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Retinal degeneration (rhodopsin mutation)
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Fundus images of retinitis pigmentosa

Retinal degeneration is the deterioration of the retina(1) caused by the progressive and eventual death of the cells of the retina.(2) There are several reasons for retinal degeneration, including artery or vein occlusion, diabetic retinopathy, R.L.F./R.O.P. (retrolental fibroplasia/ retinopathy of prematurity), or disease (usually hereditary).(3) These may present in many different ways such as impaired vision, night blindness, retinal detachment, light sensitivity, tunnel vision, and loss of peripheral vision to total loss of vision(4). Of the retinal degenerative diseases retinitis pigmentosa (RP) is a very important example. Inherited retinal degenerative disorders in humans exhibit genetic and phenotypic heterogeneity in their underlying causes and clinical outcomes* (5) (6) (7). These retinopathies affect approximately one in 2000 individuals worldwide (7). A wide variety of causes have been attributed to retinal degeneration, such as disruption of genes that are involved in phototransduction, biosynthesis and folding of the rhodopsin molecule, and the structural support of the retina.(6) Mutations in the rhodopsin gene account for 25% to 30% (30% to 40% according to(9)) of all cases of autosomal dominant retinitis pigmentosa (adRP) (5) (20– 22) in North America (10–12) There are many mechanisms of retinal degeneration attributed to rhodopsin mutations or mutations that involve or affect the function of rhodopsin. One mechanism of retinal degeneration is rhodopsin overexpression. Another mechanism, whereby a mutation caused a truncated rhodopsin, was found to affect rod function and increased the rate of photoreceptor degeneration.(9)

*For example, a single peripherin/RDS splice site mutation was identified as the cause of retinopathy in eight families; the phenotype in these families ranged from retinitis pigmentosa tomacular degeneration.(7)

Photoreceptor cell death[edit]

Illustration of the structure of the mammalian retina, including photoreceptor cells ; rod cells and cone cells

Photoreceptor cell death is the eventual outcome of retinal degeneration. Without proper function of the photoreceptor cells, vision is not possible. Irreversible loss of these cells has been attributed as a cause of blindness in many retinal degenerative disorders, including RP. The exact mechanism of photoreceptor cell death is not clearly understood. (5)(14) Among potential causes is the endocytosis of stable complexes formed between rhodopsin and its regulatory protein arrestin in certain mutants. (5) Various studies have also documented that over-expression of rhodopsin itself (mutations in genes involved in the termination of rhodopsin signaling activity have been shown to cause degeneration by persistent activation of the phototransduction cascade (14)) causes photoreceptor cell death and may induce photoreceptor cell loss in transgenic animals expressing truncated rhodopsin. Yet another mechanism may be prolonged photoreceptor responses and also abnormal rhodopsin deactivation may induce outer segment shortening and eventual photoreceptor death (9) In RP photoreceptor cell death is believed to occur by programmed cell death or apoptosis. (10) (11) (15)

Retinitis pigmentosa[edit]

Photograph of United States Supreme Court building illustrating normal visual field, prior to onset of retinitis pigmentosa

Photograph of the United States Supreme Court Building, modified to illustrate the effect of visual field loss, tunnel vision, in patients with Retinitis Pigmentosa.

Retinitis pigmentosa is a progressive neurodegenerative disorder(16), which affects 1 in 3,000 individuals (6) and affects between 50000 and 100000 people in the United States.3 Autosomal dominant RP accounts for approximately 15% of these cases. (8) Autosomal dominant retinitis pigmentosa (ADRP) is a genetically heterogeneous group of inherited retinal degenerations that cause blindness in humans. (14) RP begins with death of rod photoreceptor cells, which are the only cells in the retina to express rhodopsin and which express it as their most abundant protein. Eventually, loss of rod cells leads to loss of cone cells(cone photoreceptors), the mainstay of human vision (16). Symptoms of RP include loss of sensitivity to dim light, abnormal visual function, and characteristic bone spicule deposits of pigment in the retina. Affected individuals progressively lose visual field and visual acuity, and photoreceptor cell death can ultimately lead to blindness. (9) A prominent early clinical feature of retinitis pigmentosa is the loss of night vision as a result of death of rod photoreceptor cells. Proper expression of the wild-type rhodopsin gene is essential for the development and sustained function of photoreceptor cells.(10) Mutations in the human rhodopsin that affect its folding, trafficking and activity are the most commonly encountered causes of retinal degeneration in afflicted patients. A single base-substitution at the codon position 23 in the human opsin gene (P23H) is the most common cause of ADRP in American patients. (6)(17) ADRP due to rhodopsin mutations has a wide range of clinical presentation

and because defects in the rhodopsin gene are the most common cause of the most common inherited blinding disease.and severity. Mutations in the human rhodopsin that affect its folding. Before 1991. rhodopsin folding. and rhodopsin endocytosis. phenotypic evidence pointed to different subsets of ADRP with varying prognoses.(8) Rhodopsin and its function in vision[edit] Three dimensional structure of rhodopsin Rhodopsin is a transmembrane protein (Rh1) that is the primary visual pigment (photopigment) of rod photoreceptors (which are the only cells in the retina to express rhodopsin and which express it as their most abundant protein(16)) and forms an integral part of the visual cascade.(8)(13)(10) It is a Gprotein-coupled receptor activated by light that initiates the phototransduction cascade (visual transduction cascade taking place in photoreceptor rod outer segments (13). (10) The structure and function of rhodopsin and the gene encoding it have been the subjects of intense scrutiny for many years because rhodopsin serves as a useful model for understanding the largest receptor family in the human genome.(8)(10)(20 –22) Over 100 distinct mutations in the light-sensing molecule rhodopsin are known to cause (adRP).(30–34) Molecular classification of ADRP and further sub-classification based on the region of the mutation in the rhodopsin gene allowed better prediction of a particular disease course.) converting light signals to electrophysiologicalsignals in retinal neurons. This photo-activated signal transduction process is essential for vision. (16) (18)(19) Rhodopsin Mutation[edit] The human rhodopsin gene is the locus for numerous alleles linked to the neurodegenerative disease retinitis pigmentosa. the G protein-coupled receptors. (6) A single base-substitution mutation of codon 23 of the rhodopsin gene in which proline is changed to histidine (Pro23His) in the . the prognosis is influenced by the specific mutation itself. trafficking and activity are the most commonly encountered causes of retinal degeneration in patients afflicted with RP. retinitis pigmentosa. (16) Mutations in the rhodopsin gene account for 25% to 30% (30% to 40% according to (9)) of all cases of autosomal dominant retinitis pigmentosa (ADRP). But even within these specific subsets. (10)(24) These mutations affect rhodopsin transport to the outer segments of rod photoreceptor cells. (6)(9)(10)(13)(14) Most of these mutations aremissense mutations affecting single amino acid residues in the rhodopsin protein.

such as those at the Cterminus (sorting signal of rhodopsin) may result from inappropriate intracellular transport of the molecule. where it continues to be the most commonly described gene defect in RP.(14) Rhodopsin C-terminal mutations[edit] A fraction of rhodopsin mutations alter the C-terminal tail of the protein. the rhodopsin protein undergoes endocytosis following activation (6)(44)(45). when taken up during endocytosis these complexes causes photoreceptor cell death. each of which plays a role in terminating rhodopsin activity. P347R.The presence of truncated opsin may impair synaptic transmission or other cellular processes and eventually cause cell death. and V345M.(6) Missense mutations in the opsin gene affecting the R135 and K296 residues of the protein product cause ADRP and result in accumulation of Rhodopsin-Arrestin complexes in the photoreceptor cell (6)(46)(47). Similarly.Arrestin complexes is also reported for mutants of human rhodopsin associated with severe forms of ADRP (6)(46)(47). such as the point mutations P347L. and display endosomal abnormalities. mutations at Arg135 are associated with severe forms of retinitis pigmentosa and exhibit a high affinity for arrestin. Perturbation of endocytic regulation of rhodopsin has deleterious effects on photoreceptor cell physiology. caused light-dependent retinal degeneration.transmembrane. the Pro23His mutation of the rhodopsin gene was reported as the first mutation associated with RP. whereas mutations affecting cytoplasmic domains and retinol binding sites tend to be very severe. the . and an intron splice mutation (N88) is thought to remove the entire C-terminal tail of rhodopsin.(29–36) Intradiscal mutations tend to be less severe.3–5 In addition.human opsin gene accounts for the largest fraction of rhodopsin mutations observed in the United States and is the most common cause of ADRP in American patients. The presence of truncated rhodopsin in the outer segments causes functional abnormalities and localization of rhodopsin in the outer and/or inner segments induces increased photoreceptor cell death (9) Rhodopsin endocytosis[edit] Like many other G protein-coupled receptors.(8)(20 –22)(24–28) Multiple studies have demonstrated that the degree of severity of a given mutation in the rhodopsin gene is based in large part on its position in the rhodopsin molecule —intradiscal.(38) In this research null mutations in the rhodopsinkinase(39) and arrestin(40) genes. The formation of toxic Rhodopsin. or cytoplasmic. As mentioned these complexes have a fatal effect. P347S.(6)(8)(17) In 1990.(9)(41)(42) Data indicate that expression of truncated rhodopsin negatively affects both photoreceptor function and health. two frameshift mutations (fs 341del and fs 341-343del) are predicted to add additional residues to the C terminus. compromising rod cell survival. rhodopsin and its regulatory protein arrestin form stable complexes.(8)(20)(21)(24–26) This mutation has been described only in the United States. Large quantities of mislocalized opsin may decrease the availability of functional proteins in regions where truncated opsin is concentrated.(43) whereas Q344ter results in a C-terminal truncation. (8)(13)(37) Some research has described transgenic mouse mutants that cause degeneration by prolonged activation of the phototransduction cascade. For example. The phenotype of the RP associated with the P23H mutation is characteristically relatively mild but variable. undergo endocytosis. In certain mutants. because the internalized rhodopsin is not degraded in the lysosome but instead accumulates in the late endosomes.(30)(36) The severity of cytoplasmic mutations affecting maintenance of photoreceptor cell polarity. The R135 mutant rhodopsin is noted to form stable complex with arrestin and undergo endocytosis resulting in aberrant endocytic vesicles in HEK cell culture system (47).

