Neurological findings in patients with pseudopapilloedema with and without verified optic disc drusen

As part of a clinical examination of 200 patients with pseudopapilloedema with and without verified optic disc drusen, neuroophthalmological examinations were performed in 158, neurological examinations in 117 and electroencephalography in 109 patients. Headache and suspected papilloedema were the most frequent reasons for referral. Headache was one of the complaints in 102 patients. Migraine was diagnosed in 22 and suspected in 3 patients. Epilepsy was present in 9 patients and in addition, 1 patient had had treatment for convulsions in childhood. 2 patients had a pituitary tumour and 1 had a tumour in the hypothalamic region. Various other neurological disorders appeared in small numbers. Abnormalities in electroencephalograms (EEGs) were found in 36 patients. Progressive loss of central visual acuity unexplained by retinal pathology as well as bitemporal and homonymous hemianopic visual field defects and also evidence of papilloedema warrant a neurological examination in patients with optic disc drusen.     

Arteriovenous malformations presenting with papilloedema

Cerebral arteriovenous malformations (AVMs) are fairly common and the majority of paediatric patients with this condition also present with intracranial haemorrhage. Two patients who had an incidental finding of an AVM associated with papilloedema are described here. The first was a 13-year-old male who presented after an accidental kick to the eyes. Examination revealed bilateral papilloedema. He gave a 2-year history of intermittent headache. Brain magnetic resonance imaging (MRI) showed an unruptured AVM in the temporal lobe. Lumbar puncture revealed elevated cerebrospinal fluid pressure. Visual acuity and visual fields were normal. He was treated with acetazolamide and improved within a few weeks. He subsequently underwent stereotactic radiosurgery to the AVM. He discontinued acetazolamide due to adverse side effects and there was no recurrence of headache and papilloedema. The second patient was a 14-year-old male who had polyarticular juvenile chronic arthritis and received low-dose steroids and methotrexate. Bilateral papilloedema was discovered during routine ophthalmology surveillance and he was otherwise asymptomatic neurologically. Brain MRI revealed an AVM in the posterior fossa. He had three embolization procedures, which have resulted in significant reduction in lesion size. The papilloedema resolved completely after the first two procedures, and visual acuity and fields remained normal. Here, possible underlying mechanism of raised intracranial pressure and importance of visual assessment in those with AVMs and their management are discussed.     

Benign intracranial hypertension: A retrospective and follow-up study

Thirty-six patients with benign intracranial hypertension (BIH) were reviewed. Follow-up was obtained on 33 patients (91%) after a mean period of $\text{7}\text{1}\text{2}$ years. Precipitating factors were found in 27 patients (75%). On admission, 5 patients had retro-ocular pain, especially on eye movements, a complaint not yet described in BIH. Seven patients had nystagmus, two of them horizontal positional nystagmus. It is questionable whether all signs in BIH are caused by the raised CSF pressure.The general outcome was good. Only two patients sustained severe ultimate visual impairment. Both presented with retro-ocular pain and sudden loss of vision on admission. Papilloedema can persist for years in BIH without serious visual impairment. Sometimes “causal” treatment is possible. No symptomatic treatment which is free from complications has been proved to prevent visual failure.     

Benign intracranial hypertension (pseudotumor cerebri). 12 cases

Twelve cases of benign intracranial hypertension (BIH) are reported, there being a higher proportion of women patients and a greater frequency of those in the 3rd decade of life. Symptoms were principally headache (11 patients) and/or transient or permanent visual disorders (10 patients). Bilateral papillary edema was often associated with diminished visual acuity (5 cases) and/or amputation of the visual field (5 cases). Cerebral venous occlusion was detected in 3 patients and an infective focus in 7 cases. Three patients were receiving some treatment but none of the women was taking oral contraceptives. Two were pregnant. The CSF was abnormal in 5 of the 7 patients (mixed cell reaction and red blood cells: 1 case; pleiocytosis: 3 cases; raised protein levels: 1 case), and CT scans were normal except for reduced ventricular system size in 9 patients. Follow-up for a mean of 1 year showed total disappearance of symptoms and signs in 4 cases. Papillary edema had not totally regressed at the last ophthalmological examinations performed on 5 patients, and visual sequelae (visual acuity and/or visual field anomalies) were present in 5 cases. Two patients relapsed. The conventional acceptance of the benign nature of BIH must be tempered by the possible onset of possible serious visual disorders. Two factors appear to be determinant: the presence of severe visual disorders initially and/or the existence of arterial hypertension. Various physiopathogenic hypotheses have been evoked to explain BIH. The essential mechanism appears to be an anomaly of absorption of CSF, either from increased pressure in the dural venous sinuses or lesions of the arachnoid villi, but the role of cerebral edema and/or increased cerebral blood volume cannot be excluded. Incomplete understanding of physiopathogenic mechanisms account for the numerous treatments proposed and for some confusion in their indications.     

