Optic neuropathy and chiasmopathy in the diagnosis of systemic lupus erythematosus.

PURPOSE: To report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE). METHODS: Retrospective case series. RESULTS: All patients had a positive antinuclear antibody and elevated anti-double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications. CONCLUSIONS: Visual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.

Neuroma as a cause of oculomotor palsy in infancy and early childhood.

INTRODUCTION: Third nerve palsy in infancy and early childhood is rare. We present 5 children with isolated monocular oculomotor palsy originally believed to be idiopathic but subsequently documented to be secondary to a presumed neuroma of the third nerve, distal to the mesencephalon. METHODS: Retrospective case review of patient charts from 1995 through 1999 was performed. RESULTS: Mean age at initial presentation was 14.4 months (range, 2-36 months). Two patients were male. Initial manifestation was an incomplete paresis in all 5 cases; 2 patients presented initially with exotropia, 2 with ptosis, and 3 with anisocoria. The pupil was ultimately involved in all cases. Progression to total paresis occurred in 4 of 5 patients over a mean of 13 months (range, 4-22 months). Findings of initial neuroimaging were believed to be normal in all cases but further thin-section gadolinium-enhanced magnetic resonance imaging studies, including coronal views, revealed enlargement and/or enhancement of the subarachnoid or intracavernous portion of the oculomotor nerve. Aberrant regeneration did not develop in any case throughout follow-up (mean, 49.4 months). Visual acuity was decreased on the ipsilateral side in all cases, consistent with amblyopia. No patient had neurofibromatosis. CONCLUSIONS: Suspicion for neuroma should be high in infants and young children with acquired, isolated, apparently idiopathic third nerve palsy. Serial gadolinium-enhanced, 1- to 2-mm thin-section magnetic resonance imaging with coronal views is essential for proper diagnosis.

Pseudotumor cerebri and optic nerve sheath decompression.

OBJECTIVE: To determine the efficacy and safety of optic nerve sheath decompression in a large population of patients with pseudotumor cerebri with visual loss despite medical treatment and to suggest a treatment algorithm on the basis of these data. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: One hundred fifty-eight eyes in 86 patients with pseudotumor cerebri. INTERVENTION: Optic nerve sheath decompression. MAIN OUTCOME MEASURES: Visual acuity, visual fields, and surgical complications. RESULTS: After optic nerve sheath decompression for pseudotumor cerebri, visual acuity stabilized or improved in 148 of 158 (94%) eyes, and visual fields stabilized or improved in 71 of 81 (88%) eyes. Surgical complications, most of which were transient and benign, were seen in 39 of 86 patients. Only one eye in one patient had permanent severe visual loss secondary to an operative complication. CONCLUSIONS: In patients with pseudotumor cerebri with progressive visual loss despite maximum medical therapy, optic nerve sheath decompression is a safe and effective means of stabilizing visual acuity and the visual fields of those tested.

"Mickey Mouse" aneurysm presenting with cranial bruit: case report.

The production of a bruit associated with intracranial aneurysms is very rare. A patient with a bilobed aneurysm of the middle cerebral artery who was otherwise asymptomatic was admitted with both a subjective and an objective bruit. The bruit resolved with elective clipping of the aneurysm. After a thorough review of the literature, a total of 12 cases of proved intracranial aneurysms were found with associated bruit. Five of those were carotid artery aneurysms within the cavernous sinus. Most (67%) of those patients reviewed were admitted with hemorrhage that was present either into the cavernous sinus or in the form of subarachnoid hemorrhage, with the bruit only secondarily noted. A possible explanation for the production of bruits caused by intracranial aneurysms is discussed. We conclude that, although an audible bruit as the presenting symptom of an intracranial aneurysm is quite rare, it deserves serious surgical considerations.

Neuro-ophthalmological findings in closed head trauma.