wrongdiagnosis.edu/Education/anomalies/Retinal_degeneration. the stable rhodopsin and arrestin complexes are shown to mislocalize and accumulate in the inner segments of rod photoreceptors of the mouse model of ADRP. It was also shown that the internalized rhodopsin in dying photoreceptor cells were not degraded but instead showed characteristics of insoluble proteins. Non-rhodopsin mutations[edit] Mutations non-retina-specific ADRP genes that encode for proteins essential for pre-mRNA splicing may be a major cause of ADRP. (10) References[edit] 1. meaning that rhodopsin (RHO) is among the target splicing substrate genes for PRPF31. it was shown that rhodopsin accumulates in endosomal compartments in these mutants and leads to light-dependent retinal degeneration. Besides this. Daiger SP (1996) Inherited retinal degeneration: exceptional genetic and clinical heterogeneity.optigen.html 3.htm 5. Failure of proper protein degradation and resultant subsequent accumulation of proteins. such as PRPF31 mutations. http://www. Sullivan LS. http://www. including HPRP3. as in the case of rhodopsin-arrestin complexes. This data led to the implication of rhodopsin buildup in the late endosomal system as a novel trigger of death of photoreceptor neurons. This component senses the accumulation of rhodopsin and then engages the proper machinery to execute cell death in the retina.U5 tri-snRNP have been identified. suggesting that lysosomal turnover of rhodopsin is vital in maintaining photoreceptor viability. (10) This shows that non-rhodopsin mutations may also be critical in presentation of retinal degenerative disorders. is a wellrecognized cause of cell death in many neurodegenerative disorders. Proteins required for the formation of stable U4/U6 snRNPs and for assembly of the U4/U6. Thus it can be understood that mutations in PRPF31 can cause alternative. http://www.uwlax. Mol Med Today 2: 380–386 . PRPC8. This abnormal interaction is demonstrated to have pathological consequences in the retina. proper function of these proteins is necessary for spliceosome performance. Thus failure to degrade internalized rhodopsin in a timely manner triggers cell death of photoreceptor neurons. http://bioweb.com/r/retinal_degeneration/symptoms. (10) The rhodopsin transcript is a pre-mRNA splicing substrate affected by PRPF31 protein. It is speculated that a component innate to the endolysosomal system plays a crucial role in regulating the cell death signals emanating from the endosomes. It suggests a mechanism for retinal degeneration caused by non-retinal-specific genes.edu/bio203/s2007/berends_bets/glossary_of_terms.K296E rhodopsin is observed to bind the visual arrestin with high affinity.com/opt9_glossary.htm 2. rhodopsin. potentially non-functional. (6) The precise mechanisms regulating the pro-cell death signaling pathways and their interconnection with endocytosis is not well understood. It was shown that expression of these mutant PRPF31 proteins significantly reduced rhodopsin expression in cultured retinal cells and induced apoptosis of retinal cells. and PRPF31.tsbvi. such as retinitis pigmentosa (6)(48). forms of the rhodopsin protein.htm 4. establishing a link between mutations in proteins involved in pre-mRNA splicing and the expression of a critical retinaspecific gene.(6) Using mutants that are defective in late endosome to lysosome trafficking.

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http://www. The retina is like film. Retinal degenerative diseases affect the delicate layer of tissue that lines the inside back of the eye. which affects an estimated 1. like the iris of the eye. races and ethnicities. if the film is faulty. which is the leading cause of vision loss in Canada and North America.ffb. opens and closes to let in the right amount of light. This very large and diverse group of vision disorders affects young and old and people from many cultures. Juvenile Stargardt disease Usher disease . and age is considered to be the major risk factor for developing this eye disease.is essential for vision.html Retinal Degenerative Diseases Millions of people in North America live with varying degrees of irreversible vision loss because they have an untreatable.5 million people worldwide. blurred or impossible to see. even if a person who develops it has no previous family history of vision loss. Retinitis pigmentosa and other related conditions are inherited genetic conditions. the developed pictures may be distorted. but here are a few of the more common:      Retinitis pigmentosa Choroideremia (affects males) Retinoschisis.the retina . Age-related macular degeneration is distinguished by its prevalence in the senior population. Imagine the eye is like a camera. or age-related macular degeneration. Regardless of the perfection or quality of the rest of the camera. The lens helps to focus light on the film. The shutter.ca/eye_conditions/RD_diseases. degenerative eye disorder like retinitis pigmentosa (RP). The list of inherited retinal dystrophies (degenerations) is very long. This part of the eye -.

Age-related Macular Degeneration (referred to as AMD) usually does not develop until the sixth or seventh decade of life. The study of molecular genetics. This form accounts for 90 percent of Age-related Macular Degenerations. or drusenoid form. the vision directly in front of you. nonexcudative. Certain genes that are necessary for normal vision give faulty messages to the cells in the macula.Age-related and early onset or Juvenile and they will be explained in detail in separate sections. The macula is very small: only about three by five millimetres (about the size of a ladybug) covering about 10 percent of the retina. although early onset cases are becoming more common in patients as young as 40. The word "macula" comes from the Latin for "spot".org/eye-conditions/retinal-degenerative-conditions/amd/ What is Macular Degeneration? Macular Degeneration is the name for several similar conditions that are characterised by a breakdown of the macula. but studies are showing familial patterns of the condition. There are also other aspects of your health that are risk factors for Age-related Macular Degeneration.retina-international. it is the centre portion of the retina that makes central vision. What causes Macular Degeneration? There are two basic types of Macular degeneration . The symptoms of Macular Degeneration. like those of other retinal diseases. Because of the later onset of this disease. What is Dry AMD? Dry AMD is the more common form of Macular Degeneration. it is a condition that is programmed into your cells and not caused by injury or infection or any other external agent. Early onset Macular Degeneration appears to be largely genetic. cell biology. it is difficult to determine if it is inherited. and how one step leads to another. To understand why and how Macular Degenerations occur. that is. biochemistry. It is also referred to as atrophic. scientists must look to a variety of disciplines. possible. indicating that there may be genetic causes.http://www. leading to their progressive degeneration and eventually to vision loss. Increasingly sophisticated research at the cellular level is providing insight into the processes that cells undergo whey they die. and how these and other fields interact with each other has provided an abundance of avenues for researchers to pursue. vary greatly and range in severity from one person to another. these will be discussed later. .

