diagnosis and prevalence of subclinical hepatic encephalopathy in apparently healthy children and adolescents with cirrhosis

Subclinical or minimal hepatic encephalopathy [SHE or mHE], in con-to hepatic encephalopathy is not associated with overt neuropsychiatric symptoms but rather with subtle changes detected by special psychomotor and/or neurophysiologic tests. The purpose of this study was to elucidate the validity of the neuropsychological and neurophysiological to diagnose SHE in children and adolescents with liver cirhosis. Furthermore, to detect its prevalence in a specific period. A prospective controlled study was applied on 23 patients [10 males and 13 females with mean age 10.24 +/- 3.87 years] with different etiologies of liver cirrhosis through the period from August 2002 to August 2003. Fifteen healthy and adolescents with matched age, sex and education standard, included as a control group. All patients and controls were subjected to thorough clinical evaluation, laboratory tests of liver functions, neuropsychological assessment using the Egyptian version of Wechsler intelligence tests, visual and mapping analysis of electroencephalographic records and event related potential [P300] testing. Patients and controls were age and sex matched d showed no significant difference in periods of education. Serum bilirubin and prothrombin time were significantly affected in patients compared with controls. Verbal, performance and full scale IQ were all significantly affected in patients compared with controls; a cut off point of abnormal test performance was seated at scaled score 2 SD below th mean of the controls. Patients have sigr4flcant slowing in EEG back ground activity compared to controls [P=0.001]; a cut off point for diagnosis of slow activity was seated at 6.8 C/S. Prevalence of SHE among cirrhotic patients was 47.8% by applying two abnormal neuropsychologic tests and 65.2% by applying two abnormal psychologic tests together with significant slowing of EEG background activity. Prevalence of SHE among patients with Child-Pugh class B/C cirrhosis was significantly higher than that recorded in patients with class A cirrhosis [P<0.05] Event related potentials [P300] mean wave latency and mean wave amplitude showed no significant differences between patients and controls however, cirrhotic patients with SHE had significant prolongation of mean P300 wave latency than patients without SHE [P=0. 031]. Clinical follow up [5 +/- 1 .64months] revealed that two out of fifteen mHE diagnosed patients died after 3 and 5 months from initial testing and the two were Child-Pugh class C and overt encephalopathy was precipitated by sepsis. Minimal hepatic encephalopathy is quite prevalent in our patients [65.2%] and the diagnosis can be ascertained by special neuropsychological and/or neurophysiological tests. Minimal hepatic encephalopathy is thought to predict the development of overt hepatic encephalopathy, thus it is clinically relevant

Diagnostic yield of clinical, electromyographic and pathologic studies in muscle disorders

Muscle diseases are classified into two major groups, i.e.; myopathic which also includes muscle dystrophies and neurogenic. Investigations of muscle disease include clinical biochemistry, electromyography, muscle and cytogenetics. The aim of our study was to evaluate the diagnostic yield of clinical biochemistry, eletromyographic and pathologic in different muscle disorders-further more to detect the prevalence of different muscle disorders in our locality in a specific period. Sixty patients with generlized hypotonia and hyporeflexia [40 males and 20 with ages ranged from 2 months to 13 years were studied consequatively in addition to 15 healthy controls of matched age and sex for comparison in certain aspects. All cases were subjected to: a] thorough clinical and neurological evaluation, b] assessment of serum creatine kinase, c] electromyography and d] muscle biopsy from the most affected for histologic and histochemical studies. Frequency of muscle disorders was pathologically as: 41.7% in muscle dystrophies, 25% in congenital myopathies 8.3% in inflammatory myopathies and 25% in spinal muscle atrophy. Clinically; muscle dystrophies were found in 41.7%, however with difference in subtypes. Congenital myopathies were suggested in 30% of cases inflammatory myopathies in 8.3% and spinal muscle atrophy in 20% Electromyographic studies were compatible with the pathologic results in all cases of myopathies and spinal muscle atrophy. All cases of muscle dystrophies were evaluated as myopathic disorders. Serum creatine kinase was highly significantly elevated in the group of muscle dystrophies, significantly elevated in inflammatory myopathies and mildly elevated in spinal muscle atrophy as compared with controls. Clinical biochemistry and pathologic compatibility was found in 92% of dystrophinopathies group, 88% of myopathyies and 80% of neurogenic muscle atrophy .Apart from the group of muscle dystrophy, the EMG yield was compatible with the pathologic diagnosis of all cases of myopathies, and neurogenic muscle atrophy. Thus all measures considered concordant in diagnosis of muscle diseases