Benign intracranial hypertension

Benign intracranial hypertension (BIH) is characterized by an elevation of the intracranial pressure not associated with an intracranial process or hydrocephaly, and with normal cerebrospinal fluid (CSF) contents. The elevation of the intracranial pressure is isolated; therefore, diseases such as cerebral venous thrombosis or dural fistulas should not be considered as etiologies of BIH. The exact definition of BIH remains debated, and other terms such as "pseudotumor cerebri" or "idiopathic intracranial hypertension" are often used in the literature. Although we agree that BIH is usually not a so benign disease, we suggest that BIH is still the most appropriate term to describe this entity which should be classified as "secondary BIH" or "idiopathic BIH" depending on whether there are precipitating factors for the development of BIH or not. We also propose new diagnostic criteria emphasizing the need for investigations for the diagnostic of secondary and idiopathic BIH. The management of patients with BIH depends mainly on the presence and severity of ocular symptoms and signs on which the prognostic of the disease is based. Repeated lumbar punctures associated with acetazolamide and weight loss are usually efficient enough. However a surgical treatment (optic nerve sheath fenestration or lumboperitoneal shunt) is required when appropriate medical management does not prevent progressive alteration of vision (visual loss or visual field defect), or when the patients complains of severe, refractory headaches. Careful follow-up with repeated formal visual field testing may help preventing a devastating visual loss in these patients.     

Evaluation of the visual system in multiple sclerosis: a comparative study of diagnostic tests.

In 22 patients with clinically definite multiple sclerosis (MS) who were without visual symptoms and had a visual acuity of at least 1.0 in both eyes at the time of measurement, the following tests were performed to detect subclinical lesions in the visual system: visual evoked potential (VEP), contrast sensitivity test (CS), flight of colours test (FOC), colour vision test (Ishihara plates) (CV) and the pupillary light reflex (PLR). VEP was abnormal in 81.8%, CS in 72.7%, FOC in 36.4%, CV in 31.8%, and PLR in 52.3% of the patients. VEP and CS together were most sensitive: combining these techniques subclinical lesions of the visual system were detected in 90.9% (20/22) of these asymptomatic patients.     

Activate your online subscription

OBJECTIVE: To determine if visual function loss from vigabatrin use recovers after the drug is discontinued. BACKGROUND: Vigabatrin is an effective antiepileptic drug, but it is known to cause a variety of changes in visual function, including reductions in the visual field, visual acuity, color vision, and in electroretinogram (ERG) and electro-oculogram amplitudes. It is not known whether these changes are reversible. METHODS: Measurements of static and kinetic visual fields, visual acuity, color vision, and the ERG were recorded while patients were taking vigabatrin and again in 13 patients who had discontinued the drug because of lack of efficacy or reductions in visual field. Most of the patients had been off the drug for 3 to 6 months, although two patients had been drug-free for almost 1 year. RESULTS: Although ERG cone implicit time improved, most of the patients did not show improvement in either clinical measures of visual function (i.e., visual acuity, color vision, visual fields) or in ERG amplitudes. However, several patients who showed minimal visual field loss while on the drug had substantial recovery of ERG amplitudes. There was no statistical association between recovery of function and either duration of treatment or cumulative dosage. The multifocal ERG showed a diffuse loss of function that was not isolated to the periphery. CONCLUSIONS: Although the visual deficits in patients taking vigabatrin tend to be mild, most patients do not show improvement after they stop taking the drug. Visual field loss resulting from vigabatrin was not reversible. Visual acuity, color vision, and ERG amplitude loss may be reversible in patients with minimal or no field loss.     

