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Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia.

Early in the course of a painful crisis, a 19-year-old man with known sickle cell anemia (SCA) developed a clinical picture that resembled either early cavernous sinus thrombosis or retroorbital and bifrontal microinfarcts. A brain computer tomography scan demonstrated bilateral retroorbital hemorrhages along with a left frontal epidural hematoma. In the absence of trauma, thrombocytopenia, or any other detectable hemostatic defect, this type of hemorrhagic manifestation in the setting of SCA has not, to our knowledge, been previously reported in the literature.

Adult↗

Osteomyelitis of the skull following scalp reduction and hair plug transplantation.

A case of Staphylococcus aureus osteomyelitis of the skull following scalp reduction and hair plug transplantation is presented. Frontal and biparietal craniectomies were performed, adjacent galea and subcutaneous tissues were excised, and intravenous antibiotics were administered to eradicate the infection and forestall the development of sagittal sinus thrombosis. Persistent drainage of hair plug transplantation sites may suggest underlying osteomyelitis.

Hair↗

Diffusion-weighted imaging as a problem-solving tool in the evaluation of patients with acute strokelike syndromes.

This article addresses syndromes that clinically and/or radiologically resemble acute stroke. These syndromes generally fall into four categories. (1) Patients with acute neurological deficits with nonischemic lesions and no acute abnormality on diffusion-weighted images. These patients may have peripheral vertigo, migraines, seizures, dementia, functional disorders, amyloid angiopathy, or metabolic disorders. When these patients present, we can confidently predict that they are not undergoing infarction. (2) Patients with ischemic lesions with reversible clinical deficits. Nearly 50% of patients with transient ischemic attacks have lesions with restricted diffusion. Patients with transient global amnesia may have punctate lesions with restricted diffusion in the medial hippocampus, parahippocampal gyms, and corpus callosum. (3) Vasogenic edema syndromes that may mimic acute infarction clinically and on conventional imaging. These include eclampsia/hypertensive encephalopathy, other posterior leukoencephalopathies, human immunodeficiency virus encephalopathy, hyperperfusion syndrome following carotid endarterectomy, venous sinus thrombosis, acute demyelination, and neoplasm. These syndromes demonstrate elevated diffusion rather than the restricted diffusion associated with acute ischemic stroke. (4) Entities in which restricted diffusion may resemble acute infarction. These include pyogenic infections, herpes virus encephalitis, Creutzfeldt-Jakob disease, diffuse axonal injury, tumors with dense cell packing, and rare acute demyelinative lesions.

Diffusion↗

The role of drugs in the etiology of stroke.

Drugs of many classes have been implicated in hemorrhagic and ischemic stroke. Alcohol in moderation may have a protective effect although in greater doses may predispose to stroke. Drugs such as cocaine, amphetamines and heroin have been associated with stroke by a number of mechanisms. Antiplatelet, anticoagulant and thrombolytic therapy carry risk of hemorrhagic complications. Oral contraceptives appear to slightly increase stroke risk whereas estrogen replacement therapy may decrease it. Anabolic steroid use in athletes has been linked to stroke. The antineoplastic agent L-asparaginase has been associated with cerebral hemorrhage, ischemic infarction and venous sinus thrombosis. Infarction has been reported in association with cisplatin-based combination chemotherapy. Stroke is an infrequent but recognized complication of some forms of drug therapy and drug abuse.

Animals↗

Chronic rhinocerebral mucormycosis.

Rhinocerebral mucormycosis is a well-described fulminant fungal infection that typically presents acutely in patients with diabetic ketoacidosis or immunosuppression. Chronic presentations of rhinocerebral mucormycosis have also been described. In the chronic infection, the disease course is indolent and slowly progressive, often occurring over weeks to months. The authors report 2 cases of chronic rhinocerebral mucormycosis (CRM) treated at their institution and review 16 other cases reported in the English-language literature. In these cases, the median time from symptom onset to diagnosis was 7 months. The most common presenting features of CRM are ophthalmologic and include ptosis, proptosis, visual loss, and ophthalmoplegia. CRM occurs predominantly in patients with diabetes and ketoacidosis. The incidence of internal carotid artery and cavernous sinus thrombosis is higher in CRM patients than in those with the acute disease, although the overall survival rate for CRM patients is 83%. CRM is clinically distinct from chronic Entomophthorales infection.

