¿Qué escala debemos elegir para evaluar actividad y gravedad en la orbitopatía de Graves? / Which scale should we choose to assess activity and severity in Graves orbitopathy?

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The effect of orbital decompression surgery on refraction and intraocular pressure in patients with thyroid orbitopathy.

PURPOSE: To investigate the effect of orbital decompression surgery in thyroid orbitopathy (TO) on both refractive status and intraocular pressure (IOP). PATIENTS AND METHODS: A prospective, multicentre, consecutive audit of patients undergoing thyroid decompression surgery. Indications for surgery included cosmetically unacceptable proptosis or corneal exposure. Exclusion criteria included the following: previous orbital surgery, glaucoma, corneal disease, steroid use in the preceding 12 months, or an acute optic neuropathy. Automated refraction, keratometry, pachymetry, Hertel exophthalmometry, and IOP were recorded at 1 month pre- and 3 months postoperatively. IOP using the Tono-Pen (mean of three readings) was measured in the primary, upgaze, and downgaze positions. RESULTS: Data were collected from 52 orbits of 33 patients (East Grinstead, New York, and Adelaide). There was no significant difference between pre- and postoperative data for sphere, cylinder, or central corneal thickness (CCT). The mean spherical equivalent was -0.43 ± 1.49 D pre-operatively and -0.28 ± 1.52 D postoperatively. The steepest meridian of corneal curvature was 93.1 degrees pre- and 94.2 degrees postoperatively, with no significant difference. Mean IOP significantly decreased when measuring by Goldmann applanation tonometry (GAT) (2.28 mm Hg, (*) P=0.001) and Tono-Pen (3.06 mm Hg, (*) P=<0.0001). IOP measured in upgaze was significantly greater than that in the primary position. Regression analysis between change in IOP and either Hertel exophthalmometry or the number of orbital walls decompressed was non-significant ((*)Student's t-test). CONCLUSION: Patients with TO undergoing orbital decompression had, on average, with-the-rule astigmatism not affected by orbital decompression surgery. IOP was significantly reduced by decompression surgery although no relationship between IOP and the degree of decompression was observed.

Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review.

BACKGROUND/AIMS: Adult xanthogranulomatous disease involving the ocular tissues is rare and poorly understood. Adult onset xanthogranuloma (AOX), adult onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD) are the four syndromes within this disorder, which is diagnosed by characteristic histopathology. Experience with eight cases prompted a multi-institutional effort to study the histopathology, immunohistochemistry, clinical findings, and systemic associations in this disorder. METHODS: 22 cases, including histopathological slides, were compiled. Published reports were identified by an English language Medline search (1966-2005) and review of reference citations. Each case in this series and the literature was classified as one of four syndromes and then analysed for age onset, sex, skin xanthoma, orbital location, immune dysfunction, internal organ and bone lesions, treatment, and outcome. The histopathology in each of these cases was reviewed by two pathologists. Immunhistochemical stains (CD3, CD4, CD8, L26) were performed in 14 cases where unstained slides were available. RESULTS: 137 cases were compiled. There was no sex or age difference between syndromes. AOX, AAPOX, NBX affect the anterior orbit, ECD tends to be diffuse and intraconal. Skin lesions are found in all the syndromes. Immune dysfunction was noted in all cases of AAPOX and NBX; 11% of NBX and all ECD patients had internal organ disease. Treatment included surgery, corticosteroids, other chemotherapeutic agents, radiotherapy, and combinations of these. No AOX or AAPOX deaths occurred; 66% of ECD patients died. All 22 cases had xanthoma cells; most had Touton giant cells. Lymphocytes were present in all cases and occurred as aggregates (mostly in AAPOX) or diffuse populations mixed with fibroblasts (mostly in ECD). Immunohistochemistry revealed the majority of these to be CD8+. Necrosis was most marked in NBX. CONCLUSION: Adult xanthogranuloma of the orbit is rare, making prospective evaluation or meta-analysis impossible. The best treatment is unknown but seems to be with multiagent chemotherapy guided by histopathological, immunohistochemical, and systemic findings.

