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Cardiac abnormalities are frequently associated with myotonic dystrophy (MD), and electrocardiographic (ECG) changes are not rare in these patients. The most common ECG abnormalities are PQ or QT interval prolongation, low P wave amplitude, ST elevation or depression, negative T waves, conduction and rhythm abnormalities. We analysed ECG changes in 42 patients with MD. ⋯ We found no ST alterations. ECG changes were more frequent in patients with severe disease, but it was not statistically significant. There was no correlation between age and ECG abnormalities in our patients.
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We present the facts on kind and frequency of disorder of high cortical functions in amyotrophic lateral sclerosis. It was considered for a long time that this disease affects selectively motor neurons. The signs of degeneration found in other structures and associated signs of other neurological disorders brought the diagnosis of amyotrophic lateral sclerosis under suspicion. ⋯ The nosological status of motor neuron disease with dementia is still controversial. There is a possibility of a new clinical entity or a variant of motor neuron disease. Taking into account the fact that pathological changes of motor neurons and the natural history of amyotrophic lateral sclerosis and amyotrophic lateral sclerosis with dementia are not essentially different, as well as the fact that frontal dysfunction is detected in non-demented patients with amyotrophic lateral sclerosis, the authors consider that we are facing a variant of well-known disease of motor neuron.
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In order to define the cerebrospinal fluid (CSF) profile indicative of multiple sclerosis (MS), it is essential that each laboratory specifies the percentage of clinically definite MS patients with as well as the ranges of obtained values for each CSF parameter in these patients. CSF of 92 patients with clinically defined MS were analysed for cell count, concentrations of total protein, albumin and IgG, blood-CSF barrier function as assessed by CSF/serum albumin quotient, quantitative measurements of intrathecal IgG production (IgG index and IgG daily synthesis rate) and the presence of CSF oligoclonal IgG. For the detection of CSF oligoclonal IgG the isoelectric focusing (IEF) of unconcentrated CSF on agarose with transfer of proteins to cellulose nitrate and immunoperoxidase staining, was performed. ⋯ In 99% of MS patients, the total protein was <980 mg/L, the albumin <740 mg/L and the total cell count <20 cells per mm3. According to our data, the CSF findings that support the diagnosis of clinically definite MS are: 1) the presence of intrathecally produced CSF oligoclonal IgG by IEF; 2) normal or slightly to moderately elevated level of CSF proteins and albumin; 3) normal CSF-brain barrier function or slight to, rarely, moderate CSF-brain barrier dysfunction as assesed by CSF/serum albumin quotient, and 4) normal cell count or slight pleocytosis. If this profile is not found in a patient suspected of MS, the diagnosis should be questioned or a complication should be expected.
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Growth hormone (GH) response to dexamethasone (DEX) in 10 poorly controlled insulin dependent diabetic patients (IDDM) without clinical evidence of diabetic complications and in 10 healthy controls, was studied. GH responses to DEX were compared with pituitary GH response to growth hormone releasing hormone (GHRH). ⋯ The peak GH responses to GHRH and DEX were similar in the controls and IDDM patients (23.8 +/- 6.49 vs 38.87 +/- 7.26, p > 0.05 in GHRH test and 13.71 +/- 3.59 vs 17.33 +/- 5 23, p > 0.05 in DEX test). No significant difference between area under curve during GHRH (1386. +/- 490.69 vs 1966.89 +/- 561.46, p > 0.05) and during DEX test (1085.8 +/- 239 856 vs 501.87 +/- 847.16, p > 0 05) in the controls and IDDM patients, were established There was no significant correlation between basal and peak GH values and AUC during both tests, and HbA1C and duration of diabetes It is concluded that GH response to GHRH was normal and that our patients had preserved the integrity of the hypothalamo-pituitary axis, thanks to the suggested mechanism of dexamethasone action via somatostatin.
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During an outbreak of haemorrhagic fever with renal syndrome (HFRS) in 1989, five children (3 girls, 2 boys, aged 6.8-16 years) with severe clinical form of the disease, were treated at the Institute of Mother and Child Health of Serbia; four of them were followed-up 22-28 months thereafter. The main clinical features were: fever, headache, myalgia, abdominal and back pains, and vomiting in all, and haemorrhagic syndrome in three; renal syndrome with severe acute renal failure in all five patients. ⋯ It was not possible to differentiate these two serogroups on the basis of clinical features. This finding gave further evidence of circulation of different hantaviruses causing severe HFRS in Serbia.