An extensive cranial base meningioma extending bilaterally into Meckel's cave: case report

OBJECTIVE AND IMPORTANCE: A patient with an extensive cranial base meningioma that included bilateral invasion of Meckel's cave underwent surgical resection and had an unexpected rare complication, malocclusion from bilateral trigeminal dysfunction. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our hospital with alternating painful ophthalmoplegia. He had been blind since the age of 10 years. At the time of admission, neurological findings included bilateral visual loss and optic atrophy. Magnetic resonance images showed an extensive tumor located at the planum sphenoidale, tuberculum sellae, and bilaterally at Meckel's cave and the medial tentorial incisura. INTERVENTION: The patient underwent a two-stage operation. During the first procedure, the masses in the planum sphenoidale, tuberculum sellae, and the left side of Meckel's cave were excised intradurally. At the second operation, the mass in the right side of Meckel's cave was excised extradurally and the tentorial mass was removed intradurally. The patient's postoperative course was complicated by bilateral trigeminal nerve dysfunction, which caused malocclusion. CONCLUSION: Bilateral dysfunction of the trigeminal nerve may cause a number of problems. Thus, extreme caution must be taken to preserve the function of this nerve.     

Progressive visual loss due to glioblastoma: normal neuroroentgenorgraphic studies

A case of progressive bilateral visual loss of abrupt onset an rapid course is described. Extensive neurorentgenographic evaluation was normal, and the results of exploratory craniotomy were unremarkable, except for a slight ovality of the left optic nerve. A biopsy of the left optic nerve showed a glioblastoma, apparently originating in the anterior optic pathways.     

Pancreatitis induced by codeine: a case report with positive rechallenge

PURPOSE: To postulate a causal relation between optic nerve hypoplasia and a suprasellar teratoma. METHOD: Case report. RESULTS: A 6-month-old infant with suprasellar teratoma was visually inattentive and had searching nystagmus. He had moderately severe, bilateral optic nerve hypoplasia with the left eye being somewhat worse than the right eye. CONCLUSIONS: Optic nerve hypoplasia is a major cause of impaired vision in children and rarely has been attributed to an intracranial tumor. Our case, involving a patient with a suprasellar teratoma and optic nerve hypoplasia, supports a causal relation between the two.     

Optic nerve ganglioglioma. Case report

Gangliogliomas of the optic nerve are extremely rare. The case is reported of a 38-year-old man who presented with a visual field deficit and was discovered to have an optic nerve ganglioglioma. The possible embryological origins of this neoplasm, its histological and immunohistochemical features, and its appearance on magnetic resonance imaging are examined. The prognoses of optic nerve glioma and of gangliogliomas occurring elsewhere in the nervous system are compared.     

Autoantibodies and pregnancy loss

Patients with neurofibromatosis type 1 (NF-1) have an increased incidence of optic glioma. Although spinal dural ectasia or meningocele is well represented in the NF-1 literature, radiologists are not as familiar with dural ectasia of the optic nerve sheath as spinal dural ectasia. This is a report of a pediatric patient with NF-1 with dural ectasia of the optic nerve sheath. This is the second reported case of dural ectasia of the optic nerve sheath demonstrated by magnetic resonance imaging.     

Primary papillary meningioma of the optic nerve sheath: a case of unique location and benign pathology

I report here a case of primary papillary meningioma of the optic nerve sheath which developed in the left eye of a 54-year-old woman over a period of 20 years. A review of 123 primary papillary meningiomas, including the current instance, disclosed that they tended to occur in young patients of both sexes at an average age of 35 years, with one-fourth of them occurring in the first two decades of life, and with a female/male ratio of 3:2. These meningiomas were most frequent in the supratentorial compartment of the cranial cavity, especially on the cerebral convexity and in the parasagittal region. They were uncommon subtentorially, intraspinally, and outside the central nervous system. Most reported papillary meningiomas were claimed to be malignant, but the tumor of this particular woman was benign pathologically and clinically.     

