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Atypical Moyamoya disease associated with brain tumor.

A 4-year-old boy with right retinal hemorrhage, mental retardation, and multiple minor anomalies was referred to our hospital. Computed tomography scanning revealed a cystic brain tumor at the vermis. Angiography showed stenosis of both internal carotid arteries at the supraclinoid portion and the Moyamoya vessels. The right ophthalmic artery was dilated as wide as the internal carotid artery. Stenosis of the basilar artery was also observed. Collateral circulation via the posterior inferior cerebellar artery and Moyamoya vessels in the area of the posterior cerebral artery was observed.

Abnormalities, Multiple↗

Retinal complications during interferon therapy for chronic hepatitis C.

OBJECTIVES: Various side effects of interferon (IFN) therapy have been reported. In this study, we examined retinal change during IFN therapy. METHODS: We performed ophthalmological examinations before, during, and after therapy on 63 patients with chronic hepatitis C who were receiving either natural IFA-alpha or recombinant IFN-alpha 2a or 2b. RESULTS: No retinal lesion was detected before IFN therapy, but, during therapy, retinal abnormality or retinopathy developed in 36 (57.1%) of 63 patients, including retinal hemorrhage in 25 patients and cotton-wool spots in 28 patients. They were noted early in the course of IFN therapy, within the first 4 wk in 67% (24/36) and within 8 wk in 86% (31/36). The incidence was not influenced by the type of IFN but was higher among diabetic (11/12, 92%, p < 0.05) or hypertensive patients (4/5, 80%, not significant) than among patients without either diabetes or hypertension (24/49, 49%). There was no relation between the incidence of retinopathy and the level of ALT activity or white blood cell or platelet counts. However, retinopathy occurred in most patients receiving IFN therapy after white blood cell count and platelet count reached a nadir. The levels of LDL-cholesterol and the atherosclerotic index in patients with retinopathy were slightly higher than those in the patients without retinopathy. CONCLUSIONS: These results suggest that retinopathy often occurs in patients with chronic hepatitis C who are receiving IFN and that we should closely monitor patients for retinal complications during IFN therapy.

Adult↗

A population-based comparison of clinical and outcome characteristics of young children with serious inflicted and noninflicted traumatic brain injury.

OBJECTIVE: Diagnosing inflicted traumatic brain injury (TBI) in young children is difficult in practice. Comparisons of children with inflicted and noninflicted TBI may help to identify markers of inflicted TBI. The objective of this study was to compare inflicted and noninflicted TBI in terms of presenting complaints, clinical features, and hospital outcomes. METHODS: The presenting complaint, clinical finding, hospital course, and outcome of all children who were aged 2 years or younger in North Carolina and were admitted to a pediatric intensive care unit or died with a TBI in 2000 and 2001 were reviewed. Clinical presentation and injury types were compared between children with inflicted and noninflicted TBI. Risk ratios were used to compare clinical and outcome characteristics between the 2 groups. Among survivors, multivariate binomial regression was used to examine the adjusted risk of a poor outcome dependent on injury type. RESULTS: A total of 80 (52.6%) children had inflicted and 72 (47.3%) children had noninflicted TBI. Children with noninflicted TBI (not in a motor vehicle crash) were more likely to present to the emergency department asymptomatic (44.8% vs 8.3%) and to have a specific history of trauma than children with inflicted TBI. Retinal hemorrhage, metaphyseal fracture, rib fracture, and subdural hemorrhage were more commonly found in children with inflicted compared with noninflicted TBI. Skeletal survey and ophthalmologic examination combined would have missed 8 (10.0%) inflicted TBI cases. CONCLUSIONS: Manner of presentation and injury types are helpful in distinguishing inflicted TBI. Clinicians should not rule out inflicted TBI on the basis of skeletal survey and ophthalmoscopy alone but should proceed to computed tomography and/or magnetic resonance imaging.

Accidental Falls↗

Atypical anterior optic neuropathy caused by toxoplasmosis.

PURPOSE: To report atypical anterior optic neuropathy due to toxoplasmosis. METHODS: Interventional case report. A 33-year-old male presented with sudden painless loss of vision and floaters in the right eye. Examination demonstrated a best-corrected visual acuity of 20/200, optic nerve head edema, retinal hemorrhages, and vitreous opacities. RESULTS: Nine days later, a granuloma at the optic nerve head was apparent, and the patient was treated with pyrimethamine, sulfadiazine, folinic acid, and prednisone. Six weeks after initiating therapy, best-corrected visual acuity had improved to 20/25. CONCLUSION: Optic nerve involvement in toxoplasmosis is uncommon and, when it occurs, usually presents with a white inflammatory mass on the optic disk. The current case demonstrates the importance of including toxoplasmosis in the differential diagnosis of unilateral anterior optic neuropathy, even if a focal inflammatory mass is not apparent.