Disability in schizophrenia: clinical correlates and prediction over 1-year follow-up.

Schizophrenia may cause disability leading to restrictions on many domains of daily life such as hygiene, self-management, vocational and leisure activities, and family and social relationships. The aim of this study was to assess the level of disability with the Brief Disability Questionnaire (BDQ), developed by the World Health Organization, and to identify the clinical correlates and predictors of disability during a 1-year follow-up period in 382 patients with schizophrenia. All patients were assessed at the beginning of the study, and 168 (44%) of them were re-evaluated after 1 year. Total disability scores of the patients with schizophrenia were significantly decreased at follow-up. Female patients seemed to be more disabled than males. Disability showed a positive correlation with the total, positive symptoms and negative symptoms scores on the Brief Psychiatric Rating Scale, as well as scores on the UKU Side Effects Rating Scale. Patients with the disorganized subtype of schizophrenia and residual symptoms were more disabled than patients with other subtype diagnoses. Negative symptoms and duration of untreated psychosis were significant predictors of disability after 1 year. Early-onset schizophrenia had a twofold increased risk for developing disability. Disability in schizophrenia is a clinical phenomenon closely linked to negative symptoms and poor outcome.

Obsessive-compulsive symptoms in clozapine-treated schizophrenic patients.

The aim of the present study was to assess the occurrence of obsessive-compulsive symptoms (OCS) in schizophrenic patients treated with clozapine, and to examine the relationship between OCS and other clinical variables. The results support earlier findings which suggest that clozapine produces or unmasks OCS. In addition, the severity of OCS was not related to other dimensions of psychopathology, severity of illness, clinical improvement or dose and duration of clozapine treatment.

[Creutzfeldt-Jakob disease: a case that initiated with psychiatric symptoms]. / Creutzfeldt-Jakob Hastaligi: Psikiyatrik Belirtilerle Baslayan Bir Olgu.

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive prion disease that causes deficits involving movement, cognition, and mental status. The clinical heterogeneity of the disease can make diagnosis difficult. Thorough neurologic, cognitive, and psychiatric examinations are necessary for observing its clinical features. In this case report we describe a 62-year-old male patient who was initially followed with a diagnosis of depression and later was diagnosed with CJD. The patient had a one-year history of anhedonia, loss of interest, social withdrawal, anxiety and decrease in speech and was given paroxetin 20 mg/day with a diagnosis of depression. During follow up, neurological symptoms including ataxia and rigidity became evident and dementia and akinetic mutism developed in a rapidly progressive course. Although electroencephalography (EEG) and magnetic resonance imaging (MRI) revealed nonspecific findings initially, typical findings for CJD were seen during the follow up. The positive 14-3-3 protein in CSF supported the diagnosis. The aim of this report is to emphasize the fact that CJD may present with different psychiatric symptoms and can be initially misdiagnosed. CJD should be considered in the differential diagnosis of patients who have focal neurological signs in addition to psychiatric symptoms. Repeated neurological examinations, EEG and cranial MRI may help in the diagnosis of these patients.

Venous thromboembolism and escitalopram.

Selective serotonin reuptake inhibitors (SSRIs) are currently used as a first-line treatment for depression, as they have a favorable side-effect profile. Escitalopram, a new SSRI, is also well-tolerated and serious side effects are rarely associated with its use. There have been several reports that SSRIs might increase bleeding tendency in some patients by affecting platelet function. However, to our knowledge, there have been no reports about their relation to thrombosis. In this brief report, we present a case of venous thromboembolism associated with escitalopram in a patient with psychotic depression without any major risk factors for thrombosis. SSRIs might have a dual effect on platelet function. The immediate and early effect of SSRI use on platelets might be an increase be an in tendency for thrombosis, whereas the late effect after repeated dosing might be an increase in tendency to bleed, as suggested by previous literature.

