Neuro-ophthalmologic manifestations of rheumatologic and associated disorders

Inflammatory diseases affecting the eye, the orbital content, or the endocranium are various. The clinical or radiologic presentation are multiple and their diagnosis can be a real challenge. Most relevant studies or case reports on systemic lupus erythematosus, Wegener's granulomatosis, Churg-Strauss syndrome, giant cell arteritis, Beh?et's disease, Takayasu's arteritis, relapsing polychondritis, or inflammatory pseudotumor of the orbit are summarized in the present review.     

Neuro-ophthalmologic manifestations of epilepsy

This review summarizes the major neuro-ophthalmologic manifestations of epilepsy. Positive or negative visual manifestations such as hallucinations or visual loss may be seen. There is considerable overlap of the visual manifestations with migrainous aura and transient ischemic attack (TIA), making a detailed history important for accurate diagnosis. Oculomotor manifestations, such as tonic eye deviation, are often recognized to have localizing value. Nystagmus or repetitive blinking may be present and offer clues in clinically subtle seizures.     

Neuro-ophthalmologic manifestations of Lyme disease

Lyme disease is a tick-borne spirochetal infection characterized by skin rash, neurologic, cardiac, and arthritic findings. The authors report six patients with Lyme disease who had neuro-ophthalmologic manifestations. One patient had meningitis with papilledema, two had optic neuritis, and one had neuroretinitis. Three patients had sixth nerve paresis, two of whom cleared quickly, whereas multiple cranial nerve palsies and subsequent optic neuropathy developed in another. Early recognition of neuro-ophthalmologic findings can help in the diagnosis and treatment of Lyme disease.     

Neuro-ophthalmologic manifestations of systemic disease and neurologic disease

Neuro-ophthalmology is a difficult and challenging field for both ophthalmologists and neurologists. Unfortunately, signs and symptoms of these patients can be very subtle and easily overlooked. Although neuro-ophthalmologic cases are rare in the practice of a general ophthalmologist, missing the diagnosis can have a severe impact on the patient's health. This paper reviews the past year's publications of neuro-ophthalmologic manifestations of systemic and neurologic disease.     

Neuro-ophthalmologic manifestations of systemic disease and neurologic disease

Neuro-ophthalmology is a difficult and challenging field for ophthalmologists, orthoptists, and neurologists. Unfortunately, signs and symptoms of these patients can be very subtle and easily overlooked. Although neuro-ophthalmologic cases are rare in the practice of a general ophthalmologist, missing the diagnosis can have severe impact on the patient's health. The authors review last year's publications of neuro-ophthalmologic manifestations of systemic and neurologic disease.     

Neuro-ophthalmologic manifestations of systemic and neurologic disease.

Neuro-ophthalmology is a difficult and challenging field for both ophthalmologists and neurologists. Unfortunately, signs and symptoms of these patients can be very subtle and easily overlooked. Although neuro-ophthalmologic cases are rare in the practice of a general ophthalmologist, missing the diagnosis can have a severe impact on the patient's health. This paper reviews last year's publications on neuro-ophthalmologic manifestations of systemic and neurologic disease.     

Neuro-ophthalmologic manifestations of systemic disease: rheumatologic/Inflammatory

Rheumatologic and inflammatory systemic diseases often cause similar neurologic and neuro-ophthalmologic manifestations. A wide variety of conditions can be included in these categories. This article summarizes the most common of these conditions, including sarcoidosis, idiopathic orbital inflammation, inflammatory bowel disease, Wegener's granulomatosis, polyarteritis nodosa, Churg-Strauss syndrome, Behcet's disease, systemic lupus erythematosis, scleroderma, Sjogren's syndrome, and rheumatoid arthritis.     

Ocular manifestations of metabolic disorders

Articles published during the past year on the ocular manifestations of metabolic diseases and related issues are reviewed. The focus is on clarifying the genetic or molecular basis of various metabolic disorders. Mutations of the P gene were reported in tyrosinase-positive oculocutaneous albinism and autosomal recessive ocular albinism, and were associated with a wide range of clinical phenotypes. This finding should aid in more accurate diagnosis and facilitate genetic counseling. There is no consensus but the horizontal supranuclear gaze palsy in Gaucher's disease may aid in subtyping. The report of a family with Morquio syndrome suggests that lenticular opacities should be considered as an additional finding in this syndrome. Patients with nephropathic cystinosis, which had been a fatal disease in childhood, are now surviving to adulthood. Serious ocular complications were described in adults.     

Ocular manifestations of metabolic disorders

Articles published during the past year on the ocular manifestations of metabolic disorders and related issues are reviewed. Fewer articles on this topic were available this year than previous years. Ornithine-delta-amino transferase-deficient mice were produced by gene targeting in the hope of creating an animal model for gyrate atrophy. The mice developed unexpected hypoornithinemia in the neonatal period and died 24 to 48 hours after birth. One human infant also had hypoornithnemia without serious symptoms. Both mice and human develop hyperornithinemia later. Arginine supplementation rescued the mice, but they developed central retinal degeneration by 7 months. Coexistence of autosomal dominant congenital or early onset cataract and hyperferritinemia, not related to iron overload, was discovered in three pedigrees, two Italian and one not mentioned, by two different groups. Mutations of the ferritin L-subunit gene in the iron-responsive element were identified, with autosomal dominant inherited cataract associated with hyperferritinemia.     