Adult↗

Subretinal membranes in proliferative vitreoretinopathy.

Subretinal membranes (SRMs) are an important but rarely identified component of proliferative vitreoretinopathy (PRV). In 153 consecutive cases that had vitreoretinal surgery for this condition and were followed for at least 6 months, SRMs were encountered in 72 eyes (47%). In 20 (28%) of the 72 eyes, the SRMs prevented complete retinal reattachment and needed to be removed or excised through one or multiple retinotomies. Intraoperative complications related to the SRMs or their removal included choroidal or retinal hemorrhage in three eyes (15%), subretinal air in three eyes (15%), and unplanned extension of the retinotomies in two eyes (10%). The 20 eyes requiring SRMs removal were followed for a median of 11 months. Retinas were reattached in 13 eyes (65%), although only 4 eyes (20%) had a visual acuity of 5/200 or better. Recognizing SRMs as a component of PVR is important in helping to maximize the anatomic success rate although the effects on visual function are not fully known.

Adult↗

Classification of retinal damage by a neural network based system.

The objective of this research is to provide an ophthalmologist with a helpful system, capable of classifying a degree of patients' retinal hemorrhage. The system is composed of four modules: (a) data acquisition module, (b) image Database module, (c) image processing module, (d) image classification module. The system was trained with a modular neural network on a set of 25 images, and tested on a set of 160 images. A training performance of greater than 95% was achieved. The classifying part of the system showed 79% recognition accuracy. Since the testing images were taken from independent sources, we assume that the system should also provide an accurate classification of other image types.

Algorithms↗

Current patterns of inflicted head injury in children.

The purpose of this study was to examine the current patterns of head trauma associated with child abuse. We reviewed the records of all patients admitted to our medical center between 1995 and 1997 with a primary diagnosis of head trauma, and analyzed the clinical presentation, mechanism of injury, socioeconomic status and outcome for these patients. Head trauma was deliberately inflicted in 38/405 children (9%). There were 25 boys and 13 girls, with a median age of 5.5 months. Two thirds of the families lived in the inner city. Of the 99 children under the age of 2 years admitted for head trauma, the injury was inflicted in 32 (32%). Acute subdural hematoma was present in 22/32 (69%) of children with inflicted trauma, but in only 5/68 (7%) with accidental trauma. Retinal hemorrhages were present in 17/32 (53%) abused children, but in no cases of accidental trauma (0/68). Deliberately inflicted injury is a frequent cause of serious head trauma in young children. Head injury is a major cause of morbidity and mortality in the abused child. Child abuse cases correlated strongly with low socioeconomic status. Nonaccidental trauma must be considered strongly in children under 2 years of age who present with acute subdural hematoma in the absence of a history of a motor vehicle accident.

Acute Disease↗

[Incidence, prevention and therapy of acute mountain sickness].

The symptoms and signs of acute mountain sickness are present in about half of the tourists trekking in Nepal to an altitude of 42000 m. The most common symptoms are headache and nausea. Pulmonary rales are found in more than 10% of trekkers, while high altitude pulmonary edema is rare. Retinal hemorrhages occur almost exclusively above 5000 m. A careful history and physical examination are generally sufficient for medical evaluation of fitness for high altitude. There are no specific tests to predict performance at altitude. The most effective prophylaxis of acute mountain sickness is "slow" ascent, which is arbitrarily defined as an increase in sleeping altitude of 300-400 m per 24 hours. Sufficient fluid intake is also very important. Prophylactic administration of acetazolamide reduces the incidence and severity of acute mountain sickness. Mild forms of acute mountain sickness are treated by a rest day, whereas patients with severe disease should descend as soon as possible.

Acetazolamide↗

Pituitary ablation for diabetic retinopathy.

Pituitary ablation was performed on 26 patients with advanced diabetic retinopathy by pituitary stalk section (nine patients) or transphenoidal hypophysectomy (17 patients). After a latent period varying from six to 12 months, retinal hemorrhages disappeared, vitreous hemorrhages stopped and new vessels regressed. Eight of 10 patients followed up for more than a year had a complete remission, although in one of two patients whose ablation was incomplete the retinopathy remained active. Preoperative visual acuity levels were preserved in 47 of 50 eyes of patients followed up for a mean period of 12 months. Three patients have died, two from causes related to their operation. Twenty of the survivors have continued in their usual occupation. Pituitary ablation is effective in preventing the progressive visual deterioration usually associated with advanced diabetic retinopathy.

