom-Sign Inventory, however, consists of eighty questions which have to be presented orally. Furthermore, although the inventory covers psychotic disturbances, it is necessary to use an additional questionnaire, the Hysteroid-Obsessoid Questionnaire (Caine & Hawkins, 1963) to complete the spectrum of psychoneurotic illness. Moreover, the concept of psychiatric illness developed by these workers is individual rather than conventional in a number of respects. The Tavistock Self-Assessment Inventory (Sandler, 1954) is too long for the present purposes (876 items in six booklets). The Taylor Manifest Anxiety Scale (Taylor, 1953) is short and convenient, but it measures one dimension only. As many doctors, including psychiatrists, find it useful to think in terms of orthodox clinical categories for diagnostic, therapeutic, prognostic and research purposes, it was decided to design and attempt to validate a self-rating scale adapted to these categories, taking the patient 5–10 minutes to complete and capable of being rapidly scored by the doctor or an assistant.