Clinical review: the surgical treatment of idiopathic pseudotumour cerebri (idiopathic intracranial hypertension).

To review the literature on the surgical treatment of idiopathic pseudotumour cerebri (PTC) [idiopathic intracranial hypertension (IIH)]. When medical therapy fails or when visual dysfunction deteriorates, surgical therapies for PTC should be considered. The main procedures performed include lumboperitoneal shunt (LPS), ventriculoperitoneal shunt (VPS) and optic nerve sheath fenestration (ONSF). Recently, venous sinus stenting procedures have been performed on selected patients with PTC, especially those with venous sinus occlusive disease. The literature is summarized and appraised in the form of a narrative review. It is evident that ONSF, LPS, VPS and, in selected cases, venous sinus stenting may improve vision and prevent deterioration of vision in patients with PTC. All of the procedures have their advantages and disadvantages and may fail with time no matter what procedure is used. Various authorities have vehemently advocated one or the other of these procedures. Until a prospective, randomized study comparing ONSF with LPS or VPS for PTC is performed, and until the role of venous sinus obstruction as the aetiology of PTC is better defined, the question of which surgical procedure is best for the treatment of PTC remains unanswered.

Cerebellar degeneration with Hodgkin's disease: computed tomographic correlation and literature review.

A 62-year-old man with progressive cerebellar degeneration, corticospinal tract dysfunction, and mild dementia was found to have Hodgkin's disease. Serial computed tomographic studies revealed progressive cerebellar atrophy. The clinical features and neuroradiologic studies in nine previous cases are reviewed. A thorough search for lymphadenopathy should be made in patients with unexplained cerebellar degeneration.

The "numb cheek-limp lower lid" syndrome.

A patient developed isolated numbness, 1st confined to the lateral nose and upper lip, but later involving the cheek, lower lip, upper gingiva, and the palate. This numbness was later associated with paresis of the muscles of the upper lip and angle of the mouth and with ipsilateral lower lid droop (the "numb cheek-limp lower lid" syndrome). Squamous cell carcinoma was discovered infiltrating the infraorbital nerve and distal branches of the facial nerve. Cheek numbness associated with lower eyelid or upper lip weakness may herald a neoplasm affecting the infraorbital nerve and distal facial nerve branches.

Localization of lesions of the oculomotor nerve: recent concepts.

The anatomic features of the third cranial nerve (the oculomotor nerve) and the localization of lesions that affect it are reviewed. Recent considerations of the organization of the oculomotor fascicles in the ventral mesencephalon, the superior and inferior divisional palsies localized proximal to the cavernous sinus, and the possibility of the localization of ischemic oculomotor palsies to the mesencephalon rather than a peripheral site are emphasized. The characteristic manifestations of nuclear lesions (unilateral palsy of the third cranial nerve, weakness of the ipsilateral and contralateral superior rectus muscles, and bilateral incomplete ptosis) are described, as are other variations of nuclear involvement. Although proptosis is typically associated with orbital masses, it may also result from lesions of the cavernous sinus or (rarely) an intracranial lesion. Metastatic orbital tumors often are the first evidence of systemic spread in patients with cancer; infiltrative and mass lesions are the most common. Aberrant regeneration of the oculomotor nerve may occur months to years after the occurrence of an oculomotor lesion.

Ocular motor abnormalities in Wallenberg's lateral medullary syndrome.

The ocular motor abnormalities that commonly occur in Wallenberg's lateral medullary syndrome are often unappreciated. These abnormalities include signs of dysfunction of ocular alignment (skew deviation, ocular tilt reaction, and environmental tilt), various types of nystagmus, smooth pursuit and gaze-holding abnormalities (eye deviation, ipsipulsion or lateropulsion, and impaired contralateral pursuit), and saccadic abnormalities (ipsipulsion and torsipulsion). These impairments of ocular motor control and their proposed mechanisms are discussed.

Palsies of the trochlear nerve: diagnosis and localization--recent concepts.

