Cases reported "Pseudotumor Cerebri"

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1/50. Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri.

    PURPOSE: To describe three children with acute fourth cranial nerve palsy secondary to pseudotumor cerebri. methods: We reviewed the medical records of children younger than 18 years who were diagnosed with pseudotumor cerebri between 1977 and 1997. pseudotumor cerebri was defined by normal neuro-imaging, elevated intracranial pressure measured by lumbar puncture, and normal cerebrospinal fluid composition. RESULTS: Three children with pseudotumor cerebri presented with vertical diplopia and clinical signs of fourth cranial nerve palsy including a hypertropia of the affected eye, which increased with adduction and ipsilateral head tilt. The fourth cranial nerve palsy resolved after reduction of the intracranial pressure in all three children. CONCLUSIONS: Fourth cranial nerve palsy may occur in children with pseudotumor cerebri and may be a nonspecific sign of elevated intracranial pressure.
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2/50. pseudotumor cerebri in children receiving recombinant human growth hormone.

    PURPOSE: This article represents the first report in the ophthalmology literature of an association between pseudotumor cerebri (PTC) and recombinant human growth hormone (rhGH). DESIGN: Noncomparative case series. PARTICIPANTS: Three children receiving rhGH for short stature with turner syndrome, Jeune syndrome, or down syndrome. methods: Children underwent full ocular examination. After papilledema was identified, patients underwent lumbar puncture and imaging with either magnetic resonance imaging or computerized tomography. Treatment was under the guidance of the primary physician or neurosurgeon. The rhGH was discontinued in all children. MAIN OUTCOME MEASURES: visual acuity and evaluation of the optic nerve for resolution of papilledema were followed at each examination. RESULTS: In all three cases, papilledema resolved with the cessation of rhGH, and treatment with acetazolamide or prednisone. visual acuity was unchanged in case 1, decreased by two to three lines in case 2, and was inconsistent in case 3. One child (case 2) required a ventriculoperitoneal shunt for persistent elevation of intracranial pressure. CONCLUSION: There appears to be a causal relationship between the initiation of rhGH with the development of PTC. Children should have a complete ophthalmic evaluation if they report headache or visual disturbances. Baseline examination with routine follow-up should be instituted when children cannot adequately communicate.
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3/50. pseudotumor cerebri secondary to intermediate-dose cytarabine HCl.

    OBJECTIVE: To describe a case of pseudotumor cerebri associated with the administration of intermediate-dose cytarabine. CASE SUMMARY: An 11-year-old Hispanic boy with acute myeloblastic leukemia developed symptoms of pseudotumor cerebri (headache, diplopia, photophobia, nausea, vomiting) after receiving chemotherapy including cytarabine. The patient improved after a lumbar puncture and treatment with prednisone and acetazolamide, and is now asymptomatic. DISCUSSION: pseudotumor cerebri is a condition usually associated with obese women of child-bearing age. case reports in pediatric patients are unusual. Several medications have been implicated in causing pseudotumor cerebri, including antimicrobials (tetracycline, naladixic acid), amiodarone, lithium carbonate, vitamin a and its derivatives, growth hormone, and corticosteroids. Chemotherapy agents reported to cause pseudotumor cerebri include busulfan with cyclophosphamide, and the combination of vinblastine, cisplatin, and bleomycin. Most of the information on medication-induced pseudotumor cerebri is in the form of case reports. Different mechanisms for causing this condition have been offered for individual medications. Most of these explanations involve fluid imbalance or interference with the Na /K adenosine triphosphatase pump. Controlled studies are difficult because this condition is an unpredictable and rare occurrence. cytarabine has frequently been associated with neurologic toxicities, but few reports of pseudotumor cerebri can be found. CONCLUSIONS: The exact cause of pseudotumor cerebri in this patient is unknown, but cytarabine seems a likely cause. The mechanism by which cytarabine could cause this reaction is unclear.
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4/50. Dural sinus thrombosis and pseudotumor cerebri: unexpected complications of suboccipital craniotomy and translabyrinthine craniectomy.

