Neuromyelitis optica has a lower prevalence than idiopathic optic neuritis,and tends to become refractory. Recently,the relationship between anti-aquaporin 4(AQP4)antibody and neuromyelitis optica has been elucidated,which has provided insight into the gradual clarification of therapeutic approaches. Anti-AQP4 antibody-positive optic neuritis has an acute onset,and is generally resistant to steroids,causing diverse visual changes. Astrocytes,which are one type of glial cells,are the target cells. A high female to male ratio(9 : 1)is also a characteristic. On the other hand,in anti-myelin oligodendrocyte glycoprotein(MOG)antibody-positive optic neuritis,oligodendrocytes are the target cells. Like anti-AQP4 antibody-positive optic neuritis,damages tend to extend from the optic nerve to the optic chiasm and optic tract,and therefore manifesting visual changes similar to those of anti-AQP4 antibody-positive optic neuritis. Although the prognosis of anti-MOG antibody-positive optic neuritis is relatively favorable,some patients respond poorly to high-dose steroid therapy and the disease tends to recur. Treatment for both anti-AQP4 antibody-positive and anti-MOG antibody-positive optic neuritis should start with pulse steroid therapy initially,and switch to plasmapheresis,immunoadsorption or high-dose immunoglobulin therapy when resistance develops.

View full abstract

Thyroid eye disease is an one of autoimmune disease that is developed by hyper-,eu-,and hypothyroid-Graves' disease. Thyrotropin receptor antibodies(TRAb)and thyroid stimulating antibodies(TSAb)are well known as an anti-thyroid autoantibodies, and TSAb is more related to severity and activity of the thyroid eye disease. The treatment of thyroid eye disease is mainly the anti-inflammatory therapy, the normalization of thyroid functions and TRAb for immunological remission. Thyroid eye disease trend to worse in patients with high TRAb levels. MRI is an important examination because it can determine the activity of thyroid eye disease. We present three cases who are progressed the thyroid eye disease before the onset of Graves' disease,developed the thyroid eye disease after isotopetreatment, and relapsedafterthyroidectomy. Although new therapies for the thyroid eye disease have also been verified, the steroid therapy is the mainly treatment for anti-inflammatory and immunosuppression.

View full abstract

Myasthenia gravis(MG)is an autoimmune disease, caused by impaired neuromuscular transmission. Pathogenic autoantibodies are antibodies to acetylcholine receptor(AChR)and muscle-specific tyrosine kinase(MuSK), and recent studies have revealed that MG is also associated with other autoantibodies such as antibodies to striated muscle. A peak age has been identified in the infantile-onset group and another for the elderly-onset of MG. Recently, the incidence in the elderly population has been increasing. Antibodies to AChR and striated muscle are found more often in elderly-onset MG, suggesting that elderly-onset MG may have a different etiology from early-onset MG. Currently, there is no standard evidence-based treatment. The application of immunotherapy, such as corticosteroids, immunosuppressants, and thymectomy,have increased compared with the use of cholinesterase inhibitors duringthe last 20 years.

View full abstract

Fisher syndrome is a disorder characterized by acute onset of ataxia, areflexia, and ophthalmoplegia. It is regarded as a subtype of Guillain-Barré syndrome. Prior infection has previously been implicated, and a high proportion of patients prove positive for anti-GQ1b antibodies. An analysis of patients treated in our institution showed that this condition was more common in young men, and approximately 90% of all patients had a history of prior infection. Ophthalmoplegia rarely shows total extraocular muscle palsy, while abducens palsy accounts for the majority of cases. The mean time required for recovery from diplopia was approximately 70 days. GQ1b antigens are believed to be localized in large quantities around the nodes of Ranvier and Schwann cells of the extramedullary parts of the abducens, trochlear, and oculomotor nerves. Anti-GQ1b antibodies bind to these antigens, causing impaired transmission, which results in the onset of nerve paralysis. Recently, it was shown that antibodies to a ganglioside complex consisting of two different gangliosides, rather than a single ganglioside, are implicated in its onset. With an etiology based on the molecular mimicry hypothesis and the complement system has been reported, Fisher syndrome is becoming understood at the molecular genetic level.

