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Idiopathic intracranial hypertension.

Idiopathic intracranial hypertension results from cerebral oedema. The symptoms and signs of the condition are reviewed, especially the risks of visual failure if the condition is prolonged without adequate treatment. The most significant symptom in childhood is headache, and the most important sign is papilloedema. The difficulties of diagnosis are discussed, especially the cerebral oedema that can occur on the withdrawal of steroid therapy, and the increased intracranial pressure that may develop with minimal forms of craniostenosis. The causes of intracranial hypertension are many, and include ear infections, venous sinus thrombosis, the use of certain drugs, and as a complication of acute disseminated encephalomyelitis and Cushing's disease, and impaired renal function. The condition may remit spontaneously, but even so a careful watch must be kept on visual acuity. If treatment is needed various drugs can be tried, and if the response is unsatisfactory repeated lumbar punctures will be indicated. It is rarely necessary to consider surgical intervention such as cerebral decompression.

Child↗

Evaluation of neurologic complications by brain MRI in kidney and liver transplant recipients.

The aim of this study was to retrospectively analyze brain magnetic resonance imaging (MRI) findings in patients who developed neurologic complications after liver and kidney transplantation. The results in 216 organ transplant recipients, who had brain MRI were evaluated retrospectively. We performed 187 brain MRI on kidney recipients and 29 liver recipients. Neuroradiologic findings were classified in three groups: group 1 findings were related to transplantation; group 2 findings, to chronic parenchymal disease; and group 3 to neither transplantation nor chronic parenchymal disease. In group 1, six patients (20.6%) after liver and three (1.6%) after kidney transplantation had posterior reversible encephalopathy syndrome; two patients (1.1%) after renal and one (3.4%) after liver transplantation had tuberculosis granulomas; one patient (0.5%) after renal transplantation had osmotic demyelination syndrome; one patient (0.5%) had a Nocardia abcess and one (0.5%) focal cerebritis after renal transplantation. Among group 2, 38 patients (20.3%) had brain atrophy; 37 (20%), white matter changes; 3 (1.6%), sinus thrombosis; 8 (4.3%), lacunar infarct; 1 (0.5%), had renal osteodystrophy in the cranial bones; and 4 (2.2%), had intracranial hemorrhage secondary to end-stage renal disease. Brain atrophy in nine patients (31%), hyperintensity in the globus pallidus on T1-weighted MR images owing to manganese deposits in nine patients (31%), hyperintensity in basal ganglia on T2-weighted MR images owing to copper depositions in one patient (3.4%) were seen secondary to chronic liver disease. In group 3, three patients (1.6%) had intracranial lipomas; one (0.5%), mesial temporal sclerosis; and one (0.5%), an anterior cerebral artery aneurysm in renal transplant patients. Periventricular and subcortical white matter hyperintensities were observed on T2-weighted MR images in six liver transplant patients (20.7%). Neurologic complications after organ transplantation may be secondary to transplantation itself, to chronic parenchymal disease, or to neither transplantation nor chronic parenchymal disease.

Adult↗

Choroidal folds associated with increased intracranial pressure.

PURPOSE: To determine whether a relationship exists between increased intracranial pressure and the presence of idiopathic choroidal folds. METHODS: A prospective study in which 12 consecutive patients presenting with choroidal folds were evaluated by imaging studies (ultrasonography, magnetic resonance imaging, and/or computed tomography) to rule out known causes of choroidal folds, such as orbital disease, choroidal tumor, posterior scleritis, hypotony, and choroidal neovascular membrane. A lumbar puncture was performed on each of these patients, and measurement of opening pressure of cerebrospinal fluid was obtained. RESULTS: Twelve patients with choroidal folds included nine men and three women. Six patients (50%) presented with papilledema in the eye with choroidal folds. The other six patients (50%) presented with only choroidal folds. In this study, 10 (83%) of 12 patients had an opening pressure greater than 230 mm H(2)O. In patients presenting with only choroidal folds, five (83%) of six patients had an opening pressure greater than 230 mm H(2)O, with an average opening pressure of 290 mm H(2)O. CONCLUSION: Depending on the timing of the evaluation, papilledema may or may not be present, and only choroidal folds may be seen as a reflection of increased intracranial pressure. We believe that increased intracranial pressure from any source (that is, pseudotumor cerebri, sinus thrombosis, or intracranial mass) can present with only choroidal folds; therefore, these patients should have an appropriate work-up that should probably include a lumbar puncture before the title of "idiopathic" is given to their finding.

Adult↗

Occlusive vascular disease in asphyxiated newborn infants.

