Fourth nerve palsy in pseudotumor cerebri

A 13-year-old white female with a past medical history of facial acne treated with minocycline presented with headaches, normal visual acuity, bilaterally enlarged blind spots on visual field testing due to bilateral disc edema, and a right fourth nerve palsy. Neuroimaging studies were normal and a lumbar puncture revealed an elevated opening pressure but normal cerebrospinal fluid contents. The diagnosis of pseudotumor cerebri was made and the patient was treated with acetazolamide. The papilledema improved over several weeks and the fourth nerve palsy resolved completely. Fourth nerve palsy is an uncommonly reported nonlocalizing sign in pseudotumor cerebri.     

Fourth cranial nerve palsy following spinal anesthesia. A case report

Fourth nerve palsy has been rarely seen following lumbar puncture, myelogram, or spinal anesthesia. We report a case of 4th nerve and 6th nerve palsies following spinal anesthesia. The 4th nerve palsy was best detected by using a Maddox rod. If all 6th nerve palsies occurring after spinal anesthesia were examined with a Maddox rod, more cyclovertical palsies might be discovered.     

Bilateral congenital third cranial nerve palsy

We describe a patient in whom bilateral congenital third cranial nerve palsy was diagnosed at 2 weeks of age. The ptosis was treated at 3 weeks with bilateral eyelid suspension surgery, and surgical repair of the exotropia was done at 4 months. To our knowledge this is the fourth reported case of this condition. It was probably caused by a single lesion involving the oculomotor and possibly the trochlear nuclei in the brain stem. Magnetic resonance imaging scans supported this hypothesis and suggested other central nervous system anomalies.     

Third cranial nerve palsy in children.

PURPOSE: To report the causes and the sensory, motor, and cosmetic results after treatment for oculomotor (third cranial nerve) palsy in children. METHODS: Review of the clinical records of children with a diagnosis of third cranial nerve palsy followed up in a university-based pediatric ophthalmology practice between 1981 and 1996. RESULTS: Forty-nine children with 53 affected eyes were followed up for a mean of 5.5 years. Third cranial nerve palsy was partial in 31 children (32 eyes) and complete in 18 children (21 eyes). The palsy was congenital in 20 eyes and caused by postnatal trauma in 17 eyes. Seventeen eyes had aberrant regeneration and four eyes with partial third cranial nerve palsy had spontaneous resolution. Thirty-six children (38 eyes) were affected before visual maturation (age 8 years), and 25 (27 eyes) had amblyopia. Of the five amblyopic eyes with quantifiable visual acuity, none had measurable improvement of Snellen visual acuity during the follow-up period. Overall, visual acuity was between 6/5 and 6/12 at the last follow-up visit in 31 eyes (58%). Ocular alignment was greatly improved after strabismus procedures, with a mean of 1.5 procedures for patients with partial third cranial nerve palsy and 2.3 procedures for those with complete palsy. Binocular function was difficult to preserve or restore but was achieved for some patients with partial third cranial nerve palsy. CONCLUSIONS: Surgical treatment of third cranial nerve palsy is frequently necessary, especially in cases of complete palsy. Multiple strabismus procedures are often needed to maintain good ocular alignment. Surgery can result in cosmetically acceptable alignment of the eyes, but it rarely results in restoration or achievement of measurable binocular function. Treatment of amblyopia is effective in maintaining the level of visual acuity present at the onset of the third cranial nerve palsy, but improvement in visual acuity is difficult to achieve.     

Optical coherence tomography as a tool for monitoring pediatric pseudotumor cerebri

PURPOSE: To evaluate optical coherence tomography (OCT) measurement of peripapillary retinal nerve fiber layer (RNFL) and macular thickness in children with pseudotumor cerebri. METHODS: Prospective observational series of children with pseudotumor cerebri compared against controls matched for age and gender. We included 11 pediatric subjects with clinical pseudotumor cerebri and 37 normal controls. Subjects underwent a complete eye examination including stereo disk photographs and OCT. Peripapillary RNFL and macular thickness were evaluated using the Stratus OCT 3000. The peripapillary RNFL was evaluated with two protocols: Fast RNFL Map protocol with values divided in eight sections, made up of four quadrants, each with an inner and outer ring, and Fast RNFL Thickness (3.4) protocol, using values presented for four quadrants. A Fast Macular Thickness Map was also obtained, and results reported similarly included eight sections, made up of four quadrants, each with an inner and outer ring. RESULTS: The Fast RNFL Map protocol showed an increased RNFL thickness (pseudotumor cerebri vs controls) in the temporal and superior quadrants. The Fast RNFL Thickness (3.4) protocol showed a thicker average RNFL for pseudotumor cerebri eyes compared with controls (125.7 vs 106.5 microm, p < 0.0001). The Fast Macular Thickness Map showed an increased RNFL thickness in the nasal quadrant for the inner ring. The macular volumes for pseudotumor cerebri versus control eyes were 7.21 versus 6.97 mm(3), respectively (p = 0.005). CONCLUSIONS: OCT shows increased RNFL and macular thickness in pseudotumor cerebri and may be a useful clinical tool in these children.     