Losses of hemifield contrast sensitivity in patients with pituitary adenoma and normal visual acuity and visual field.

OBJECTIVE: To detect early losses of contrast sensitivity (CS) in patients with pituitary adenomas, before the occurrence of visual acuity and visual field defects. METHODS: CS has been evaluated in both hemifields of 28 patients with different kinds of pituitary adenoma (mainly intrasellar) and normal visual acuity and visual field, as well as in 15 age-matched controls. Two different stimuli were used: a coarse (0.3 c/deg) dynamic (10 Hz) grating and a finer (2 c/deg) static grating. RESULTS: On average, CS and/or hemifield asymmetry were reduced in patients, whereas perimetric sensitivity was normal. CS losses were more frequent for 2 c/deg static-, as compared with 0.3 c/deg, 10 Hz stimuli. However selective losses for either stimuli were also found. CS losses did not correlate with anatomical measurements (size, chiasm involvement) of tumors as established by MRI scans. CONCLUSIONS: CS evaluation may provide a simple and effective tool for early detection and monitoring of visual dysfunction in patients with pituitary adenoma. The lack of correlation between CS losses and chiasm involvement suggests causes different from chiasmal compression for visual dysfunction.     

Benign intracranial hypertension: correlation of cerebral blood flow with disease severity

Benign intracranial hypertension (BIH) is characterized by symptoms and signs of raised intracranial pressure in the absence of an intracranial mass lesion, infection or hydrocephalus. The purpose of this study was to evaluate the effect of disease severity on cerebral blood flow in patients with BIH on acetazolamide therapy. METHODS: 11 patients (nine females, two males; mean age 30.5 years; range 22-29 years) with BIH were studied. All patients underwent CT and MRI scanning which were normal. The CSF pressure of all patients was above 200 mm H2O. All patients were under treatment with acetazolamide (1 g/day). Disease severity was determined by visual field examination and by clinical symptoms. Five patients were categorized into mild to moderate BIH (group I) and six patients had severe BIH (group II). All patients underwent perfusion brain SPECT with 740 MBq of Tc-99m-HMPAO. RESULTS: Brain perfusion abnormalities were observed in six of the 11 patients. One out of five patients in group I (20%) and five out of six patients (83%) in group II, had abnormal SPECT findings (P<0.04). In four patients of group II the left parietal lobe was involved and another patient had a right occipital abnormality. The single patient from group I with SPECT abnormalities demonstrated focal decreased perfusion in the left temporal area and decreased perfusion in the left caudate nucleus. CONCLUSION: Patients with severe degree of BIH have a higher incidence of cerebral perfusion abnormalities. This group may have an increased risk of cerebrovascular complications. The continuous administration of acetazolamide which affects the vascular autoreactivity may contribute to the regional hypoperfusion. Further studies are recommended to evaluate the natural course of disease versus iatrogenic treatment effects.     

Doxycycline and intracranial hypertension

The authors report seven patients from six neuro-ophthalmology referral centers who developed pseudo-tumor cerebri during treatment with doxycycline. All four female patients and one of three male patients were obese. Vision was minimally affected in most patients, but two had substantial visual acuity or visual field loss at presentation. Discontinuation of doxycycline, with or without additional intracranial pressure-lowering agents, yielded improvement, but permanent visual acuity or visual field loss occurred in five patients.     

Aneurysmal compression of the anterior visual pathways

Ten patients with aneurysmal compression of the anterior visual pathways had visual loss, unilateral in 4 and bilateral in 6. There was no typical clinical presentation. Visual loss was acute or gradual, acuity sometimes fluctuated, and visual field testing was highly variable. The aneurysms were supraclinoid (four patients), carotid-ophthalmic (two), anterior communicating-anterior cerebral (three), and intracavernous carotid (one). Nine patients had successful clipping of their aneurysm, and in one, ipsilateral common carotid ligation was performed. Postoperatively, visual acuity was improved in six cases, unchanged in three, and worse in one.     

