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Show Journal of Neuro- Ophthalmology 16( 4): 241- 246, 1996. 1996 Lippincott- Raven Publishers, Philadelphia " Normal Pressure" Pseudotumor Cerebri Jeffrey P. Green, M. D., Nancy J. Newman, M. D., Zachary N. Stowe, M. D., and Charles B. Nemeroff, M. D., Ph. D. We present a case report of a patient with clinical features suggestive of pseudotumor cerebri ( PTC), without a documented elevated measurement of intracranial pressure ( ICP). Chart review was done of one patient's clinical course over a 28- month period. The patient was treated for PTC even though she never had a documented elevated ICP. Her signs and symptoms, including headache, disc edema, and visual field loss, all showed improvement with standard PTC therapy, which ultimately included optic nerve sheath fenestration ( ONSF). Her presenting symptoms of clinical depression were also relieved with this treatment. PTC may present without an elevated ICP as defined by current standards. Some patients may be more susceptible to lower levels of ICP and develop this syndrome, and it may be responsive to standard PTC therapy. Further investigation may warrant that clinical depression be included as another minor symptom of PTC. Key Words: Pseudotumor cerebri- Optic nerve sheath fenestration- Depression- Intracranial pressure. Pseudotumor cerebri ( PTC) is a syndrome of raised intracranial pressure of unknown etiology. The modified Dandy criteria for the diagnosis of PTC are well known: ( a) signs and symptoms of increased intracranial pressure; ( b) absence of localized findings on neurologic examination; ( c) normal neuroradiologic studies; ( d) awake and alert patient; ( e) no other cause of increased intracranial pressure present ( 1). The major symptoms of PTC include headache, transient visual obscurations, blurred vision, pulsatile tinnitus, and diplopia ( 1,2). Other minor symptoms include paras-thesias, back and leg pain, arthralgias, and unsteady gait ( 3). Johnston et al. ( 4) called attention to some cases that bear a close resemblance to PTC but fail to comply with one or more of the accepted diagnostic criteria. These authors proposed that the concept of PTC be broadened to include other atypical forms. One of these atypical forms was that of so-called " normal pressure pseudotumor" syndrome. We report a patient who presented with symptoms of clinical depression and headache and possible " normal pressure pseudotumor cerebri." Her symptoms and signs improved after optic nerve sheath fenestration. Manuscript received February 23, 1996; accepted April 21, 1996. From the department of Ophthalmology ( J. P. G., N. J. N.), Neurology ( N. J. N.), Neurosurgery ( N. J. N.), Psychiatry and Behavioral Sciences ( Z. N. S., C. B. N.), and Gynecology and Obstetrics ( Z. N. S.), Emory University, Atlanta, Georgia, U. S. A. This work was supported in part by a departmental grant from Research to Prevent Blindness Inc. Address correspondence and reprint requests to Dr. Nancy J. Newman, Emory Eye Center, Neuro- Ophthalmology Unit, 1327 Clifton Road NE, Atlanta, GA 30322, U. S. A. CASE REPORT An 18- year- old, 66- inch tall, 145- pound woman with a mildly overweight body habitus, was admitted to the general psychiatric and clinical research unit with a history of depression for 15 months and chronic fatigue syndrome for approximately 3 years diagnosed by a local psychiatrist. Her symptoms included complaints of sadness, lack of motivation, hypersomnolence ( up to 16 h per day), hyperphagia, fatigue, difficulty concentrating, and a history of morbid thoughts. She had b e e n t r e a t e d for 10 m o n t h s with fluoxetine 241 242 /. P. GREEN £ T AL. ( Prozac) up to 60 mg/ day, with only a partial remission of symptoms. The fluoxetine was discontinued and the patient was started on desipramine ( Norpramin) up to 100 mg/ day, which was discontinued secondary to a rash. Upon admission her medications included lorazepam ( Ativan) 1 mg at bedtime, and an oral contraceptive, Ortho Novum 1/ 35. Her admission Beck Depression Inventory ( BDI) ( 5) was 15, and she was started on sertraline ( Zoloft) 50 mg/ day. On her admission physical examination the psychiatrist noted that she had bilateral disc edema. Further questioning revealed that the patient had been experiencing recurrent headaches and eye pain with no complaints of decreased visual acuity or visual field loss. She denied transient visual obscurations or diplopia. She also denied the use of vitamin A, steroids, or tetracycline. On examination her vision was 20/ 20 in both eyes. Pupils were normal. Intraocular pressure was 16 in both eyes, and motility and slit lamp examinations were unremarkable. Fundus examination revealed bilateral disc elevation ( Fig. 1), with blurred margins and mild hyperemia in both eyes. There were no spontaneous venous pulsations. Automated perimetry revealed superonasal defects, right eye worse that left. ( Fig. 2). Magnetic resonance imaging with and without gadolinium was normal. A lumbar puncture revealed an opening pressure of 150 mm HzO, no cells, protein less than 10 mg/ dl, normal glucose, negative stains and cultures, and negative rapid plasma reagin ( RPR). Complete blood count, chemistries, pregnancy test, thyroid function tests, RPR, fluorescent trepenomal antibody ( FTA), human immunodeficiency virus ( HIV), cytomegalovirus ( CMV), and urinalysis were all negative. The antinuclear antibody ( ANA) was elevated at 1/ 320, but subsequent rheumatologic evaluation disclosed no specific collagen vascular disorders. The day following the lumbar puncture, the patient reported increased energy, decreased desire to sleep, markedly reduced headaches, and she was able to participate in group activities. Her psychiatrist and nursing staff noted a decrease in her depressive symptoms, specifically decreased tearfulness, improved mood, increased energy and group attendance, and an absence of somatic complaints. She was discharged 7 days later with a BDI of 4. Twelve days following lumbar puncture, the patient was seen in neuro- ophthalmologic follow-up. She reported a recurrence of her headaches and psychiatric symptoms but her examination was unchanged. Fluorescein angiogram revealed bilateral disc leakage and no evidence of autofluo-rescence. Four months later the patient had continued symptoms. Her weight had not fluctuated. Her examination was notable for progression of her visual field defects ( Fig. 3). Repeat lumbar puncture revealed an opening pressure of 110 mm HzO, with normal CSF contents. Ten minutes after the lumbar puncture, the patient noted a decrease in her headache, and headaches did not recur until one week later. She was begun on Diamox 500 mg twice per day. One month later she reported an increase in her headaches. Her medications included Zoloft 150 mg/ day, Diamox 500 mg twice per day and Ortho Novum 1/ 35. She still reported no transient visual obscurations and no diplopia. Her neuro- ophthalmologic examination was unchanged, with the exception of further visual field loss. Her Diamox was increased to 500 mg three times per day. Five weeks later her symptoms and her visual fields were unchanged. Her weight had still not FIG. 1. Left: right eye; right: left eye. Ophthalmoscopic appearance at presentation showing disc elevation, blurred margins, and mild hyperemia. ( Green and Newman). / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 PSEUDOTUMOR CEREBRI 243 FIG. 2. Visual fields at presentation showing superonasal defects, right eye greater than left. ( Green and Newman). changed. Diamox was discontinued, and she was admitted to the hospital for bolt monitoring of her intracranial pressure. Over a 48- h period, bolt monitoring revealed ICP that fluctuated between 0 and 180 mm H20. No sedation was administered while the intracranial bolt was in place. When the bolt was placed, the patient reported a decrease in headache intensity. There was no notable CSF leak around the bolt. Two days after bolt placement an optic nerve sheath fenestration was performed through a standard medial orbitotomy ( 6). There were no complications. One day postoperatively her visual acuity was unchanged, and there was no diplopia. Her intracranial pressure was monitored for a 24- h period postoperatively and was noted to fluctuate between 0 and 80 mm H20. One month postoperatively, the patient reported continued decrease in headaches since the surgery. She was still taking her Zoloft, and Ortho Novum 1/ 35, but was not on any Diamox. Her weight remained the same. Examination was notable for improvement in her disc edema ( Fig. 4), and significant improvement in her visual fields ( Fig. 5). The patient, her family, and her psychiatrist reported a dramatic improvement in her mood with resolution of her depressive symptoms. Her Zoloft was tapered and discontinued with no recurrence of her depressive symptoms. Over the subsequent 20 months, on no medications, without any weight change, the patient reported one mild headache per month and has remained eu-thymic. She has returned to school and has maintained a good sleep pattern and energy level. She has only sought medical attention for routine health care, and her neuro- ophthalmologic examination has remained stable. FIG. 3. Visual fields, four months after presentation showing progression of field defects in both eyes. ( Green and Newman). / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 244 /. P. GREEN ET AL. FIG. 4. Left: right eye; right: left eye. Ophthalmoscopic