Six full-term severely asphyxiated newborn infants underwent evaluation with digital intravenous angiography. All infants were comatose and flaccid and had seizures, depressed brainstem function, and signs of intracranial hypertension. An initial brain CT scan revealed diffuse hypodensities and compressed ventricles. Follow-up noncontrast CT scans showed areas of increased attenuation. Digital intravenous angiography demonstrated venous sinus thrombosis in five infants, two of whom also had arterial thrombosis, and hypervascularity in the sixth infant. Our data document that occlusive vascular disease is a prominent feature of severe perinatal asphyxia.

Arterial Occlusive Diseases↗

Heparin and oral anticoagulants in the treatment of brain ischemia.

The use of heparin, heparin analogues, and oral anticoagulation in the treatment of brain ischemia remains controversial. We review the mechanism of action of the heparins and warfarin as well as the data regarding their efficacy and complications in patients with ischemic stroke and venous sinus thrombosis. None of these drugs have proven effective for all forms of cerebral ischemia. Further studies examining the efficacy of anticoagulants in different stroke subgroups will be necessary in order to clarify optimal clinical practice.

Administration, Oral↗

Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Acute Disease↗

Post-traumatic orbital cellulitis.

Orbital cellulitis is uncommon. It may arise as a sequel to eyelid infection, or from direct spread of infection from the paranasal sinuses; it may be of odontogenic origin and has been reported after meningitis and after nasoorbital fractures with pre-existing sinusitis. Clinically, orbital cellulitis is of great importance, as it is a severe disease with potentially disastrous consequences. It may lead to optic neuritis, optic atrophy, blindness, cavernous sinus thrombosis, superior orbital fissure syndrome, meningitis, subdural empyema, and even death. We report two cases of severe post-traumatic orbital cellulitis with subperiosteal abscesses. These were managed surgically and vision was preserved. We describe the anatomy, a classification of orbital infection, and the importance of multidisciplinary management of these cases.

Adult↗

Rhinocerebral mucormycosis: a case of a rare, but deadly disease.

Rhinocerebral mucormycosis is a rare fungal infection of the nasal cavity and sinuses that can spread to the orbits and cranium within days. Its presentation can be confused with those of sinusitis, viral infections, diabetic ketoacidosis, or carotid sinus thrombosis, and it is often missed at early presentation. Survival is directly linked to early detection and treatment. We present a case of rhinocerebral mucormycosis and discuss the literature on its early signs and symptoms, pathophysiology, and treatment options.

Adult↗

Thrombolytic therapy in neurointensive care.

Thrombolytic therapy has been studied in acute ischemic stroke, intracranial hemorrhage, intraventricular hemorrhage, subarachnoid hemorrhage, and sagittal sinus thrombosis. This form of therapy has an evolving role in contemporary neurologic practice, and increased investigational fervor will ensure more exacting therapeutic alternatives for stroke victims in the future.

Brain Diseases↗

Complications of otitis media requiring surgical intervention.

BACKGROUND: Although the incidence of complications of otitis media that require surgical interventions has decreased substantially over the past few years, it is a prevailing condition for which clinicians should remain vigilant. METHODS: We conducted a 3-year review [June 1998 to June 2001] in our hospital of surgical records of patients with complications of otitis media that were treated surgically. RESULTS: There were 16 patients with complications of otitis media, of which nine [56%] were intracranial; brain abscess and lateral sinus thrombosis were the most common intracranial complications. Extracranial complications were present in 15 [94%] of the patients; mastoid abscess [40%] was the most common extracranial complication. Seven [44%] patients had two or more concomitant complications. All patients with intracranial complications recovered well with no neurological deficits after aggressive antibiotic therapy and initial surgical treatment by neurosurgeons. Modified radical mastoidectomy was the most common surgical otological procedure that was performed in these cases. CONCLUSIONS: Aggressive antibiotic therapy and combined management of cases by otologists and neurosurgeons are the key to reducing the morbidity and mortality of the serious complications of otitis media.

Adolescent↗

Pediatric neurotology.

Pediatric neurotology is a subspecialty encompassing the medical and surgical treatment of conditions involving the middle ear, inner ear and lateral skull base. Multiple otologic and neurotologic diseases exist. In the pediatric patient the most common neurotologic conditions are vertigo, complications of otitis media, cholesteatoma, hearing loss and facial paralysis. Vertigo is differentiated into peripheral and central vestibular abnormalities. Peripheral vestibular causes include Meniere's disease, benign paroxysmal positional vertigo, vestibular neuronitis, otitis media and labyrinthitis. The predominant lesions in our field causing central vestibular disorders are brainstem and posterior fossa tumors. Mastoiditis, meningitis, petrositis, sigmoid sinus thrombosis, extradural abscess, brain abscess, otitic hydrocephalus, cholesteatoma and cholesterol granuloma are complications of otitis media and middle ear disease. Sensorineural hearing loss is now readily treated with cochlear implantation. Facial nerve paralysis may result from infectious and other etiologies.