Aged↗

Neurological features of cranial vault lymphomas: report of two cases.

Two patients who had focal neurological deficits as the initial manifestation of a malignant non-Hodgkin's lymphoma involving the skull are described. Soft-tissue masses and variable bone destruction were the predominant computed tomographic findings, and magnetic resonance imaging studies revealed meningeal involvement in one case and sinus thrombosis in the other. Malignant non-Hodgkin's lymphomas initially appearing in the skull are rare, but these lesions should be considered in patients with a palpable scalp mass who have focal neurological signs.

Adult↗

Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage.

OBJECTIVE: An excruciating headache of instantaneous onset is known as a thunderclap headache. A subarachnoid hemorrhage is the prototypical cause, but other serious disorders may also present with a thunderclap headache, including cerebral venous sinus thrombosis, carotid artery dissection, and pituitary apoplexy. We report a group of patients with thunderclap headaches as the initial manifestation of spontaneous intracranial hypotension caused by a spinal cerebrospinal fluid leak. METHODS: Among 28 patients with spontaneous intracranial hypotension due to a documented spinal cerebrospinal fluid leak, four (14%) initially experienced an excruciating headaches of instantaneous onset. RESULTS: The mean age of the four patients (two men and two women) was 35 years (range, 24-45 yr). Nuchal rigidity was present in the three patients who sought early medical attention, and they underwent emergency computed tomographic scanning, lumbar puncture, and cerebral angiography to rule out an aneurysmal subarachnoid hemorrhage. The delay between the onset of headache and diagnosis of intracranial hypotension ranged from 4 days to 5 weeks. A fourth patient did not seek medical attention until 1 month after the ictus. CONCLUSION: Spontaneous intracranial hypotension should be included in the differential diagnosis of thunderclap headache, even when meningismus is present.

Adult↗

99Tcm-HMPAO brain SPET in patients with pseudotumour cerebri.

The aim of this study was to look for regional cerebral blood flow (rCBF) abnormalities in patients with a recent diagnosis of pseudotumour cerebri (PTC) and to compare 99Tcm-hexamethylpropylene amine oxime (99Tcm-HMPAO) cerebral single photon emission tomographic (SPET) findings with those of X-ray computed tomography (CT) and magnetic resonance imaging (MRI). Seventeen consecutive patients aged 14-50 years (mean = 29 years) without previous drug treatment for PTC underwent 99Tcm-HMPAO SPET, cranial CT and MRI studies. No patient had evidence of intracranial mass lesions on the CT or MRI scans. Qualitative analysis of the 99Tcm-HMPAO SPET studies revealed impaired rCBF in nine (53%) patients. The CT and MRI studies revealed an empty sella in two patients, dural sinus thrombosis in one and a slit ventricle in another. Eight of the 13 patients with normal CT and MRI results had rCBF abnormalities. In contrast, three patients with CT and MRI abnormalities had a normal 99Tcm-HMPAO scan. Our results suggest that patients with PTC have functional abnormalities that are revealed by 99Tcm-HMPAO brain SPET.

Adolescent↗

Otitis media complications and treatment failures: implications of pneumococcal resistance.

Classic complications of untreated otitis media include meningitis, lateral sinus thrombosis and chronic suppurative otitis media. In the past, in countries where otitis media is usually treated, complications have been rare, because of the good activity of almost all orally administered antibiotics against the most common cause of complications, Streptococcus pneumoniae. Treatment failures were usually caused by beta-lactamase-producing nontypable Haemophilus influenzae or by Moraxella (Branhamella) catarrhalis and were rarely associated with serious systemic infections. With the advent of multidrug-resistant pneumococci, however, serious and fatal infections can occur in the face of our most potent antimicrobial agents. The consequences of the emergence of multidrug-resistant pneumococci are likely to include more persistent purulent otitis media, increased usage of broad-spectrum antibiotics, an increase in surgical treatment rates for otitis media and, eventually, an increase in suppurative complications of otitis media. Medical treatment failures probably already surpass eustachian tube dysfunction as the most common reason for tympanostomy tube insertion. Multidrug-resistant pneumococci may be expected to change the way in which primary and secondary care is currently administered.

Child↗

Severe hypernatremic dehydration and death in a breast-fed infant.

