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Descrevemos 2 casos de coroidopatia lúpica, uma manifestação ocular incomum do lúpus eritematoso sistêmico, caracterizada por elevação serosa do epitélio pigmentário retiniano e/ou retina sensorial e moteamento do epitélio pigmentário. We describe 2 cases of lupus choroidopathy, an unusual ocular manifestation of systemic lupus erythematosus, characterized by serous elevation of the retinal pigment epithelium and/or sensory retina and pigment epithelium mottling.

Congenital infection by Toxoplasma gondii is a leading cause of visual impairment in children and the most frequent infectious etiology. Although congenital toxoplasmosis may affect many organs, eye manifestations are the most common. Lesions of retinochoroiditis affect visual function according to their localization relative to the macula and to the optic disc. After the neonatal period, scarred lesions of retinochoroiditis may reactivate or new lesions may appear. Therefore, in cases of congenital toxoplasmosis, even when fundus examination is normal at birth, a careful long-term ophthalmic follow-up is necessary.

The menopausal syndrome consists of a variety of psychic and physical signs of different intensity. At our outpatient department for problems in menopause and prevention of osteoporosis (1. Dept. of Gynaecol. and Obstetrics, Vienna) 1287 women who were consulting this department for the first time because of prevailing complaints or prophylactic reasons, were registered during the years 1988 and 1989. In the course of this work we paid particular attention to the symptom "eye complaint" as a sign of the beginning of the menopausal period. 98 out of 430 patients with eye complaints were examined ophthalmologically. Referring to our patient population we tried to analyse epidemiologically the frequency and the degree of severity of menopausal eye complaints as well as the therapeutical response to a hormone substitution therapy. To what extent an endocrinological explanation can be found and which facts lead us assume such a statement, we have tried to demonstrate in the discussion.

Trichinellosis is a parasitic disease, which is caused by the ingestion of raw meat containing infective larvae of the parasitic nematode trichinella. The severity of the clinical course depends on parasitic factors such as the species involved, the number of living larvae ingested, and host factors such as sex, age, ethnic group, and immune status. Eye manifestations of trichinellosis are wide-ranging from mild to severe. We report an exceptional occurrence of severe bilateral proptosis due to trichinellosis shown by a CT scan that was done before and MRI that was done after treatment with steroids and showed noteworthy improvement. Radiology images are extremely important in the evaluation of proptosis and should be used more often in support of its differential diagnosis and treatment assessment.

Giant cell arteritis is a disease of the elderly which is more common than previously recognized. It is important to be aware of this condition because treatment effectively relieves symptoms and prevents serious complications. The disease is suggested when an elderly patient complains of constitutional symptoms, headache, jaw claudication, or the musculoskeletal manifestations of polymyalgia rheumatica. Abnormalities in temporal arteries or other cranial arteries, or evidence of large vessel involvement may be detected by physical examination. A markedly elevated sedimentation rate in association with other clinical features of the disease strongly suggests giant cell arteritis, but a biopsy should be performed to confirm the diagnosis. Corticosteroid therapy should be started promptly in high doses in order to prevent blindness. Prolonged treatment with lower dose corticosteroids is generally necessary for up to 1 to 2 years, and sometimes longer, for continued symptomatic relief. Long-term follow-up of treated patients has demonstrated no detectable effect on survivorship.

The roentgenologic findings have been documented in 10 patients with enzymatically diagnosed deficiency of cystathionine synthase. Patients responsive to massive doses of pyridoxine have the largest amount of residual cystathionine synthase activity. Those who are unresponsive show virtually no residual activity. The pyridoxine-responsive patients show the mildest and fewest roentgenologic findings. Similarly, those patients unresponsive to pyridoxine are the most severely affected roentgenologically, although the type of involvement is variable.Calcific spicules and streaks in the radius or ulna may be diagnostically useful as an early finding in children. In young adults with thromboembolic disease, the presence of one or more of the diverse skeletal changes may suggest the diagnosis of homocystinuria.

Human volunteers were exposed in a controlled-environment chamber to propylene glycol dinitrate vapor at concentrations of 0.03, 0.1, 0.2, 0.35, 0.5, and 1.5 ppm. Physiological and central nervous system responses to the exposures were monitored. Exposure to concentrations of 0.2 ppm or greater produced disruption of the organization of the visual evoked response (VER) and headache in the majority of subjects. Subjects repeatedly exposed to 0.2 ppm for 8 hr on a daily basis developed a tolerance to the induction of headaches, but the alteration in VER morphology appeared cumulative. Marked impairment in balance became manifest after exposure to 0.5 ppm for 6.5 hr, while 40 min of exposure to 1.5 ppm added eye irritation to the list of symptoms.

