Wilson disease: Description of 282 patients evaluated over 3 decades

Medicine

Volumn 86, Issue 2, 2007, Pages 112-121

  Taly, Arun B ^a   Meenakshi Sundaram, S ^a   Sinha, Sanjib ^a   Swamy, H S ^a   Arunodaya, G R ^a  

^a NATIONAL INSTITUTE OF MENTAL HEALTH AND NEUROSCIENCES   (India)

Author keywords

[No Author keywords available]

Indexed keywords

CALCIUM; CERULOPLASMIN; COPPER; DIAZEPAM; DIMERCAPROL; LEVODOPA; PENICILLAMINE; POTASSIUM DERIVATIVE; POTASSIUM SULFIDE; PYRIDOXINE; TRIHEXYPHENIDYL; UNCLASSIFIED DRUG; ZINC SULFATE;

ADOLESCENT; ADULT; AGE DISTRIBUTION; ARTICLE; ATHETOSIS; BEHAVIOR DISORDER; BIOCHEMISTRY; CEREBELLUM DISEASE; CERULOPLASMIN BLOOD LEVEL; CHILD; CHOREA; CLINICAL FEATURE; COHORT ANALYSIS; COMPUTER ASSISTED TOMOGRAPHY; CONSANGUINITY; DEATH; DETERIORATION; DISEASE DURATION; DYSTONIA; ELECTROPHYSIOLOGY; EXTRAPYRAMIDAL SYNDROME; FAMILY HISTORY; FEMALE; FOLLOW UP; HUMAN; LIVER DISEASE; MAJOR CLINICAL STUDY; MALE; MEDICAL RECORD REVIEW; MENTAL DISEASE; MUSCULOSKELETAL DISEASE; MYOCLONUS; NEUROIMAGING; NEUROLOGIC DISEASE; NEUROPATHOLOGY; NUCLEAR MAGNETIC RESONANCE IMAGING; PARKINSONISM; PHENOTYPE; PRIORITY JOURNAL; PYRAMIDAL SIGN; URINARY EXCRETION; WILSON DISEASE;

ADOLESCENT; ADULT; ATHETOSIS; BRAIN; CERULOPLASMIN; CHELATING AGENTS; CHILD; CHILD, PRESCHOOL; CHOREA; COHORT STUDIES; CONSANGUINITY; COPPER; DYSTONIA; ELECTROENCEPHALOGRAPHY; FEMALE; FOLLOW-UP STUDIES; HEPATOLENTICULAR DEGENERATION; HUMANS; INDIA; MAGNETIC RESONANCE IMAGING; MALE; MIDDLE AGED; MYOCLONUS; OUTCOME ASSESSMENT (HEALTH CARE); PARKINSONIAN DISORDERS; PENICILLAMINE; ZINC SULFATE;

EID: 34247567771     PISSN: 00257974     EISSN: None     Source Type: Journal    
DOI: 10.1097/MD.0b013e318045a00e     Document Type: Article

References (43)

