choroideremia disease is caused by what the?
majority of patients with genetic factors.
(B) the pathogenesis
2. immune factors from the histological and ultrastructural changes were found in macrophage phagocytosis of photoreceptor outer segments and pigment, the pigment in swallowing may cause allergic reactions in the protein, the collapse of the choroidal stroma, Bruch membrane and blood vessels disappear, the occurrence of full-thickness choroidal atrophy.
choroideremia disease early symptoms?
1. as early onset of functional changes may have been born, and even vision loss decreased to light perception. Concentric narrowing field of vision for sexual orientation. Atrophic changes began in the periphery, extend to the center with age, the last remnants of the central part, had become the tubular vision. Usually 10 to 30 years old with moderate visual acuity decreased, but remained the central field of vision, 40 to 50 years old involving the macula, in order to control the TV or associated with peripheral island, the last vision disappeared. To the center depending on the temporal side of the island and the remaining patients, as the island disappears completely blind. Early-onset blind every night, was shot cone degeneration, rod dark adaptation final threshold was progressively increased, late undetectable dark adaptation curve. color vision disorder, as the red-green blindness. Electrophysiological measurement of the eye, early normal photopic ERG, erg dark adaptation for low-wave, off late. EOG low wave or no wave. Male patients with this disease for the disease early erg changes that adapt to the part of the erg may be normal, but the dark-adapted part of a wave and b-wave amplitude decreased, b-wave latency, late erg off. Women with vision, vision, dark adaptation, color, EOG, ERG, mostly normal, but the occasional exception. female carriers show significantly eroded in the eyes like pigment disorders and the accumulation of cases, the erg response is still normal, erg amplitude can be reduced or increased. EOG changes of the disease is more obvious than the ERG, no visual function in patients with choroidal an unusual feature of the baseline EOG potential decreased, the late potential is almost undetectable baseline EOG light peak completely disappeared. More female patients no abnormal changes in visual function, but a few patients may have abnormalities.
(1) early stage: mild atypical peripheral pigmented retinopathy, due to degeneration of retinal pigment epithelium, equatorial and peripheral fundus showed flare yellow, deep pigment particles, pigment zone between depigmentation, it was pepper-like eyes. pigment was not like bone cells.
(2) medium: the lesions gradually developed from the periphery back pole, the inner layer of the retina without pigment, then there choroidal and RPE atrophy, showing a small area of choroidal large vessels exposure.
(3) late: the choroid and RPE to the posterior pole retinal atrophy. retinal pigment epithelium was almost completely destroyed, lost, and choroidal atrophy (Figure 1), sometimes leaving only a small macular choroid, its clear boundary. At the same time can be left a peripheral choroidal island, but after the age of 50 to 60 also gradually disappear. As the pigment epithelium and choroidal blood vessels disappear, exposing the sclera of the eyes white reflective. Residual islands can be brownish-red, with the peripheral circular or irregular pigmentation spots, but all eyes are rarely seen white, no visible choroidal vessels. Although choroidal lesions significantly, often to maintain normal retina and optic nerve. Late disc can atrophy, retinal blood vessels may be a little thin.
female patients as carriers, typical fundus changes in patients with similar young men. But the fundus into the stationary nature of light, and normal vision. Retinal depigmentation and can be expressed as pigment hyperplasia, atrophy was salt and pepper, and more at the equator of the fundus, pigment particle size, arranged in clusters and the surrounding strip for the radiation. Is a very peripheral hypopigmentation. Some cases there is a small macular pigmentation, retinal and optic disc normal.
