In September 2002 the European Medicines Evaluation Agency (EMEA) approved a licence for alteplase (rt-PA) in the treatment of ischaemic stroke within 3 h of symptom onset. One of the conditions required by the EU regulatory authorities for the official definitive approval of thrombolytic therapy was that treatment safety should be monitored over a period of three years by entering all treated patients in the SITS-ISTR (Safe Implementation of Thrombolysis in Stroke -Thrombolysis Register) web register, in accordance with a study protocol, the SITS Monitoring Study - SITS-MOST. The aims of this study are to evaluate the efficacy (in terms of functional independence for activities of daily living at three months after treatment) and safety (in terms of symptomatic intracerebral haemorrhage and death) of the thrombolytic treatment in real routine clinical practice, outside the ideal conditions of an experimental setting, and to compare the results with a systematic review from the randomised clinical trials.
Allgrove syndrome is a rare autosomal recessive disorder characterised by childhood onset, alacrima, oesophageal achalasia, adrenocortical insufficiency, neurological and occasionally autonomic involvement. Although the disease has been associated with mutations in the ALADIN gene on chromosome 12q13, it is genetically heterogeneous. The case we report is interesting because of its onset in adulthood, long duration of disease and prominent neurological dysfunctions. After the onset of neurological abnormalities the diagnosis went unrecognised for years until the patient presented for evaluation of dysphagia. The presence of achalasia with dysphagia, adrenal insufficiency, reduced tear production, optic atrophy and peripheral motor-sensory neuropathy with axonal loss led us to clinically diagnose Allgrove syndrome even though a genetic study showed no mutations in the ALADIN gene exons. The case we report shares many clinical features with Allgrove syndrome and, even with the limitations of a single case, underlines the variability in this syndrome and the need for appropriate investigations along with a multidisciplinary approach.
A longitudinal electroencephalographic study of 100 patients suffering from acute leukaemia is reported. An high incidence of EEG abnormalities indicates the central nervous system involvement with no clinical manifestations. The patients have been divided in two groups: A) with normal EEG and B) with pathological (cerebro-meningeal localisation) but many causes (metabolic, toxic or microcirculatory disturbances) can produce the abnormalities. Moreover therapy may cause CNS disturbances with EEG evidence. The most damaging seems to be the simultaneous association of radiation therapy and methotrexate.
Rasmussen's encephalitis (RE) is a chronic, progressive disease that typically occurs in childhood, rarely in adulthood. When it does occur in adulthood, it may be associated with atypical electro‐clinical features, and neuroimaging alterations usually help diagnosis. Unlike childhood forms, in which epilepsia partialis continua is usually observed, the EEG pattern in adult variants may be aspecific. We describe a highly interesting case of late‐onset RE in which an electrical status epilepticus was not detected by the surface EEG, but by a recording with subdural electrodes.
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