In this review, the anatomy of the trochlear nerve, the diagnosis of palsies of the trochlear nerve, and the localization of lesions of the trochlear nerve are discussed. Paresis of the superior oblique muscle is often not evident on duction testing; therefore, subjective diplopia testing with use of a Maddox rod is often necessary. The torsional component of the deviation may be evaluated by double Maddox rod testing. Palsies of the trochlear nerve must be distinguished from other causes of vertical diplopia, such as oculomotor palsy, skew deviation, myasthenia gravis, and Graves' ophthalmopathy. Trauma is the most common cause of isolated, unilateral or bilateral, acquired palsies of the trochlear nerve when a cause can be determined. The localization of lesions of the trochlear nerve to the nucleus or fascicles (or both), subarachnoid space, cavernous sinus and superior orbital fissure, or orbit depends on the associated damage to neighboring neurologic structures. Myokymia of the superior oblique muscle is usually idiopathic and benign but may rarely be an isolated manifestation of tectal disease.

Optic disk edema with a macular star.

Optic disk edema with a macular star is a descriptive term encompassing a heterogeneous group of disorders. The clinical features include sudden visual loss, swelling of the optic disk, peripapillary and macular exudates that may occur in a star pattern, and cells in the vitreous. Herein we describe the clinical features, potential etiologic factors, differential diagnosis, work-up, and natural history of this entity. Although optic disk edema with a macular star is usually idiopathic, infectious causes, especially syphilis, Lyme disease, cat-scratch disease, and toxoplasmosis, should be considered. The macular exudate likely results from primary optic nerve disease, not from inflammation of the retina; therefore, we prefer the term "idiopathic optic disk edema with a macular star" for idiopathic cases rather than "neuroretinitis." When optic disk swelling and macular star are associated with focal or multifocal inflammatory lesions in the retina (retinitis), especially if an infectious cause is documented, the term "neuroretinitis" is appropriate. The prognosis for visual recovery is usually good, but residual visual loss may be severe in a few cases. Patients with a recurrent type of the disease may not experience pronounced improvement in optic nerve function. The macular exudate may not develop in cases of disk edema until 2 weeks after the patient's initial assessment; thus, patients who have acute papillitis with a normal macula should be reexamined within 2 weeks for development of a macular star. The presence of a macular star militates strongly against subsequent development of multiple sclerosis.

Acquired binocular horizontal diplopia.

Binocular horizontal diplopia is an important symptom that may foretell or help localize and characterize various neurologic and neuromuscular disorders. An appropriate evaluation requires a careful and complete neuro-ophthalmic history and examination. This review focuses on the differential diagnosis of binocular horizontal diplopia.

Binocular vertical diplopia.

The assessment of a patient with binocular vertical diplopia begins with a thorough history and neuro-ophthalmologic examination. The neuro-ophthalmologic examination includes observation for a compensatory head, face, or chin position; ocular ductions and versions in the nine cardinal positions of gaze; the three-step test; the double Maddox rod test; indirect ophthalmoscopy to observe the location of the fovea in relationship to the optic nerve head to determine cyclodeviation; and the forced ductions test. Binocular vertical diplopia may be due to supranuclear processes, ocular motor nerve dysfunction, neuromuscular junction disease, diseases of eye muscle, mechanical processes causing vertical eye misalignment, and even retinal disease. In this article, the differential diagnosis of these processes is outlined.

Detection of migratory silicone pseudotumor with use of magnetic resonance imaging.

A patient with silicone gel breast implants, who experienced capsular contracture of the left breast that was treated by closed capsulotomy, sought medical attention because of numbness and pain in the left medial forearm and hand. Inflammatory masses subsequently developed in the left anterior axillary and antecubital regions. Magnetic resonance imaging revealed silicone pseudotumors, and mammography confirmed implant rupture and gel extrusion along fascial planes into the axillary region.

Simultanagnosia as the initial sign of degenerative dementia.

In a study of 10 patients with degenerative brain disease that manifested as simultanagnosia, our aims were (1) to elucidate their clinical, neuropsychologic, and radiologic findings to determine whether these patients might represent a group distinguishable from those with typical Alzheimer's disease and (2) to help clinicians recognize this entity. All patients were initially examined by ophthalmologists because of visual difficulties, and the simultanagnosia remained undiagnosed until nonophthalmologic complaints developed. Optic ataxia developed in six patients, and all patients had mildly impaired eye movements. All 10 patients could identify colors appropriately. Nine patients had language deficits (anomia, decreased auditory comprehension, alexia, and agraphia) but were fluent and had relative preservation of sentence repetition, and four performed in the normal range on a test of associative fluency. Two patients scored in the normal range on memory tests, all had preserved insight, and nine had no family history of degenerative dementia. The mean age at onset of the disorder was 60 years (range, 50 to 69). Neuroimaging disclosed prominent bilateral occipitoparietal atrophy in nine patients and generalized atrophy in one. With this unusual but consistent clinical, neuropsychologic, and anatomic profile, these patients are clinically distinguishable from those with typical Alzheimer's disease, but until a specific cause has been found, we cannot be certain that they constitute a specific biologic entity. Clinicians should consider this diagnosis in relatively young patients who have slowly progressive nonocular visual complaints.

