Subacute myelo-optic neuropathy | |
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Specialty | Neurology |
Subacute myelo-optic neuropathy (SMON) is an iatrogenic disease of the nervous system [1] leading to a disabling paralysis, blindness and even death. [2] Its defining manifestation was as an epidemic in Japan during the 1960s: the Japanese government estimated 11,000 were affected; however, the College of Medicine at the University of Tokyo put the number at 30,000, citing a lack of preservation of medical records for longer than five years and a lack of cooperation from doctors as the reasons for the discrepancy. [3] On August 3, 1978, the Tokyo District Court ruled that the cause of SMON is Clioquinol. Its manufacturer, Ciba-Geigy, has publicly stated that "Medical products manufactured and sold by us have been responsible for the occurrence of [SMON] in Japan, we extend our apologies." [3]
SMON was first observed and diagnosed in Sweden 1966, by the pediatrician and neurologist Olle Hansson. [4] Clioquinol was marketed as a prophylaxis to tourist diarrhoea. Dr. Olle Hansson was in the front line, fighting for a ban of clioquinol. Doctors in many countries boycotted Ciba-Geigy for many years. Not until 1985 was the pharmaceutical withdrawn. Dr Hansson died a few months later. The day of his death, May 23, is observed as the Anti-Hazardous Drug Day in several parts of the world.[ citation needed ]
Optic neuritis describes any condition that causes inflammation of the optic nerve; it may be associated with demyelinating diseases, or infectious or inflammatory processes. It is also known as optic papillitis, neuroretinitis when there is a combined involvement of optic disc and surrounding retina in the macular area and retrobulbar neuritis. It is most often associated with multiple sclerosis, and it may lead to complete or partial loss of vision in one or both eyes. Other causes include:
Novartis International AG is a Swiss multinational pharmaceutical company based in Basel, Switzerland. It is one of the largest pharmaceutical companies in the world.
Idebenone is a drug that was initially developed by Takeda Pharmaceutical Company for the treatment of Alzheimer's disease and other cognitive defects. This has been met with limited success. The Swiss company Santhera Pharmaceuticals has started to investigate it for the treatment of neuromuscular diseases. In 2010, early clinical trials for the treatment of Friedreich's ataxia and Duchenne muscular dystrophy have been completed. As of December 2013 the drug is not approved for these indications in North America or Europe. It is approved by the European Medicines Agency (EMA) for use in Leber's hereditary optic neuropathy (LHON) and was designated an orphan drug in 2007.
Leber's hereditary optic neuropathy (LHON) is a mitochondrially inherited degeneration of retinal ganglion cells (RGCs) and their axons that leads to an acute or subacute loss of central vision; it predominantly affects young adult males. LHON is transmitted only through the mother, as it is primarily due to mutations in the mitochondrial genome, and only the egg contributes mitochondria to the embryo. LHON is usually due to one of three pathogenic mitochondrial DNA (mtDNA) point mutations. These mutations are at nucleotide positions 11778 G to A, 3460 G to A and 14484 T to C, respectively in the ND4, ND1 and ND6 subunit genes of complex I of the oxidative phosphorylation chain in mitochondria. Men cannot pass on the disease to their offspring.
Clioquinol (iodochlorhydroxyquin) is an antifungal drug and antiprotozoal drug. It is neurotoxic in large doses. It is a member of a family of drugs called hydroxyquinolines which inhibit certain enzymes related to DNA replication. The drugs have been found to have activity against both viral and protozoal infections.
Posterior ischemic optic neuropathy (PION) is a medical condition characterized by damage to the retrobulbar portion of the optic nerve due to inadequate blood flow (ischemia) to the optic nerve. Despite the term posterior, this form of damage to the eye's optic nerve due to poor blood flow also includes cases where the cause of inadequate blood flow to the nerve is anterior, as the condition describes a particular mechanism of visual loss as much as the location of damage in the optic nerve. In contrast, anterior ischemic optic neuropathy (AION) is distinguished from PION by the fact that AION occurs spontaneously and on one side in affected individuals with predisposing anatomic or cardiovascular risk factors.
SMON may stand for:
Sensory ataxia is both a symptom and a sign in neurology. It is a form of ataxia caused not by cerebellar dysfunction but by loss of sensory input into the control of movement.
Neuritis is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired conduction of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.
Frank Henry Netter was an American surgeon and medical illustrator. The first edition of his Atlas of Human Anatomy — his "personal Sistine Chapel" — was published in 1989; he was a fellow of the New York Academy of Medicine where he was first published in 1957.