subretinal neovascularisation. or disciform degeneration. It may be some time before the second eye is seriously affected enough for an individual to notice vision problems. However. New fragile blood vessels develop which may leak fluid and blood. resulting in a dark or empty area in the centre of their field of vision. If you experience a sudden vision loss or distortion. new abnormal blood vessels begin to grow beneath the macula. beneath the macula. These changes. Others do notice a sudden loss of vision. It is also called choroidal neovascularisation. . They may also notice distortions of lines and shapes. Often if one eye has dry AMD. The layer of photo receptor cells in the macula begin to atrophy. crooked door frames) or in tests given by the eye doctor. either in everyday objects (e. it is important that you see your eye care professional immediately. exudative form. How are macular degenerations diagnosed? The early signs of Macular Degeneration are usually detectable in a thorough eye exam even before the disease begins affecting vision. the other eye will also show some signs of the condition. there is marked disturbance of vision in the affected eye. and it is the only blood supply for the macula. This causes the retinal layer to blister under the macula. Because Age-related Macular Degeneration could begin in one eye. the opening in the centre of the iris through which light rays enter the eye. the remaining good eye will take over on its own to compensate for vision loss. which help to show the interior of the eyeball through the pupil. in a thin layer of tissue called the choroid. in turn. although peripheral vision and night vision usually remain unaffected. may. or die. People with Macular Degeneration may have blind spots. The choroid is the main source of oxygen and nutrients for the retinal photo receptors.g. and the photo receptor cells to degenerate. result in a distortion of vision that is most apparent when reading. small yellowish deposits. This is the test that people are most familiar with and typically involves a standard eye chart. and then cause the choroid and retina to deteriorate. Tests for Macular Degeneration include:  Acuity tests which measure the accuracy of your reading and perception vision at specific distances in specific lighting situations. as some of the cells break down.Dry AMD is characterised by the build-up of drusen. In wet AMD. Colour vision may also be diminished. The doctor will examine the eye with special lenses. dry AMD does not usually cause total loss of reading vision. What are the symptoms? The most common symptoms are blurring of vision with particular difficulty discerning details. What is Wet AMD? Wet AMD accounts for 10 percent of patients with AMID. both up close and from a distance. At this stage.

light skin and eye colour. your doctor will be better able to determine the health of these cells through your performance on these tests. including the blood vessels in the eyes. which will identify new blood vessel growth and leakage from blood vessels. Both men and women are at risk for this disease. smoking. Check your vision with an Amsler grid The Amsler grid test may be helpful in revealing signs of wet Age-related Macular Degeneration (AMD). Photos are then taken of the retina and macula. There are several types of colour tests which measure various aspects of vision. It is not a substitute for regularly scheduled eye exams. Look at the centre dot at all times. distorted. . The presence of these risk factors in people with AMD has been noted. Are there any specific risk factors related to AMD? As we have seen. which is explained in detail in the section of this booklet describing the different forms of Macular Degeneration. New blood vessel growth is a feature of wet age-related Macular Degeneration. AMD is related to ageing. but the relationship of the various factors to the disease itself has not been systematically studied and it is not clear just why the links are present. Sit at a comfortable reading distance (12” or 30cm) from the grid in a well lit room. These include a history of hypertension (high blood pressure) and/or cardiovascular disease. Information from this test can help the doctor to better understand the current function of your rod cells. Cover one eye with your hand. If you notice any area on the grid that is blurred. but it certainly does not affect all older people. discoloured or missing. you may be exhibiting signs of AMD and should contact your ophthalmologist promptly.  A fluorescein angiogram which allows inner eye structures to be visualised. which are the retinal cells responsible for night vision. There is a growing body of evidence that suggests genetic factors can determine whether or not someone will get AMD. A non-toxic dye is injected into the patient's arm and it moves through the bloodstream. a family history of AMD.  A dark adaptation test which will measure how well your eyes adjust to changes in lighting. Directions: Wear your reading glasses (if you use them). Colour testing which can help to determine the status of your cone cells. hyperopia (farsightedness). Other studies have looked for additional conditions that may be associated with AMD and have found a number of factors besides age that are associated with an increased risk of developing AMD. Since cone cells are the retinal cells that interpret colour. and lens opacities (cataracts).

If I have drusen. regardless of acuity. Most people start accumulating some drusen after the age of 40. How quickly does AMD progress? Most cases of AMD progress very slowly over a period of years. the number of those affected by AMD will increase as well. will I develop AMD? Not necessarily. and many patients retain a reasonable amount of peripheral vision. and hard drusen . is restricted to a 20 degree diameter (10 degree radius). However. Do Macular Degenerations lead to total blindness? Most people with Macular Degeneration do retain some peripheral vision. and it is the major cause of legal blindness in individuals over the age of 65. It is important to remember that your driving affects not only you but other drivers and pedestrians. It is not known how they form but it has been suggested that they are undigested waste products from RPE cells.How common is AMD? Researchers estimate that one in 2000 people in the developed world are affected by AMD. Drusen are deposits that contain complex lipids (fats) and calcium and can accumulate as a person ages. However. round. and that the results of an error can ultimately be life-threatening. Vision may remain stable between annual eye examinations. Among non-diabetics. solid deposits that sit under the RPE without causing structural damage. Many people with mild forms of Macular Degeneration do drive legally. or whose visual field. There are two basic types of drusen: "hard" and "soft". although the presence of drusen may indicate that your eyes are at some risk for developing AMD. The majority of people who experience severe vision loss from AMD have the wet form. As the population enjoys a longer life. Often people with Macular and other retinal degenerations speak of "near miss" accidents that force them to confront their vision loss and acknowledge that it is affecting their driving. if needed) is 20/200 or worse in their better eye. Legally blind individuals are those whose best visual sharpness or acuity (with glasses or contact lenses. Driving is a symbol of independence for many people. wet AMD typically progresses much more rapidly than dry AMD. Hard drusen are small. Each case differs. many will be classified as legally blind". it is the most common problem affecting the retina. and individuals with progressive vision loss may be unwilling to face the fact that their vision impairment may impede save driving. and they learn to optimize the use of their remaining vision. Can people with Macular Degeneration drive? This is a difficult question. It would be best to discuss your visual limitations and their effect on driving with your eye care professional. and do not have problems.

What are retinal detachments and tears? Retinal detachment occurs when the photo receptors (the rods and cones) separate from their underlying tissue layers. particularly if you have a family history of Macular Degeneration. Retinal detachments and tears may be caused by physical injury or may be inherited. you should continue to seek eye care regularly. they are not necessarily a consequence of the disease.alone do not impair vision. Retinitis Pigmentosa is associated with night blindness and a progressive loss of peripheral vision leading to "tunnel" vision. These symptoms are not the same. Are cataracts associated with Macular Degeneration? It is not unusual for an older individual with a Macular Degeneration to develop a cataract. which is a clouding of the lens of the eye. There are many people with drusen in both eyes and no impairment to their vision at all. Is AMD related to other retinal diseases? Age-related Macular Degeneration is just one type of a group of retinal degenerations that are characterised by a breakdown of the photo receptor cells. While cataract surgery cannot improve the vision loss due to retinal degeneration. This causes a loss of vision in the area of the separation. hard drusen may advance to soft drusen. so if your eye doctor tells you there are drusen in your eyes. Whether or not surgery improves the vision often depends on the extent of retinal changes. and contain other cellular substances in addition to lipids. Development of soft drusen may cause the RPE to separate from other eye tissue layers. as well as use an Amsler grid to monitor your vision yourself. . Retinal tears are splits in the retina that may cause vision disturbances. Soft drusen are more likely to be associated with vision loss. involve a larger area under the RPE. Retinal detachments can sometimes be repaired or partially repaired with surgery. Cataracts that significantly interfere with vision can be surgically removed. there is increasing evidence that they are related. For example. it can alleviate the added loss caused by the cataract. it is important to discuss the details of your individual case with an eye care professional. it has been found that different defects of the same gene are responsible for certain forms of RP and Macular Degeneration. Because surgery is not advised for everyone. it is not known who will go on to develop a vision problem and who will not. such as flashes of light or floaters. While AMD is characterised by a loss of central vision. Although they may occur in people with Macular Degenerations. It is important to understand the significance of drusen. but as researchers get closer to uncovering the causes of these conditions. although this does not always happen. They are less uniform. leading to impaired vision. However. However.

AMD presents a special challenge for genetic researchers because it is difficult to determine the exact role of genetics when a disease occurs late in life. occurrence of AMD is being compared in groups of identical and non-identical twins. those with degenerative vision loss in one eye. soft drusen.for example. How can nutrition influence the occurrence of AMD? Researchers found that people who consumed the highest quantity of spinach. there are studies that suggest that adjustments in lifestyle may help reduce the risk of developing AMD. However. In one study. were less likely to have the advanced form of AMD. or positive family history . You can get the best information about the likely pattern of the disease in your own family by consulting with a genetic counsellor or an eye care professional who specializes in hereditary retinal degenerations. A number of research projects are now underway to look specifically at the role of genetics in the development of AMD. They can help you learn how the disease is inherited in your family and the chance of passing it on to your children. each with its own particular variations. a person may not realize his or her vision is impaired until the disease has reached advanced stages. Genes from blood samples of people with Macular Degeneration are being searched for disease-causing changes. compared with people in the study who ate the least amounts of these foods. but an assortment. The findings also suggest that people should not rely on vitamin supplements as . the hereditary pattern of these diseases differs from family to family. at present there is no known method of preventing its occurrence. Regular eye exams may allow for early diagnosis of AMD. Individuals at risk . However. Because it is not the same gene that causes Macular Degeneration in everyone with the condition.Will others in my family be affected? The late onset forms of Macular Degeneration are not known to be inherited in a straightforward pattern such as autosomal dominant or recessive. From these and other studies. Without self-monitoring. indicating that inheritance is involved to some extent. Some surveys have estimated that 15 to 20 percent of AMD patients have one or more first-degree relatives who are also affected. Is there any way to prevent AMD? While research supported by the member organisations of Retina International and other private and government agencies worldwide is adding to new understandings of AMD.should have regular eye examinations by an eye care professional after the age of 50 and self-monitor their vision daily with the use of an Amsler grid. familial patters have been observed. scientists hope to learn more about the relationship between genes and Macular Degeneration. collard greens and other dark green leafy vegetables foods that are rich in carotenoids. although the gene mutation will be consistent within one family.

