Ординатура / Офтальмология / Английские материалы / Neuro-Ophthalmology_Kidd, Newman, Biousse_2008

152Neuro-Ophthalmology: Blue Books of Neurology

67.Hely MA, McManis PG, Doran TJ, et al: Acute optic neuritis: A prospective study of risk factors for multiple sclerosis. J Neurol Neurosurg Psychiatry 1986;49:1125–1130.

68.Frith JA, McLeod JG, Hely M: Acute optic neuritis in Australia: A 13 year prospective study. J Neurol Neurosurg Psychiatry 2000;68:246.

69.Optic neuritis study group: High and low risk profiles for the development of multiple sclerosis within 10 years after optic neuritis. Arch Ophthalmol 2003;121:944–949.

70.Meadows SP: Retrobulbar and optic neuritis in childhood and adolescence. Doyne memorial lecture. Trans Ophthalmol Soc UK 1969;89:603–639.

71.Luccinetti CF, Kiers L, O’Duffy A, et al: Risk factors for developing multiple sclerosis: after childhood optic neuritis. Neurology 1997;49:1413–1418.

72.Morrisey SP, Miller DH, Kendall BE, et al: The significance of brain magnetic resonance imaging abnormalities at presentation with clinically isolated syndrome suggestive of multiple sclerosis: A five year follow up study. Brain 1993;116:135–146.

73.O’Riordan JI, Thompson AJ, Kingsley DPE, et al: The prognostic value of brain MRI in clinically isolated syndromes of the CNS. A ten-year follow up. Brain 1998;121:495–503.

74.Brex PA, Ciccarelli O, O’Riordan JI, et al: A longitudinal study of abnormalities on MRI and disability from multiple sclerosis. N Engl J Med 2002;356:158–164.

75.Kutzelnigg A, Lucchinetti C, Stadelman C, et al: Cortical demyelination and diffuse white matter injury in multiple sclerosis. Brain 2005;128:2705–2712.

76.Zeman ASJ, Kidd D, McClean BN, et al: A study of oligoclonal band negative multiple sclerosis. J Neurol Neurosurg Psychiatry 1996;60:27–30.

77.Optic neuritis study group: The predictive value of CSF oligoclonal banding for MS 5 years after optic neuritis. Neurology 1998;51:885–887.

78.Soderstrom M, Jin YP, Hillert J, Link H: Optic neuritis: Prognosis for multiple sclerosis from MRI, CSF and HLA findings. Neurology 1998;50:708–714.

79.Rawson MD, Liversedge LA, Goldfarb G: Treatment of acute retrobulbar neuritis with corticotrophin. Lancet 1966;ii:1044–1046.

80.Bowden AM, Bowden PMA, Friedman AI, et al: A trial of corticotrophin gelatine injection in acute optic neuritis. J Neurol Neurosurg Psychiatry 1974;37:869–873.

81.Gould ES, Bird AC, Leaver PK, McDonald WI: Treatment of optic neuritis by retrobulbar injection of triamcinolone. BMJ 1977;1:1495–1497.

82.Hickman SJ, Kapoor R, Jones SJ, et al: Corticosteroids do not prevent optic nerve atrophy following optic neuritis. J Neurol Neurosurg Psychiatry 2003;74:1139–1141.

83.Optic neuritis study group: A randomised controlled trial of corticosteroids in the treatment of acute optic neuritis. N Engl J Med 1992;326:581–588.

84.Wakakura M, Mashimo K, Oono S, et al: Multicenter clinical trial for evaluating methylprednisolone pulse treatment of idiopathic optic neuritis in Japan. Optic neuritis treatment trial multicenter cooperative research group (ONMRG). Jpn J Ophthalmol 1999;43:133–138.

85.Brusaferri F, Candelise L: Steroids for multiple sclerosis and optic neuritis: A meta-analysis of randomised controlled clinical trials. J Neurol 2000;247:435–442.

86.Kaufman DI, Trobe JD, Eggenberger ER, Whitaker JN: Practice parameter: The role of corticosteroids in the management of acute monosymptomatic optic neuritis: Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2000;54:2039–2044.

lupus erythematosus has led to the availability of a greater range of therapies, particularly in severe cases.

The syndromes of recurrent relapsing bilateral optic neuropathy, including CRION and autoimmune optic neuropathy, respond well to chronic immunosuppression.