Abnormal somatosensory evoked potentials in two patients with conversion disorder.

On clinical grounds, somatosensory evoked potentials (SEP) and motor evoked potentials (MEP) are currently used to discriminate between hysterical and neurological conditions. The present paper reports on two patients with severe gait disturbance who had the near-total absence of SEP responses on the scalp during the symptomatic period, which normalized after recovery. These findings, along with others, may shed light on the brain correlates of conversion phenomena.

Bone mineral density in premenopausal women with major depressive disorder.

This cross-sectional study investigated whether a group of unmedicated patients with major depressive disorder, single episode, had decreased bone mineral density (BMD). The BMD at the lumbar spine and proximal femur in 25 premenopausal women with major depressive disorder and 15 normal women was measured by dual-energy X-ray absorptiometry. Bone turnover markers and serum cortisol levels were also evaluated for each subject. As compared with values in the normal women, the mean BMD in the depressed women was significantly lower at the lumbar spine and at all sites of the proximal femur. There was no statistically significant difference between serum cortisol levels and bone turnover markers except for significantly higher urinary excretion of deoxypyridinoline cross-links in the patients compared with the controls. In conclusion, depressed women may have decreased BMD even at the very early stages of the illness, and this possibility should be taken into consideration in treatment.

Neurological soft signs in schizophrenic patients and their nonpsychotic siblings.

OBJECTIVE: (a) To investigate the prevalence of neurological soft signs (NSS) in schizophrenic patients and their nonpsychotic siblings and (b) to examine the clinical correlates of NSS in the schizophrenic group. METHODS: Ninety-nine schizophrenic patients, 80 of their nonpsychotic siblings and 59 healthy controls were included in the study. NSS were assessed with the Neurological Evaluation Scale (NES). Psychiatric assessment of the patients was conducted with the Positive and Negative Syndrome Scale (PANSS). Siblings and the control group were evaluated with Schedules for Clinical Assessment in Neuropsychiatry (SCAN) to determine the presence of any past or current psychotic disorder. RESULTS: Schizophrenic patients had significantly higher scores overall and on each subscale of NES than the sibling and control groups. The sibling group's scores were intermediate between those of the schizophrenic patients and those of the healthy controls. All subscale scores and the total NES scores correlated positively with the negative symptoms subscale scores of PANSS. The general psychopathology subscale scores of PANSS also showed a positive correlation with all subscale scores of NES, except the 'sequencing of complex motor acts' subscale. The total NES scores of the patients as well as their scores for the 'sequencing of complex motor acts' and 'others' subscales were significantly correlated with the respective scores of their own siblings. CONCLUSIONS: These results support the findings of previous studies suggesting that there might be common genetic and/or environmental factors in the pathogenesis of neurological impairment in schizophrenic patients and their siblings. They also indicate that neurological soft signs in schizophrenic patients are associated with prominent negative symptoms.

Reversal of dysthyroid optic neuropathy following orbital fat decompression.

AIMS: To document the successful treatment of five patients with dysthyroid optic neuropathy by orbital fat decompression instead of orbital bone decompression after failed medical therapy. METHODS: Eight orbits of five patients with dysthyroid optic neuropathy were selected for orbital fat decompression as an alternative to bone removal decompression. Treatment with systemic corticosteroids and/or orbital radiotherapy was either unsuccessful or contraindicated in each case. All patients satisfied clinical indications for orbital bone decompression to reverse the optic neuropathy. High resolution computerised tomographic (CT) scans were performed in all cases and in each case showed signs of enlargement of the orbital fat compartment. As an alternative to bone decompression, orbital fat decompression was performed on all eight orbits. RESULTS: Orbital fat decompression was performed on five patients (eight orbits) with optic neuropathy. Optic neuropathy was reversed in all cases. There were no cases of postoperative diplopia, enophthalmos, globe ptosis, or anaesthesia. All patients were followed for a minimum of 1 year. CONCLUSIONS: In a subset of patients with an enlarged orbital fat compartment and in whom extraocular muscle enlargement is not the solitary cause of optic neuropathy, fat decompression is a surgical alternative to bony decompression.