C. What treatment options are available for AMD? Retina International and its members support extensive multi-disciplinary research programmes in an effort to find the causes prevention. Laser Photocoagulation Laser photocoagulation is a surgical procedure involving the application of a hot laser to seal and halt or slow the progression of abnormal blood vessels. Little is known about what causes the conversion from dry to wet AMD. and possible treatment for Macular Degeneration and other forms of retinal degenerations. minerals and nutrients. Wet AMD At present people with macular degeneration have three possible treatment options: thermal (heat laser).their main source for vitamins. for example. or anti-VEFG drugs. Does smoking influence the onset of AMD? Cigarette smoking has been linked to increased risk of developing AMD. bright sunlight may help to reduce the retinal degeneration due to AMD. hats and visors can help people to protect their eyes from the sun. research related to RP may also benefit those with Macular Degeneration. Listed below are details of some of the latest advances in research and treatments for AMD. Good quality sunglasses. . but instead should eat a balanced diet that includes a wide range of vegetables. is being marketed in Canada as a treatment for dry AMD by OccuLogix Inc. Check back regularly for updates to this website as research studies are published. Researches studying retinal degenerative diseases may contribute to the understanding of others so that. and this is the subject of ongoing research studies. In the 1990's laser treatment was the only therapy available for AMD. very little is available to help patients with atrophic or "dry" AMD to prevent progression to more serious stages of debilitating disease. A blood filtration process. aside from cessation of smoking and a healthy diet of dark green leafy vegetables and fruits supplemented by zinc and antioxidant vitamins (Vitamins E. Dry AMD Although several new drugs are being investigated and approved by regulatory agencies around the world for the treatment of the exudative (wet) type of AMD. Photodynamic Therapy. It is recommended that persons stop smoking to decrease their chance of developing AMD. Information from research projects is shared with others in the field in order to advance the goal of understanding all forms of retinal degeneration. and beta carotene). Does sunlight influence the onset of AMD? Avoiding intense. called Rheopheresis.

because once vision is lost due to of the growth of abnormal blood vessels. pegaptanib sodium (trade name Macugen) is approved for use in Canada. FDA approval came following successful clinical trials demonstrating that the drug reduced vision loss in 70 per cent of clinical trial patients. from Genetech and Novartis. the US Food and Drug Administration (FDA) announced approval of Lucentis (Ranibizumab). . This decision means that treatment with Lucentis will now be widely available in the USA through retinal specialists. Anti-angiogenesis Therapies As of February 2006. Anti-VEGF drugs are delivered directly to the eye by an injection. it cannot be reclaimed by either treatment. This approval is based on the evidence presented from several years of rigorous clinical trials. This treatment does not produce a blind spot on the retina. which effectively makes available in the USA a ground breaking treatment for wet age related macular degeneration. though immediate. Pegaptanib sodium (trade name Macugen) is what researchers call an anti-VEGF drug. a drug which works by targeting the proteins which act to trigger abnormal blood vessel growth and leakage. It therefore reduces damage to normal surrounding tissue and allows the treatment to be given again as needed. Introduction of Lucentis in Europe is expected to follow in the coming year. A scar forms as a result of the treatment. and this scar creates a permanent blind spot in the field of vision. laser photocoagulation seals the choroidal neovascularization (CNV) and inhibits the leaky blood vessels growth.Through the use of a high-energy light that turns to heat when it hits the parts of the retina to be treated. and improve vision in approximately 30 to 40% of trial participants. The United States Food and Drug Administration (FDA) approved Macugen for treatment of neovascular (wet) age-related macular degeneration. some visual distortion will disappear after laser treatment. the United States and Europe. Photo Dynamic Therapy (PDT) Photodynamic Therapy (PDT) (trade name Visudyne) uses a non-thermal (or cold) laser with an intravenous light-sensitive drug to seal and halt or slow the progression of abnormal retina blood vessels. AMD Alliance International (AMDAI) loudly applauded the decision. However. However. in which Lucentis was shown to maintain vision in 95% of trial participants. In many cases. 2006. It is also very encouraging that the drug is effective for all kinds of wet AMD. On June 30. The light is shone directly at the targeted tissue and the drug accumulates in these cells. loss of vision following laser treatment. preventing further vision deterioration. Other anti-VEFG drugs on the horizon include ranubizimab (trade name Lucentis). or in other words. which is repeated every four to six weeks. early diagnosis of AMD is key. is generally less severe than the eventual loss of vision that usually occurs if laser treatment is not done. whether in the early or late stages. Fighting Blindness and the AMD . Vision does not usually improve after laser treatment and may even be somewhat worse. The FDA approval of course only covers the USA.

which bring hope and help to those with macular degeneration. is showing promise. and the other will help to prevent any future developments. Combination treatments pair one or more existing or new AMD treatments to see if the end result might be greater than what could be achieved individually. scientists working on the treatment believe this may be a result of drug delivery problems. On May 24. called angiostatic therapies. Unlike the anti-VEGF treatments. from Alcon Laboratories.3 mg and 1 mg pegaptanib sodium experienced less vision . Inc. Usually the idea is that one kind of treatment will take care of existing AMD in the patient. This class of drugs propose yet another approach to treatment of AMD. One possible angiostatic treatment is anecortave acetate (Retaane).) Pegaptanib Sodium Phase 3 clinical trials for pegaptanib sodium demonstrated that after 1 year of treatment. and safety of anti-vascular endothelial growth factor (VEGF) therapies for use in the treatment of age-related macular degeneration (AMD) were first established by clinical trials of pegaptanib sodium. to the back of the eye. (Macugen. which combine traditional PDT therapy with new drugs to increase the effectiveness of PDT. Position Statement on Avastin (bevacizumab) The role. in this case by administering a type of steroid to stop the abnormal growth of blood vessels in the eye. individuals who were treated with 0. Genentech. efficacy. not the drug itself and are making adjustments. including combination therapies. [OSI] Eyetech/Pfizer) and later by clinical trials for ranibizumab (Lucentis. Combination Therapies Other investigations are also showing promise. Angiostatic Therapies In other research developments. More and more medical practitioners believe that combination methods are the way of the future for wet AMD treatment. from regulatory consideration. the clinical studies submitted with the NDA and other ongoing clinical studies for RETAANE® suspension to determine the steps necessary to gain final approval for the wet AMD indication. applauds the introduction of new treatments. a completely different class of AMD drugs.Alliance International of which it is a member. by Alcon. angiostatic drugs are delivered through a canula. a request for market approval in Europe was withdrawn. In early March 2006. Alcon recently reported that their researchers and officials will "meet with the FDA to discuss the approvable letter. Although early clinical results were not as stellar as hoped. basically meaning that the drug is approvable but some further study is required." Retaane has received market approval for use in Australia. the USA Food and Drug Administration released what is called an "approvable" letter. 2005.

