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Indian Journal of Medical Sciences, Vol. 60, No. 11, November, 2006, pp. 471-472 Letter To Editor Wilson's hepatitis: Unusual presentation and survival Dixit VK, Vashistha P, Kate MP, Abhilash VB, Mohapatra A, Jain AK Department of Gastroenterology, Institute of
Medical Sciences, Banaras Hindu University,
Varanasi - 221 005, UP, India Code Number: ms06070 Sir, Neurological manifestations of Wilson disease (WD) typically occur in the early twenties or later, usually presenting as movement disorders[1] without any sensory symptoms except for headache, which is not a presenting feature. Hepatic manifestations occur usually in the first decade (83%), with acute liver failure occurring only in 10% of cases, of which < 1% survive without orthotopic liver transplantation.[2] A 7-year-old girl (nonconsanguineous parents) presents with jaundice, nausea and vomiting since 3 days. She developed drowsiness, which rapidly progressed to coma in 1 day. There was history of headache for the last 8 months, for which she was being treated for migraine (excluding refractory errors/ structural brain /seizure disorders); she received valproate and topiramate, with inconsistent improvement in symptoms, following which she stopped the therapy on her own (2 months prior to admission). On examination, the subject was in altered sensorium (Glasgow coma scale 9/15); asterixis was present with deep icterus and mild pallor with no stigmata of chronic liver disease. Liver span (5 cm) was decreased. On torch light eye examination, a peripheral corneal ring was suspected, which was confirmed by slit lamp examination to be Kayser-Fleischer ring in both the eyes. Investigations revealed hemoglobin 6.1 gm%, total leukocyte count 11,200/cmm with neutrophil 75% and lymphocyte 23%. Reticulocyte production index was 2.94 and peripheral blood picture showed hypochromicity microcytosis with anisopoikilocytosis. Total bilirubin was 38.4 mg%; direct bilirubin, 32.1 mg%; AST, 147 IU/l; ALT, 73I U/l; alkaline phosphatase, 272 IU/L; prothrombin time, (I.N.R.) 2.7 (Nazer prognostic index was -9).[3] HBsAg was negative; IgM HEV negative; IgM HAV negative; Se Ceruloplasmin 14 mg/dl; Coomb's test, direct and indirect, negative; serum uric acids 3.2 mg/dl. Ascitic fluid examination revealed total cell count - 20/cmm;, lymphocytes - 90%; albumin - 0.3 g/dl; SAAG-2 and 24 h urine copper without challenge - 331μg/L. The younger the age at presentation, more acute are the manifestations and higher the mortality in patients with WD, except in asymptomatic siblings.[4] Our case developed neurological symptoms at the age of 7 years, which is unusual for this subset of patients. Moreover, the patient presented with headache, which is an uncommon presenting feature in WD. After a period of 8 months of from initial presentation, the subject developed acute liver failure. Acute liver failure is a less common (10%) but a devastating presentation of WD.[2] In our case, the patient improved only with conservative management, which is an unusual feature. Following gradual improvement, she was started on copper chelation therapy in the second week; penicillamine 250 mg b.i.d. was started along with zinc 25 mg thrice daily. Patient responded very well in the form of rapidly resolving jaundice and improvement in headache over 4 weeks.[4] Headache or behavior symptoms may be the presenting manifestations of WD even in the absence of clinical evidence of hepatic involvement. The combination of involvement of any of the organ systems with evidence of liver dysfunction suggests WD at any age.[5] It is highlighted that screening for WD is warranted in unexplained headache or unexplained behavior disorder not responding to usual medications. Simple test in the form of AST and ALT, with ophthalmologic examination for K-F ring and serum ceruloplasmin would be helpful as early pointers towards the hepatic involvement of WD. References
Copyright 2006 - Indian Journal of Medical Sciences |
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