Absence of seasonal variation in Graves disease.

PURPOSE: To determine whether there is an identifiable pattern of seasonal variation in the onset of symptoms of orbitopathy and thyrotoxicosis in patients with Graves disease. METHODS: This retrospective, noncomparative case series reviewed 305 randomly selected office records of patients referred to one author (M.K.) for evaluation of Graves orbitopathy between July 1990 and June 1998. All patients met inclusion criteria for the diagnosis of Graves orbitopathy. During initial evaluation for orbitopathy, patients identified the date of onset of orbital symptoms as well as the earliest date of either onset of thyroid symptoms or documented thyroid abnormality. Patients were excluded from analysis of seasonal variation if they could not recall the month of symptom onset or were euthyroid. The onset of orbital symptoms and identification of dysthyroid state were analyzed by calendar month and season. The chronological relationship of the development of orbital and thyroid symptoms was evaluated. RESULTS: No significant seasonal variation appeared in the onset of orbital symptoms or identification of dysthyroidism. Out of 148 patients, 115 (78%) developed symptoms of orbital disease within 18 months of the identification of dysthyroidism. The most common presenting orbital symptoms were swelling of the lid or prominence of the globe. CONCLUSIONS: This study fails to provide evidence for a seasonal influence on the incidence of Graves disease and the associated orbitopathy.

Management of conjunctival cicatrizing diseases and severe ocular surface dysfunction.

Physical or chemical injuries, infections, immunologic oculocutaneous disorders, drugs, and various systemic disorders may cause scarring of the conjunctiva and disturbances of the ocular surface. Trichiasis, lid margin malposition, and dry eye may result in persistent ocular irritation. The cornea may be primarily or secondarily involved. If severe, disturbances of the ocular surface may lead to significant visual impairment. Thorough evaluation of patients and of the underlying disease process is required for optimal management. Treatment may be challenging and should be comprehensive, combining medical measures and surgical correction of structural changes. Suppression of exogenous irritants, treatment of dry eye, antiinflammatory therapy, and immunosuppressants are paramount to control the underlying disease and allow optimal surgical results. Surgical correction of trichiasis and lid margin malposition, conjunctival grafting, mucous membrane transplantation, limbal stem cell transplantation, amniotic membrane transplantation, and penetrating keratoplasty help reestablish a physiologic ocular surface. Severe cases may require keratoprosthetics for visual rehabilitation. Corneal ulceration or perforation requires prompt attention to maintain ocular integrity. Special measures should be considered for patients who require cataract or glaucoma surgery.

Magnetic resonance imaging of optic perineuritis.

Optic perineuritis, an uncommon variant of orbital pseudotumor, may be clinically indistinguishable from retrobulbar optic neuritis. Because treatment and prognosis for these two entities are different, early diagnosis is important. We report a case of a 47 year-old woman with clinical findings suggestive of retrobulbar optic neuritis, but whose magnetic resonance images suggested optic perineuritis. A dramatic clinical response to oral corticosteroids was observed. Optic perineuritis should be considered in cases of presumed retrobulbar optic neuritis. MRI may differentiate these two entities in the acute stage, and should be considered before treatment is decided.

The role of postoperative irradiation in the management of sphenoid wing meningiomas. A preliminary report.

