Ocular Syndromes and Systemic Diseases

Ward Syndrome (Epitheliomatous Phakomatosis; Nevus-Jaw Cyst Syndrome) 1324

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Ward Syndrome (Epitheliomatous Phakomatosis; Nevus-Jaw Cyst Syndrome) 1324

General: Autosomal dominant.

Ocular: Hypertelorism; dystopia canthorum; nevi of eyelids; congenital cataracts; congenital corneal opacities; colobomata.

Clinical: Basal cell nevi with multiple basalomatous nodules on face, neck, and trunk; epithelioma adenoides cysticum.

Font RL, Ferry AP. The phakomatoses. Int Ophthalmol Clin 1972; 12:1.

Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid ribs. N Engl J Med 1960; 262:908.

Water Intoxication Syndrome (Overhydration Syndrome) 1325

General: Administration of water in excess of kidney excretion capacity; psychogenic water drinker; may be chronic, with slow accumulation of water, or acute.

Ocular: Lacrimation.

Clinical: Chronic symptoms include weakness, sleepiness, apathy, anorexia, nausea, vomiting, sialorrhea, diarrhea, perspiration, behavioral changes, seizures and coma, tendon hyporeflexia, pitting edema, hemiplegia, pulmonary edema, and congestive heart failure; acute symptoms include tendon hyporeflexia, decreased attention, confusion, aphasia, muscle twitching, hemiplegia, incoordination, apathy, violent behavior, and marked muscle weakness.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Weber Syndrome (Weber-Dubler Syndrome; Cerebellar Peduncle Syndrome; Alternating Oculomotor Paralysis; Ventral Medial Midbrain Syndrome) 1326

General: Lesion of the peduncle (crus), pons, or medulla, which interrupts the third nerve before it emerges from the peduncle and interrupts fibers in the pyramidal tract above the level of the third nuclei; hemorrhage and thrombosis; tumor of the pituitary region, extending posteriorly; also may result secondary to cerebrovascular disease.

Ocular: Ptosis; homolateral third nerve palsy (usually complete); fixed, dilated pupil.

Clinical: Contralateral hemiplegia; contralateral paralysis of face and tongue (supranuclear type).

Kistler JP, Ropper AH, Martin JB. Cerebrovascular diseases. In: Isselbacher KJ, et al., eds. Harrison’s Principles of Internal Medicine, 13th ed. New York: McGraw-Hill, 1994:2242.

Newman NJ. Third, fourth and sixth-nerve lesions and the cavernous sinus. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. V. Philadelphia: WB Saunders, 1994:2451.

Miller NR, ed. Walsh and Hoyt’s Clinical Neuro-Ophthalmology, vol. 4, part 1, 4th ed. Baltimore: Williams & Wilkins, 1991:2384.

Weber HD. A contribution to the pathology of the crura cerebri. Med Chir Trans 1863; 46:121.

Wolf S, et al. The posterior inferior cerebellar artery on vertebral angiography. AJR Am J Roentgenol 1962; 87:322.

Weber-Christian Syndrome (Pfeiffer-Weber-Christian Syndrome) 1327

General: Etiology unknown; subcutaneous inflammatory lesions; occurs at any age; no gender dominance.

Ocular: Secondary glaucoma; anterior uveitis; acute exudative central choroiditis.

Clinical: Generalized distribution of subcutaneous nodular lesions that vary in size and are located predominantly on the trunk, arms, and legs; recurrent attacks of fever; anorexia; hepatosplenomegaly; malaise; oropharyngeal infections; myocardosis; liver cirrhosis; retroperitoneal fibrosis; ulcerative colitis; myalgia; cardiac dilatation with congestive heart failure.

Christian HA. Relapsing febrile nodular non-suppurative panniculitis. Arch Intern Med 1928; 42:338.

Lemley DE, et al. Cardiac manifestation of Weber-Christian disease: report and review of the literature. J Rheumatal 1991; 18:756-760.