View full abstract

We report a case diagnosed with acute disseminated encephalomyelitis(ADEM)presenting as papilledema followed by optic neuritis. A 7-year-old girl who had not recovered from mycoplasma sinusitis and complained of diplopia was referred to our hospital. Visual acuity was good, but bilateral optic disc edema was observed. No abnormal findings were found on MRI. Cerebrospinal fluid examination showed increased pressure and pleocytosis. She was hospitalized with a diagnosis of papilledema due to intracranial hypertension. The symptoms were improved with diuretics, but an acute bilateral loss of vision developed with relative afferent pupillary defect,bilateral optic disc erythema, and edema. Subsequent MRI findings and clinical course met the criteria for ADEM. A diagnosis of bilateral optic neuritis due to ADEM was made. Following steroid pulse therapy, her vision returned to normal. This case indicates that the initial episode of optic disc edema was papilledema caused by meningitis and that the second episode was optic neuritis caused by ADEM. ADEM is an acute demyelinating disorder of the central nervous system, which follows certain viral infections or vaccinations. It is very rare that the two different clinical episodes in this case occur successively. Distinctive brain MRI findings helped with the diagnosis of ADEM and with its follow-up. The case presented here is a good example of the consideration that should be paid to the relation between clinical course and MRI findings.

View full abstract

We report a case of orbital myositis combined with optic neuropathy. A 65-year old woman complained of lid swelling and visual disturbances in her right eye. Corrected vision in this eye was 0.6 and the critical flicker fusion frequency was slightly decreased. MRI showed swelling and inflammation of the right medial and superior rectus muscles and of the superior oblique muscle. The medial rectus muscle pressed against the optic nerve, and there was inflammation around the optic nerve. Based on these findings, we diagnosed the patient with orbital myositis combined with optic neuropathy. Methylprednisolone of 80 mg per day was given and the dose was then gradually reduced. Methylprednisolone of 10 mg per day was given for about four months. Vision in the right eye improved immediately, and the swelling of the right extraocular muscles decreased at two weeks compared with that before medication. Optical neuropathy may have occurred by the mechanical compression due to the swelling orbital muscles and the inflammatory involvement from these muscles.

View full abstract

A 43-year-old male, who was blinded in his righteye in a traffic accident10 years earlier,complained of visual disturbances in his left eye. He was initially diagnosed with psychogenic visual impairment in the left eye caused by the stress of a divorce. Two years later, he visited our clinic again with a left orbital fracture, having been punched in the left eye during a dispute with a customer, and complained once again of visual loss in his left eye. No accompanying organic lesions were found. He persistently demanded a visual handicap certification. MRI showed diffuse brain atrophy, and intelligence tests revealed decreased impressibility. We suspect that his psychogenic visual impairment became malingering as time passed. The higher brain dysfunction might have been overlooked for 10 years, which made difficult to diagnose this case.

View full abstract

We reviewed the evolution of three journals dedicated to neuro-ophthalmology, Neuro-ophthalmology, Journal of Clinical Neuro-ophthalmology/Journal of Neuro-ophthalmology, and Neuro-ophthalmology Japan, witha particular focus on the first issue of publication, and performed bibliometric and citation analyses of full articles using the Web of Science to reveal the trends in research in the field over the past thirty years.

View full abstract

Leber's hereditary optic neuropathy(LHON)is a mitochondrial genetic disease characterized by the selective loss of retinal ganglion cells, the degeneration of optic nerve axons, and visual loss. LHON occurs most frequentry in young males. There have been remarkable advances in our understanding of the clinical features, genetics, biochemical effects, and even the pathophysiology of LHON in past two decades. However, the potential therapies for LHON and other mitochondrial disorders are still under investigation. This instruction course provides an update on the recent advances in various therapies, such as quinone derivatives or gene therapy, for patients with LHON.

View full abstract