Breast milk is acknowledged as the best source of nutrition for neonates. We present the case of a full-term newborn who was fed solely breast milk and developed severe dehydration and hypernatremia. The patient developed cerebral edema, transverse sinus thrombosis, and died. The literature on the uncommon entity of breast-feeding hypernatremia and dehydration is reviewed, and management strategies are presented.

Adult↗

Intracranial complications of ear disease in a pediatric population with special emphasis on subdural effusion and empyema.

Between the years 1963 to 1982, 84 consecutive cases of intracranial complications of ear disease in pediatric patients were diagnosed and treated at the North Carolina Baptist Hospital. This group consisted of 65 patients with otitic meningitis, four with otitic brain abscess, four with otitic hydrocephalus, three with lateral sinus thrombosis, three with otitic subdural empyemas, and five with an otitic subdural effusion of fluid. The advent of antibiotics has drastically altered the natural history of middle ear infections. We present these 84 cases to review diagnostic problems and therapeutic options, and to offer a plea for continued awareness of the middle ear as a potential source of central nervous system complications.

Acute Disease↗

Persistent nausea and anorexia after marrow transplantation: a prospective study of 78 patients.

BACKGROUND: Persistent nausea, vomiting, anorexia, and poor oral intake are common after hematopoietic cell transplantation. In the past, herpesvirus infections and acute intestinal graft-versus-host disease (GVHD) were the most common causes. METHODS: We studied 76 patients with 78 episodes of these symptoms to discern the causes. Diagnoses were based on histology of skin and intestinal biopsy specimens, viral cultures, and responses to therapy. RESULTS: The mean day of study entry was day 57+/-31.3 posttransplant. Acute GVHD was the most common cause of symptoms, affecting 63 patients (81%) as the sole cause of symptoms and an additional 4 patients (5%) who had other concurrent causes. Patients with GVHD had marrow donors who were unrelated or HLA-mismatched in 27/63 cases. Gastric edema, erythema, and apoptotic epithelial cells were the most useful findings for the diagnosis of GVHD. Prednisone therapy (1-2 mg/kg/day) was effective in 58 of 63 patients (92%). Infection by herpes simplex virus, cytomegalovirus, or Candida was found in six patients, three of whom had concurrent GVHD. Other causes of symptoms were medications (one patients), parenteral nutrition (one patient), and sagittal sinus thrombosis (one patient). CONCLUSIONS: Acute GVHD is now the dominant cause of persistent nausea and anorexia in marrow transplant patients who are beyond day 20 posttransplant. The diagnosis can be made clinically in most cases and confirmed by endoscopic biopsy of gastric mucosa. Infections, medications, and rare cases of central nervous system disease are much less common.

Adolescent↗

Interventional neuroradiology.

Interventional neuroradiology continues to play an increasing role in the treatment of intracranial aneurysms, the treatment of vasospasm related to subarachnoid hemorrhage, and the treatment of vascular malformations and fistulae of the brain and spine. New techniques for aneurysm embolization are examined, such as detachable coil systems, as are reports of intracranial angioplasty and superselective papaverine infusion for vasospasm following aneurysm rupture. Results of investigations of arteriovenous malformation embolizations and recent analyses of risk factors associated with arteriovenous malformations are discussed. New approaches to tumor embolization are presented, and there are new data on angioplasty for cerebral atherosclerotic disease. Local fibrinolytic therapy in the treatment of patients with acute intracranial arterial or venous sinus thrombosis is evaluated.

Angioplasty, Balloon↗

Behçet's syndrome and the nervous system.

PURPOSE OF REVIEW: Behçet's syndrome (BS) is a multi-system, vascular-inflammatory disease of unknown origin, involving the nervous system in a subgroup of patients. The syndrome is rare, but as patients with BS are young and frequently present with an acute or subacute brainstem syndrome or hemiparesis, as well as with other various neurological manifestations, the syndrome is often included in the differential diagnosis of multiple sclerosis, stroke of the young adult, and another wide range of neurological disorders. The present review summarizes the neurological involvement in BS, and emphasizes recent clinical concepts and ethiopathogenetic findings. RECENT FINDINGS: Over the last years the growing clinical and imaging evidence had suggested that neurological involvement in BS may be subclassified into two major forms: one, which is seen in the majority of patients, may be characterized as a vascular-inflammatory CNS disease, with focal or multifocal parenchymal involvement; the other, which has few symptoms and a better neurological prognosis, may be caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. These two types rarely occur in the same individual, and their pathogenesis is likely to be different. A nonstructural vascular type headache is relatively common, whereas isolated behavioral syndromes and peripheral nervous system involvement are rare. SUMMARY: The involvement of the nervous system in BS is heterogeneous as clinical and imaging data reveal. Currently it is unknown which factors determine or have a role in the development of neurological involvement, but some progress has been achieved in understanding the neurological spectrum of the syndrome, which may lead to a better management of these patients.