Adolescent↗

Leukemic ophthalmopathy in children.

Abnormalities of the eye were detected in 52 of 657 children (9%) suffering from acute leukemia. The treatment for leukemia was single agent chemotherapy administered sequentially. "Prophylactic" treatment of the central nervous system was not administered. Major manifestations of leukemic ophthalmopathy comprised retinal hemorrhage (in 19) and infiltration of the optic nerve, retina, iris or orbit (29). The ophthalmopathy was treated by topical dexamethasone and radiation therapy. Concurrent bone marrow relapse occurred in most patients. Twenty-seven of 29 patients whose cerebrospinal fluid was examined before or at the time of the onset of leukemic ophthalmopathy demonstrated meningeal leukemia. In contrast, this complication has not been observed in recent patients who received "prophylactic" treatment for meningeal leukemia. Leukemic invasion of the eye should receive appropriate recognition; the posterior pole should be included in the treatment of the central nervous system as a pharmacologic sanctuary.

Child↗

Bilateral optic disk edema and blindness as initial presentation of acute lymphocytic leukemia.

PURPOSE: To report bilateral optic disk edema and blindness as the unusual initial presentation of acute lymphocytic leukemia (ALL) in an adult. METHODS: A 19-year-old man presented with a history of headaches, back pain, and 10 days of worsening vision that progressed to blindness. Ocular examination revealed light perception acuity in the right eye and no light perception in the left eye. Fundus examination revealed bilateral profound optic disk edema, tortuous vessels, and retinal hemorrhages. Acute lymphocytic leukemia was diagnosed with complete blood count and bone marrow biopsy. Head computed tomography and magnetic resonance imaging, were normal. Lumbar puncture revealed normal opening pressure. Ocular ultrasonography showed bilateral optic nerve enlargement. DESIGN: Interventional case report and literature review. RESULTS: The presumptive diagnosis of leukemic infiltration of the optic nerves was made, and urgent radiotherapy, intrathecal methotrexate, and intravenous daunorubicin were instituted. Visual acuity improved to hand motions in the right eye. CONCLUSIONS: Acute lymphocytic leukemia can rarely present in adults as visual changes due to leukemic optic nerve infiltration. Radiation treatment should be considered as an urgent treatment modality for this rare condition.

Adult↗

Inflicted versus accidental head injury in critically injured children.

OBJECTIVES: To assess the frequency of inflicted head injury in critically injured children; the severity of neurologic injury; the neurologic outcome; and the historical, socioeconomic, physical, and radiologic factors associated with inflicted head injury. DESIGN: Prospective clinical study. SETTING: Multidisciplinary pediatric intensive care unit (ICU). PATIENTS: Consecutive cases (n = 40) of severe head injury admitted to a pediatric ICU. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Fourteen (35%) of 40 cases of head injury were due to inflicted head injury. Eleven (79%) of 14 inflicted head injury cases were due to child abuse and three (21%) were due to neglect. The severity of neurologic injury, as measured by the admission Glasgow Coma Scale, was worse in cases of inflicted head injury (7.1 +/- 0.7 [SE] [inflicted] vs. 9.9 +/- 0.8 [accidental]; p = .04). Glasgow Outcome Scores were worse after inflicted head injury (2 +/- 1 inflicted] vs. 4 +/- 1 [accidental]; p = .004). In victims of child abuse, we found the combination of any two of the following three factors was associated with inflicted head injury: an inconsistent history/physical examination; retinal hemorrhages; or parental risk factors (alcohol or drug abuse, previous social service intervention within the family, or a past history of child abuse or neglect). CONCLUSIONS: This study confirms that severity of neurologic injury and neurologic outcome in cases of inflicted head injury are worse than in any other type of childhood head injury. We believe that a combination of any two of the above three risk factors may prove to be a reliable marker of inflicted head injury in children admitted to a pediatric ICU and will lead to an early and definitive diagnosis.

Accidents↗

An international classification and grading system for age-related maculopathy and age-related macular degeneration. The International ARM Epidemiological Study Group.