Nozue M, et al. Ulcerative colitis associated with Weber-Christian panniculitis and musculitis: a case report. J Gastroenterol 1994; 29:84-87.

Ohara S, et al. Myalgia as the major symptom in systemic panniculitis (Weber-Christian disease). Eur Neurol 1992; 32:321-323.

Popoff N, Weelock M. Relapsing febrile nodular nonsuppurative panniculitis. Pfeiffer- Weber-Christian disease: report of three cases. Arch Intern Med 1956; 97:39.

Weber FP. A case of relapsing non-suppurative nodular panniculitis, showing phagocytosis of subcutaneous fat cells by macrophages. Br J Dermatol Syphil 1925; 37:301.

WEBINO Syndrome (Wall-Eyed Bilateral Internuclear Ophthalmoplegia) 1328

General: Differential diagnosis includes demyelinating disease, arteriosclerotic cerebrovascular disease, trauma, Arnold-Chiari malformation, syphilis, periarteritis nodosa, glioma, cryptococcal meningitis, and premature infants; usually represents midline involvement of oculomotor nucleus.

Ocular: Wall-eyed internuclear ophthalmoplegia (bilateral internuclear ophthalmoplegia with exotropia).

Clinical: Depends on cause; association with multiple sclerosis in young patients.

Daroff RE, Hoyt WE Supranuclear disorders of ocular control systems in man. In: Bach-Y-Rita P, Collins CC, eds. The control of eye movements. Orlando, FL: Academic Press, 1971:223.

Lepore FE, Nissenblatt MJ. Bilateral internuclear ophthalmoplegia after intrathecal chemotherapy and cranial irradiation. Am J Ophthalmol 1981; 92:851-853.

Miller NR, ed. Walsh and Hoyt’s Clinical Neuro-Ophthalmology, vol. 5, part 1, 4th ed. Baltimore: Williams & Wilkins, 1995:4323.

Wegener Syndrome (Wegener Granulomatosis) 1329

General: Etiology unknown; occurs in fourth and fifth decades of life; persistent rhinitis or sinusitis; three characteristic features are necrotizing granulomatous lesions in the respiratory tract, generalized focal arthritis, and necrotizing thrombotic glomerulitis,

Ocular: Exophthalmos; lid and conjunctival chemosis; papillitis; conjunctivitis; corneal ulcer; corneal abscess; optic atrophy; optic neuritis; orbital cellulitis; episcleritis; sclerokeratitis; cataract; peripheral ring corneal ulcers; ptosis; dacryocystitis; retinal periphlebitis; cotton-wool spots; retinal and vitreous hemorrhages; rubeosis iridis; neovascular glaucoma.

Clinical: Severe sinusitis; pulmonary inflammation; arteritis; weakness; fever; weight loss; bony destruction; granulomatous vasculitis of the upper and lower respiratory tracts; glomerulonephritis; diffuse pulmonary infiltrates; lymphadenopathy; diffuse pulmonary hemorrhage; overlap with giant cell arteritis.

Collins JF. Handbook of Clinical Ophthalmology. New York: Masson, 1982.

Flach AJ. Ocular manifestations of Wegener's granulomatosis [Letter]. JAMA 1995; 274:1199-1200.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Haynes BF, et al. The ocular manifestations of Wegener's granulomatosis. Fifteen years' experience and review of the literature. Am J Med 1977; 63:131-141.

Leavitt RY, Fauci AS. Less common manifestations and presentations of Wegener's granulomatosis. Curr Opin Rheumatol 1992; 4:16-22.

Robinson MR, Lee SS, Sneller MC, et al. Tarsal-conjunctival disease associated with Wegener’s granulomatosis. Ophthalmology. 2003; 110: 1770-1780.

Straatsma BR. Ocular manifestations of Wegener's granulomatosis. Am J Ophthalmol 1957; 44:789.

Weil Disease (Leptospirosis) 1330

General: Acute severe infection caused by Leptospira transmitted by ingestion of food contaminated by the reservoir bacterium.