Electrophysiology versus psychophysics in the detection of visual loss in pseudotumor cerebri

This study compared visual evoked potentials (VEP) and contrast sensitivity (CS) as methods of detecting visual loss in 15 women with acute onset of pseudotumor cerebri (PTC). A total of 24 out of 30 eyes (14 of 15 patients) had a visual disturbance. Neuro-ophthalmologic examination demonstrated abnormal Goldmann visual fields in 13 of 30 eyes. CS was abnormal in 18 eyes; VEP was abnormal in five eyes. Nine eyes had visual loss detected by CS only, five by perimetry only, and one had the VEP as the sole abnormality. CS was a valuable adjunct to perimetry in PTC whereas VEP was rarely helpful.     

Chiari I malformation and intra-cranial hypertension:a case-based review

OBJECTIVE: To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed. CLINICAL PRESENTATION: A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI. INTERVENTION AND FOLLOW-UP: Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy. CONCLUSION: IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.     

Intracranial hypertension causing visual failure in cryptococcus meningitis.

Thirty four patients with cryptococcal meningitis seen in the University of Malaya medical centre since 1980 were reviewed. Eleven patients had bilateral papilloedema and visual impairment but eventually survived. Seven patients had intensive aggressive measures, including shunting to reduce intracranial hypertension irrespective of ventricular size shown in CT scan, and showed substantial improvement in vision. It is concluded that papilloedema and visual failure in cryptococcal meningitis reflects raised intracranial pressure and that this should be treated vigorously.     

Visual manifestations of visible and buried optic disc drusen.

BACKGROUND: It has been reported that visible optic disc drusen are associated with decreased visual acuity and loss of peripheral visual field. Patients with buried optic disc drusen have not been as well characterized. METHODS: An observational, retrospective review was made of 92 eyes with funduscopic or ultrasonographic evidence of optic disc drusen. Demographics, presenting symptoms, visual acuity, refractive error, intraocular pressure, presence of an afferent pupillary defect, cup-to-disc ratio, appearance of the optic nerve, and visual field were recorded. RESULTS: Fifty-one (55%) of the eyes were symptomatic; among them, 63% had symptoms of visual acuity loss, and 49% had symptoms of visual field loss. Seventy-nine (86%) of the optic discs appeared abnormal on ophthalmoscopy, but only 42% of these had visible drusen. Forty-five (49%) of the eyes had a visual field defect, and 73% of these were nerve fiber bundle in type. Overall, 73% of optic discs with visible drusen had abnormal visual fields compared with only 36% of optic discs with buried drusen (P < 0.05). CONCLUSIONS: Among 92 eyes with optic disc drusen, only slightly more than 50% produced visual symptoms. Fewer than 50% of drusen were visible ophthalmoscopically. Buried optic disc drusen identified by ultrasound had a significantly lower frequency of associated visual field defects than did visible optic disc drusen.     

Magnetic resonance imaging in optic nerve lesions with multiple sclerosis

Magnetic resonance imaging (MRI) of the optic nerves was performed in 10 patients with multiple sclerosis (MS) using short inversion time inversion recovery (STIR) pulse sequences, and the results were compared with the visual evoked potentials (VEP). The 10 patients had optic neuritis in the chronic or remitting phase together with additional symptoms or signs allowing a diagnosis of clinically definite or probable MS. Sixteen optic nerves were clinically affected and 4 were unaffected. MRI was performed using a 0.5 tesla superconducting unit, and multiple continuous 5 mm coronal and axial STIR images were obtained. A lesion was judged to be present if a focal or diffuse area of increased signal intensity was detected in the optic nerve. In VEP, a delay in peak latency or no P 100 component was judged to be abnormal. With regard to the clinically affected optic nerves, MRI revealed a region of increased signal intensity in 14/16 (88%) and the VEP was abnormal in 16/16 (100%). In the clinically unaffected optic nerves, MRI revealed an increased signal intensity in 2/4 (50%). One of these nerves had an abnormal VEP and the other had a VEP latency at the upper limit of normal. The VEP was abnormal in 1/4 (25%). In the clinically affected optic nerves, the degree of loss of visual acuity was not associated with the longitudinal extent of the lesions shown by MRI. The mean length was 17.5 mm in optic nerves with a slight disturbance of visual acuity and 15.0 mm in nerves with severe visual loss.(ABSTRACT TRUNCATED AT 250 WORDS)     