3. fluorescein angiography showed early RPE defects in a wide range of strong fluorescent area, then the retinal pigment epithelium atrophy and choroidal capillaries disappear, only to see the filling of the choroidal fluorescence great vessels. Late shows a wide range of non-fluorescent zone, where the sparse remnants of large choroidal vessels. female gene carriers of the fluorescein angiography shows RPE atrophy, was see through a wide range of strong fluorescence or fluorescence.
male patients with this disease, clinical features of night blindness, concentric narrowing of vision, blue vision defects, dark adaptation threshold was elevated and progressive full choroidal and RPE atrophy. according to the typical fundus changes, electrophysiological changes and family history, can make the correct diagnosis.[Aftertreat]
intractable pain should be how to prevent?
prevention: (a) dorsal rhizotomy:
adaptation levy: the origin of peripheral nerves commonly used in the neck Department, trunk and abdominal pain, head and neck pain often require root resection and underwent bilateral posterior cranial sensory branch transection, only an estimated survival of patients is not long. thoracotomy after peripheral nerve injury leading to persistent pain after root resection is the preferred application of levy. Refractory coronary ischemia induced by bilateral pain in the dorsal root resection or resection of the dorsal root ganglia may also be alleviated. Include: intradural dorsal root resection. Epidural spinal nerve root resection. spinal dorsal root ganglion rhizotomy. Percutaneous spinal nerve root damage after surgery.
(b) the pain of stereotactic surgery:
stereotactic surgery in the treatment of pain is the pain transmission principle regulation of pain pathways and intracranial structures to create damage foci, thus blocking pain transmission or pain center regulate the control system to achieve the purpose of pain relief. Adaptation levy: nociceptive pain such as pain, especially in advanced cancer pain caused by the higher of a wide range or location. Neuropathic pain is less effective, but there was intermittent neuralgia, or abdominal pain, significant hyperalgesia, may consider surgery. Such as the hypothalamus, central pain syndrome, outer medullary plate syndrome, limb pain after brachial plexus stripping or postherpetic pain, stereotactic surgery for postoperative remission rate for up to 50%.
(1) failed back surgery syndrome.
(2) limb pain after amputation.
(6) peripheral nerve injury pain.
(1) complete transection of spinal cord injury.
(2) cancer pain.
(4) post-herpetic neuralgia.
(5) severe mental disabilities.
effect: more than 50% pain relief accounted for 50% to 60%, to stimulate the failure or ineffective in 20% to 50%.
(3) no obvious mental illness.
(4) in the appropriate pre-implantation to stop taking the drug.
(5) has the ability to identify the entire process.
(6) morphine - a satisfactory response to naloxone test.
DBS control of intractable pain about 62% of the long-term success rate. For back pain, deafferentation type of pain and the efficacy of trigeminal neuralgia is yes. Of the thalamus pain syndrome, post-herpetic neuralgia and traumatic spinal cord injury pain syndrome caused by ineffective. chronic brain stimulation in western Europe and other developed countries have become the primary treatment of refractory pain means. However, at home, due to the stimulation device is expensive, difficult to get popular in the country.
(2) peripheral deafferentation pain, such as trigeminal neuralgia, limb pain.
no special treatment.
(b) the prognosis
progressive disease progresses, patients can be blind.
intractable pain should do what check?
diagnosis: pain (pain) is the body of damaged tissue or potential damage resulting from an unpleasant reaction, is a complex physiological and psychological activities , is the most common clinical symptoms. It consists of pain and pain response of two parts. pain can harm the body as a warning, causing the body to protect a series of defensive responses. On the other hand, the pain has its limitations as an alarm (such as cancer pain, has been too late). And some long-term pain, the body has become an unbearable torment.[Diff]
diagnosis: pain (pain) is the body of damaged tissue or potential damage resulting from an unpleasant reaction, is a complex physiological and psychological activity, is the most common clinical symptoms. It consists of pain and pain response of two parts. pain can harm the body as a warning, causing the body to protect a series of defensive responses. On the other hand, the pain has its limitations as an alarm (such as cancer pain, has been too late). And some long-term pain, the body has become an unbearable torment.[Disease]
intractable pain related diseases
More Other symptoms
"frozen" phenomenon "three fears" (water, sound, light 21 - hydroxylase deficiency igm antibodies sm accumulation increased belching scar insomnia and daytime sleepiness, nighttime physical leukocytosis leukopenia signs of leukemic cell infiltration in sepsis to maintain sleep disorder galactosemia lost hold of a passive position overeating addiction rage stolen or hypochondriacal paranoia delusions
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