Onchocercal microfilaruria was studied in the course of an epidemiologic investigation of diseases, infections, and general health conditions in a rural village on the Adamaoua Plateau in northern Cameroon. Microfilariae of Onchocerca volvulus were observed in nearly one half of the skin snips taken from the village residents. The prevalence of onchocerciasis as diagnosed from skin snips increased with age, and was greater for males than females. The number of microfilariae per skin snip also increased with age. The presence of subcutaneous nodules, inguinal lymphadenopathy, microfilariae in the urine, ocular lesions and prolonged itching all were related to both age and the presence of microfilariae in skin biopsy specimen, as well as to intensity of infection. Microfilaruria was not restricted to individuals with severe onchocerciasis.

The physical properties of lightning are given, including a description of the different observed lightning forms. The wide variety of effects of lightning on humans is reviewed. In the prehospital care of those struck by lightning, emphasis is upon immediate resuscitation of those who appear unresponsive. Recommendations for emergency department evaluation, treatment, and disposition are given. Guidelines to prevent humans from being struck by lightning are discussed.

Adriamycin (Doxorubicin) was perfused through the ventriculo-cisternal (VC) and ventriculo-lumbar (VL) spaces of Rhesus monkeys at inflow concentrations ranging from 1.5 to 100 μg/ml at an average inflow rate of 128 μl/min for 190 min. Animals died or became moribund within 10 days following perfusion of Adriamycin within the range of inflow concentrations of 6.0 to 100 μg/ml. General neurotoxicity consisted of general body weakness, tremors, severe to slight hypokinesia (especially of the hindlimbs), and signs of excitation, nervousness, or depression after a perfusion. Other adverse responses were a significant decrease in water and food intake, mydriasis, and loss of the wink reflex in one or both eyes. With the exception of isolated instances, only trasient minor changes occurred in heart rate and rhythm, respiratory rate, body temperature, cortical EEG, opening ventricular pressure, CSF formation rate, and inulin clearance. Conditioned avoidance behavior was not significantly affected in otherwise normal animals; although, at times, motor deficits of the arms may have interfered with performance. In the brains of three monkeys perfused at 100, 12.5, or 6.0 μg/ml, there was a distinctive necrotizing angiopathy that was noninflammatory and for which it is difficult to find an exact parallel in human pathology. In view of the particular findings shown in this study, it is not recommended that this drug be administered intrathecally in man.

The authors report of Argyll-Robertson-like pupils in three patients presenting the neural type of Charcot-Marie-Tooth (CMT) disease with peroneal muscular atrophy. Up to now the light-near dissociation had been reported in the hypertrophic variety of CMT disease. Stress is laid upon the fact that the presence of the light-near dissociation in patients with CMT disease does not help in clinically differentiating the neural from the hypertrophic type of this disease.

Two hundred and sixty-one neurologically asymptomatic patients were examined by computed tomography (CT) following a variable number of transient ischaemic attacks (TIAs) experienced over different time intervals. Cerebral infarcts were discovered in 101 patients. Dominant hemisphere infarcts were usually small, of recent origin and most often associated with less than 5 TIAs and a short history; whereas non-dominant hemisphere infarcts were found twice as frequently and were larger and older and correlated with repeated TIAs over many months. All infarcts, independent of size, were confined to a single vascular territory, suggesting haemodynamic rather than embolic causes. By comparing the age and size of the infarcts with the number of TIAs and their chronicity, the hypothesis emerged, suggesting that infarcts may occur early on and progressively enlarge with repeated attacks.

General examination of the eye was carried out in 22 patients with systemic lupus erythematosus (SLE) and in 10 with scleroderma. 3 of the SLE and 2 of the scleroderma patients had keratoconjunctivitis sicca. Fluorescein angiography showed abnormalities of the retinal vasculature in one of a subgroup of 12 SLE patients and one of 10 scleroderma patients. None of the 12 SLE patients had abnormalities of the choroidal vasculature, while 5 of the 10 scleroderma patients had patchy areas of nonperfusion of the choroidal capillary bed.

Clinically visible corneal opacities were observed in a patient with an extremely severe form of mucopolysaccharidosis II. In a second patient with an unusually mild form of mucopolysaccharidosis II, discrete corneal opacities were detected by slit-lamp examination. Thus clear corneae can no longer be regarded as a hallmark of mucopolysaccharidosis II.

Hooded rats react to intracutaneous injections of complete Freund's adjuvant with an adjuvant disease, which is externally perceivable by massive polyarthritis, by skin and ear nodules, and by other known symptoms. In contrast to Wistar rats, however, they show no tendency to develop clinically or histologically provable eye manifestations such as an uveitis anterior (adjuvant uveitis). This fact has been connected with the genetic differences between the two strains of rats.

The author found abnormalities of ocular movement including abnormal blink rate and blink reflex to glabellar tap in 34 of 44 medication-free schizophrenic patients. It is not clear whether these signs represent part of the pathology of schizophrenia or result from withdrawal of neuroleptic treatment.