appearance one month postoperatively showing improvement of disc elevation. ( Green and Newman). DISCUSSION We believe this case represents a form of PTC with two interesting features, namely normal intracranial pressure and presenting symptoms of depression relieved by PTC therapy. One of the strict Dandy criteria for PTC is increased intracranial pressure ( 1). Corbett has said that " under no circumstances should the diagnosis of PTC be made without finding elevated spinal fluid pressure . . . " ( 7). Normal intracranial pressure has been defined as 136 mm H2 0 ( SD ± 37.6) in patients of normal weight, and 167 mm HzO ( SD ± 36.46) in obese patients; however, it should be pointed out that there was no statistical difference between these two groups using Duncan's multiple range test ( 8). At no time was our patient documented to have an elevated CSF pressure, including two lumbar punctures and during two days of intracranial bolt monitoring. Johnston and Pater-son ( 9) have observed wide fluctuations of CSF pressure in patients with PTC, using continuous ICP monitoring. Others have confirmed these fluctuations ( 10,11). The medical treatment of PTC includes weight loss, carbonic anhydrase inhibitors, and possibly systemic steroids. If these modalities are unsuccessful in alleviating the symptoms or curtailing the visual dysfunction caused by PTC, then surgical intervention is indicated. Most authorities now agree that optic nerve sheath fenestration is the procedure of choice for progressive visual field loss and lumboperitoneal shunt the choice for intractable headaches with some overlap between the two ( 12- 18). The importance of accurately diagnosing PTC is underscored by the fact that although these treatments are often highly effective, they are not without inherent side effects and complications ( 19,20). We believe that our patient represents a variation of PTC that has been called " normal pressure FIG. 5. Visual fields one month postoperatively showing improvement in field loss in both eyes. ( Green and Newman). / Neuro- Ophthalmol, Vol. 26, No. 4, 1996 PSEUDOTUMOR CEREBRI 245 PTC." Johnston et al., in a series of atypical PTC patients, reported a 13- year- old boy who was followed for four years after presenting with a unilateral scotoma ( 4). He had disc edema with progressive visual field and acuity loss, normal neurologic examination, and normal CT and MRI scans of the brain. His CSF pressure was 130 mm HzO at presentation with a normal composition. One year later CSF pressure monitoring via lumber subarachnoid catheter was normal over a 36- h period. The patient's visual fields and acuity declined and, after treatment with systemic steroids was ineffective, percutaneous lumboperitoneal shunt was performed. Surgery was successful in halting visual field loss, and there was rapid resolution of disc edema. Two episodes of recurrent disc edema and worsening of visual acuity were associated with shunt obstructions, both resolving with shunt revision. This case was very similar to ours in that both patients were felt to have PTC without having a measured elevated ICP even with continuous ICP monitoring. Both patients had relief of their visual deterioration and resolution of their optic disc swelling after CSF diverting procedures. Johnston et al. proposed two possible explanations for the presence of papilledema in the absence of measured elevated ICP. Firstly, it was proposed that the situation could be likened to normal- pressure hydrocephalus, in which there is a definite abnormality of CSF circulation and volume relieved by drainage, without a demonstrable abnormality of CSF pressure ( 4,21). Secondly, it was hypothesized that local abnormalities in the region of the optic nerve sheath are responsible for the development of papilledema, with relatively normal ICP allowing a local buildup of pressure that is not reflected in the pressure measurement elsewhere in the subarachnoid space ( 4). It is possible that both these mechanisms played a role in our patient's clinical course. The former theory may have more merit than the latter, as our patient had other non-visual symptoms that resolved upon optic nerve sheath fenestration, including relief of headache, and improvement in her psychiatric symptoms. Our case supports the theory that unilateral optic nerve sheath fenestration acts as a CSF filter ( 14,22). The patient had bilateral improvement in her visual fields and bilateral resolution of her papilledema. This bilateral effect after unilateral surgery has been reported in up to 73% of cases in other series ( 15- 18). The continuous ICP monitoring also reflected a lower average ICP postoperatively. Her clinical course and response to treatment were typical of PTC. It is likely