which was caused by the injection. The improvement in visual acuity endpoints in the ranibizumabtreated groups (0. It is important to note that these clinical studies were not conducted as randomised clinical trials. At 2 years. The use of bevacizumab in the eyes. VEGF.3% of cases) was endophthalmitis.loss than those who were treated with a placebo. Ranibizumab Phase 3 clinical trials for ranibizumab demonstrated superior results after 1 year of treatment.5 Based on these results. at least 90% of individuals who were treated with ranibizumab maintained or improved vision compared to approximately 53% of individuals who were treated with sham injections. Their first study was called Systemic Avastin for Neovascular AMD (SANA). In early 2004. PhD.the drug blocks the production of VEGF. individuals who were clinically followed reported improvements in visual acuity comparable to ranibizumab with no serious adverse events. Ranibizumab is a molecular fragment of an antibody. initiated the use of bevacizumab in the treatment of AMD. It is reasoned conjecture on the part of the AMD Alliance International that the off-label use of bevacizumab was first suggested for reasons of economy and availability in the face of a significant unmet need. Until the June 30. affected less than 3% of individuals. and included conjunctival hemorrhage. and bevacizumab is a full-length antibody. The most common side effect (occurring in approximately 1.4. or. which consisted of intravitreal injections of bevacizumab. Broadening the Anti-VEGF Theory Ranibizumab was developed by Genentech. Bevacizumab binds with VEGF and interferes with its ability to stimulate blood vessel growth. also known as angiogenesis. Bevacizumab for use in cancer therapy is currently being investigated. Philip Rosenfeld. while individuals in the control group continued to experience vision deterioration. Individuals who were treated with pegaptanib sodium experienced lasting results for 2 years. They are both thought to work by a similar principle . Inc. treatment with ranibizumab was not available unless an individual was registered in a clinical trial.5 mg) was maintained at year 2. and colleagues at the Bascom Palmer Eye Institute in Miami. MD. Fla.3 mg and 0. an indication for which it is not approved. increased IOP. prompts the abnormal growth of blood vessels. Treatment side effects were mild to moderate. In this and subsequent studies. Inc. The company had previously developed bevacizumab (Avastin. Genentech. as is . vitreous floaters. the use of bevacizumab for the treatment of AMD appears to have been broadly accepted by retinal specialists around the world. is called off-label use.) an anti-VEGF drug that is currently approved by the Food and Drug Administration (FDA) as an intravenous therapy for metastatic colorectal cancer patients. 2006 FDA approval in the USA. and endophthalmitis. and showed that the majority of individuals who were treated with ranibizumab improved or maintained vision 2 years later. which is also produced by cancer cells.

which of these is helpful to AMD is not yet known. However. but unfortunately has no relationship to the problems in the eye caused by Macular Degeneration. the research done so far has been promising enough for the American Foundation Fighting Blindness to expand a grant award programme aimed at . called oxidation. However. including beta-carotene. What you may have heard referred to as an "eye transplant" is probably the process of corneal transplantation. Free radicals are thought to result. They cause cellular damage by taking electrons from molecules in healthy cells. It is simply impossible to reconnect the nerves leading from the eye to the brain. This process. and these are helped by antioxidants that we ingest through food or vitamin supplements. Such research might take several more years. E nd carotenoids. from exposure to sunlight and other forms of ultraviolet light. receives the treatment on what is called a 'named-patient' basis. there is not yet conclusive evidence that retinal cell transplants or similar procedures in animals with a retinal degeneration result in long-term improved or restored vision. although it is important to emphasize that this is not yet a treatment available for use in humans. although it is still in its early experimental stages Retinal cell transplantation is described below. Recommendations regarding nutritional supplementation and light avoidance for patients with Macular Degeneration are expected to emerge from studies now in progress. long-term beneficial effects must be proven and possible side effects must be determined. Can an eye transplant cure Macular Degeneration? No. the body produces its own antioxidants. Vitamins C. Retinal cell transplantation is still in preliminary stages of investigation in the laboratory. Medical technology is not yet advanced enough to transplant the entire eye. The work investigating a link between vitamins and Macular Degeneration is still in preliminary stages. which is a valuable vision saving procedure for some people. in part. However.possible in some European countries. are examples of potent antioxidants. Before a procedure can be tested in humans. Retinal Cell Transplantation Transplantation of retinal cells has shown some encouraging results in animals. retinal cell transplantation is a procedure that may have promise for people with Macular Degeneration in the future. The good news is that studies so far have found that when photo receptor cells are transplanted into the retinas of animals. some features of normal photo receptors are either maintained or develop after transplantation. Nevertheless. Antioxidants and Vitamin Therapies One working hypothesis is that a cause or contributing factor to Macular Degeneration involves the formation of chemicals in the body called free radicals. has been linked to a variety of health problems including heart disease and cancer. Substances called antioxidants may counteract the oxidation process.

While this may sound simple. Finally. While all of the above factors make gene therapy a promising future approach for treating Macular Degeneration. define risk factors. There are a number of reasons that retinal degenerations are diseases that seem particularly suited to the use of gene therapy. is working to define normal ageing. there are still many obstacles. analyse eye tissue layers and how they interact. the actual procedure of gene therapy is very complex. develop new diagnostic techniques. Gene therapy might be described as a form of drug therapy in which the "good" gene itself is the drug. One key question is how to actually introduce the DNA of the good gene into diseased cells. The American Foundation. some of the defective genes for early onset inherited Macular Degeneration have been identified. giving researchers the ideal conditions for conducting a controlled scientific experiment. a treated eye can be compared to an untreated eye in the same patient. . there are a number of applicable animal models in which gene therapy can be tested for effectiveness and safety. and which may be predictive of AMD. which is introduced into the body to replace the "bad" gene. What assistance is available to help cope with AMD? This information is helpful in learning how to physically cope with Macular Degeneration and similar diseases. First and foremost. and all body tissues undergo changes associated with ageing. Also. along with the American National Eye Institute. Gene therapy is what many scientists feel is the answer of the future for many forms of retinal degenerations. And the outcomes of gene therapy can be tested by reliable and non-invasive visual examination of the retina. Gene Therapy As researchers identify more of the mutant genes that contribute to Macular Degeneration. define genetic components. and develop animal models that imitate human AMD. replace it with one that is not defective.scientists who are investigating several areas of basic science that could lead to new therapies that might repair or replace damaged retinal cells. which seem to be particularly good targets for this type of gene transfer. it is difficult to determine which eye findings are normal in those over the age of 50. But what about emotional aspects? What assistance is available to help me and my family cope with Macular Degeneration? There are many devices and techniques that help people with Macular Degeneration maximize the use of their remaining vision. it becomes possible to think about curing the defect at the most basic cellular level. Researchers have found that a neutralized virus can act as a transporter of the gene to the degenerating photo receptor cells. It is based on a simple logic: if a gene is defective. Special challenges in AMD research Because AMD does not develop until late in life. classify AMD types.

These include Corning and NOIR glasses. To determine which aids may be most useful for you. and talking computers. The person with the conditions may still able to manoeuvre around obstacles. reading machines. can see a white piece of fluff on a dark carpet and yet we will walk right by a neighbour or best friend without recognising them. that they cannot see that kind of detail." They will sometimes fake "seeing" because it is easier than explaining that they have no central vision. making it possible to easily enlarge type on the screen or provide an audio or Braille version to go with what is shown on the screen. An increasing number of computer programmes are addressing the needs of the visually impaired. These include closed-circuit televisions (CCTV). If a companion says. largely because the eyes of a person with AMD look normal. People with Macular Degenerations may rind it helpful to discuss their questions and concerns with other people who have similar experiences. Are there other ways that people with this condition can enhance the quality of their lives? It is important that individuals and families know that there are resources available to help them cope with the life-changing conditions Macular Degeneration may bring. that is. yet again. Also it is difficult to locate items for example. one could ask a family member 'Have you seen my umbrella' . Optical aids are devices that work to improve your vision to some extent. and some people with Macular Degeneration find it a useful navigational aid if their vision loss progresses beyond a certain stage. Those with AMD want people to understand that they are "visually impaired. some of these devices are becoming increasingly sophisticated and offering new opportunities for people with retinal degenerations to maximize their usable vision. The RP Foundation Fighting Blindness can help people get together to exchange information about these common issues in their lives and explore possible solutions for some of their problems. Contact the RP Foundation for more information on low vision services in your area. There are also electronic aids.The white cane is probably the most visible aid. the Fresnel prism. With advancing technology. While research findings provide hope for the future." not "clumsy. "Just look at that picture. "Oh yes!" rather than explaining. it does not have an actual effect on your eyes. You may also wish to speak with a mental health professional to assist you and/or your family in dealing with the many changes that can be related to Macular Degenerations." or "illiterate. telescopes and magnifiers. Other non-optical aids include guide dogs. and large print books." many will reply. it is suggested that you get a thorough low vision evaluation from a specialist." "standoffish. audio tapes. although it may help you see or cope with vision loss. The cane is a form of non-optical aid. there is no actual treatment for people with macular degeneration. How can I assist a person with AMD? Age-related macular degeneration (AMD) is a difficult condition to understand.