A patient with hydrocephalus due to aqueductal occlusion is described in whom the Sylvian aqueduct syndrome appeared during a sudden increase in intracranial pressure. The ocular signs resolved completely when the hydrocephalus was relieved. Marked dilatation of the posterior part of the third ventricle and of the rostral aqueduct with axial displacement of these structures was demonstrated radiologically. It is suggested that the ocular signs in this case were the result of periaqueductal dysfunction due to assimilation and dilatation of the aqueduct, with secondary tentorial block. This abnormality may be the cause of the similar abnormalities commonly found in noncommunicating hydrocephalus in both infants and adults.

Eighteen cases of Sweet's syndrome are described. This is the largest series so far reported and the first from the tropics. The essential features are the characteristic morphology, the histological appearances, the dramatic response to corticosteroids and the absence of scarring. Attention is drawn to the frequent involvement of the eyes and joints during the course of the illness. Fever and neutrophilia were found less frequently than a raised ESR or a preceding upper respiratory tract or skin infection. The term Sweet's syndrome is preferred to acute febrile neutrophilic dermatosis. The frequency of occurrence of different clinical manifestations is discussed.

A total of 45 patients with craniopharyngioma are reviewed, with particular reference to the presenting clinical features and neuro-ophthalmological findings. Of these 50 per cent had been referred in the first instance to an eye clinic. More than half the children presented with the clinical picture of raised intracranial pressure, although one-third of these had optic atrophy rather than papilloedema. One-third of the children had a history of concomitant strabismus, and only one patient had a paretic squint. The majority of the adults presented with visual failure and optic atrophy. Bitemporal hemianopia was fairly frequently found (27 per cent of our patients at the time of diagnosis) but was asymmetrical and unpredictable in its evolution. Homonymous hemianopia was relatively common, a presenting feature in II per cent of patients in this study. Full fields were found initially in nine patients (20 per cent), a high incidence compared with pituitary adenomas or suprasellar meningiomas. We consider pleomorphism, that is a distinct change from one type of field defect to another with progress of the disease, to be a characteristic feature of the tumour (as indeed are fluctuations in the clinical state and visual acuity); it was detected in 22 per cent of our patients. Endocrine disorders were common, especially in adults, and mental deterioration was a frequent presenting feature in patients over 30 years of age. Straight x rays of the skull are practically diagnostic of craniopharyngioma in children, but in adults a normal x ray does not exclude the diagnosis.

Six juvenile and adult patients with progressive neurological diseases and beta-galactosidase deficiency were reported. Any diseases known to date were denied. These cases together with ten case reports in the literature were reviewed and were classified into three groups from clinical and biochemical points. Group 1 patients were characterized by progressive ataxia and myoclonus with gargoyl changes and macular cherry-red spots. In this syndrome beta-galactosidase activity seems to be secondarily affected by other biochemical defects. A group 2 patient showed similar neurological manifestations without gargoyle changes or macular cherry-red spots. Patients with these clinical features not associated with beta-galactosidase deficiency have also been described in the literature. Group 3 patients had progressive pyramidal and extrapyramidal disease without gargoyl changes or macular cherry-red spots. These cases may represent juvenile and adult type GM1-gangliosidosis. Accumulation of GM1 has not yet been demonstrated.

Summary The eye manifestations of Graves' disease are usually mild and self-limiting. Occasionally they follow a progressive course leading to visual loss and total blindness. The ocular manifestations bear no relationship to the metabolic state and may appear before, during or after onset of thyrotoxicosis. Characteristically they become evident at about the time of onset of hypermetabolism. Various factors responsible for the failure of visual acuity are discussed with case illustrations.

A 43-year-old black woman showed ophthalmoscopic evidence of retinal arteriolitis two weeks after being treated for uniocular panuveitis. Angiographic examination suggested that these deposits were not intraluminal or endothelial atherosclerotic emboli or plaques, but were deposits in the outer walls of retinal arterioles. Sequential ophthalmoscopic and angiographic examinations at one-month intervals for 12 months showed no progression or change in location of these deposits. Results of clinical and laboratory investigations suggested the diagnosis of syphilis. We believe it is rare for syphilitic infection to be implicated in the diagnosis of isolated retinal arteriolitis without periphlebitis.

Despite advances in the control of the disease in animals, brucellosis is a zoonosis with important economic impact in the United States. It is estimated that cases of brucellosis in humans are underdiagnosed and underreported and that the disease continues to be a major human health hazard. Nine active cases of brucellosis in humans were documented in Houston, Texas between June 1981 and June 1982. These cases, plus an additional case of chronic brucellosis, served as the impetus for this review of the protean manifestations of the disease in humans.