Ocular: Acute conjunctivitis; episcleritis; fibrinous iridocyclitis with vitreal haze; hypopyon; keratitis; pain on ocular movement; uveitis; optic neuritis; cataract; hemorrhagic retinitis; ptosis.

Clinical: Jaundice; fever; headaches; chills; vomiting; anemia; psychologic disturbances.

David R, Barkay S. Anterior uveitis and leptospirosis. Ann Ophthalmol 1976; 8:958-962.

den Haan PJ, et al. Weil's disease as a cause of jaundice. Neth J Med 1993; 42:171-174.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Sanford JP. Leptospirosis. In: Isselbacher KJ, et al., eds. Harrison's Principles of Internal Medicine, 13th ed. New York: McGraw-Hill, 1994:743.

Weissenbacher-Zweymuller Syndrome 1331

General: Probably neonatal expression of Stickler syndrome; autosomal dominant.

Ocular: Myopia; congenital glaucoma; cloudy cornea; buphthalmos; Descemet membrane tears; retinal detachment; cataracts.

Clinical: Flattened occiput; flat facies; low nasal bridge; anteverted nostrils; flat vascular nevus-covered glabellar area; small mouth with protruding tip of tongue; high-arched palate; short neck; limbs shorter proximally; dumbbell-shaped long bones; posterior defects in vertebral bodies thoracic region; platyspondylic evidence of coronal cleft lumbar area; deafness; small size at birth; mid face hypoplasia; parietooccipital encephalocele; hearing loss; dwarfism.

Galil A, et al. Weissenbacher-Zweymuller syndrome: long-term follow-up of growth and psychomotor development. Dev Med Child Neurol 1991; 33:1104-1109.

Ramer JC, et al. Identical twins with Weissenbacher-Zweymuller syndrome and neural tube defect. Am J Med Genet 1993; 45:614-618.

Scribanu N, et al. The Weissenbacher-Zweymuller phenotype in the neonatal period as an expression in the continuum of manifestations of the hereditary arthro-ophthalmopathies. Ophthalmic Paediatr Genet 1987; 8:159-163.

Weissenbacher G, Zweymuller E. Gleichzeitiges Vorkommen Eines Syndroms von Pierro Robin und einer Fetalen Chondrodysplasie. Mschr Kinderheilkd 1964; 112:315-317.

Winter RM, et al. The Weissenbacher-Zweymuller and Marshall syndromes: further evidence for their identity. Am J Med Genet 1983; 16:189-199.

Werlhof Disease (Hemophilia and Thrombocytopenic Purpura) 1332

General: Hemorrhagic disease of unknown etiology.

Ocular: Retinal hemorrhages; degeneration or severe intraocular hemorrhages with resultant retinitis proliferans.

Clinical: Petechiae and ecchymoses of skin and mucous membranes; tendency to bruise; decreased level of circulating platelets with a normal clotting time.

Kobayashi H, Honda Y. Intraocular hemorrhage in a patient with hemophilia. Metab Ophthalmol 1985; 8:27-30.

Pau H. Differential diagnosis of eye diseases. New York: Thieme, 1987.

Werlhof PG. Disquisitio Medica et Philogica de Variolis et Anthracibus. Brunswick: 1735.

Wermer Syndrome (Multiple Endocrine Neoplasia 1; MEN1; Multiple Endocrine Adenomatosis 1;
MEA1; Endocrine Adenoma-Peptic Ulcer Complex; Pluriglandular Adenomatosis n 1333

General: Autosomal dominant; high degree of penetrance; both sexes affected; onset after the first decade (see Zollinger-Ellison Syndrome).

Ocular: Visual field defects secondary to pituitary adenoma.

Clinical: Parathyroid adenomas or hyperplasia; pancreatic adenomas; pituitary adenomas; thyroid adenomas; adrenocortical adenomas; subcutaneous lipomas; hypoglycemic crisis; headaches; amenorrhea; diarrhea; weight loss; acromegaly; Cushing syndrome; hyperthyroidism; ulcer; cerebral aneurysm.