Now where is over there? The person may be pointing in the general direction but the affected individual can not see where they are pointing so needs to a more detailed explanation. Is it left. it's over there'. we urge you to:       Become informed. Upon diagnosis painful emotions such as disbelief. As a carer it is important to be as informed as you can about the condition of the person receiving care and educate their network of family and friends. What can really help an individual to come to terms with vision loss is an increase in confidence and skill level .and that takes time. and willing to accept help when needed without feeling dependent on others.answer 'yes. When a person is diagnosed with AMD. So sometimes the entire family will need to be reeducated. and share that information with the people they are caring for and their families. Ensure the person you are caring for receives skills training and assessments for adaptive tools. or is it right etc. panic. it changes not only their own lives. those with AMD or any form of vision loss can live with dignity.adjusting to vision loss takes time. By working together with caregivers and family members. anger. learn as much as you can about the condition. Caregivers need to get as much information as they can about vision loss. Be patient . in control. If you are a caregiver to someone with any form of vision loss. these steps will help the affected individual feel confident. Meet other caregivers. confidence. Contact a member organization of Retina International. safety. . but the lives of their families and friends. and frustration can be experienced by an individual and often family and caregivers are on the receiving end of these feelings. Meet other people with the same condition. Eventually. and a strong feeling of self-worth.

dan sel kerucut.5) Faktor resiko gangguan ini selain karena usia tua.4) Degenerasi makula menyebabkan kerusakan penglihatan yang berat (misalnya kehilangan kemampuan untuk membaca dan mengemudi) tetapi jarang menyebabkan kebutaan total. terapi laser bisa membersihkan pembuluh darah abnormal sehingga kekaburan penglihatan dapat dicegah. 5.3) Diagnosis dapat ditegakkan berdasarkan gejala klinis dan hasil pemeriksaan mata.com/2009/02/02/referat-degenerasi-makula/ Referat : DEGENERASI MAKULA Posted on Februari 2. merupakan lapis aselular merupakan tempat sinaps sel bipolar. 8.2) Berdasarkan American Academy of Oftalmology penyebab utama penurunan penglihatan atau kebutaan di AS yaitu umur yang lebih dari 50 tahun. Lapis sel ganglion yang merupakan lapis badan sel daripada neuron kedua.(1. merupakan tubuh sel bipolar. Kadang gejala awalnya berupa gangguan penglihatan pada salah satu mata. Untuk beberapa kasus basah.4. merupakan susunan lapis nucleus sel kerucut dan batang. ANATOMI DAN FISIOLOGI RETINA Anatomi Retina Retina atau selaput jala merupakan bagian mata yang mengandung reseptor yang menerima rangsang cahaya. Data di Amerika Serikat menunjukkan. Sejauh ini belum ada terapi untuk degenerasi makula tipe kering. tetapi kadang berkembang secara progresif. 6. 7. Lapisan nucleus luar. tidak semua kasus bisa diatasi dengan terapi laser. 4.7) 1.(1. Gejala klinis biasa ditandai terjadinya kehilangan fungsi penglihatan secara tiba-tiba ataupun secara perlahan tanpa rasa nyeri. Lapisan pleksiform dalam. sel amakrin dengan sel ganglion.5) II. . Lapisan epitel pigmen 2. Terdapat 2 jenis tipe dasar dari penyakit-penyakit tersebut yakni Standar Macular Degeneration dan Age Related Macular Degeneration (ARMD). Lapisan fotoreseptor merupakan lesi terluar retina terdiri atas sel batang yang mempunyai bentuk ramping.wordpress. Degenerasi makula terjadi sebagai akibat dari kerusakan pada epitel pigmen retina. dinilai garis yang sesungguhnya lurus terlihar bergelombang. sel horizontal dan sel Muller. Lapisan pleksiform luar merupakan lapis aselular dan merupakan tempat sinapsis sel fotoreseptor dengan sel bipolar dan sel horizontal.4) Degenerasi makula terkait usia merupakan kondisi generatif pada makula atau pusat retina. Lapis nucleus dalam. Membran limitan eksterna yang merupakan membrane ilusi. yang terkena hanya penglihatan pada pusat lapang pandang.http://idmgarut. Retina berbatas dengan koroid dengan sel epitel pigmen retina dan terdiri atas lapisan : (6. Kondisi ini biasanya berkembang secara perlahan-lahan. 2009 by idmgarut PENDAHULUAN Degenerasi macula adalah suatu keadaan dimana macula mengalami kemunduran sehingga terjadi penurunan ketajaman penglihatan dan kemungkinan akan menyebabkan hilangnya fungsi penglihatan sentral. Penglihatan pada tepi luar dari lapang pandang dan kemampuan untuk melihat biasanya tidak terpengaruh.(2. 15 persen penduduk usia 75 tahun ke atas mengalami degenerasi makula itu. Macula adalah pusat dari retina dan merupakan bagian yang paling vital dari retina yang memungkinkan mata melihat detil-detil halus pada pusat lapang pandang. ras kaukasia serta merokok.(1. Tetapi. Saat ini sedang dikembangkan berbagai obat dan prosedur operasi baru antara lain terapi foto dinamik.2. Terdapat 2 macam degenarasi makula yaitu tipe kering (atrofik) dan tipe basah (eksudatif). juga riwayat keluarga (genetik).3. Suplemen seng hanya mampu membantu memperlambat progresivitas gangguan. 3.(3. Bentuk yang paling sering terjadi adalah ARMD.3. Kedua jenis degenerasi tersebut biasanya mengenai kedua mata secara bersamaan.(1. sehingga menyebabkan kehilangan penglihatan yang sangat berat pada satu atau kedua bola mata. Tanda utama dari degenerasi pada makula adalah didapatkan adanya bintik-bintik abu-abu atau hitam pada pusat lapangan pandang.

banyak fotoreseptor dihubungkan ke sel ganglion yang sama. . Lapis serabut saraf. dan akhirnya di tepi ora serrata. Setiap sel fotoreseptor kerucut mengandung redopsin. digunakan terutama untuk penglihatan perifer dan malam (skotopik).5 mm di belakang garis Schwalbe pada system temporal dan 5. (6.7) Fisiologi Retina Untuk melihat. bulat. Retina membentang ke depan hampir sama jauhnya dengan korpus siliare. fotoreseptor. Seiring dengan waktu. Pada bentuk penglihatan adaptasi gelap ini. Makula bertanggung jawab untuk ketajaman penglihatan yang terbaik dan untuk penglihatan warna. ora serrata berada sekitar 6. Sewaktu retina telah beradaptasi penuh terhadap cahaya. sensitivitas spektral retina bergeser dari puncak dominasi rodopsin 500 nm ke sekitar 560 nm. Retina adalah selembar tipis jaringan saraf yang semitransparan. -hijau. drusen dapat membesar. dan –merah. Secara histopatologis sebagian besar drusen terdiri dari kumpulan lokal bahan eosinifilik yang terletak di antara epitel pigmen dan membran Bruch. terdapat hubungan hampir 1:1 antara fotoreseptor kerucut. dan sebagai suatu transducer yang efektif. yang merupakan suatu pigmen penglihatan fotosensitif yang terbentuk sewaktu molekul protein opsin bergabung dengan 11-sis-retinal. Penyerapan cahaya puncak oleh terjadi pada panjang gelombang sekitar 500 nm. dan sclera. yang sebagian besar terdiri dari fotoreseptor batang. Di retina perifer. epitel pigmen retina. senjakala oleh kombinasi sel kerucut dan batang. dan penglihatan malam oleh fotoreseptor batang. dengan ukuran bervariasi di belakang epitel pigmen dan tersebar di seluruh makula dan kutub posterior. terlihat bermacam-macam nuansa abu-abu. termasuk lapisan pleksiformis luar dan lapisan inti luar. Permukaan luar retina sensorik bertumpuk dengan membran Bruch. mata harus berfungsi sebagai suatu alat optis. yang terletak di daerah biru-hijau pada spektrum cahaya. Sewaktu foton cahaya diserap oleh rodopsin. dan 575 nm masing-masing untuk sel kerucut peka-biru. dan lapisan epitel pigmen retina.7 mm di belakang garis ini pada sisi nasal. (7) Penglihatan skotopik seluruhnya diperantarai oleh fotoreseptor sel batang. membran Bruch. Di fovea sentralis. PATOFISIOLOGI Degenerasi makula yang terkait usia tipe kering ditandai oleh adanya atrofi dan degenerasi retina bagian luar.8) Retina menerima darah dari dua sumber : khoriokapiler yang berada tepat di luar membrana Bruch. Pada orang dewasa. Sel-sel batang dan kerucut di lapisan fotoreseptor mampu mengubah rangsangan cahaya menjadi suatu impuls saraf yang dihantarkan oleh lapisan serat saraf retina melalui saraf optikus dan akhirnya ke korteks penglihatan. dan hal ini menjamin penglihatan yang paling tajam.7) Fotoreseptor kerucut dan batang terletak di lapisan terluar yang avaskuler pada retina sensorik dan merupakan tempat berlangsungnya reaksi kimia yang mencetuskan proses penglihatan. 540. tetapi warna tidak dapat dibedakan.9.(7) III. Suatu benda akan berwarna apabila benda tersebut mengandung fotopigmen yang menyerap panjang-panjang gelombang dan secara selektif memantulkan atau menyalurkan panjang-panjang gelombang tertentu di dalam spektrum sinar tampak (400-700 nm). mengalami kalsifikasi dan meningkat jumlahnya. dan muncul sensasi warna.1 mm pada ora serrata dan 0. (6. 11-sis-retinal segera mengalami isomerisasi menjadi bentuk ali-trans. dan sebagian besar selnya adalah sel kerucut. Penelitian-penelitian sensitivitas spektrum fotopigmen kerucut memperlihatkan puncak penyerapan panjang gelombang di 430. merupakan membrane hialin antara retina dan badan kecil. Ditengah-tengah retina posterior terdapat makula. Di tengah makula terdapat fovea yang secara klinis merupakan cekungan yang memberikan pantulan khusus bila dilihat dengan oftalmoskop. dan koriokapilaris dengan derajat yang bervariasi. Retina menpunyai tebal 0. sebagai suatu reseptor kompleks. khoroid. Redopsin adalah suatu glikolipid membran yang separuh terbenam di lempeng membram lapis ganda pada segmen paling luar fotoreseptor. Akibat dari susunan seperti itu adalah bahwa makula terutama digunakan untuk penglihatan sentral dan warna ( penglihatan fototopik) sedangkan bagian retina lainnya. menyatu. 10. Dari perubahanperubahan di epitel pigmen retina dan membran Bruch yang dapat dilihat secara oftalmoskopi adalah drusen yang sangat khas. dan multilapis yang melapisi bagian dalam dua per tiga posterior dinding bola mata. Penglihatan siang hari terutama diperantarai oleh fotoreseptor kerucut. sel ganglionnya. dan diperlukan sistem pemancar yang lebih kompleks. dan serat saraf yang keluar. merupakan lapis akson sel ganglion menuju kearah saraf optic. yang mendarahi sepertiga luar retina. Drusen adalah endapan putih kuning. Membran limitan interna.(3. serta cabang-cabang dari arteri retina sentralis yang memperdarahi dua per tiga sebelah dalam. Fotopigmen sel kerucut terdiri dari 11-sis-retinal yang terikat ke berbagai protein opsin. diskret.23 mm pada kutub posterior.