Optic neuropathy is damage to the optic nerve from any cause. Damage and death of these nerve cells, or neurons, leads to characteristic features of optic neuropathy. The main symptom is loss of vision, with colors appearing subtly washed out in the affected eye. On medical examination, the optic nerve head can be visualised by an ophthalmoscope. A pale disc is characteristic of long-standing optic neuropathy. In many cases, only one eye is affected and patients may not be aware of the loss of color vision until the doctor asks them to cover the healthy eye.
Nitroxoline is an antibiotic that has been in use in Europe for about fifty years, and has proven to be very effective at combating biofilm infections. Nitroxoline was shown to cause a decrease in the biofilm density of P. aeruginosa infections, which would allow access to the infection by the immune system in vivo. It was shown that nitroxoline functions by chelating Fe2+ and Zn2+ ions from the biofilm matrix; when Fe2+ and Zn2+ were reintroduced into the system, biofilm formation was reconstituted. The activity of biofilm degradation is comparable to EDTA, but has a history of human use in clinical settings and therefore has a precedent with which to allow its use against “slimy” biofilm infections.
Iatrogenesis is the causation of a disease, a harmful complication, or other ill effect by any medical activity, including diagnosis, intervention, error, or negligence. First used in this sense in 1924, the term was introduced to sociology in 1976 by Ivan Illich, alleging that industrialized societies impair quality of life by overmedicalizing life. Iatrogenesis may thus include mental suffering via medical beliefs or a practitioner's statements. Some iatrogenic events are obvious, like amputation of the wrong limb, whereas others, like drug interactions, can evade recognition. In a 2013 estimate, about 20 million negative effects from treatment had occurred globally. In 2013, an estimated 142,000 persons died from adverse effects of medical treatment, up from an estimated 94,000 in 1990.
Sohan Singh Hayreh is an ophthalmologist, clinical scientist, and professor emeritus of ophthalmology at the University of Iowa. As one of the pioneers in the field of fluorescein angiography, he is generally acknowledge to be a leading authority in vascular diseases of the eye and the optic nerve. For over 60 years, Hayreh has been actively involved in basic, experimental, and clinical research in ophthalmology, publishing over 400 original peer-reviewed articles in various international ophthalmic journals, 6 classical monographs and books in his field of research, and more than 50 chapters in ophthalmic books. He has made many seminal observations dealing with the ocular circulation in health and disease, the optic disc and the optic nerve, retinal and choroidal vascular disorders, glaucomatous optic neuropathy, fundus changes in malignant arterial hypertension, ocular neovascularization, rheumatologic disorders of the eye, and nocturnal arterial hypotension. He is an elected fellow of the National Academy of Medical Sciences.
Behçet's disease is recognized as a disease that cause inflammatory perivasculitis, inflammation of the tissue around a blood or lymph vessel, in practically any tissue in the body. Usually, prevalent symptoms include canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In addition, patients experience severe headache and papulopustular skin lesions as well. The disease was first described in 1937 by a Turkish dermatologist, Dr. Hulusi Behçet. Behçet's disease is most prevalent in the Middle East and the Far East regions; however, it is rare in America regions.
Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful mouth sores, genital sores, inflammation of parts of the eye, and arthritis. The sores typically last a few days. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go.
Mitohondrial optic neuropathies are a heterogenous group of disorders that present with visual disturbances resultant from mitochondrial dysfunction within the anatomy of the Retinal Ganglion Cells (RGC), optic nerve, optic chiasm, and optic tract. These disturbances are multifactorial, their aetiology consisting of metabolic and/or structural damage as a consequence of genetic mutations, environmental stressors, or both. The three most common neuro-ophthalmic abnormalities seen in mitochondrial disorders are bilateral optic neuropathy, ophthalmoplegia with ptosis, and pigmentary retinopathy.
Akira Nakajima was a Japanese ophthalmologist.
Klaus von Grebmer, descendant of an old Austrian family, is a Swiss-German economist and one of the pioneers of the Global Hunger Index. He is currently a Research Fellow Emeritus and Strategic Adviser at the International Food Policy Research Institute since 2012. Klaus von Grebmer joined the International Food Policy Research Institute as Director of the Communications Division in 1999. During 2013 von Grebmer served as Acting Director for Communications and Marketing at WorldFish.
John David "Jerry" Spillane (1909–1985) was a Welsh neurologist and a pioneer of tropical neurology.