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 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient developed the unusual combination of a supranuclear downward gaze paralysis and bilateral ptosis. It was caused by a single midbrain glioma. Other ocular motor functions were intact. The neuropathologic examination showed a tumor growing mainly around the third ventricle and the aqueduct. The findings agree with recent experimental evidence that a network of neural elements involved in eyelid control lies in the supraoculomotor area immediately dorsal to the oculomotor nucleus.
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PMID:Ptosis and supranuclear downgaze paralysis. 292 47

A 70 year old woman underwent an anterior rightsided temporal lobectomy for a mixed glioblastoma-fibrosarcoma. Six months later, she presented with rightsided zygomatic and maxillary swellings, associated with rightsided ptosis, chemosis, and nerve palsies of the III, IV, VI, and VII cranial nerves. A computerized scan (CT) of the head revealed tumor erosion of the skull base of the right middle fossa with extension into the maxillary sinus. This was confirmed at surgery. Pathologic examination revealed a predominantly small cell anaplastic glioblastoma associated with sarcoma elements, similar to the original intracranial tumor. This case documents a rare and atypical behavior of malignant glial tumors.
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PMID:Gliosarcoma with cranial penetration and extension to the maxillary sinus. 406 29

We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.
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PMID:Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl. 1070 32

We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.
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PMID:Nasal chondromesenchymal hamartoma of infancy clinically mimicking meningoencephalocele. 1536 5

We report two children with post radiation midbrain damage causing severe neurological symptoms. A twelve-year-old boy with a four year history of hydrocephalus was diagnosed with tectal glioma, which endoscopic biopsy revealed to be low grade. He underwent gamma knife radiation surgery (central 24 Gy/peripheral 12 Gy). Two months later bilateral ptosis followed by total oculomotor palsy and drowsiness developed. Despite pulsed-steroid therapy the tumor size increased up to 4.6 times in volume. The tumor was totally removed and was diagnosed as an early delayed radiation reaction pathologically. His symptoms disappeared except for a slight upper gaze palsy. The second patient was a six-year-old girl with a medulloblastoma. Following total resection and a VP shunt she received conventional radiation therapy along with chemotherapy. After the final irradiation she became comatose (JCS II-2) and MRI revealed diffuse midbrain damage with acute aqueduct obstruction, which recovered in two weeks. Reports of irradiation injuries of the midbrain in childhood are rare but it should be considered as a possible cause of fulminant symptoms requiring emergency treatment. Because of midbrain anatomical complexity, midbrain radiation therapy requires great care, especially in children.
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PMID:[Fulminating midbrain irradiation injury of pediatric brain tumor]. 1624 69

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with highly variable expression generally ascribed to random factors. However, evidence is presented for patterns suggesting non-stochastic processes as follows: (1) We have seen a MZ twin pair concordant for renal vascular hypertension, and another for unilateral ptosis. Other concordances have been reported, including both malformations and tumors, and combinations as well. (2) Four children were seen with a distinct ipsilateral association of glaucoma or iris anomaly, optic glioma, plexiform neurofibromas arising from the trigeminal nerve and its branches, and sphenoid dysplasia. Other cases in the literature support milder forms of this association. (3) We saw six children with apparent gynecomastia or premature thelarche without endocrine abnormalities. Tissue samples from four of these showed an unusual fibrous plexiform neurofibroma. Interestingly, five of the six cases were African Americans, and constitutional factors affecting fibrous reactions may also be involved here.A tentative hypothesis is presented suggesting vascular fields involving defined areas that can: (1) Support tumor growth. They would be the "soil" determining the ability and the extent of growth. There would, however, still be a need for a "second hit" tumor transformation. (2) Affect blood supply to organs, creating structural anomalies. NF1 involves a vasculopathy, and would predispose to vulnerabilities of such fields. Genetic factors could induce superimposed susceptibilities of specific fields, leading to twin concordances. "Hits" affecting specific fields would increase the likelihood of multiple abnormalities that could include both tumors and structural findings. Finally, tumors may follow the contours of existing fields. The breast is an area normally primed for growth, and the observation of clitoromegally secondary to tumor involvement suggests that such fields exist elsewhere.
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PMID:Non-random associations and vascular fields in neurofibromatosis 1: a pathogenetic hypothesis. 1677 Aug 9

A rare case of neurosarcoidosis presenting as an isolated quadrigeminal plate mass without systemic manifestation of this disease is reported. This 26-year-old man presented with symptoms of acute intracranial hypertension including headache, morning vomiting as well as a right homonymous hemianopsia. Magnetic resonance imaging (MRI) showed an expansive tectal mass causing hydrocephalus secondary to an aqueductal obstruction. An external ventricular drainage was inserted and the mass, postulated to be a glioma, was removed through an occipital transtentorial craniotomy. Histopathological examination revealed numerous sarcoid granulomas. Postoperative course was relevant for bilateral hypoacusis and tinnitus, blurred vision, bilateral palpebral ptosis and bilateral internuclear ophthalmoplegia. Chest X-ray was normal. Postoperative thoracic computed tomography (CT) scan showed mediastinal adenopathies. Lung function tests were normal. Angiotensin converting enzyme (ACE) cerebrospinal fluid (CSF) blood ratio was normal. Postoperative treatment and follow-up included corticosteroids, serial lung function tests and cerebral MRI. Neurosarcoidosis may present with protean clinical manifestations and unusual radiological features. This rare diagnosis has to be kept in mind when facing isolated intracerebral mass lesions.
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PMID:Neurosarcoidosis presenting as an isolated mass of the quadrigeminal plate. 1863 66

We report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas.
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PMID:Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass. 2130 32

Gliomatosis cerebri is a rare, diffuse glioma of neuroepithelial origin involving more than two cerebral lobes. Clinical presentation of gliomatosis cerebri is variable and depends on the degree, extent, and location of cortical involvement. Signs and symptoms related to supratentorial cortical involvement predominate and the diagnosis is reached through a combination of clinical, radiographic, and histopathological evaluations. This is a report of a young man who presented with visual problems and bilateral ptosis, which were eventually attributed to gliomatosis cerebri. Standard radiation and chemotherapy were administered but the patient eventually succumbed to the disease. The unique clinical presentation is discussed in light of this rare neoplasm of the central nervous system.
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PMID:Bilateral ptosis as initial presentation of gliomatosis cerebri: case report. 2342 31