A common detection and classification system is needed for epidemiologic studies of age-related maculopathy (ARM). Such a grading scheme for ARM is described in this paper. ARM is defined as a degenerative disorder in persons > or = 50 years of age characterized on grading of color fundus transparencies by the presence of the following abnormalities in the macular area: soft drusen > or = 63 microns, hyperpigmentation and/or hypopigmentation of the retinal pigment epithelium (RPE), RPE and associated neurosensory detachment, (peri)retinal hemorrhages, geographic atrophy of the RPE, or (peri)retinal fibrous scarring in the absence of other retinal (vascular) disorders. Visual acuity is not used to define the presence of ARM. Early ARM is defined as the presence of drusen and RPE pigmentary abnormalities described above; late ARM is similar to age-related macular degeneration (AMD) and includes dry AMD (geographic atrophy of the RPE in the absence of neovascular AMD) or neovascular AMD (RPE detachment, hemorrhages, and/or scars as described above). Methods to take and grade fundus transparencies are described.

Aged↗

An experimental approach to the study of intraocular Toxocara canis.

An experimental study of nematode endophthalmitis due to T canis and review of the literature has been presented. Six owl monkeys were infected either by nasogastric tube using embryonated T canis eggs or by carotid or intravitreal injection of second stage larvae. The clinical manifestations, especially ocular, were observed and various diagnostic tests performed. Only minimal or no intraocular changes were seen after systemic infection but significant abnormalities such as retinal hemorrhages and venous dilation were noted after intravitreal infection. Motile larvae were observed in the lenses of three eyes and in the vitreous of five eyes and, probably a sixth, after intravitreal injection. The intensity and timing of the intraocular reaction seemed to correlate with the infecting dose and apparent disappearance of larvae from the eye. Pathologic confirmation of larvae in the lens was obtained in one eye. A marked inflammatory reaction occurred in eyes receiving intraocular infection but none was seen in eyes with only systemic infectin. Various laboratory and serologic studies were performed, including the ELISA test, which were used to evaluate systemic as well as intraocular responses to infection with T canis. The two monkeys infected by nasogastric tube gave a positive ELISA response in the serum but intraocular fluids gave a negative response in all monkeys including those infected syst:mically and/or intraocularly. Problems in the understanding of clinical aspects of the disease, laboratory diagnosis and treatment are discussed. The need for future experimental studies is emphasized.

Adult↗

The shaken baby syndrome. A clinical, pathological, and biomechanical study.

Because a history of shaking is often lacking in the so-called "shaken baby syndrome," diagnosis is usually based on a constellation of clinical and radiographic findings. Forty-eight cases of infants and young children with this diagnosis seen between 1978 and 1985 at the Children's Hospital of Philadelphia were reviewed. All patients had a presenting history thought to be suspicious for child abuse, and either retinal hemorrhages with subdural or subarachnoid hemorrhages or a computerized tomography scan showing subdural or subarachnoid hemorrhages with interhemispheric blood. The physical examination and presence of associated trauma were analyzed; autopsy findings for the 13 fatalities were reviewed. All fatal cases had signs of blunt impact to the head, although in more than half of them these findings were noted only at autopsy. All deaths were associated with uncontrollably increased intracranial pressure. Models of 1-month-old infants with various neck and skull parameters were instrumented with accelerometers and shaken and impacted against padded or unpadded surfaces. Angular accelerations for shakes were smaller than those for impacts by a factor of 50. All shakes fell below injury thresholds established for subhuman primates scaled for the same brain mass, while impacts spanned concussion, subdural hematoma, and diffuse axonal injury ranges. It was concluded that severe head injuries commonly diagnosed as shaking injuries require impact to occur and that shaking alone in an otherwise normal baby is unlikely to cause the shaken baby syndrome.

Battered Child Syndrome↗

Occult intracranial injury in infants.