Leber hereditary optic neuropathy. Electron microscopy and molecular genetic analysis of a case

BACKGROUND: Leber hereditary optic neuropathy (LHON) is a mitochondrial genetic disorder characterized by bilateral central visual loss typically in early adulthood. Few histopathologic studies, including ultrastructural and molecular genetic analysis, have been reported. METHODS: Ocular tissue was obtained postmortem from an 81-year-old woman with LHON from the Queensland 1 pedigree characterized by mutations at nucleotide positions 4160 and 14484. Routine histopathologic studies, electron microscopy, electron-probe analysis, and molecular genetic analysis were performed. RESULTS: Marked atrophy of the nerve fiber and retinal ganglion cell layers and optic nerves was present. Results of electron microscopic examination demonstrated 1.2 microns electron-dense, double-membrane-bound inclusions, consisting of calcium by electron-probe analysis, in retinal ganglion cells. The optic nerve was homoplasmic for mutations 4160 and 14484. CONCLUSION: Optic nerve and inner retinal atrophy in LHON may be a result of metabolic mitochondrial dysfunction leading to intramitochondrial calcification. Homoplasmy for mitochondrial mutations 4160 and 14484 in the leukocyte/platelet fraction of whole blood may correlate with homoplasmy in the optic nerve.     

Determination of blood cystatin C in pregnant women during labor and in their newborns

BACKGROUND: Monozygotic (MZ) or "identical" twins arise from a single fertilized egg, which divides into two embryos at an early stage of development. As a result, MZ twins have identical genomes and are always of the same sex. METHODS: A case of optic nerve hypoplasia and anisometropia, in association with mirror-image presentation in a set of 12-year-old identical twins, is reported. The monozygotic twinning event responsible for identical twins--as well as the rare phenomenon of mirror imaging--is described. RESULTS: The combined occurrence of anisometropia and optic nerve hypoplasia in mirror-image presentation in a set of monozygotic twins provides a unique opportunity to study the genetic versus environmental influences on the development of optic nerve hypoplasia. CONCLUSIONS: Although the cause of optic nerve hypoplasia remains unclear, its associated mirror-image presentation in this case suggests a possible genetic predisposition.     

Bilateral disc edema in retinitis pigmentosa

Retinitis pigmentosa (RP), one of the most common forms of hereditary retinal degeneration, is characterized by night blindness and constricted visual fields. In addition to bone spicule pigmentation, other ocular findings may include posterior subcapsular cataracts, cystoid macular edema, and hyaline bodies or drusen of the optic nerve. Rarely, optic nerve head (ONH) edema has been reported to be associated with RP. A 44-year-old white male with RP and neurosensory hearing loss (Usher's syndrome type II) presented to our clinic for routine examination. A dilated fundus examination revealed bone spicule pigmentation, vessel attenuation, several flame hemorrhages on or adjacent to the nerves, and ONH edema in the right eye. B-scan ultrasonography revealed drusen of the right ONH but not of the left. Late stage fluorescein angiography showed hyperfluorescence and dye leakage from both optic discs which was more pronounced in the right eye than the left. Computed tomography (CT) of the head and orbits and cerebrospinal fluid (CSF) examination by lumbar puncture were normal. The differential diagnosis of bilateral ONH edema in this case included ONH drusen or papilledema secondary to increased intracranial pressure. This patient was found to have RP with asymmetric, bilateral ONH edema of unknown cause. One theory regarding the cause of the ONH edema is disc vessel leakage secondary to an inflammatory reaction caused by rapid photoreceptor and retinal pigment epithelium (RPE) degeneration.     

Transantral-ethmoidal decompression of optic canal fracture

A case of indirect trauma to the optic nerve was successfully treated with transethmoidal decompression. The literature was reviewed, and reports of poor results from neurosurgical procedures are cited. Improved results from transethmoidal decompression of the optic nerve have been reported by Japanese authors but, in our opinion, without proper selection of cases. Early diagnosis of traumatic compression of the intracanalicular optic nerve as evidenced by delayed and/or progressive vision loss following injury, coupled with prompt transantral-ethmoidal surgical decompression, should yield gratifying results in the treatment of this uncommon condition.     

Ectopic pregnancy after a laparoscopically-assisted vaginal hysterectomy

PURPOSE/METHODS: Rubinstein-Taybi syndrome is a constellation of clinical findings characterized by mental and motor retardation, broad thumbs and broad first toes, marked growth retardation, microcrania, typical facies, high-arched palate, and cryptorchidism in males. Ocular and adnexal abnormalities are quite common and include antimongoloid slant of the palpebral fissures, epicanthal folds, congenital obstruction of the lacrimal excretory system, ptosis, strabismus, and severe ametropia. Macrocornea, microophthalmos, colobomas of the iris and of the optic nerve head, congenital cataract, and optic nerve atrophy have also been described. Congenital glaucoma is a rare complication. We examined a patient with Rubinstein-Taybi syndrome with bilateral congenital glaucoma. RESULTS/CONCLUSIONS: Examination of this patient revealed bilateral antimongoloid slants of the palpebral fissures, and bilateral congenital glaucoma. Gonioscopic examination revealed the iris to be inserted flatly into the trabecular meshwork. This case emphasizes the importance of detailed, complete ocular examinations in patients with Rubinstein-Taybi syndrome, and also highlights the occurrence of ocular abnormalities rarely associated with this disease.     

