Wilson's disease: Brain MRI, Tc-99m HMPAO SPECT and clinical correlation

Introduction: Wilson's disease is a genetic disease characterized by excess deposition of copper in the liver, brain, cornea and kidneys. Brain damage leads to several psychiatric and neurological manifestations. However, a little research has been published about the role of anatomo-functional diagnostic modalities in the evaluation of the clinical course of the disease

Aim of the study: to evaluate and compare the role of brain MRI and Technetium-99m hexamethylpropylene-amine oxime [Tc-99m HMPAO] SPECT in assessing brain involvement in Wilson's disease and correlating these findings with the clinical presentations of the disease

Patients and Methods: eighteen patients with established Wilson's disease [10 males and 8 females] aged between 21 to 53 years were included in the study. The clinical picture was of neurologic type [nWD] in 11 patients [61.1%], and of the hepatic type [hWD] in 7 patients [38.9%]. All patients were subjected to careful general and neurological assessment, cognitive assessment using M.M.S.E scale and Trial Making test, ophthalmic slit lamp examination, biochemical tests including urinary copper and serum ceruloplasmin levels, abdominal ultrasound, liver biopsy [only in 5 patients], MRI of the brain and Tc-99m HMPAO brain SPECT

Results: brain MRI was abnormal in 11/18 patients [sensitivity 61.1 %]. It was abnormal in 9/11 nWD patients [sensitivity 81.8%], while it was positive in only 2/7 of hWD patients [sensitivity 28.6%]. Brain Tc-99m HMPAO SPECT was abnormal in 15 out of 18 patients [sensitivity 83.3%]. The scan was abnormal in 10/11 patients with nWD [sensitivity 90.9%] and in 5/7 of patients with hWD [sensitivity 71.4%]. The correlation between the clinical presentation of the disease, radiological, and SPECT findings still not clear in most cases

Conclusion: brain Tc-99m HMPAO SPECT and brain MRI are useful tools for evaluating patients with Wilson's disease. Brain SPECT has a higher sensitivity than brain MRI in detecting brain abnormalities, however the clinico-anatomical correlation still not established yet in many cases

Progressive supranuclear palsy: New brain stem measurement and its clinical correlations

Objective: to detect the value of mid-sagittal MRI measurements of the midbrain and pons in the diagnosis of Progressive Supranuclear Palsy [PSP] and to differentiate it from mimics [Parkinson Disease [PD] and Multiple-System Atrophy of the Parkinson Type [MSA-P]], and to what extend these radiological measurements correlate with the clinical aspects of PSP

Methods: MRI of 13 patients with PSP, 25 patients with PD, and 10 patients with MSA-P, as well as 20 age-matched controls were prospectively studied. The areas of the midbrain tegmentum and the pons were measured on mid-sagittal MRI using the display tools. The ratio of the area of the midbrain to the area of the pons was also evaluated in all subjects. Patients were also evaluated clinically using Hoehn and Yahr Scale, UPDRS and MMSE scale in addition to full general, neurological assessment and routine laboratory investigations

Results: the average midbrain area of the patients with PSP [57 mm[2]] was significantly smaller than that of the patients with PD [101 mm[2]] and MSA-P [97.9 mm[2]] and that of the age-matched control group [116.9 mm[2]]. There was no overlap between patients with PSP and patients with PD or normal control subjects regarding midbrain area values. However, patients with MSA-P showed some overlap of the values of individual areas with values of individual areas with values from patients with PSP. The ratio of the area of the midbrain to the area of pons in patients with PSP [0.123] was significantly smaller than that in those with PD [0.207] and MSA-P [0.265] and in normal control subjects [0.235]. Also, use of the ratios allowed differentiation between PSP groups and the MSA-P group. Good significant correlation was observed between various radiological measurements studied and disease duration, staging and severity

Conclusion: the mid-sagittal MRI measurements of the midbrain area can differentiate between the PSP from PD, MSA-P and normal aging and these radiological measurements correlated well with the clinical aspects of the syndrome of PSP

Evaluation of self care program of mentally retarded children at Alwafaa volunteering social center, Sohar Wilayat, Sultanate of Oman

The increasing number of mentally retarded children from 26 per 1000 to 38 per 1000 in Oman according to the census of 1995, as being the last statistical data, is a growing concern in the medical field. It may be due to cultural practices such as consanguineous marriage, as the family considers it has significant social benefits. Therefore, the children have complex needs that differ from those of unimpaired age mates and need special attention and care, based on their physical and mental abilities. Several programs to help those children are offered. One of these programs is the self care program for mentally retarded children to promote independence self care. So, the objective of this study was to evaluate the effectiveness of self care program on mentally retarded children. The study was conducted at Alwafa handicapped center, Sohar wilayat Sultanate of Oman. A purposeful sample of 107 mentally retarded children were selected, aged between 8 and 18 years and were instituted during the period from June 2004 to June 2005. Data were collected by interview with the teachers of those children, child health file, and direct observation of children while performing activities by using observation check list. Data analysis was done and the results revealed that most of mentally retarded conditions were of moderate type. On admission, most of them [81.3%] were not able to do any physical activities, while after program implementation, they showed progressive improvement in their performance of daily care activities. The degree of improvement on top of intervention showed significant relation to the degree of mentally retarded conditions. Regarding to the duration of stay in the center, the results showed that long stay with more training to the child lead to more improvement in his ability to perform self care activities. Based on the present results, the following recommendation are requested: the importance of continuing educational program with continuous assessment to mentally retarded children to evaluate their progress. Also, the importance of involving the teachers and families in the program to improve their performance