The 20/20 eye in multiple sclerosis

Using clinical and electrophysiologic measures, we evaluated the visual pathway of patients who had multiple sclerosis, 20/20 Snellen acuity, and no history of optic neuritis. Delayed latencies were found in the transient visual evoked potentials (VEPs) of 38% of the patients, and interocular latency differences were abnormal in 67%. Contrast VEPs were abnormal in 46%. Psychophysical determinations of contrast sensitivity were abnormal in 78%. Only 17% of the patients had dyschromatopsia, 36% had afferent pupillary abnormalities, and 59% had optic nerve pallor or nerve fiber layer loss. Psychophysical contrast evaluations and VEP studies were superior to other clinical evaluations in demonstrating visual dysfunction in these patients.     

Treatment of visual loss in pseudotumor cerebri associated with uremia

Ten patients with pseudotumor cerebri associated with renal insufficiency had papilledema and elevated intracranial pressure, but neurologic examinations and CT were normal. The 40% frequency of severe visual loss, 20/100 or worse, was higher than expected for pseudotumor cerebri. While furosemide was effective in 3 patients with renal transplants, administration of corticosteroids to 2 other patients did not halt progressive loss of vision. The visual function of 2 patients deteriorated even after lumboperitoneal shunting. Five patients underwent fenestration of the optic nerve sheath. This procedure improved the visual acuity of 3 patients and stabilized vision in the others. While the pathogenesis of pseudotumor cerebri in renal insufficiency is unknown, optic nerve sheath fenestration is the surgical treatment of choice for visual loss unresponsive to medical therapy.     

Treatment of benign intracranial hypertension by dehydrating agents with particular reference to the measurement of the blind spot area as a means of recording improvement.

A series of 30 patients (28 females : 2 males) presenting with benign intracranial hypertension is reported. It is shown that the papilloedema may be resolved in as little as six weeks by the use of dehydration therapy. Evidence is presented that this method can restore to normal a visual acuity which is as low as 6/24 or even 6/36. It is concluded that surgery is never required in the management of this condition. It is strongly recommended that serial blind-spot measurements should be used routinely to assess the effectiveness of therapy. Fluorescein angiography has a place in diagnosis among a small proportion of these patients.     

神经内镜经鼻入路手术治疗颅咽管瘤的疗效评价

目的探讨神经内镜经鼻入路手术治疗颅咽管瘤的临床疗效。方法回顾性分析2018年10月至2020年4月首都医科大学附属北京天坛医院神经外科采用神经内镜经鼻入路治疗102例颅咽管瘤患者的临床资料,其中原发肿瘤82例,复发肿瘤20例。术后每3个月门诊随访患者的视力、视野,评估垂体-靶腺轴损伤数量以及有无尿崩症,复查头颅MRI观察肿瘤有无复发、进展。根据术后3个月的头颅MRI结果,评估肿瘤的切除程度结果102例患者中,92例(90.2%)肿瘤全切除,10例(9.8%)次全切除;其中原发肿瘤全切除77例,复发肿瘤全切除15例术后5例患者发生脑脊液漏并行神经内镜经鼻修补术,3例患者发生中枢神经系统感染。无一例患者死亡或出现昏迷。102例患者随访时间的中位数(范围)为12(6~19)个月。至末次随访,96例术前视力、视野受损的患者中,62例视力明显改善,27例稳定,7例恶化;术前视力正常的6例患者中,仅1例术后视力短暂下降,术后3个月复查时视力恢复正常。99例合并尿崩症,其中72例尿崩症持续时间≤3个月,27例持续时间>3个月至末次随访,73例术前存在垂体-靶腺轴功能障碍的患者,垂体-靶腺轴功能均无改善;29例术前垂体-靶腺轴功能正常的患者中,22例出现垂体-靶腺轴功能障碍102例患者随访期间复查头颅MRI均未见肿瘤复发或进展。结论采用神经内镜经鼻入路手术可更充分地观察肿瘤与视神经、垂体柄及下丘脑等结构的界限,为术者提供更清晰的术野,肿瘤全切除率较高,部分患者术后视力、视野改善,但术后尿崩症和垂体-靶腺轴功能障碍发生率较高。