Introduction

Subacute optic neuropathies that develop with and without pain may arise in a diverse and wide-ranging series of inflammatory disorders. This chapter makes note of all those disorders in which optic neuropathy may arise, however rare. It will be noted that vasculitic disorders are included. Some optic neuropathies arise as a consequence of ischemia following thrombotic occlusion of the ophthalmic artery or its branches, and it is clear that recovery of visual function under these circumstances may merely be a forlorn hope. Giant cell arteritis is an example. However, other vasculitic disorders may present with symptoms and signs of an optic neuropathy indistinguishable on clinical (and often too on imaging) grounds and prompt treatment of which leads often to a substantial improvement of visual function. Wegener’s granulomatosis, Churg-Strauss syndrome, and lupus are examples of this circumstance. It is important, therefore, to understand that vigorous and urgent investigation of such cases may lead to instigation of treatment sufficiently early to allow a recovery to take place. This is poorly understood and it is hoped that the information included leads to an improvement in the management of these uncommon but important disorders.

The relationship between the ophthalmic and the neurologic complications of these disorders is also underlined; the use by neurologists of an ophthalmic consultation and vice versa as a mandatory collaboration rather than a test under these circumstances is an extremely important message to understand.

Optic Neuritis Associated with Infections

Optic neuritis may complicate a wide variety of infective disorders (Table 7–1). In general, the natural history of the disorder is the same as that of a primary demyelinating optic neuritis, although in the majority of cases a prodrome suggestive of an infective illness is apparent. The optic neuropathy may occur in isolation but may also complicate other neurologic or ophthalmic complications.

VIRAL DISORDERS

More common in children than in adults and more often bilateral, the optic neuritis that complicates viral disorders usually follows a symptomatic infective prodrome some 1 to 3 weeks before. The clinical syndrome is the same as a primary demyelinating optic neuritis, and the disc appearances vary in the same way. Other neurologic

TABLE 7–1 Viral, Bacterial, and Fungal Infections Known to Have Caused Optic Neuritis

CMV, cytomegalovirus; EBV, Epstein-Barr virus; HHV, human herpes virus; HSV, herpes simplex virus; VZV, varicella zoster virus.

complications may arise simultaneously, such as ataxia and meningoencephalitis. Imaging reveals no abnormalities in the brain when the optic neuropathy occurs on its own but shows white matter lesions when meningoencephalitis occurs at the same time. The cerebrospinal fluid (CSF) shows a modest protein elevation and lymphocytosis in most cases. Recovery is usually good, although the discs are seen to become pale.1 Some advocate treatment with corticosteroids,2 although there is no evidence that recovery is enhanced were this to be undertaken.

Table 7–2 summarizes the principle viral infections that are associated with neuro-ophthalmic complications. It is not an exhaustive summary, and interested readers are strongly urged to read the superb chapter by Paul Brazis and Neil Miller in Walsh and Hoyt’s Clinical Neuro-Ophthalmology3 for a comprehensive and clear account of all the known neurologic, neuro-ophthalmic, and ophthalmologic complications of viral infections.

Human Immunodeficiency Virus Infection

Human immunodeficiency virus (HIV) infection may cause a range of ocular and neurologic complications by virtue of the resultant immunosuppression leading to the acquisition of infection. Primary complications in the eye include a retinal microangiopathy leading to cotton wool spots and microaneurysm formation leading to retinal hemorrhage. A retinopathy in which slowly progressive enlarging focal field defects associated with abnormalities of the pattern electroretinogram (ERG) may also occur.

Optic neuropathy resulting from a direct toxic effect of HIV-1 on retinal ganglion cells and optic nerve axons is rare and associated with a progressive decline in visual function that is commonly unresponsive to treatment, although Newman and Lessell5 reported two patients in whom a diagnosis of HIV-associated optic neuropathy made an incomplete response to zidovudine (AZT) and Sweeney and colleagues6 reported a similar case in whom a unilateral optic neuropathy arose and enhancement of the optic nerve was seen at magnetic resonance

imaging (MRI). The patient made an incomplete recovery without treatment. Other cases in which a response to corticosteroids7 and to highly active antiretroviral treatment (HAART)8 occurred have also been reported. In a recent series from the Democratic Republic of Congo,9 optic neuropathy was seen in 52 of 166 patients studied, of whom 25 showed signs of neuroretinitis, 16 papillitis, and 11 retrobulbar optic neuropathy. In this series, the majority were found to have infective causes, including toxoplasmosis (17/25 cases of neuroretinitis were associated with this infection), cryptococcus, tuberculosis, and varicella. Seven cases showed no evidence for an alternative cause to HIV itself: 4% of the study population. Abnormalities of the visual evoked potential were seen in 42% of HIV infected patients without neurologic complications, suggesting that subclinical involvement of the anterior visual pathway is common. This may reflect axonal degeneration in optic nerve fibers resulting from viral proteins and to neurotoxic factors particularly tumor necrosis factor alpha (TNFa).10

There is also a relationship between the manifestation of Leber’s hereditary optic neuropathy and HIV infection,11,12 particularly presenting at the time of

commencement of antiretroviral treatment (see Chapter 8).