    OBJECT: The goal of this study was to document the hazards associated with pseudotumor cerebri resulting from transverse sinus thrombosis after tumor resection. Dural sinus thrombosis is a rare and potentially serious complication of suboccipital craniotomy and translabyrinthine craniectomy. pseudotumor cerebri may occur when venous hypertension develops secondary to outflow obstruction. Previous research indicates that occlusion of a single transverse sinus is well tolerated when the contralateral sinus remains patent. methods: The authors report the results in five of a total of 107 patients who underwent suboccipital craniotomy or translabyrinthine craniectomy for resection of a tumor. Postoperatively, these patients developed headache, visual obscuration, and florid papilledema as a result of increased intracranial pressure (ICP). In each patient, the transverse sinus on the treated side was thrombosed; patency of the contralateral sinus was confirmed on magnetic resonance (MR) imaging. Four patients required lumboperitoneal or ventriculoperitoneal shunts and one required medical treatment for increased ICP. All five patients regained their baseline neurological function after treatment. Techniques used to avoid thrombosis during surgery are discussed. CONCLUSIONS: First, the status of the transverse and sigmoid sinuses should be documented using MR venography before patients undergo posterior fossa surgery. Second, thrombosis of a transverse or sigmoid sinus may not be tolerated even if the sinus is nondominant; vision-threatening pseudotumor cerebri may result. Third, MR venography is a reliable, noninvasive means of evaluating the venous sinuses. Fourth, if the diagnosis is made shortly after thrombosis, then direct endovascular thrombolysis with urokinase may be a therapeutic option. If the presentation is delayed, then ophthalmological complications of pseudotumor cerebri can be avoided by administration of a combination of acetazolamide, dexamethasone, lumbar puncture, and possibly lumboperitoneal shunt placement.
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5/50. pseudotumor cerebri caused by all-trans-retinoic acid: a case report.

    pseudotumor cerebri is a condition characterized by increased intracranial pressure with normal cerebrospinal fluid composition. It is usually associated with many medical conditions and drugs. It has been reported that all-trans-retinoic acid can induce pseudotumor cerebri. The authors report a 35-year-old patient diagnosed with acute promyelocytic leukemia who developed pseudotumor cerebri after receiving all-trans-retinoic acid. The patient was treated with 60 mg/day of all-trans-retinoic acid. Two weeks later, she had severe headache and blurred vision. The neurological examination revealed papilledema and decreased visual acuity. Other physical examinations were unremarkable. brain CT was normal. Additionally, lumbar puncture was performed and the CSF finding was normal except for high CSF pressure. According to modified Dandy criteria, the diagnosis was pseudotumor cerebri. The patient's symptoms disappeared after all-trans-retinoic acid was discontinued. To our knowledge, this is the first case report in thailand.
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6/50. Idiopathic intracranial hypertension and hemophilia a.

    OBJECTIVE: A patient with hemophilia a and long-standing recurrent symptoms of idiopathic intracranial hypertension is described. During his relapses, he experienced headache, and attention and language disturbance, but no visual symptoms. BACKGROUND: hemophilia a is a rare inherited coagulation disorder secondary to factor viii deficiency. Idiopathic intracranial hypertension has been reported in association with prothrombotic conditions and iron deficiency anemia, but not in patients with hemophilia a. Recurrent or chronic headache is not a typical symptom of hemophilia, but headache is a presenting sign of intracranial bleed in persons with hemophilia. methods: Medical history review, clinical neurologic examination, brain magnetic resonance imaging, computed head tomography, and electroencephalogram were performed. RESULTS: neurologic examination revealed bilateral papilledema during relapses of idiopathic intracranial hypertension. Multiple lumbar punctures preceded by the intravenous administration of factor viii early in the course of the illness confirmed the presence of elevated cerebrospinal fluid pressures and absence of subarachnoid blood. He had no complications from lumbar punctures. Initial electroencephalograms showed background slowing but later normalized. magnetic resonance imaging of the brain and computerized tomography of the head were normal. Relapses of idiopathic intracranial hypertension were eventually controlled with the administration of acetazolamide. CONCLUSION: Idiopathic intracranial hypertension may develop in patients with hemophilia a in the absence of visual symptoms. Therapeutic and diagnostic lumbar punctures were safe to perform on this patient, following the administration of factor viii.
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7/50. When does low mean high? Isolated cerebral ventricular increased intracranial pressure in a patient with a Chiari I malformation.