mengalami kalsifikasi dan meningkat jumlahnya. Cairan serosa dari koroid di bawahnya dapat bocor melalui defek defek kecil di membran Bruch sehingga mengakibatkan pelepasan-pelepasan lokal epitel pigmen. ETIOLOGI Degenerasi macula dapat disebabkan oleh beberapa factor dan dapat diperberat oleh beberapa factor resiko. Meskipun degenerasi makula dapat terjadi pada orang muda.9) 1.7.8. 5. menyatu. Bintik tersebut berlokasi di belakang mata pada level retina bagian luar. Obesitas dan kadar kolesterol tinggi V. Tipe ini bersifat multipel. dan koriokapilaris dengan derajat yang bervariasi. Kebanyakan kasus ini bisa memberikan efek berupa kehilangan penglihatan yang sedang.9) IV. Riwayat keluarga. Adapun lesi klasik yang bisa ditemukan adanya atrofi geografik. 3. sebagian besar pasien yang menderita gangguan penglihatan berat akibat penyakit ini mengalami bentuk eksudatif akibat terbentuknya neovaskularisasi subretina dan makulopati eksudatif terkait. membran Bruch.13. drusen adalah yang paling khas. Paparan terhadap sinar Ultraviolet 8. bintik putih kekuningan yang di sebut drusen dan merupakan kunci identifikasi untuk tipe kering. penelitian menunjukkan bahwa umur di atas 60 tahun beresiko lebih besar terjadi di banding dengan orang muda. epitel pigmen retina. drusen dapat membesar. tapi resiko ini meningkat 30% pada orang yang berusia di atas 70 tahun. penyebab kerusakan makula adalah CFH.(11. dengan ukuran bervariasi di belakang epitel pigmen dan tersebar di seluruh makula dan kutub posterior. Degenerasi Makula tipe non-eksudatif (tipe kering) Rata-rata 90% kasus degenerasi makula terkait usia adalah tipe kering. 2% saja yang dapat menderita degenerasi makula pada orang muda. Pelepasan epitel pigmen retina dapat secara spontan menjadi datar dengan bermacam-macam akibat penglihatan dan meninggalkan daerah geografik depigmentasi pada daerah yang terkena.10) . darah atau perembesan cairan.7. faktor resiko yang paling berperan pada terjadinya degenerasi makula adalah umur. 4. 2. Dari perubahan-perubahan di epitel pigmen retina dan membran Bruch yang dapat dilihat secara oftalmoskopis. Dapat terjadi pertumbuhan pemubulu-pembuluh darah baru ke arah dalam yang meluas ke koroid sampai ruang subretina dan merupakan perubahan histopatologik terpenting yang memudahkan timbulnya pelepasan makula dan gangguan penglihatan sentral yang bersifat ireversivel pada pasien dengan drusen. Genetik. gen yang telah bermutasi atau faktor komplemen H yang dapat dibawa oleh para keturunan penderita penyakit ini. Terdapat endapan pigmen di dalam retina tanpa disertai pembentukan jaringan parut . dan hanya 12 % pada mereka yang tidak memiliki hubungan dengan degenerasi makula. Drusen adalah endapan putih kuning.(4. bulat. atau tekanan darah tinggi gara-gara mudah pecahnya pembuluh-pembuluh darah kecil (trombosis) sekitar retina. Degenerasi Makula menyerang para penderita penyakit diabetes. diskret. drusen mencerminkan pelepasan fokal epitel pigmen.7.(7. Merokok.(4. Peningkatan cairan tersebut dapat semakin menarik retina sensorik di bawahnya dan penglihatan biasanya menurun apabila fovea terkena. Ras kulit putih (kaukasia) adalah sangat rentan terjadinya degenerasi makula di banding dengan orang Afrika atau yang berkulit hitam.9) Walaupun pasien dengan degenerasi makula biasanya hanya memperlihatkan kelainan non eksudatif. Umur. Secara histopatologis sebagian besar drusen terdiri dari kumpulan lokal bahan eosinifilik yang terletak di antara epitel pigmen dan membran Bruch.drusen mencerminkan pelepasan fokal epitel pigmen. Hipertensi dan diabetes.14) Degenerasi makula terkait usia noneksudatif ditandai oleh atrofi dan degenerasi retina bagian luar. Pembuluh pembuluh darah ini akan tumbuh dalam konfigurasi roda-roda pedati datar atau sea-fan menjauhi tempat masuk ke dalam ruang sub retina. kecil. bulat. KLASIFIKASI 1. Trombosis mudah terjadi akibat penggumpalan sel-sel darah merah dan penebalan pembuluh darah halus. resiko seumur hidup terhadap pertumbuhan degenerasi makula adalah 50% pada orangorang yang mempunyai hubungan keluarga penderita dengan degenerasi makula. CFH terkait dengan bagian dari sistem kekebalan tubuh yang meregulasi peradangan.12. 7. diantaranya : (6. Merokok dapat meningkatkan terjadinya degenrasi makula. 6. Seiring dengan waktu.