Evaluation of optic nerve sheath fenestration in pseudotumor cerebri using automated perimetry

Evaluation of the efficacy of optic nerve sheath fenestration in pseudotumor cerebri has been hindered by a lack of quantitative indicators of visual function before and after treatment. Nine patients (14 eyes) were treated who had pseudotumor with optic nerve sheath fenestration, in whom automated perimetry was used as a major parameter in the decision to undertake surgery and in evaluating the results. Visual field results demonstrated significant improvement in this group of patients. Seven of 8 eyes with total loss less than 1500 dB had improvement or no further deterioration. Using results of the last preoperative examination as baseline, it was found that six of the eight had improvement over 150 dB. In one patient, a slight deterioration occurred postoperatively. Those eyes with total loss above 1500 dB demonstrated stabilization of fields postoperatively. Statistical analysis of total loss preoperatively and postoperatively for this group of patients demonstrated significant improvement for five of the six comparisons investigated. Average preoperative total loss and average postoperative total loss were not significantly different. Comparisons of results of the last preoperative examination to those of the first postoperative and most recent follow-up examinations, and evaluation of average total loss postoperatively showed significant improvement had occurred. Results of the first postoperative examination and those of the most recent follow-up examination showed significant improvement from the average preoperative total loss.     

Biopsy-negative cranial arteritis with complete oculomotor nerve palsy.

Cranial arteritis (CA) presenting as sudden blindness is well documented, and early recognition of this entity in an elderly patient with visual loss or diplopia is of critical importance. This entity presents a difficult diagnostic problem when temporal artery biopsy is negative, as in our case, or when the erythrocyte sedimentation rate is normal. The present report details an unusual patient with "occult temporal arteritis" who sustained abrupt monocular visual loss and subsequent ipsilateral ophthalmoplegia involving all functions of the oculomotor nerve. Despite negative biopsies of each temporal artery, other probable etiologies of the symptom complex were excluded, and the diagnosis of cranial arteritis is warranted. The patient is unique in that the oculomotor palsy is complete and permanent. This report emphasizes cranial arteritis masquerading as an intracranial aneurysm.     

Plasma renin activity and aldosterone determination in patients with pseudotumor cerebri.

A group of 6 women diagnosed as suffering from pseudotumor cerebri had measurement of their plasma renin activity and aldosterone levels at baseline, following plasma volume contraction by furosemide and after dexamethasone therapy. In each instance, the values recorded fell within the normal range and a normal renin-aldosterone axis is postulated.     

Multiple cranial nerve palsy in an HIV-positive patient

We report the case of a 32 -year- old HIV-positive Indian male who presented with sixth nerve (bilateral), ninth, tenth and twelfth nerve palsies; cerebellar and posterior column involvement. CT scan showed gyriform enhancement in the right occipital lobe and nodular leptomeningeal enhancement in the left frontal lobe. Cytomegalovirus serology was positive and the patient was treated as presumed CMV. HIV can present with multiple cranial neuropathy and varied neurological involvement.     

Contrast sensitivity testing in pseudotumor cerebri

Twelve patients with pseudotumor cerebri underwent sensory visual testing and the results were analyzed. Contrast sensitivity loss was detected with use of Arden gratings in nine of the twelve patients (75%) and in 13 of 24 eyes (54%). Snellen acuity was worse than 20/20 in 21% of eyes. Contrast sensitivity scores improved with resolution of the patient's papilledema whereas there was no significant improvement of Snellen acuity. Contrast sensitivity testing with Arden plates is a useful procedure for detecting visual loss and serially following patients with pseudotumor cerebri.     