that certain patients have different susceptibilities to varying levels of ICP. On four separate occasions ( two lumbar punctures, subarachnoid monitor placement, and ultimately optic nerve sheath fenestration), our patient experienced improvement in her headaches as well as her depressive symptoms. We believe that there was an undetectable amount of CSF that leaked around the subarachnoid monitor. This is a known effect that occurs with the placement of these types of monitors ( 23). If the bolt had a tight seal, we would not have expected persistent headache relief with its insertion. Failure to document an elevated pressure could have been due to leakage around the subarachnoid monitor. This improvement in her headaches and depressive symptoms was noted subjectively by the patient, and following the initial LP, by both the patient and her psychiatrist. While temporally related, it is unlikely that the reduction in her headaches alone accounted for her improved mood. The patient's primary complaints focused on her depressive symptoms and the headaches and eye pain were elicited only on a careful review of systems. It is reasonable to assert that the headaches may have contributed to the clinical presentation and decline in function; however, the patient's salutatory improvement in numerous aspects of her life are difficult to attribute to alleviation of the subjectively mild headaches. It is possible that other confounding factors such as the placebo effect of her treatments and a desire to please her physician may have contributed to the relief of her headaches and depressive symptoms. Such confounds are equally unlikely since the patient had been involved in a therapeutic relationship with a previous psychiatrist and had failed to respond to antidepressant treatment. In addition, the course of psychiatric treatment with an antidepressant mechanistically very similar to previous medications, the relatively short duration of such treatment, and the maintenance of a euthymic state suggests a relationship in this patient between PTC and depressive symptoms. Our patient met diagnostic criteria for major depression ( 24), and her symptoms were consistent with the atypical subtype of major depression. While her initial BDI score was mild to moderately severe, the BDI does not reliably assess patients with atypical symptom patterns. Furthermore, the patient demonstrated considerable social, academic, and occupational impairment indicative of a major affective disorder. The majority of these symptoms were relieved transiently by lumbar punctures and persistently by optic nerve sheath fenestration. The classic symptoms and signs of / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 246 /. P. GREEN ET AL. PTC include headache, transient visual obscurations, pulsatile intracranial noises, diplopia secondary to sixth cranial nerve paresis, and papilledema ( 1,2). Round and Keane reported on other minor symptoms of PTC, including neck stiffness, tinnitus, distal extremity parasthesias, joint pains, low back pain, and gait ataxia ( 3). Lessell ( 25), in his major review of pediatric PTC, reported that young children, excluding adolescents, may present with symptoms of irritability. He also cited the case of an infant with PTC who presented with somnolence ( 26). Other investigators have noted the association of PTC and psychiatric impairment ( 27,28). We feel that this case illustrates that clinical depression can be a presenting complaint of PTC and should be listed as another " minor symptom" of PTC. The preference of both PTC and depression to present in women during their child-bearing years underscores the need to investigate the relationship between these two diseases. The visual sequalae of untreated PTC can be devastating. Approximately 90% of patients with this disorder have some visual field defect, in at least one eye, and serious visual field loss or loss of visual acuity is seen in approximately 10- 25% of carefully studied patients ( 2,12,29). Diagnosis and prompt therapy may be visually preserving. We believe that a patient presenting with a clinical picture indicative of PTC ( complying with the modified Dandy criteria), without evidence of ICP greater than 180 mm H2 0 on at least two separate measurements, should undergo more aggressive evaluation. If clinical signs or symptoms are unresponsive to established tolerable medical therapy, 48- h continuous subarachnoid ICP monitoring must be considered. If the three separate ICP measurements including the subarachnoid monitor have all been 180 mm H2 0 or less, the diagnosis of " normal pressure pseudotumor cerebi" should be made. An accurate prevalence of this particular variant is unknown. 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