eksudat. drusen diffus ( konfluent).4. DIAGNOSIS BANDING Degenerasi macula khususnya tipe eksudat dapat di diagnosis banding dengan: (4) 1.7. Kasus inflamasi 5. Pembuluh darah ini bisa mengalami perdarahan dan menyebabkan terjadinya scar yang dapat menghasilkan kehilangan pusat penglihatan.(4.9) 1. penyakit ini tidak menyebabkan hilangnya penglihatan perifer. Kadang-kadang dilakukan angiografi dengan zat warna fluoresein. Selain drusen. Neovaskularisasi koroid merupakan perkembangan abnormal dari pembuluh darah pada epitel pigmen retina pada lapisan retina. GEJALA KLINIS Gejala-gejala klinik yang biasa didapatkan pada penderita degenerasi makula antara lain : (1. Ada daerah kosong atau gelap di pusat penglihatan 5. VII. (2. Secara tiba-tiba ataupun secara perlahan akan terjadi kehilangan fungsi penglihatan tanpa rasa nyeri. PENATALAKSANAAN Tidak ada terapi khusus untuk AMD noneksudatif Penglihatan dimaksimalkan dengan alat bantu penglihatan termasuk alat pembesar dan teleskop. lesi koroid hijau abu-abu di makula.2.10) 2.4.5) 1. DIAGNOSIS Diagnosis dapat ditegakkan berdasarkan gejala klinik dan hasil pemeriksaan oftalmoskopi yang mencakup ruang lingkup pemeriksaan sebagai berikut : (1. Test Amsler Grid. e VIII. Tumor kecil seperti melanoma koroid IX. Degenerasi Makula tipe eksudatif ( tipe basah) Degenerasi makula tipe ini adalah jarang terjadi namun lebih berbahaya di bandingkan dengan tipe kering.Drusen dapat di bagi berdasarkan klinik dan histopatologi yakni drusen keras ( nodular).4. Pada pemeriksaan fundus.11. Ini penting karena banyak pasien takut mereka akan menjadi buta total. Kehilangan kemampuan membedakan warna dengan jelas 4. Dokter spesialis mata menyuntikan zat warna kontras ini ke lengan penderita yang kemudian akan mengalir ke mata dan dilakukan pemotretan retina dan makula. Vaskulopati koroid polipoid 3.8. dan drusen kalsifikasi . Membrane vascular subfovea dapat diobliterasi dengan terapi fotodinamik (PDT) karena laser argon konvensional akan merusak fotoreseptor di atasnya. kata-kata terlihat kabur atau berbayang 6. untuk melihat apakah penderita masih dapat membedakan warna. Tipe ini ditandai dengan adanya neovaskularisasi subretina dengan tanda-tanda degenerasi makula terkait usia yang mendada atau baru mengalami gangguan penglihatan sentral termasuk penglihatan kabur. Makroneurisme 2.15. Test penglihatan warna. Khorioretinopati serous sentral 4. mungkin dapat dilakukan obliterasi membrane tersebut dengan terapi laser argon. terlihat darah subretina.9) Pada sebagian kecil pasien dengan AMD eksudatif yang pada angiogram fluorosen memperlihatkan membrane neovaskular subretina yang terletak eksentrik (tidak sepusat) terhadap fovea.3. dimana pasien diminta suatu halaman uji yang mirip dengan kertas milimeter grafis untuk memeriksa luar titik yang terganggu fungsi penglihatannya. dapat muncul secara progresif gumpalan-gumpalan pigmen yang tersebar secara tidak merata di daerah-daerah depigmentasi atrofi di seluruh makula. Scar ini disebut dengan Scar Disciform dan biasanya terletak di bagian sentral dan menimbulkan gangguan penglihatan sentral permanen. Kemudian retina diteropong melalui lampu senter kecil dengan lensa khusus. 3. Kira kira didapatkan adanya 10% dari semua degenerasi makula terkait usia dan 90% dapat menyebabkan kebutaan. dan tes-tes lain untuk menemukan keadaan yang dapat menyebabkan kerusakan pada makula. Pasien diyakinkan bahwa meski penglihatan sentral menghilang. distorsi atau suatu skotoma baru.16) VI. PDT dilakukan dengan menyuntikkan secara intravena bahan kimia serupa porfirin yang diaktivasi oleh sinar laser nontermal . Garis-garis lurus mengalami distorsi (membengkok) terutama dibagian pusat penglihatan 3. Zat warna ini memungkinkan melihat kelainan pembuluh darah dengan lebih jelas. Distorsi penglihatan. Kesulitan membaca. obyek-obyek terlihat salah ukuran atau bentuk 2.(7. drusen halus ( granular ). 2.8.

..K.http://www.. tetapi belum ada terapi yang bernilai efektif sehingga kemungkinan untuk sembuh total sangat kecil. neovaskularisasi rekuren di dekat atau jauh dari jaringan parut laser dapat dapat terjadi pada separuh kasus dalam 2 tahun. (4. [Online].9) Apabila tidak ada neovaskularisasi retina.. Maturi R. (2. (4.(10) DAFTAR PUSTAKA 1. 8. 2006 10. Chew C. IDI Online-Iptek Kedokteran. Sayuran hijau terbukti bisa mencegah terjadinya degenerasi makula tipe kering. Prognosis dapat didasarkan pada terapi.. Available from : URL: http://www. Namun apabila terdapat membrane neovaskular subretina ekstrafovea yang berbatas tegas (? 200 um dari bagian tengah zona avaskular fovea).. Medicastore Online. Liesegang TJ. Januari 17th ]. [Online]. misalnya. Degenerasi Makula dan Mata Anda. vitamin C. Yakarta : Penerbit Erlangga. Retina dan Tumor Intraokuler. Dalam : Vaughan D. belum terbukti efektif untuk penyakit ini.php?news_id=623 4. Retina and Vitreous. Rekurensi sering disertai penurunan penglihatan berat sehingga pemantauan yang cermat dengan Amsler grid. Januari 17th ]. Dengan angiografi dapat ditentukan dengan tepat lokasi dan batas-batas membrane neovaskular yang kemudian diablasi secara total oleh luka-luka bakar yang ditimbulkan oleh laser.162.stlukeseye. Pasien dengan gangguan penglihatan sentral di kedua matanya mungkin memperoleh manfaat dari pemakaian berbagai alat bantu penglihatan kurang. asam cupric dan zinc). Januari 17th ]. Dalam : Ilmu Penyakit Mata Edisi kedua. Jakarta : Widya Medika. Available from : URL: http://www. Januari 17th ]. Fotokoagulasi juga menghancurkan retina di atasnya tetapi bermanfaat apabila membrane subretina dapat dihentikan tanpa mengenai fovea. [ Cited on 2007. Sayangnya kondisi ini dapat terjadi kembali bahkan setelah terapi laser. James C. Sidarta I. Macular Degeneration.php?id=&iddtl=983&idktg=16&idobat=&UID=2007030619264 9125. http://www.com/. karena diduga dapat memperbaiki dan mencegah terjadinya degenerasi makula.http://www. http://www.com/. Januari 17th ]. Klinik Mata Nusantara Online.115 2. [Online].eyemdlink.saat sinar laser berjalan melalui pembuluh darah di membrane subfovea. Hardy RA.255. Editor.8. Setelah fotokoagulasi membrane neovaskular subretina berhasil dilakukan.com/med/detail_pyk.15) X.medicastore.org/.com/ 13. Cantor LB. [ Cited on 2007. Dalam : Oftalmologi Edisi Kesembilan.idionline. 11. 12. tidak ada terapi medis atau bedah untuk pelepasan epitel pigmen retina serosa yang terbukti bermanfaat.8) Fotokoagulasi laser krypton terhadap neovaskularisasi subretina avaskular fovea (? 200 um dari bagian tengah zona avaskular fovea) dianjurkan untuk pasien nonhipertensif. beta caroten. IDI Online-Iptek Kedokteran.com/. Aging Relation Macular Degeneration. 5.G. 2000. [ Cited on 2007. Pemakaian interferon alfa parenteral. Age-Related Macular Degeneration. Available from : URL:http://www. Degenerasi Makula. Selain itu kebiasaan merokok dikurangi dan dan pembatasn hipertensi. Selain itu dengan mengkomsumsi multivitamin dan antioksidan ( berupa vitamin E . Degenerasi Makula. Jakarta : BP-FKUI. 2003-2004.. oftalmoskopi dan angiografi perlu dilakukan.. Anatomi dan Fisiologi Mata. 2002 7.php (diakses tanggal 6 Maret 2007) 9.wikipedia.nationaleyeinstitute. PROGNOSIS Bentuk degenerasi makula yang progresif dapat menyebakan kebutaan total sehingga aktivitas dapat menurun. [ Online ]. Oftalmologi Umum Edisi 14. Retina dan Koroid.. 3. Molekul yang teraktivasi menghancurkan pembuluh darah namun tidak merusak fotoreseptor. Skuta GL.Section 12 . Macular Degeneration. California : American Academy of Ophthalmology. San Fransisco. diindikasikan fotokoagulasi laser.org/iptek-isi. Degenerasi Makula.emedicine. . In : Eye Condition. Prognosis dari degenerasi makula dengan tipe eksudat lebih buruk di banding dengan degenerasi makula tipe non eksudat.php?news_id=623 6.idionline.4. Available from : URL: http://www. Basic and Clinical Course.8) Selain itu terapi juga dapat dilakukan di rumah berupa pembatasan kegiatan dan follow up pasien dengan mengevaluasi daya penglihatan yang rendah. Bron A. Macular Degeneration. Asbury T. [ Cited on 2007.Available from : URL: http://en. Riordan E.com/degenerasi.klinikmatanusantara. [Online]. [ Cited on 2007.(4.P.org/iptek-isi.

New York : Grune & Stratton. In: Ryan SJ.nih. 15. Cure Macular Degeneration-Prevent Age Related Macular Degeneration.. Patterns in Macular Degeneration. [Online].nei.google. Macular Degeneration. Sarks SH.14. Januari 15th].emedicinehealth. 1985.com/. . Retina. [ Cited on 2007. Retinal Diseases. Little HL.Editor. Available from:URL: http://www.[Cited on 2007. Januari 17th ] . Januari 17th ]. Available from : URL:http://www. Image of Eye. Killingsworth MC. [Online]. 17. Inc. 5th Edition.[ Cited on 2007.. 16. Driei DV.gov/health.. Dawson AK. Penfold PH. and Laser Theraphy of Macular Degeneration.com/. [Online].Available from : URL: http://www.