Relationship among social and intrapersonal risk, alcohol expectancies, and alcohol usage among early adolescents

We evaluated 3 patients with biopsy-proven hypertrophic cranial pachymeningitis apparently unrelated to any systemic disease. Each patient had chronic headache, cranial neuropathy, an elevated ESR, and a mild CSF pleocytosis. Neuro-ophthalmic findings included bilateral sixth nerve palsies in two patients and the third had bilateral optic neuropathies. MR imaging revealed thickened dura that enhanced with Gd-DTPA administration. Histologic examination showed thickened, fibrotic dura with a sterile, chronic, nongranulomatous inflammation. The response to treatment was variable with corticosteroids, immunosuppressive drugs, or radiation. The distinctive MR appearance should help physicians recognize this rarely reported disease.     

Diabetic papillopathy: two case reports in individuals with adult onset diabetes mellitus

BACKGROUND: Diabetic papillopathy is a benign unilateral or bilateral optic neuropathy with transient optic disk edema and minimal reduction in visual function. The optic disk edema typically resolves in a few months with no resulting optic atrophy and minimal or no decrease in acuity. The exact etiology of the disk edema is unknown, but theories include retinal vascular leakage into and surrounding the optic nerve and disruption of axoplasmic flow resulting from microvascular disease of the optic nerve head vasculature. CASE REPORTS: Two adult patients receiving insulin for type II diabetes mellitus manifested bilateral disk edema and minimal visual dysfunction. Both patients showed funduscopic evidence of mild-to-moderate nonproliferative diabetic retinopathy O.D. and O.S., and one patient had clinically significant macular edema in both eyes. The diagnosis in both cases was diabetic papillopathy. Both patients had significant resolution of their disk edema in 3 to 6 months, with stable acuities and no signs of optic atrophy. CONCLUSIONS: Although diabetic papillopathy is a well-known clinical entity in patients with type I diabetes, the clinical profile can be expanded to include individuals with type II diabetes.     

CMA leads drive to improve physicians'' management skills

PURPOSE: To report bilateral aplasia of the optic nerves, chiasm, and tracts in an otherwise healthy infant. METHOD: Case report. RESULTS: Ophthalmologic examination disclosed bilateral microphthalmos and optic nerve aplasia. Physical, neurologic, and genetic evaluations were otherwise normal. Magnetic resonance imaging of the brain and orbits disclosed bilateral aplasia of the optic nerves, chiasm, and tracts. CONCLUSION: Bilateral aplasia of the optic nerves, chiasm, and tracts may occur in an otherwise healthy infant.     

A case of thoracic meningioma presenting paraplegia at 4.5 years after removal of a falx meningioma

A case of a thoracic meningioma presenting paraplegia 4.5 years after removal of a falx meningioma is reported. A 73-year-old woman, complaining of diplopia, was admitted to our department. Neurological examination revealed right abducens palsy. CT demonstrated a well-enhanced right frontal mass beneath the falx. The mass was totally removed under right frontal craniotomy. Its histology was transitional meningioma with rich fibroblasts. 4.5 years after craniotomy, she complained of progressing gait disturbance and nocturnal leg pain. Neurological examination revealed paraplegia, complete loss of leg sensation, loss of patellar and ankle reflex, bilateral positive Babinski reflex and urinary disturbance. Rectal function and anal reflex were preserved. Thoracic MRI demonstrated an intradural extramedullary mass which was well enhanced with Gd-DTPA at Th6-7. Under laminectomy, the mass was totally removed. Its histology was transitional meningioma with rich psammoma bodies and whirl formations. 4 months after removal, her palsy and sensory loss were almost completely recovered. We were able to find 15 cases of combined intracranial and spinal meningiomas in the literature. A young woman of neurofibromatosis suffered from tentorial, intraventricular and C1-2 meningiomas. Of 15 cases without neurofibromatosis including our case, 4 cases were of young boys and 11 cases were of women. Their initial symptoms originated from intracranial meningiomas in 8 cases. Multiple intracranial meningiomas were revealed in only 4 cases. In 9 cases, one case presented a combination of one intracranial meningioma and one spinal meningioma. Histology of intracranial meningioma was almost the same that of spinal meningioma in almost half of the 10 cases. These findings suggest the multi sentricity theory of multiple meningiomas originating in other neuroaxial compartments. Severe spinal dysfunction was recovered after removal in our case. Rectal function and anal reflex were preserved. These anorectal findings suggest that spinal dysfunction is either complete or incomplete. Motor evoked potentials are hopeful tools which can select reversible spinal motor dysfunctions.     