Quantitative evaluation of optic disc pallor in pseudotumor cerebri patients

We quantified optic disc pallor in patients with pseudotumor cerebri with the Rodenstock Optic Nerve Head Analyzer before and after optic nerve sheath decompression. Mean frequency distribution of pallor peaked between the pallor values of 0.10 and 0.20 in four pseudotumor cerebri patients and between 0.50 and 0.60 in five normal subjects. The red dominant pallor reflectance in pseudotumor cerebri patients decreased significantly and shifted toward the reflectance of normal subjects gradually during the 9 weeks after successful optic nerve sheath decompression, coinciding with the ophthalmoscopic resolution of papilledema. The computerized pallor measurement with the Rodenstock Optic Nerve Head Analyzer allows an objective and quantitative assessment of papilledema in pseudotumor cerebri patients. Offprint requests to: D.H. ShinThe authors have no commercial interest in the Rodenstock Optic Nerve Head Analyzer     

Juxtapapillary subretinal hemorrhages in pseudotumor cerebri

We report two cases of pseudotumor cerebri with subretinal hemorrhages. Bilateral juxtapapillary subretinal neovascular membranes were present in one. A unilateral hemorrhage occurred adjacent to a small anomalous optic disc in the other. Subretinal hemorrhages in papilledema may occur from juxtapapillary subretinal neovascular membranes that evolve rapidly and then involute. The relationship of the appearance and subsequent evolution of such juxtapapillary subretinal neovascular membranes to the course of the intracranial pressure is discussed. Occurrence in one eye may presage occurrence in the other eye, making control of intracranial pressure important once one eye is afflicted. Fortunately, severe permanent visual loss remains unreported. Some anomalous small optic discs may be particularly at risk for subretinal hemorrhage from papilledema because preexisting axonal crowding is aggravated by the swelling of the optic nerve from elevated intracranial pressure. Conditions related to the Valsalva maneuver that chronically or intermittently elevate intracranial pressure further and produce elevated cephalic venous pressure should be avoided.     

Treatment of pseudotumor cerebri by primary and secondary optic nerve sheath decompression

We performed optic nerve sheath decompression in 53 patients (101 eyes) with pseudotumor cerebri and visual loss. Sixty-nine eyes (85 patients) with acute papilledema uniformly had improved visual function after optic nerve sheath decompression. Of 32 eyes with chronic papilledema (18 patients), only ten had improved visual function after optic nerve sheath decompression. This difference was significant (P = .0001). Thirteen eyes required secondary or tertiary optic nerve sheath decompression after an initial successful result. Eleven of 13 eyes had improved visual function after repeat optic nerve sheath decompression. We believe that patients with acute papilledema and visual loss should be offered optic nerve sheath decompression, and if symptoms recur, repeat optic nerve sheath decompression is a safe and effective treatment option.     

Subretinal neovascularization and papilledema associated with pseudotumor cerebri

A 31-year-old man, on routine ocular examination, was found to have bilateral papilledema. Neurologic evaluation confirmed elevated cerebrospinal pressure with no mass lesion and a diagnosis of pseudotumor cerebri was made. A tiny subretinal hemorrhage adjacent to the right optic disk was found to be secondary to subretinal neovascularization. Over the course of several months, the papilledema resolved. However, the neovascular membrane extended further toward the fovea and was subsequently obliterated with argon laser photocoagulation.     

Bilateral juxtapapillary subretinal neovascularization associated with pseudotumor cerebri

A 32-year-old obese woman with hypertension and a three-year history of pseudotumor cerebri developed bilateral juxtapapillary subretinal neovascular membranes. To our knowledge, this is the first reported case of bilateral subretinal neovascular membranes complicating the course of this disease. The subretinal neovascular membrane in the left eye spontaneously involuted, but because the membrane in the right eye threatened the foveola, the patient underwent argon-laser photocoagulation. The subretinal fluid and hemorrhage progressively resolved, the membrane was replaced by fibrous tissue, and visual acuity improved. The pathogenesis of the subretinal neovascular membranes was presumably secondary to pressure deformity of the border of Bruch's membrane at the optic disk, creating a discontinuity of normal anatomic apposition of the chorioretinal layers. This anatomic dehiscence, coupled with hypoxia created by axonal tissue swelling and resultant impaired vascular perfusion of the tissues, led to the development of subretinal neovascular membranes.