Adachi K, et al. Cerebral aneurysm associated with multiple endocrine neoplasia, type l: case report. Neurol Med Chir 1993; 33:309-311.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981:860.

Wermer P. Genetic aspects of adenomatosis of endocrine glands. Am J Med 1954; 16:363-371.

Werner Syndrome (progeria of Adults) 1334

General: Etiology unknown; recessive inheritance; consanguinity; second and third decades; possible mechanisms have been proposed to explain mutation of a gene causing inhibition of deoxyribonucleic acid (DNA) synthesis and early cellular senescence.

Ocular: Absence of eyelashes and scanty eyebrows; blue sclera; juvenile cataracts; bullous keratitis; trophic corneal defects; paramacular retinal degeneration; proptosis; telangiectasia of lid; astigmatism; nystagmus; presbyopia; uveitis.

Clinical: Leanness; short stature (160 cm maximum); thin limbs; short, deformed fingers; small mouth; early baldness; stretched, atrophic skin (scleropoikiloderma); telangiectasia and trophic indolent ulcers on toes, heels, and ankles; arteriosclerosis with secondary heart failure.

Bristow JH, Whiting DA. Werner's syndrome: Clinical features and cataract surgery. S Afr Med J 1973; 47:566-569.

Bullock JD, Howard RO. Werner syndrome. Arch Ophthalmol 1973; 90:53-56.

Collins JF. Handbook of Clinical Ophthalmology. New York: Masson, 1982.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/.

Thweatt R, Goldstein S. Werner syndrome and biological aging: a molecular genetic hypothesis. Bioessays 1993; 15: 421-426.

Wernicke Syndrome I (Superior Hemorrhagic Polioencephalopathic Syndrome; Hemorrhagic Polioencephalitis Superior Syndrome; Encephalitis Hemorrhagica Superioris; Avitaminosis B;
Thiamine Deficiency; Beriberi; Gayet-Wernicke Syndrome; Wernicke-Korsakoff Syndrome) 1335

General: Lack of vitamin B or thiamine; focal vascular lesions in the gray matter around third and fourth ventricles and sylvian aqueduct; alcoholics (adults); beriberi of all ages.

Ocular: Ptosis; acute bilateral nuclear ophthalmoplegia; complete ophthalmoplegia; retinal hemorrhages; optic atrophy; optic neuritis; conjunctivitis; blepharitis; nutritional amblyopia; central scotoma; papilledema; nystagmus; absolute pupillary paralysis or Argyll Robertson pupils; accommodative palsy.

Clinical: Early prostration; lethargy; irritability; stupor; delirium; mental disturbances to Korsakoff psychosis; ataxia; tremors; peripheral neuritis; anorexia; vomiting; insomnia; perspiration; tachycardia; hallucinations; retrograde amnesia; apathy; anxiety; fear; defective concentration; cardiomyopathy.

Brust JCM. Environmental neurology: Wernicke Korsakoff syndrome. In: Rowland LP, ed. Merritt’s Textbook of Neurology, 9th ed. Baltimore: Williams & Wilkins, 1995:971-972.

Cogan G, Victor M. Ocular signs of Wernicke's disease. Trans Am Ophthalmol Soc 1953; 51:103.

Djoenaidi W, et al. Beriberi cardiomyopathy. Eur J Clin Nutr 1992; 46:227-234.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes. 2nd ed. Philadelphia: JB Lippincott, 1981:861-862.

Shimojyo S, et al. Cerebral blood flow and metabolism in the Wernicke-Korsakoff syndrome. J Clin Invest 1967; 46:849.

Wernicke C. Lehrbuch der Gehirnkrankeiten fur Aerzte und Studirende. Kassel: T. Fischer, 1881.

Yuen TS, Simon RP. Wernicke's encephalopathy. In: Bradley WG, et al., eds. Neurology in Clinical practice, 2nd ed. Boston: Butterworth-Heinemann, 1995:1382-1383.