    OBJECTIVE: To present an unusual case of pseudotumor cerebri with increased intracranial pressure isolated to the cerebral ventricles resulting from a Chiari I malformation. MATERIALS AND methods: The patient received a complete ophthalmologic examination on initial presentation and subsequent visits, including visual acuity, pupillary examination, intraocular pressures, dilated fundus examination with assessment of degree of papilledema, and visual field testing. intracranial pressure was measured by lumbar puncture and subsequently by intracranial pressure bolt monitoring. magnetic resonance imaging (MRI) was used to diagnose the Chiari I malformation. RESULTS: The patient initially presented with bitemporal headaches, elevated opening pressure on lumbar puncture, and mild papilledema with a normal MRI. After lumboperitoneal shunt placement and several revisions, the patient presented with decreased vision OD secondary to Terson syndrome and worsening papilledema. Subsequent evaluation revealed normal lumbar opening pressures and a Chiari I malformation. She underwent ventriculoperitoneal shunt placement with resolution of her symptoms. CONCLUSIONS: Tonsillar herniation is a well-documented complication of lumboperitoneal shunt revision. Obstruction of cerebrospinal flow through the foramina of Magendie and Luschka can result in increased intracranial pressure isolated to the cerebral ventricles. In a patient with signs and symptoms of increased intracranial pressure but normal lumbar opening pressure, a Chiari I malformation should be suspected, particularly with a history of multiple lumboperitoneal shunt revisions.
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8/50. Lumboperitoneal shunting as a treatment for slit ventricle syndrome.

    OBJECTIVE: slit ventricle syndrome (SVS) has been described in hydrocephalus patients who continue to have shunt malfunction-like symptoms in the presence of a functioning shunt system and small ventricles on imaging studies. These symptoms usually present years after shunt placement or revision and can consist of headache, nausea and vomiting, lethargy and decreased cognitive skills. Treatments offered range from observation, medical therapy (migraine treatment) and shunt revision to subtemporal decompression or cranial vault expansion. We describe a subset of patients with SVS who were symptomatic with high intracranial pressure (ICP) as measured by sedated lumbar puncture and whose symptoms completely resolved after lumboperitoneal shunt (LPS) placement. methods: Seven patients with a diagnosis of SVS underwent lumboperitoneal shunting. The age at shunting ranged from 3 to 18 years. Most had undergone recent ventriculoperitoneal shunt (VPS) revisions for presentation of shunt malfunction-like symptoms. Despite this, all remained symptomatic and underwent a sedated lumbar puncture to measure opening pressure (OP). All had high OP in spite of a functional VPS and underwent LPS placement. RESULTS: All 7 patients had a prolonged period of overdrainage symptoms after lumboperitoneal shunting that resolved completely over several weeks. The initial etiology of hydrocephalus was reported to include trauma, aqueductal stenosis and intraventricular hemorrhage of prematurity. Two patients required revision of their LPS, after which their symptoms again resolved. CONCLUSION: In a certain subset of patients with SVS who are symptomatic from increased ICP, placement of an LPS is an effective treatment option. It appears that this subgroup of patients previously treated with ventriculoperitoneal shunting behave in a fashion similar to pseudotumor cerebri patients and respond well to lumboperitoneal shunting.
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9/50. Asymptomatic idiopathic intracranial hypertension in young children.

    Presenting symptoms of idiopathic intracranial hypertension are known to vary with age. Older children may complain of headache, neck pain, diplopia, intracranial noises, or transient visual obscurations. Younger children may present with apathy or irritability. This report describes three young children with no obvious relevant symptoms in whom papilledema was newly found on routine follow-up eye examination for unrelated problems. At presentation, all had early papilledema with negative cranial neuroimaging studies. All remained apparently asymptomatic, but the papilledema progressed. Sedated lumbar puncture showed elevated cerebrospinal fluid pressure in all three. Two were felt to have truly idiopathic intracranial hypertension, whereas the third had jugular venous obstruction. The papilledema responded to treatment with either acetazolamide or furosemide in all three. An apparent lack of symptoms does not rule out chronic increased intracranial pressure in young children.
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10/50. pseudotumor cerebri in a patient with Goldenhar's and Duane's syndromes.

    A 4-year-old boy presented with Goldenhar's syndrome, Duane's syndrome and bilateral papilledema. magnetic resonance imaging of the brain was unremarkable. On lumbar puncture, the cerebrospinal fluid (CSF) pressure measured 36 cm H(2)O. CSF examination was normal. The diagnosis of pseudotumor cerebri was made, and treatment with acetazolamide was started. As the papilledema did not resolve, steroids were added to the treatment. Lumbar puncture was repeated after 1 month, and pressure was found to be 30 cm H(2)O. Because medical treatment was not effective in lowering the CSF pressure, optic nerve sheath fenestration was performed. papilledema resolved over the next 2 months. To the best of our knowledge, this is the first case of Goldenhar's syndrome associated with pseudotumor cerebri.
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