1. Akil M, Brewer GJ. Psychiatric and behavioral abnormalities in Wilson's disease. Adv Neurol. 1995;65:171-178.
2. Bittles AH, Devi ARR, Savithri HS, Sridhar NR, Rao NA. Consanguineous marriage and postnatal mortality in Karnataka, South India. Man. 1987;22:736-745.
3. Brewer GJ, Yuzbasiyan-Gurkan V. Wilson disease. Medicine (Baltimore). 1992;71:139-164.
4. Brewer GJ. Penicillamine should not be used as initial therapy in Wilson's disease. Mov Disord. 1999;14:551-554.
5. Brewer GJ. The treatment of Wilson's disease. In: Leone A, Mercer JFB, eds. Copper Transport and Its Disorders. Molecular and Cellular Aspects. New York: Plenum; 1998:115-126.
6. Brewer GJ. Wilson's Disease. A Clinician's Guide to Recognition, Diagnosis and Management. Boston: Kluwer Academic; 2001.
7. Brewer GJ, Terry CA, Aisen AM, Hill GM. Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy. Arch Neurol. 1987;44:490-493.
8. Chu N-S, Chu CC, Tu SC, Huang C-C. EEG spectral analysis and topographic mapping in Wilson's disease. J Neurol Sci. 1991;106:1-9.
9. Chu N-S, Haung T-P. Geographical variations in Wilson's disease. J Neurol Sci. 1993;117:1-7.
10. Czlonkowska A, Rodo M. Late onset of Wilson's disease. Report of a family. Arch Neurol. 1981;38:729-730.
11. Dastur DK, Manghani DK, Wadia NH. Wilson's disease in India. I. Geographic, genetic and clinical aspects in 16 families. Neurology. 1968;18:21-31.
12. Dening TR, Berrios GE, Walshe JM. Wilson's disease and epilepsy. Brain. 1988;111:1139-1155.
13. Dening TR, Berrios GE. Wilson's disease: clinical groups in 400 cases. Acta Neurol Scand. 1989;80:527-534.
14. Dobyns WB, Goldstein NP, Gordon H. Clinical spectrum of Wilson's disease (hepatolenticular degeneration). Mayo Clin Proc. 1979;54:35-42.
15. Dufour J-F, Kaplan MM. Muddying the water: Wilson's disease challenge will not soon disappear. Gastroenterology. 1997;113:348-350.
16. Fujitha M, Hosoki M, Miyazaki M. Brainstem auditory evoked responses in spinocerebellar degeneration and Wilson's disease. Ann Neurol. 1981;9:42-47.
17. Grimm G, Prayer L, Oder W, Ferenci P, Madl C, Knoflach P, Schneider B, Imhof H, Gangl A. Comparison of functional and structural brain disturbances in Wilson's disease. Neurology. 1991;41:272-276.
18. Harris ZL, Gitlin JD. Genetic and molecular basis for copper toxicity. Am J Clin Nutr. 1996;63:836-841.
19. Hitoshi S, Makoto I, Yoshikawa K. Mid-brain pathology of Wilson's disease. MRI analysis of three cases. J Neurol Neurosurg Psychiatry. 1991;54:624-626.
20. Hoogenraad TU. Wilson's disease. In: Major Problems in Neurology, Vol. 30. WB Saunders; 1996.
21. Jha SK, Behari M, Ahuja GK. Wilson's disease: clinical and radiological features. J Assoc Physicians India. 1998;46:602-605.
22. Joshua GE. Hepatolenticular degeneration (Wilson's disease) and rickets in children. Indian J Med Res. 1973;61:1876-1884.
23. Madden JW, Ironside JW, Triger DR, Bradshaw JPP. An unusual case of Wilson's disease. Q J Med. 1985;216:63-73.
24. Medalia A, Scheinberg IH. Intellectual functioning in treated Wilson's disease [letter]. Ann Neurol. 1991;29:573-574.
25. Murthy BS, Murthy JMK, Krishnaveni A, Reddy MVR, Das SM. Wilson's disease in South India and experience with zinc therapy. J Assoc Physicians India. 1988;36:417-419.
26. Oder W, Prayer L, Grimm G, Spatt J, Ferenci P, Kollegger H, Schneider B, Gangl A, Deecke L. Wilson's disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology. 1993;43:120-124.
27. Prashanth LK, Taly AB, Sinha S, Arunodaya GR, Swamy HS. Wilson's disease: diagnostic errors and clinical implications. J Neurol Neurosurg Psychiatry. 2004;75:907-909.
28. Saatci I, Topcu M, Baltaoglu H, Kose G, Yalaz K, Renda Y, Besim A. Cranial MR findings in Wilson's disease. Acta Radiol. 1997;38:250-258.
29. Satishchandra P, Swamy HS. Visual and brainstem auditory evoked responses in Wilson's disease. Acta Neurol Scand. 1989;79:108-113.
30. Scheinberg IH, Sternlieb I. Wilson's Disease. Philadelphia: WB Saunders; 1984.
31. Scheinberg IH, Sternlieb I. Treatment of the neurologic manifestations of Wilson's disease. Arch Neurol. 1995;52:339-340.
32. Sinha S, Jha DK, Sinha KK. Wilson's disease in Eastern India, a study of 49 cases. J Assoc Physicians India. 2001;49:881-884.
33. Sinha S, Taly AB, Ravishankar S, Prashanth LK, Venugopal KS, Arunodaya GR, Vasudev MK, Swamy HS. Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 2006;48:613-621.
34. Starosta-Rubinstein S, Young AB, Kluin K, Hill G, Aisen AM, Gabrielsen T, Brewer GJ. Clinical assessment of 31 patients with Wilson's disease. Correlations with structural changes on magnetic resonance imaging. Arch Neurol. 1987;44:365-370.
35. Steindl P, Ferenci P, Dienes HP, Grimm G, Pabinger I, Madl C, Maier-Dobersberger T, Herneth A, Dragosics B, Meryn S, Knoflach P, Granditsch G, Gangl A. Wilson's disease in patients presenting with liver disease: a diagnostic challenge. Gastroenterology. 1997;113:212-218.
36. Vrabelova S, Letocha O, Borsky M, Kozak L. Mutation analysis of the ATP7B gene and genotype/phenotype correlation in 227 patients with Wilson disease. Mol Genet Metab. 2005;86:277-285.
37. Walshe JM. Penicillamine, a new oral therapy for Wilson's disease. Am J Med. 1956;21:487-495.
38. Walshe JM, Yealland M. Chelation treatment of neurological Wilson's disease. Q J Med. 1993;86:197-204.
39. Walshe JM, Yealland M. Wilson's disease: the problem of delayed diagnosis. J Neurol Neurosurg Psychiatry. 1992;55:692-696.
40. Walshe JM. Penicillamine: the treatment of first choice for patients with Wilson's disease. Mov Disord. 1999;14:545-550.
41. Walshe JM. Wilson's disease. In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology, Vol. 49. Amsterdam: Elsevier; 1986:223-238.
42. Williams FJB, Walshe JM. Wilson's disease an analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents. Brain. 1981;104:735-752.
43. Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain. 1912;34:295-509.