Westphal-Strumpell Disease (Pseudosclerosis of Basal Ganglion) 1336

General: Part of Wilson disease; mostly in men; onset at ages 11 to 25 years (see Wilson Disease).

Ocular: Brown-yellow-green ring in Descemet membrane, about 2 mm wide, which begins directly at the limbus and fades away toward the center; deposition of copper.

Clinical: Jaundice; difficulty in swallowing, speaking, and mastication; extensive muscular rigidity; coarse tremors.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Pau H. Differential diagnosis of eye diseases. New York: Thieme, 1987.

West Nile Virus Infection 1337

General: Zoonotic disease transmitted by a mosquito vector with wild birds serving as its reservoir; seen worldwide but first seen in North America in 1999

Ocular: Photophobia; chorioretinal lesions; mild vitreous inflammatory reaction; retinal hemorrhage; optic disk swelling

Clinical: Muscle rigidity; meningeal inflammation; encephalitis; poliomyelitis; acute flaccid paralysis

Hershberger VS, Augsburger JJ, Hutchins RK, et al. Chorioretinal lesions in nonfatal cases of west nile virus infection. Ophthalmology. 2003; 110: 1732-1736.

Khairallah M, Yahia SB, Ladjimi A, et al. Chorioretinal involvement in patients with west nile virus infection. Ophthalmology. 2004; 111: 2065-2070..

West Syndrome (Massive Myoclonia; Jackknife Convulsion) 1338

General: Brain damage from trauma, anoxia, degenerative and metabolic factors and infective agents; onset in first year of life.

Ocular: Nystagmus.

Clinical: Convulsion; nodding of head; opisthotonos; mental retardation.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

West WJ. On a peculiar form of infantile convulsions. Lancet 1840-1841; 1:724-725.

Weyers Syndrome (2) (Weyers IV Syndrome; lridodental Dysplasia; Dentoirideal Dysplasia;
Dysgenesis Iridodentalis; Dysgenesis Mesodermalis Corneae et Irides with Digodontia) 1339

General: Present from birth; hereditary; etiology unknown.

Ocular: Dysplasia; small perforation of iris; pupillary synechiae; microphthalmia; corneal opacity.

Clinical: Dwarfism; myotonic dystrophy; microdontia; oligodontia; hypoplasia of dental enamel.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981:864.

Weyers H. Dysgenesis Irido-dentalis. Ein Neues Syndrome mit obweichdendem Chromosomalen Geschlecht bei Weiblichen Merkmalträgern. Presented at the Meeting of Deutsche Gessellschaft fuer Kinderheilkunde, Kassel, Germany: 1960.

Whipple Disease (Intestinal Lipodystrophy) 1340

General: Multisystem disorder; prominent in males; onset between fourth and seventh decades; etiology unknown.

Ocular: Ophthalmoplegia (vertical gaze involved more than horizontal gaze); papilledema; intraocular inflammation; vitreous opacities; bilateral panuveitis; small, round grayish retinal lesions; chemosis; supranuclear ophthalmoplegia; myoclonic ocular jerks; pendular nystagmus.

Clinical: Pneumonia; pleurisy; tonsillitis; sinusitis; cystitis; arthritis; fever; leukocytosis; diarrhea; malabsorption; death; dyspnea; weight loss; lymphadenopathy; polyserositis; gray pigmentation of skin; dementia; facial jerks; rhythmic movement of the mouth, jaw, and extremities.

Durant WJ, et al. Vitrectomy and Whipple's disease. Arch Ophthalmol 1984; 102:848-851.

Font RL, et al. Ocular involvement in Whipple's disease: light and electron microscopic observations. Arch Ophthalmol 1978; 96:1431-1436.

Gartner J. Whipple's disease of the central nervous system, associated with ophthalmoplegia externa and severe asteroid hyalitis: a clinicopathologic study. Doc Ophthalmol 1980; 49:155-187.