STUDY OBJECTIVES: The objectives of this study were as follows: (1) to determine whether clinical symptoms and signs of brain injury are sensitive indicators of intracranial injury (ICI) in infants admitted with head trauma, (2) to describe the clinical characteristics of infants who have ICI in the absence of symptoms and signs of brain injury, and (3) to determine the clinical significance of those ICIs diagnosed in asymptomatic infants. METHODS: We conducted a retrospective analysis of all infants younger than 2 years of age admitted to a tertiary care pediatric hospital with acute ICI during a 6(1/2)-year period. Infants were considered symptomatic if they had loss of consciousness, history of behavior change, seizures, vomiting, bulging fontanel, retinal hemorrhages, abnormal neurologic examination, depressed mental status, or irritability. All others were considered to have occult ICI. RESULTS: Of 101 infants studied, 19 (19%; 95% confidence interval [CI] 12%, 28%) had occult ICI. Fourteen of 52 (27%) infants younger than 6 months of age had occult ICI, compared with 5 of 34 (15%) infants 6 months to 1 year, and none of 15 (0%) infants older than 1 year. Eighteen (95%) infants with occult ICI had scalp contusion or hematoma, and 18 (95%) had skull fracture. Nine (47%) infants with occult ICI received therapy for the ICI. No infants with occult ICI (0%) (95% CI 0, 14%) required surgery or medical management for increased intracranial pressure. Only 1 subject (5%) with occult ICI had any late symptoms or complications: a brief, self-limited convulsion. CONCLUSION: We found that 19 of 101 ICIs in infants admitted with head trauma were clinically occult. All 19 occult ICIs occurred in infants younger than 12 months of age, and 18 of 19 had skull fractures. None experienced serious neurologic deterioration or required surgical intervention. Physicians cannot depend on the absence of clinical signs of brain injury to exclude ICI in infants younger than 1 year of age.

Acute Disease↗

Essential hypertension in a dog.

Severe hypertension was diagnosed in a dog that initially was referred for evaluation of visual deficits and retinal hemorrhage and eventually was donated for medical treatment of hypertension. Initial blood pressure measured by direct methods was markedly high (systolic, 275 mm of Hg; diastolic, 170 mm of Hg). Measures of renal function were within normal limits, with the exception of hypotonic urine. A test protocol was designed to exclude possible secondary causes of hypertension; negative results of such tests allowed the diagnosis of essential hypertension. The consistency of the hypertension and its response to medical control were studied for 5 years. Blood pressure while the dog was untreated during those years was 240 +/- 24 mm of Hg (systolic) and 146 +/- 14 mm of Hg (diastolic). Plasma renin activity was within normal limits, and the response of the renin-angiotensin system to varied salt intake was normal. The most effective medications used to lower blood pressure were propranolol and captopril, both of which were more effective than salt restriction alone. Five years after the diagnosis of hypertension, the dog was euthanatized because of chronic renal failure secondary to pyelonephritis. Hypertension was less severe as the condition progressed into chronic renal failure. Complete necropsy did not reveal an obvious cause of the hypertension, and histopathologic changes were limited to the cardiovascular system, eyes, and kidneys.

Animals↗

Elevated lactate as an early marker of brain injury in inflicted traumatic brain injury.

BACKGROUND: Traumatic brain injury is a major cause of disability and death in the pediatric population. The metabolic and neurochemical abnormalities that underlie traumatic brain injury remain poorly understood, but hypoxia-ischemic injury might play an important role. OBJECTIVE: This study evaluated children with inflicted traumatic brain injury using magnetic resonance spectroscopy (MRS). We postulated that children with hypoxic-ischemic injury indicated by elevated lactate in the acute phase of injury will have worse early neurological status and short-term clinical outcomes than those without lactate upon MRS. MATERIALS AND METHODS: This prospective study employed proton MRS to sample bilaterally the frontal lobes and the parasagittal cortex within the parietal and occipital lobes of 11 patients with inflicted traumatic brain injury who were undergoing a clinical MRI examination. Patients' measured clinical course while hospitalized included initial neurological evaluation, presence of seizure activity, need for admission to the pediatric intensive care unit (PICU), number of days hospitalized, presence of retinal hemorrhages and presence of bone fractures. Measurement of outcome was determined using the Pediatric Overall Performance Category Scale (POPCS; 1=good performance; 6=death). RESULTS: Four children demonstrated elevated lactate and diminished N-acetyl aspartate (a neuronal marker) within several regions, indicating global ischemic injury (lactate-positive global group). These four children all had seizure activity and abnormal initial neurological examinations and required admission to the PICU. The mean POPCS for this group was 3.25. In four other children, lactate was detected within at least one region, indicating a focal ischemic injury (lactate-positive focal group); two of these children had seizure activity, and two had an abnormal initial neurological examination. The mean POPCS score was 1.5 for this group. The remaining three children had no evidence of lactate upon MRS (lactate-negative group). These children did not have seizure activity, did not require admission to the PICU, nor did they have initial abnormal neurological examinations. The mean POPCS score was 1.3 for this group. SUMMARY: Patients with inflicted traumatic brain injury and evidence of hypoxic-ischemic injury as indicated by elevated lactate on MRS tend to have worse early neurological status and early outcome scores. Lactate levels as sampled by MRS might predict early clinical outcome in inflicted traumatic brain injury.

Aspartic Acid↗