Ocular manifestations of metabolic disorders

Major advances in the molecular basis of oculocutaneous and ocular albinism have been published. In addition to mutations of the P gene, some patients, with so-called autosomal recessive ocular albinism, were found to have a tyrosinase gene mutation in one allele and a nucleotide substitution of the same gene, which was considered as a polymorphism, in another allele. This finding has great impact on the diagnosis and genetic counseling of albinism. Flash visually evoked potentials may be a useful tool to show the asymmetry pattern in albino patients especially in infants. Some reports suggested that there may be an overlap between corneal amyloidosis and other forms of corneal dystrophy. Lattice dystrophy, granular dystrophy, and Avellino (granular-lattice) dystrophy were mapped on the same locus of chromosome 5q. Histopathologic findings supported that these different phenotypes were derived from defects of the same gene. Gelatinous droplike dystrophy and spheroidal band-shaped degeneration were also suggested to be allelic disorders by clinical and histopathological findings.     

Ocular manifestations of rheumatologic disorders

In the past year, advances have been made in our understanding of the genetics of Beh?et disease. Indocyanine green angiography has allowed us to look at the pathologic changes seen in Beh?et disease and sarcoidosis in more detail. New steroid-sparing treatment strategies for the control of ocular inflammatory disease are becoming better understood and better used, and their applications in sarcoidosis and juvenile chronic arthritis are reviewed here. The role of antiphospholipid antibodies in ocular disease has received attention, particularly in the context of systemic lupus erythematosus. Outcomes in HLA-B27-positive patients with uveitis and in ocular disease associated with the systemic vasculitides are discussed here, and scalp necrosis in giant-cell arteritis is reviewed.     

Neuro-ophthalmologic complications of cataract surgery

Neuro-ophthalmologic complications from cataract surgery are uncommon and include central nervous system toxicity, binocular diplopia, traumatic optic neuropathy and ischemic optic neuropathy. Retrobulbar blocks may be accidentally injected into the subarachnoid space with diffusion to the brainstem. This leads to cardiovascular, respiratory, and mental status compromise. Most patients have complete recovery with adequate support. Post-operative, binocular diplopia may occur secondary to anisometropia or previously unrecognized misalignment. Periocular injection may cause paresis or fibrosis of extraocular muscles. Anterior or posterior ischemic optic neuropathy can occur in the first 6 weeks after cataract surgery with or without periocular injection. The risk to the other eye is high with subsequent contralateral cataract extraction. Post-operative vision loss associated with direct traumatic needle injury is recognized immediately. Therefore, an orbital MRI may be warranted for a patient with an optic neuropathy in the first 24 hours after cataract surgery using periocular anesthesia. If evidence of needle injury is present on neuroimaging, a trial of steroids should be considered.     

Neuro-ophthalmologic complications of cataract surgery

Neuro-ophthalmologic complications from cataract surgery are uncommon and include central nervous system toxicity, binocular diplopia, traumatic optic neuropathy and ischemic optic neuropathy. Retrobulbar blocks may be accidentally injected into the subarachnoid space with diffusion to the brainstem. This leads to cardiovascular, respiratory, and mental status compromise. Most patients have complete recovery with adequate support. Post-operative, binocular diplopia may occur secondary to anisometropia or previously unrecognized misalignment. Periocular injection may cause paresis or fibrosis of extraocular muscles. Anterior or posterior ischemic optic neuropathy can occur in the first 6 weeks after cataract surgery with or without periocular injection. The risk to the other eye is high with subsequent contralateral cataract extraction. Post-operative vision loss associated with direct traumatic needle injury is recognized immediately. Therefore, an orbital MRI may be warranted for a patient with an optic neuropathy in the first 24 hours after cataract surgery using periocular anesthesia. If evidence of needle injury is present on neuroimaging, a trial of steroids should be considered.     

Neuro-ophthalmologic presentations of hemicrania continua

PURPOSE: We describe a series of patients with hemicrania continua, a rare indomethacin-responsive primary headache syndrome, who presented for evaluation with neuro-ophthalmologic signs and symptoms. DESIGN: Observational case series. METHODS: Nine patients between the ages of 29 and 58 years were seen with various neuro-ophthalmologic findings and a unilateral continuous headache. A detailed history was taken from each patient, followed by a focused ophthalmologic and neurologic examination. The risks and benefits of treatment with indomethacin were discussed. Patients were instructed to call after several days of treatment to report any change in their headache and neuro-ophthalmologic symptoms, in addition to any adverse side effects. RESULTS: All patients responded favorably to indomethacin, with rapid near-complete or complete resolution of headache and autonomic symptoms, and treatment was initiated as early as possible. CONCLUSIONS: Because of its absolute response to indomethacin, recognizing the neuro-ophthalmologic symptoms of hemicrania continua as a component of the headache syndrome is critical for prompt initiation of treatment.