Knox DL, et al. Cerebral ocular Whipple's disease: a 62-year odyssey from death to diagnosis. Neurology 1995; 45:617-625.

Leland TM, Chambers JK. Ocular findings in Whipple's disease. South Med J 1978; 71:335-338.

Schrenk M, et al. Augenbeteiligung be Morbus Whipple. Klin Monatsbl Augenheilkd 1994; 204:538-541.

Simpson DA, et al. Oculofacial-skeletal myorhythmia in central nervous system Whipple's disease: additional case and review of the literature. Mov Disord 1995; 10:195-200.

Whipple GH. A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Bull Johns Hopkins Hosp 1907; 18:382-391.

Wildervanck Syndrome (Cervicooculoacousticus Syndrome; Franceschetti-Klein- Wildervanck
Syndrome; Wildervanck-Waardenburg Syndrome; Cervicooculofacial Dysmorphia;
Cervicooculofacial Syndrome) 1341

General: Etiology unknown; female preponderance; similarities to Klippel-Feil syndrome; onset at birth.

Ocular: Abducens paresis; nystagmus; heterochromia iridis.

Clinical: Deafness or deaf-mutism; torticollis with short, webbed neck; epilepsy; mental retardation; cleft palate; scoliosis; ventricular septal defect; ectopic kidney; hydrocephalus; hypoplastic thumb and growth retardation.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Jensen J, Rousing H. Dysplasia of the cochlea in a case of Wildervanck's syndrome. Adv Otorhinolaryngol 1974; 21:32.

Kose G, et al. Cholelithiasis in cervico-oculo-acoustic (Wildervanck's) syndrome. Acta Paediatr 1993; 82:890-891.

Wildervanck LS. A cervico-oculo-acoustic-nerve syndrome. Ned Tijdschr Geneeskd 1960; 104:2600.

Williams-Beuren Syndrome (Supravalvular Aortic Stenosis; Beuren Elfin Face; Hypercalcemia Supravalvular Aortic Stenosis; Hypercalcemic Face) 1342

General: Onset at birth or early infancy; occurs in both sexes; etiology unknown; possible abnormality of vitamin D metabolism.

Ocular: Bilateral corneal opacities; hypertelorism; prominent epicanthal folds; strabismus.

Clinical: Anorexia; slow weight gain; retarded physical and mental development; elfin face; absent aortic systolic click; harsh ejection systolic murmur; tooth enamel hypoplasia; malocclusion; cavities.

Greenberg L, Lewis A. The Williams' syndrome, spectrum, and significance of ocular features. Ophthalmology 1988; 95:1608-1612. ,

Kapp ME, et al. Strabismus in Williams syndrome. Am J Ophthalmol 1995; 119:355-360.

Roy FH, et al. Infantile hypercalcemia and supravalvular aortic stenosis-ocular manifestations of seven cases. J Pediatr Ophthalmol 1971; 8:188.

Williams JCP, et al. Supravalvular aortic stenosis. Circulation 1961; 24: 1311-1318.

Wilson Disease (Hepatolenticular Degeneration) 1343

General: Lesion in the putamen and lenticular nucleus; familial; occurs in first decade of life; reduced liver function; increase in copper content in tissue.

Ocular: Night blindness; golden, grayish-green, or ruby-red peripheral posterior corneal stromal deposit (Kayser-Fleischer ring); sunflower cataract (same as with retention of copper foreign body); nystagmus; extraocular muscle palsies.

Clinical: Liver cirrhosis; jaundice (early); difficulties in speaking (early); difficulties in swallowing and mastication; extensive muscular rigidity; mouth usually open with salivation ("fixed grin"); coarse tremor.

Johnson BL. Ultrastructure of the Kayser-Fleischer ring. Am J Ophthalmol 1973; 776:455.

Tso MOM, et al. Kayser-Fleischer ring and associated cataract in Wilson's disease. Am J Ophthalmol 1975; 79:479.

Walshe JM. The eye in Wilson's disease. Birth Defects 1976; 12:187.

Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34:295.

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