PURPOSE: To determine whether postoperative radiation therapy decreases recurrence rates in subtotally excised and recurrent sphenoid wing meningiomas. METHODS: Patients with primary subtotally excised and recurrent sphenoid wing meningiomas who underwent surgery between 1981 and 1994 (n = 105) were prospectively followed for recurrence. Postoperative radiation was not recommended in patients who had complete excision; therefore, their recurrence rates were not evaluated in this study. Patients with malignant meningiomas also were excluded from analysis. Recurrence was defined as evidence of tumor growth on neuroimaging with or without clinical symptoms. RESULTS: Follow-up information was available for 86 patients; 69 had primary subtotally excised tumors and 17 had recurrent tumors. Follow-up information was unavailable in the remaining 19 patients. Tumor location and histopathology, type of surgery performed, and patient sex and age were similar in the irradiated and nonirradiated subgroups. Postoperative irradiation was delivered to 31 patients with primary tumors and 11 with recurrent tumors; none of these 42 patients had recurrence during a mean observation period of 4.2 and 3.5 years, respectively. The nonirradiated group consisted of 38 patients with primary tumors and 6 with recurrent tumors; 16 of 18 patients who had primary meningiomas had a recurrence and 5 of 6 who had recurrent tumors had another relapse (mean interval between resection and recurrence, 4.4 years and 14 months, respectively). CONCLUSIONS: Postoperative radiation appeared to delay recurrence in subtotally excised and recurrent sphenoid wing meningiomas during the time frame of this study.

Varicella-zoster virus retrobulbar optic neuritis in a patient with human immunodeficiency virus.

PURPOSE: To determine the cause of bilateral retrobulbar optic neuritis followed by progressive outer retinal necrosis in a patient with human immunodeficiency virus (HIV). METHODS: Extensive ophthalmologic, neurologic, infectious disease, rheumatologic, and radiologic examinations were performed. RESULTS: Cerebrospinal fluid samples taken after the onset of bilateral retrobulbar optic neuritis and before the development of clinical progressive outer retinal necrosis disclosed varicella-zoster virus from polymerase chain reaction and viral culture. CONCLUSION: Ophthalmologists and neurologists should consider varicella-zoster virus optic neuritis as a potential precursor of progressive outer retinal necrosis and as a cause of retrobulbar optic neuritis in patients infected with HIV.

Prominent proptosis in childhood thyroid eye disease.

BACKGROUND: Orbital signs and symptoms occur in approximately one half of children with Graves disease, but the symptoms are usually minor and limited to the eyelids. Prominent proptosis is uncommon in children with this disorder. METHODS: Review of eight children with prominent proptosis associated with thyroid eye disease. Four patients were treated at the Children's Hospital of Philadelphia, the other four at the Columbia Presbyterian Medical Center. RESULTS: At initial presentation, children ranged in age from 3 to 16 years. There were five girls and three boys. Seven of eight children had hyperthyroidism at ophthalmic presentation. Four patients had restrictive myopathy, and all of the seven patients who underwent neuroimaging had extraocular muscle enlargement. Five patients were treated with lubrication. Two underwent orbital fat decompression. One patient had thyroid eye disease and myasthenia gravis. CONCLUSIONS: Proptosis in childhood thyroid eye disease usually is associated with a hyperthyroid state. The proptosis may be dramatic, but corneal exposure and restrictive myopathy are seen in only some of the patients. Neuroimaging shows enlarged extraocular muscles. Most children with this complication can be treated conservatively with topical lubrication, but orbital fat decompression may be considered in patients with more advanced conditions.

Endoscopy and biopsy of the orbit.

Flexible endoscopes produce high-quality images, are small in size, and can deliver microsurgical instruments or laser probes. Early attempts at orbital endoscopy were limited by the relatively large size and poor visualization of rigid endoscopes. We performed endoscopic orbital exploration using the Olympus HYF flexible endoscope in four live dog orbits. We achieved excellent visualization of orbital structures including the globe, blood vessels, extraocular muscles, intermuscular septa, optic nerve, and fat. Visualization, hemostasis, and dissection were aided by the use of hyaluronic acid infused through the endoscope. We biopsied fat and extraocular muscle without complication using Olympus endoscopic cup biopsy forceps (confirmed by histopathologic examination). Our experience indicates that orbital endoscopy aided by viscoelastic hydrodissection may permit a less invasive approach to optic nerve sheath fenestration, tumor biopsy and treatment, and the removal of foreign bodies.