Tropicamide eyedrops cannot be used for reliable diagnosis of Alzheimer's disease.

OBJECTIVE: To evaluate the mydriatic effect of tropicamide eyedrops as a diagnostic test for Alzheimer's disease. MATERIAL AND METHODS: In a double-blind, placebo-controlled study, we assessed pupillary responses in 22 normal control subjects, 23 patients with probable Alzheimer's disease, 4 patients with isolated memory difficulty, and 6 patients with non-Alzheimer's dementia. Three separate studies were performed, the second and third on a subset of the original group. With use of infrared binocular pupillography, after 5 minutes of dark adaptation, we averaged pupil size during a 1-minute interval for baseline determinations. We then instilled 0.01% tropicamide into one eye. In the first two studies, we averaged pupil size for a 1-minute period at 5-minute intervals for 30 minutes, followed by a pupil light reflex test. In the third study, we measured pupil size every 5 minutes for 45 minutes and omitted the light reflex test. RESULTS: No significant difference was noted in pupil dilatation between normal subjects and patients with Alzheimer's disease and between patients with non-Alzheimer's dementias and the Alzheimer's disease group in all three studies. Furthermore, on reperformance of the test in the same patients, more than 50% changed from a group above or below 13% pupil dilatation (a cutoff reported to distinguish Alzheimer's disease from normal control subjects) to the opposite group. CONCLUSION: Results of this study indicate that pupil measurement after instillation of tropicamide cannot be used as a reliable diagnostic test for Alzheimer's disease. Moreover, test-retest reliability with use of dilute tropicamide eyedrops is questionable.

Temporal arteritis: a clinical approach.

OBJECTIVE: To develop a clinical guide to the evaluation of temporal arteritis. DESIGN: A Medline English-language search of the literature from 1966 to 1998, including more than 300 articles about temporal arteritis, was performed to develop a guide to the evaluation of temporal arteritis. RESULTS: A user-friendly guide to the evaluation of temporal arteritis was developed based on the following criteria: (1) clinical suspicion, (2) laboratory testing, and (3) temporal artery biopsy. CONCLUSION: A clinical guide to the evaluation of temporal arteritis may assist clinicians in the care of patients with this condition.

The natural history and results of treatment of superior oblique myokymia.

OBJECTIVE: To investigate the clinical presentations and long-term course of patients with superior oblique myokymia (SOM). METHODS: The medical records of all 16 patients with the diagnosis of SOM seen in the Neuro-Ophthalmology Unit of The Wilmer Ophthalmological Institute, The Johns Hopkins Hospital, Baltimore, Md, between 1976 and 1993 were reviewed. Follow-up information was obtained for 14 (88%) of the 16 patients. RESULTS: Of the 16 patients with SOM, nine (56%) were male and seven (44%) were female. The age of onset of symptoms ranged from 22 to 50 years (mean age, 34 years). All patients were otherwise healthy with no history of neurologic illness. Seven (44%) of the 16 patients complained of paroxysms of uniocular "shimmering," "fluttering," or oscillopsia lasting seconds, three (19%) complained of vertical and torsional diplopia, and six (38%) had both types of symptoms. Five (31%) of the 16 patients underwent neuroimaging studies within 1 year of onset of symptoms. All imaging study results were normal. Follow-up information was obtained for 14 patients (88%). The time from onset of symptoms to our most recent contact was 3 to 29 years. Of the seven patients who received no treatment, five (71%) continue to have symptoms to date. Three patients received medical treatment only; one of the three has experienced lasting benefit with carbamazepine. Four patients underwent superior oblique tenectomy combined with inferior oblique myectomy after not responding to medical treatment. All four patients experienced resolution of all ocular symptoms after surgery. CONCLUSIONS: Because SOM is a much more chronic disease than formerly realized and because of the poor long-term effects and potential side effects of the medications used, medical treatment of SOM is not the optimum way to manage the disease. Extraocular muscle surgery is the treatment of choice when symptoms of SOM are intolerable to the patient.