Visual abnormalities following closed head trauma are common and can affect any part of the visual pathway. We reviewed 181 consecutive patients referred with visual complaints following closed head trauma. A motor vehicle accident was the most common etiology of trauma in 57% of cases, direct trauma to the head in 15%, and injuries sustained from a fall in 13%. Sixty-three percent of patients lost consciousness and 26% suffered a skull fracture. Thirty-five percent of all patients had visual field defects with functional (tunnel) fields the most common. Over 88% of eyes had 20/20 or better visual acuity. Thirty-three percent of patients suffered a cranial nerve palsy, with 75% resolving without intervention. The severity of head injury was directly related to the lack of proper seat belt and helmet use. Most visual complaints were improved with a simple refraction. Most binocular diplopia cleared with time, with only 15 cases requiring surgical correction.

Acetazolamide in the treatment of abnormal oculovestibular response.

We treated seven patients with incapacitating vertigo elicited by walking down a grocery store aisle or driving a car. Results of neurologic, neuro-ophthalmic, and neuroradiologic examinations were normal. Episodic vertigo secondary to an abnormal oculovestibular response was diagnosed. Each patient was given a trial of 250 to 500 mg of acetazolamide daily. Symptoms resolved completely in four patients, two patients had near resolution of symptoms, and one patient had no relief. Carbonic anhydrase activity has been demonstrated in the inner ear, and acetazolamide has been shown to affect the ion balance of the inner ear fluids.

Pupil-sparing oculomotor nerve palsy due to midbrain infarction.

Vasculopathic oculomotor nerve palsies with pupillary sparing are thought to be due to ischemic damage to the nerve in the subarachnoid space or the cavernous sinus. We present two cases of patients with isolated pupil-sparing oculomotor nerve palsies due to midbrain infarcts. Focal ischemic midbrain lesions should be considered in cases of pupil-sparing oculomotor nerve palsies.

Intracanalicular lipoma.

We present a patient with unilateral progressive painless loss of vision leading to optic atrophy and blindness. High-resolution computed tomography and magnetic resonance imaging demonstrated adipose tissue extending from the anterior portion of an enlarged optic canal posteriorly to involve the ipsilateral optic chiasm. To our knowledge, this is the first reported case of an intracanalicular lipoma.

Ophthalmic complications of sinus surgery.

Seven patients with orbital complications of sinus surgery seen over a 10-year period are reported. Severe intraoperative orbital hemorrhage occurred in three patients while undergoing external or intranasal ethmoidectomy. In one patient, an avulsed anterior ethmoidal artery was identified as the source of bleeding, whereas in two others bleeding was due to snaring of orbital tissues. In two of these three cases, intraoperative recognition and prompt treatment of the expanding hematoma resulted in preservation of vision. Motility disturbances due to extraocular muscle injury occurred in two patients after intranasal ethmoidectomy and in one patient after a Caldwell-Luc procedure. Bilateral blindness resulted from transection of both optic nerves in one patient during bilateral intranasal endoscopic ethmoidectomies. Recommendations for prevention, intraoperative recognition, and management of such ophthalmic complications of sinus surgery are given.

Bilateral postinfectious optic neuritis and intravenous steroid therapy in children.

Six patients presented with acute, simultaneous, bilateral optic neuritis. Five of the six patients admitted to a recent history of a brief upper respiratory or gastrointestinal illness, presumably viral in nature. Visual acuity was severely reduced in all patients. Five of the six patients also demonstrated marked neurologic deficits, including seizure activity and cerebellar dysfunction. Three patients demonstrated enhancing intracranial lesions on magnetic resonance imaging (MRI) consistent with demyelinative plaques, whereas lumbar puncture was abnormal in three patients. HLA tissue typing was performed on five of the six patients. All patients were treated with intravenous methylprednisolone, followed by a 2-month tapering course of oral prednisone. Each patient experienced a rapid and nearly complete recovery of vision during treatment.

Primary chiasmal germinoma. A case report and review of the literature.