Child↗

Unusual and aggressive thrombotic onset in two children with antiphospholipid-antibody syndrome and systemic lupus erythematosus.

We report two pediatric patients with unusual, aggressive initial manifestation of antiphospholipid antibody syndrome secondary to systemic lupus erythematosus. The first patient, a 13-year-old girl, presented with bilateral amaurosis and ischemic cerebral lesions. The second, another 13-year-old girl, presented with cerebral venous sinus thrombosis and membranous glomerulonephritis. Both patients improve after treatment with anticoagulants and immunosuppressive drugs, two therapies that are aimed at modulating the immune response or towards preventing thromboembolic events. However, there is no consensus regarding the duration and intensity of oral anticoagulation in children with antiphospholipid antibody syndrome.

Adolescent↗

Central nervous system complications of acute tonsillitis.

Acute tonsillitis is a common infection of early childhood which may even run a self-limiting course without antibiotic therapy in some cases. Complications are encountered infrequently and rarely assume a life-threatening propensity. Central nervous system involvement has not to our knowledge been described in association with tonsillitis without local abscess formation. We describe three cases in which acute tonsillitis/peritonsillitis was complicated by major central neurological sequelae. The neurological complications encountered in young previously healthy adults were: facial palsy and hemiplegia; superior sagittal sinus thrombosis with communicating hydrocephalus and papilloedema; Guillain-Barré syndrome and facial palsy. The pathogenesis and management is discussed. All patients made satisfactory recoveries, though with minor residual neurological disabilities.

Adolescent↗

Lemierre's--the sinister sore throat.

Lemierre's syndrome is a rare and sometimes life threatening condition that requires prompt management. A case is reported of a previously healthy young male with Lemierre's syndrome. He developed internal jugular vein and cavernous sinus thrombosis, metastatic abscesses in the temporal lobe and lungs, temporal lobe venous infarction and severe thrombocytopaenia. Discussed are aspects of clinical presentation, diagnosis and management issues.

Adult↗

Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey.

OBJECTIVES: Pseudotumor cerebri (PC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. MATERIAL AND METHOD: A total of 62 patients diagnosed with PC, who were on follow-up for a period ranging from 4 to 60 months, were investigated retrograde from 1990 to 1998 and then anterograde from 1998 to 2001 to find out the etiological factors, symptoms and signs and the prognosis in the western part of Turkey. RESULTS: There were 47 (76%) women and 15 (24%) men. The age of onset of symptoms was 32.7 +/- 9.9 (range 18-56) years. Obesity was found in only 17 (30%) of them. There were eight patients (13%) with venous sinus thrombosis causing PC. Five patients (8%) had Behçet's disease. The most common symptom was headache, recorded in 93% of the patients, which was followed by transient visual obscurations (60%). Snellen visual acuity was disturbed in 17 patients (27%) at the initial visit. Visual loss determined by automated perimetry was present in 71% of the cases. Three patients (3%) became blind in both eyes. Of the 62 patients, 41 were on follow-up during the study. Twenty-one (51%) showed regression of the visual field grade, nine patients (22%) worsened and 11 (27%) were stationary. CONCLUSION: Obesity was not as frequent as reported in western countries but Behçet's disease was found to be a frequent cause. Perimetry was the most reliable method to follow-up the patients.

Adolescent↗

Intracranial meningioma with progesterone positive receptors presenting in late pregnancy.

A 36-year-old female presented with seizures and transient dysphasia in her 31 week of pregnancy. Neuroradiological investigations revealed a large falx meningioma. A decision was taken to deliver the infant and excise the tumor before term because of the risk of an increase in the size of the tumour and the risk of sinus thrombosis. Therefore, she underwent in her 32 week of pregnancy an elective Caesarean section followed by a craniotomy to remove the meningioma. There were no neonatal complications and she made an uneventful recovery. The final pathology report confirmed the diagnosis of meningioma with progesterone positive receptors.

Adult↗

Nonaneurysmal thunderclap headache with diffuse, multifocal, segmental, and reversible vasospasm.

OBJECTIVE: To highlight the clinical profiles and angiographic findings of two patients with recurrent thunderclap headache (TCH) without subarachnoid hemorrhage (SAH) and to present modified diagnostic criteria for this unusual syndrome. BACKGROUND: TCH may be a benign recurrent headache disorder or it may represent a serious underlying process such as SAH or venous sinus thrombosis. The pathophysiology of this disorder in the absence of underlying pathology is not well understood and its potential angiographic features are not well appreciated. METHODS: Two case descriptions with illustrative angiography. RESULTS: Both cases demonstrated the potential for reversible intracranial vasospasm without intracranial aneurysm or SAH and a benign clinical outcome. CONCLUSIONS: Primary TCH has a distinctive clinical and angiographic profile and must be distinguished from central nervous system vasculitis and SAH.

Headache↗