Acute bilateral amaurosis in sphenoid empyema caused by Streptococcus pneumoniae

PATIENT: A 14-year-old boy suffered from an acute bilateral blindness which occurred in 24-h time, accompanied by headache and raised temperature, with inconspicuous optic nerve head and fundus. After diagnosis of empyema with magnet resonance tomography (MRT) the sphenoid sinus was fenestrated and streptococcus pneumoniae isolated. Liquor and serology being inconspicuous, there was no evidence of leucaemic or autoimmune disease, intoxication or intracranial tumor. CLINICAL COURSE: The condition of the patient improved under systemic antibiotic therapy. The bilateral amaurosis remained and opticus atrophy developed. CONCLUSION: A bilateral amaurosis with descending opticus atrophy as a consequence of a sphenoiditis and spreading inflammation to the meninges and the optic nerve in the area of the chiasm is a rare event. The imaging technique of the MRT offers new opportunities for an early and more pointed diagnosis and therapy.     

Orbital compression syndrome in sickle cell disease

BACKGROUND: Orbital complications are an uncommonly reported finding in sickle cell disease. METHODS: The authors review the reported orbital manifestations of sickle cell disease and discuss a patient with hemoglobin sickle beta(0) thalassemia in whom rapidly progressive bilateral orbital compression developed. RESULTS: Computed tomography of the orbits in a patient with fever, headache, orbital swelling, and optic nerve dysfunction displayed bilateral superior subperiosteal cystic masses. Surgical exploration showed bilateral liquefied hematomas, which were evacuated. Recovery was complete 13 days after surgery. A mild recurrence 14 months later resolved with conservative treatment. The literature contains 11 reports of 16 young patients with sickle cell disease (15 sickle cell disease [Hb SS] and 1 hemoglobin sickle cell disease [Hb SC]) with rapidly developing findings ranging from frontal headache, fever, and eyelid edema to bilateral complete orbital compression syndrome. Including our patient, 60% had orbital hemorrhage on computed tomography. Ten of 12 patients tested were found to have orbital bone marrow infarctions. Sixteen of 17 patients had complete recovery; 13 were treated conservatively and 4 surgically. Only 2 of 17 had recurrence. CONCLUSIONS: Orbital complications in sickle cell disease are unusual manifestations in which a vaso-occlusive process in the marrow space around the orbit results in frontal headache, fever, eyelid edema, and often orbital compression syndrome. Subperiosteal hematomas are common and appear to result from bone marrow infarctions. Appropriate management requires a thorough evaluation to exclude other hemorrhagic, infectious or neoplastic processes, as well as vigilant ophthalmic monitoring. Supportive care is effective, unless optic nerve dysfunction or large hematomas are present, which would indicate that surgical evacuation is warranted to prevent loss of vision and to speed recovery.     

Traumatic bilateral hypoglossal nerve palsy

A case of bilateral hypoglossal nerve palsy following a road traffic accident is reported. The possible aetiology is discussed.     

Bilateral traumatic hip dislocation with bilateral sciatic nerve injury

Bilateral traumatic dislocation of the hip is a rare injury. In fact, only about 50 cases have been reported previously. We report a case of traumatic bilateral posterior hip dislocation with bilateral sciatic nerve damage suffered in a traffic accident by a 65-year-old woman.     

Diffuse cerebral artery vasospasm following total resection of posterior fossa meningioma: a case report]

Diffuse cerebral artery vasospasm following brain tumor resection is a rare complication. The authors reported a case of symptomatic diffuse cerebral artery vasospasm of early phase following resection of a left posterior fossa meningioma. A 50-year-old female patient was admitted to our hospital complaining of headache. No neurological deficits were detected at the time of admission. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large mass in the left posterior fossa. Cerebral angiography demonstrated mildly diffuse stenosis of the bilateral internal carotid artery. The tumor was resected totally. CT after operation showed a small amount of subarachnoid hematoma in the superior aspect of the cerebellum. Pathological specimen of the tumor showed fibrous meningioma. One day after this radical operation, the patient was found to have weakness in her left leg. Then she developed left hemiparesis, weakness in the right leg and left homonymous hemianopsia. MRI showed ischemic lesions in the bilateral parietal and the occipital lobe. Angiography demonstrated diffuse severe vasospasm throughout the whole cerebral artery. Ten days after the operation, angiographical findings were improved. This case indicates that vasospasm may occur even after resection of brain tumors which are localized outside the suprasellar area.