A case is presented in which an adult man with painless progressive loss of vision subsequently was found to have a primary suprasellar/perichiasmal germinoma (ectopic pinealoma). A review of the literature revealed 93 similar cases of germinoma occurring in the perichiasmal region and these are tabulated. The diagnosis and management of this lesion are discussed, including the recognition of the characteristic neuroendocrinologic triad of diabetes insipidus, visual changes, and hypopituitarism. Of 64 cases from the literature wherein presenting symptoms were reported, 56 (87.5%) had diabetes insipidus, 53 (82.8%) visual changes, and 36 (56%) hypopituitarism. A review of the literature suggests that diabetes insipidus is usually the initial symptom in suprasellar germinoma. However, we wish to emphasize the ophthalmologic presentation of this entity, because of ophthalmologic presentation of this entity, because patients in the age group most affected (adolescents) will often not recognize symptoms of diabetes insipidus, but will first seek medical attention for painless progressive loss of vision suggestive of chiasmal compression. The radiosensitivity of this lesion is also discussed. Of 61 patients receiving irradiation therapy, 42 (68%) were surviving at the time of their individual case report. No patient in the review survived without irradiation therapy.

High-signal periventricular lesions in patients with sarcoidosis: neurosarcoidosis or multiple sclerosis?

The vast majority of periventricular abnormalities visualized with MR imaging in patients less than 50 years old represents multiple sclerosis (MS) lesions. There are many other causes of periventricular lesions, most of which can be differentiated from MS on the basis of history and physical or MR findings. Five cases of biopsy- or Kveim test-proved sarcoidosis with MR findings consistent with MS are reported. Each of these patients, diagnosed as having sarcoidosis, had symptoms identical to those seen in MS. Although these patients have not had histologic characterization of the intraparenchymal lesions seen on MR, they illustrate the difficulty of differentiating sarcoidosis with CNS involvement from MS in some patients on the basis of clinical, radiographic, electrodiagnostic, or CSF testing. This series contributes to a growing body of evidence that neurosarcoidosis probably should be included in the differential diagnosis of isolated periventricular lesions in patients less than 50 years old.

Acidophil stem-cell pituitary adenoma in a prepubescent female. Case report.

Acidophil stem-cell pituitary adenomas account for less than 5% of pituitary tumors. Only 15 cases have previously been reported, with a mean age of occurrence of 38.7 years. A case of this unusual tumor is reported in a prepubertal girl. Clinical symptoms included prominent behavioral disturbance with associated headache and visual disturbance. There was marked elevation of serum growth hormone concentration without clinical features of growth hormone excess, suggesting that this tumor has the capacity to excrete biologically inactive hormones. The clinical and pathological features of this unusual invasive pituitary tumor are reviewed; the age spectrum for this neoplasm must be expanded to include prepubertal children.

Neuro-ophthalmologic findings in vestibulocerebellar ataxia.

A young adult who presents with periodic vertigo, diplopia, and tinnitus, later followed by progressive ataxia, may not have multiple sclerosis as might initially be suspected, but rather may represent a type of familial spinocerebellar degeneration previously described by Farmer and Mustian as "vestibulocerebellar ataxia." We recently encountered a patient who presented with these symptoms, and who was found to have downbeat nystagmus, ocular dysmetria, skew deviation, optokinetic dissociation, and a vertical gain bias on horizontal eye movements. Although family involvement was specifically denied by the patient, seven members of her family representing four generations were examined, and found to have similar findings. The importance of a careful neuro-ophthalmologic examination in each available family member of any patient presenting with this constellation of symptoms is emphasized. A discussion of the spinocerebellar degenerations, differential diagnosis, and literature review are included.

The nasal junction scotoma in giant aneurysms.

Thirteen giant aneurysms in ten patients were retrospectively reviewed. Careful visual field testing of both peripheral and central fields revealed an ipsilateral nasal scotoma in 7 of the 13 aneurysms (54%), or six of the ten patients. A review of 47 giant carotid aneurysms reported in the literature revealed 16 ipsilateral nasal scotomas (34%). The more common intraocular causes of ipsilateral nasal field defects were excluded by careful funduscopic examination. In the absence of intraocular pathologic characteristics, an ipsilateral nasal junction scotoma should alert the clinician to suspect the presence of an otherwise silent internal carotid artery aneurysm.