BACTERIAL DISORDERS

The optic neuropathy that may arise with bacterial and fungal disorders is a result of direct infection of the optic nerve during meningitis or during compression from an abscess, from compression itself, or from an infective endarteritis. In meningitis, an optic neuropathy may arise in the syndrome of optic perineuritis (see later). The development of hydrocephalus or raised intracranial pressure resulting from abscess formation or venous sinus thrombophlebitis may also lead to optic neuropathies.

Many case reports exists in the literature that suggest that, although rare, many bacterial infections may be associated with isolated unilateral or bilateral optic neuritis, including Shigella, Salmonella, and Francisella tularensis (Table 7–1). Those more commonly associated with optic neuritis are noted later.

Mycoplasma infection has been associated with a bilateral optic neuritis in a few published reports, usually in association with other neurologic complications, such as acute disseminated encephalomyelitis (ADEM), meningitis, brainstem encephalitis, and acute polyradiculoneuropathy.13,14 An optic perineuritis (see later) is more common than optic neuritis itself. Recovery tends to be good in time.

Brucella infection of the nervous system can be associated with meningoencephalitis, radiculopathies, and cranial neuropathies, including an optic neuritis that appears to be similar to noninfectious optic neuropathies.15,16 Neurobrucellosis can also cause a syndrome of intracranial hypertension, so the optic neuropathy may arise as a result of this.

Patients with syphilis may have a whole host of neuro-ophthalmologic complications, including an acute optic neuritis or perineuritis, neuroretinitis, and papilledema as a consequence of meningitis or choroidoretinitis.17 Symptoms tend to arise in the secondary stages of the disease and in children with congenital infection. The ophthalmic complications include a keratitis, iridocyclitis with pupillary abnormalities, uveitis, and chorioretinitis. Optic atrophy is commonly seen in the tertiary phase, both in tabes dorsalis and in general paresis.

Another spirochete, Borrelia burgdorferi, causes Lyme disease and is associated with neurologic complications in 5% of cases,18 including isolated cranial neuropathies, meningitis, and polyradiculopathies. Optic neuritis and neuroretinitis may occur but are rare.19

In tuberculosis an optic neuropathy may arise as a result of infiltration of the optic nerve or, more commonly, the chiasm by granulomatous inflammation,20 by compression from a tuberculoma21 or complicating uveitis, and during treatment with ethambutol, particularly in those with renal impairment. Neuroretinitis has also been reported. The ocular manifestations consist of a chronic bilateral granulomatous uveitis, but posterior involvement leading to choroidoretinitis and choroidal tubercles may also occur.22

Leptospira has also been reported to be associated with the development of an isolated optic neuritis23 alongside meningitis, uveitis, and interstitial keratitis. These neuro-ophthalmic complications tend to occur only in conjunction with meningitis.

Bartonella henselae causes cat scratch disease and is a common cause of neuroretinitis24; it may be associated with other ophthalmic complications such as pars planitis, branch arteriolar or venular occlusions, focal choroiditis, serous retinal detachments, focal retinal vasculitis, and white spot syndrome. Most patients with the infection develop a mild illness with regional lymphadenopathy, but the incidence of other complications is about 2%,25 including pneumonia, lytic bone lesions, organ enlargement, and the neurologic and ophthalmic complications. Meningitis, encephalitis, and isolated cranial neuropathies have all been reported.26

Although Whipple’s disease may present with neurologic symptoms resulting from meningoencephalitis and the distinctive oculomasticatory myorhythmia,27 optic neuritis has not been reported. Ocular manifestations include a chronic anterior uveitis, vitreous opacities, and choroidoretinitis.28

FUNGAL DISORDERS

Optic neuropathy may complicate meningitis resulting from a variety of molds and yeasts whose prevalence varies throughout the world. The prevalence of these disorders increases in immunocompromised or immunosuppressed patients with diabetes, lymphoreticular disorders, or acquired immunodeficiency syndrome (AIDS). Many of the organisms can cause a meningitis or cerebral abscess, for example, mucormycosis, histoplasmosis, and candidiasis.

Aspergillus

This organism is found throughout the world and is acquired by inhalation of airborne spores. Optic neuropathy may arise when an aspergilloma develop within a nasal sinusitis in which the infective material passes out of the ethmoid or sphenoid sinuses into the extradural space. Patients therefore present with an optic neuropathy that is either subacute or progressive, often painful with other signs of an orbital apex syndrome. Invasive aspergillosis can arise in severely immunocompromised patients with evidence for systemic disease, meningitis, and arteritis. It may occasionally arise in nonimmunocompromised patients, and the prognosis can be very poor.29