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Computed tomography in hepatolenticular degeneration (Wilson's disease).

Fifteen cases of hepatolenticular degeneration have been studied by means of computerized cranial tomography (CT). The common abnormalities were hypodense areas in the regions of the basal ganglia, in 9 cases; ventricular dilatation, in 7 cases; cortical atrophy, in 5 cases and brain-stem atrophy, in 4 cases. CT abnormalities were most common and most marked in patients with neurological presentations, but 1 of 3 asymptomatic patients discovered by the genetic screening of the affected family had hypodense areas in bilateral lenticular nuclei.

Atrophy↗
PubMed1978

Osteoarthropathy of hepatolenticular degeneration.

Ninety-six joints of 25 patients with hepatolenticular degeneration have been studied by means of scintigraphs with technetium (99mTc), a method which can disclose early synovial inflammation and postinflammatory articular degeneration. Signs of synovitis were found in 22 joints of 10 patients (40%) and degenerative changes were present in 15 joints of eight patients (32%). Eleven patients complained of joint pains; in four of them they were the presenting symptom. The radiological study showed osteoporosis in 22 patients (88%); flexion deformity was found in two cases, anomalous osteophytes in two, and signs of growth arrest, features of rheumatoid arthritis and aseptic necrosis in one case each. The pathogenesis of osteoarticular changes in Wilson's disease is discussed.

Adolescent↗
PubMed1997

[Contrast analysis of brainstem auditory evoked potential in untreated and treated hepatolenticular degeneration patients with treatment of combining traditional Chinese and Western medicine].

OBJECTIVE: To observe the effect of combination therapy of traditional and Western medicine (TCM-WM) on brainstem auditory evoked potential (BAEP) in hepatolenticular degeneration patients. METHODS: BAEP was recorded and analyzed in 65 patients with hepatolenticular degeneration (HLD) before and after decoppered TCM-WM treatment. RESULTS: The peak latency (PL) and interpeak latency (IPL) of BAEP in untreated patients were greatly increased compared with those of control group, especially in the prolongations of III, V, III- V and I-V. The PL and IPL improved as improvement of clinical symptoms in HLD patients treated with TCM-WM than that in untreated patients. The abnormal rates of men and women in BAEP were decreased from 21.97% and 19.05% in untreated patients to 9.09% and 6.75% in treated patients respectively. CONCLUSION: BAEP not only has an complementary diagnosis but also judge the efficacy of treatment on the HLD disease.

Adolescent↗
PubMed1976

Skin lesions induced by penicillamine. Occurrence in a patient with hepatolenticular degeneration (Wilson Disease).

A 41-year-old patient with hepatolenticular degeneration (Wilson disease), who had been treated for 15 years with penicillamine, developed small white papules at sites of venipuncture in the antecubital fossae and at surgical suture sites. Histologically, these papules showed focal areas of connective tissue degeneration in the dermis, but there was no evidence of inclusion cysts. The changes most likely resulted from the effect of penicillamine on new connective tissue formation at the sites of injury. The patient also developed crinkling of the skin of her face and neck while on the penicillamine regimen, and these changes were attributed, at least in part, to the effects of this drug on connective tissue.

Adult↗
PubMed1977

[Clinical polymorphism and ceruloplasmin variants in hepatolenticular degeneration].

The relationship between differences in the clinical polymorphism of hepatolenticular degeneration (Wilson's disease) and characteristics of CP (ceruloplasmin) structural changes were investigated. The comparative study of Wilson's disease patients revealed two forms of clinical development of this disease which differ from each other by the expression of the visceral symptoms preceding the establishment of the typical neurological picture. The peptide map analysis of tryptic hydrolysates of the CP from individual patients has demonstrated the altered peptide patterns in five cases. Clinical and genetic heterogeneity of Wilson's disease is discussed.

Adolescent↗
PubMed2001

[Study on the clinical misdiagnosis of hepatolenticular degeneration].

OBJECTIVE: To study the proportion and frequency of misdiagnosis of hepatolenticular degeneration (HLD), diseases easily confused with HLD, and the relation between misdiagnosis and curative effect so as to sum up experience and improve diagnosis. METHODS: 1 011 cases with the confirmed diagnosis of HLD who were hospitalized in the Hospital Affiliated to the Institute of Neurology, Anhui College of TCM from November 1993 to June 2000 were analyzed. Those patients who had attended two or more than two hospitals and had been misdiagnosed as other diseases or failed to get a clear diagnosis for 3 months and over before hospitalization were included in the group of clinical misdiagnosis or the group without a definite diagnosis. Relevant data were analyzed. RESULTS: 516 cases (51.04%) were misdiagnosed, 193 cases (19.09%) failed to be diagnosed as a specific disease, only 302 cases (29.87%) had been diagnosed as HLD within 3 months after the onset. The 516 cases had been misdiagnosed as more than 100 different diseases, including all types of hepatitis, cirrhosis, splenomegaly, hepatomegaly, arthritis, nephritis, encephalitis, encephalopathy, psychosis, anaemia, and so on. The curative effect was much better in the group with early diagnosis than in the groups with misdiagnosis and without a clear diagnosis (P < 0.01). CONCLUSION: HLD is often misdiagnosed as other diseases or fails to get a clear diagnosis. Early diagnosis and treatment without delay is critical to the prognosis. Patients with symptoms described in this article should be examined for the presence of corneal K-F ring and copper metabolism teat should be made when necessary.

Adolescent↗