When do optic disc edema and peripheral neuropathy constitute poetry?

A patient with chronic, bilateral optic disc edema developed multiple systemic manifestations of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes). A serum immunoelectrophoresis showed an abnormal serum IgG lambda protein, and urine immunoelectrophoresis revealed a monoclonal lambda protein plus an IgG lambda fragment. Bone survey and MRI scan revealed a sclerotic lesion of the first lumbar vertebra, and lymph node biopsy showed changes consistent with Castleman's disease. Thus, his optic disc edema was the presenting feature of the POEMS syndrome and osteosclerotic myeloma.

Optic disk swelling with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome).

We reviewed charts of patients seen at Mayo Clinic Rochester and Mayo Clinic Jacksonville to determine the importance and frequency of optic disk swelling in patients with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). A computer search identified 148 patients who were classified as having a monoclonal gammopathy and peripheral neuropathy. A review of the 148 charts disclosed that 11 patients had POEMS syndrome. Eight of these 11 patients had optic disk swelling identified by an ophthalmologist. In all eight patients, optic disk swelling was either the initial sign or was present the first time we saw the patient. Optic disk swelling may be an early sign in POEMS syndrome.

A case of bilateral optic neuropathy in a patient on tacrolimus (FK506) therapy after liver transplantation.

PURPOSE: To report a case of bilateral optic neuropathy in a patient receiving tacrolimus (FK 506, Prograf; Fujisawa USA, Inc, Deerfield, Illinois) for immunosuppression after orthotropic liver transplantation. METHOD: Case report. In a 58-year-old man receiving tacrolimus after orthotropic liver transplantation, serial neuro-ophthalmologic examinations and laboratory studies were performed. RESULTS: The patient had episodic deterioration of vision in both eyes, with clinical features resembling ischemic optic neuropathies. Deterioration of vision occurred despite discontinuation of the tacrolimus. CONCLUSION: Tacrolimus and other immunosuppressive agents may be associated with optic nerve toxicity.

Ishihara color plates as a test for simultanagnosia.

PURPOSE: To alert ophthalmologists to the use of Ishihara color plates in the detection of simultanagnosia. METHODS: We examined seven patients referred for impaired vision. Evaluation included color plate testing with Ishihara color plates. RESULTS: All seven patients had simultanagnosia, with marked difficulty in identifying the numbers in Ishihara color plates despite adequate visual acuity and the ability to name all of the colors in the plates correctly. One of these patients was referred with the diagnosis of a cone dystrophy because of her poor performance on the Ishihara test. All of the patients had bilateral occipitoparietal damage or atrophy on magnetic resonance imaging. CONCLUSIONS: Ophthalmologists must be aware that a poor performance with Ishihara plates may not be attributable to an impairment of color vision but rather to occipitoparietal brain damage associated with simultanagnosia.

Recurrent idiopathic familial facial nerve palsy and ophthalmoplegia.

INTRODUCTION: Idiopathic facial nerve palsy is a common neurologic condition but episodes of ophthalmoplegia in these patients are uncommon. MATERIALS AND METHODS: Retrospective case series. RESULTS: Three patients with facial nerve palsy with unusual features, including ophthalmoplegia, are described. CONCLUSION: Recurrent episodes, familial tendency, and associated ophthalmoplegia are uncommon in patients with idiopathic facial nerve palsy.

An imaging guide to the evaluation of third cranial nerve palsies.

PURPOSE: To devise a cost-effective imaging guide for the evaluation of third nerve palsies (TNP), based on an extensive review of the literature. METHODS: A review of the pertinent English language literature was performed to devise a guideline for the evaluation and neuroimaging of TNP. The authors also report a retrospective review of the cost of imaging studies performed on 91 patients with TNP. RESULTS: On the basis of the available literature, an imaging guide was developed and applied to a retrospective chart review of 91 patients from a single tertiary care center (Baylor College of Medicine). The cost effectiveness as well as the efficacy of the imaging guide was analyzed. CONCLUSIONS: The evaluation of TNP can be difficult; however, using such guides may allow for more appropriate and cost-effective evaluation of these patients.