Cases reported "Encephalocele"

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1/23. Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients.

    Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.
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keywords = headache
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2/23. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache.

    A 43-year-old man had been suffering from exertional headache for 10 years. Sagittal sections on magnetic resonance imaging showed a Chiari type 1 malformation and a cerebellar arachnoid cyst. This syndrome, named hindbrain hernia headache, disappeared with oral acetazolamide. This treatment should be tried in patients with hindbrain hernia headache prior to considering surgical decompression of the foramen magnum.
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keywords = headache
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3/23. Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues.

    Chiari Malformation (CM) encompasses several patterns of congenital or acquired cerebellar herniation through the foramen magnum. This may result in brain-stem compression that impacts control of breathing and is associated with obstructive and central apneas. A high clinical suspicion for sleep-disordered breathing is needed in the care of such patients after as well as before corrective surgery. To introduce a review of CM with a focus on the relevance to sleep medicine, we present a case of a 13-year-old female who was diagnosed with CM Type 1 in the course of an evaluation of symptomatic central sleep apnea. After initial improvement following surgery there was recurrence of brain-stem compression. The only clinical expression of which was polysomnographically evident recurrence of sleep apnea.
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keywords = breathing
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4/23. Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: a case report and review of the literature on de novo growth of cavernomas.

    BACKGROUND: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior.CASE DESCRIPTION: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery.CONCLUSION: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation.
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keywords = headache
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5/23. life-threatening intracranial hypotension after diagnostic lumbar puncture.

    intracranial hypotension syndrome as a complication of diagnostic lumbar puncture is a rarely observed entity. intracranial hypotension syndrome is characterized by postural headache, neck pain/stiffness, blurred vision, nausea, vomiting, clouding of consciousness, dizziness and vertigo. The majority of cases resolve spontaneously with conservative treatment. Rarely, epidural blood patch is required. We report a 41-year-old man with multiple sclerosis, who developed intracranial hypotension syndrome after diagnostic lumbar puncture and who did not respond to conservative treatment. A subdural hematoma was subsequently found, when the patient showed considerable worsening of clinical conditions with life-threatening symptoms. Surgical evacuation of the subdural hematoma was not sufficient to improve significantly the patient's conditions, while complete symptoms remission was achieved 12 hours after epidural blood patch. We stress the need for epidural blood patch in any case of post-diagnostic lumbar puncture postural headache which does not resolve with conservative therapy.
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keywords = headache
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6/23. Bacterial meningitis secondary to a transethmoidal encephalocele presenting to the emergency department.

    We present the case of a patient seen in the Emergency Department (ED) at the height of enteroviral meningitis season with the chief complaint of the worst headache of his life. He was subsequently found to have pneumococcal meningitis as the result of an encephalocele located within the left ethmoid sinus. The key features of the patient's past medical history, the steps to diagnosis, and a discussion of this exceedingly rare entity are detailed.
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keywords = headache
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7/23. Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases.

    OBJECTIVE AND IMPORTANCE: Gliomatosis cerebri (GC) is a rare type of primary brain tumor that diffusely infiltrates more than two lobes of the brain while the normal cerebral architecture is maintained. To the best of our knowledge, the association between an acquired tonsillar herniation and GC has never been reported. In this article, we describe three patients with progressive gliomatosis of the cerebellar hemispheres who subsequently showed signs and symptoms secondary to tonsillar herniation. Early recognition of this potentially life-threatening complication allowed us to recommend prompt surgical intervention. CLINICAL PRESENTATION: One patient with primary, or Type I, GC presented with suboccipital headaches, and two patients with secondary, or Type II, GC presented with the signs and symptoms of progressive myelopathy. Serial imaging studies demonstrated progressive involvement of the cerebellum, descent of the cerebellar tonsils through the foramen magnum, and cervicomedullary spinal cord compression. INTERVENTION: Once the tonsillar herniation was recognized, all three patients underwent posterior fossa decompression, a cervical laminectomy to the lowest level of the tonsillar herniation, and duraplasty. All three patients experienced immediate improvement in their conditions. CONCLUSION: Early recognition of tonsillar herniation, a possibly overlooked cause of death in patients with GC, allows for early surgical intervention as a potentially lifesaving procedure and significant improvement in the patient's condition.
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keywords = headache
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8/23. Intrasphenoidal encephalocele associated with cerebrospinal fluid fistula and subdural hematomas: technical case report.

    OBJECTIVE AND IMPORTANCE: Intrasphenoidal encephalocele is a rare clinical entity that is often complicated by rhinorrhea, recurrent meningitis, and headache, but in no case has the association of rhinorrhea with subdural hematomas been described. A surgical procedure to stop persistent cerebrospinal fluid leakage is reported. CLINICAL PRESENTATION: A 59-year-old man sought care for intractable rhinoliquorrhea of 6 months' duration. Cranial computed tomographic and magnetic resonance imaging scans revealed a basal posterior frontal bony defect and an evocative image suggesting intrasphenoidal encephalocele. INTERVENTION: A transnasal transsphenoidal surgical procedure was performed; the encephalocele was removed, and the sphenoid sinus was filled with an inflatable pouch made of synthetic dura mater containing abdominal fat. Postoperative reduction of the rhinoliquorrhea, but not its total disappearance, was observed. Total disappearance was achieved only after endonasal, transmucosal inflation of the pouch with human fibrin glue. One of the subdural hematomas disappeared spontaneously, and the other was treated by a surgical procedure. CONCLUSION: The possible role of the presented technique in the treatment of cerebrospinal fluid leakage is discussed.
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keywords = headache
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9/23. naegleria fowleri hemorrhagic meningoencephalitis: report of two fatalities in children.

    Two cases of hemorrhagic meningoencephalitis secondary to naegleria fowleri infection confirmed by postmortem analysis are described. The first patient is a 5-year-old boy who presented with a severe headache, neck stiffness, and lethargy. His neurologic examination was significant for somnolence and nuchal rigidity. cerebrospinal fluid studies and structural neuroimaging were consistent with hemorrhagic meningoencephalitis. Another 5-year-old boy presented to a different institution 2 miles away in the same week with similar complaints. Both patients declined rapidly and expired within 48 hours of admission secondary to transtentorial herniation caused by the mass effect of inflammation, edema, and hemorrhage with displacement of the brain stem. Histopathologic and immunochemistry analysis of brain tissue revealed the presence of Naegleria trophozoites in both cases.
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keywords = headache
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10/23. Acute haemorrhage into a microcystic meningioma leading to cerebral herniation.

    Low-grade (WHO level I) meningiomas are slow-growing, benign tumours typically presenting with unspecific symptoms (e.g. headache), seizures, cranial nerve compression and neuropsychological symptoms determined by location and size of the lesion. Haemorrhagic onset and sequelae are rare, and have been described infrequently. This is a case of a 50-year-old male presenting with signs of tentorial herniation secondary to hyperacute intratumoural haemorrhage (ITH) into a previously undiagnosed meningioma. Emergency surgical decompression and exstirpation of the lesion helped to achieve a favourable outcome. ITH has been described in all including benign intracranial neoplasms. Factors associated with a higher risk for haemorrhage in meningiomas are discussed. Though haemorrhages associated with meningiomas have been reported, ITH into low-grade meningiomas leading to herniation remains a rarity. Bearers of known lesions and their treating physicians who opt for conservative or delayed treatment should be aware of this remote complication.
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