Adrenal Cortex Hormones↗

Call-Fleming postpartum angiopathy in the puerperium: a reversible cerebral vasoconstriction syndrome.

BACKGROUND: The postpartum angiopathy (Call-Fleming syndrome) is a rare, reversible cerebral vasoconstriction syndrome. Unlike in eclampsia, neither proteinuria nor hypertension have been observed in the Call-Fleming syndrome. CASE: A 17-year-old woman developed headache, seizures, confusion, cortical blindness, and denial of visual loss (optic anosognosia, Anton syndrome) on the first postpartum day. Transcranial Doppler demonstrated severely elevated blood velocities. Magnetic resonance angiography showed multifocal segmental narrowing of cerebral arteries. Magnetic resonance imaging revealed occipitotemporal edema. After symptomatic treatment, the clinical deficits disappeared completely within 2 weeks. The follow-up neuroimaging performed after 3 months was unremarkable. CONCLUSION: The case shows that the Call-Fleming syndrome is a reversible condition with excellent prognosis representing an important differential diagnosis of eclampsia, dural sinus thrombosis, and intracranial bleeding in puerperium.

Adolescent↗

Neurologic disorders in pregnancy.

BACKGROUND: Neurologic dysfunction, coma, and seizures are common in obstetric patients in the intensive care unit. OBJECTIVE: To review common neurologic disorders resulting in critical illness in pregnancy. REVIEW: Obstetric disorders causing coma and seizures include eclampsia, acute fatty liver of pregnancy, and amniotic fluid embolism. Preexisting disorders such as epilepsy may worsen in one-third of pregnant patients, and seizures are common during labor. Changes in hemodynamics, blood volume, and hormonal effects on the vessel wall increase risk of bleeding from berry aneurysms and arteriovenous malformations during pregnancy and the postpartum period. Acute intermittent porphyria produces seizures and hypertension, closely mimicking eclampsia. Cerebral venous sinus thrombosis is common in postpartum patients, especially in developing countries. Brain tumors invariably enlarge during pregnancy because of fluid retention and the presence of estrogen and progesterone receptors on tumor cells. Infections such as cerebral malaria and acute viral hepatitis with fulminant hepatic failure are common causes of coma and seizures during pregnancy in tropical regions of Asia, Africa, and Latin America. Patients may be admitted to the intensive care unit with type II respiratory failure due to myasthenic crisis, Guillain-Barre syndrome and spinal cord disease. Relapses of multiple sclerosis are infrequent during pregnancy but increase in the postpartum period. CONCLUSIONS: In all instances, the effects of the disorders, diagnostic tests, and treatment on the fetus must be carefully weighed. Prompt delivery may be lifesaving for mother and fetus in conditions such as eclampsia and acute fatty liver of pregnancy; expectant treatment may be more appropriate in others.

Critical Care↗

A review of 50 cases of intracranial complications from otogenic infection between 1961 and 1977.

The records of 50 patients with intracranial complications of middle ear disease treated over the past 16 years have been reviewed. Virtually all the known intracranial consequences of middle ear infection have been encountered, and include cerebral abscess, cerebellar abscess, meningitis, lateral sinus thrombosis, otitic hydrocephalus and cortical thrombophlebitis. The clinical features, neurological signs and investigative data for each group have been compared and contrasted. Treatment in the past has consisted of radical and modified radical mastoidectomy. It is proposed that these techniques will continue to be preferred for the management of chronic otitis media in the presence of a condition as serious as an intracranial complication. It is concluded that with a quoted incidence of postoperative cholesteatoma as high as 40% intact canal wall techniques are not justified in these patients at present.

Bacterial Infections↗