A 57 year old woman with post-traumatic complex partial seizures was admitted because of recurrent episodes of altered mental state over the preceding 4 years, each lasting up to 5 days. There was a history of dietary protein intolerance since childhood and two of her daughters had died in the neonatal period from unexplained encephalopathies. In hospital she developed fluctuating confusion, amnesia, and sudden episodes of unresponsiveness. An EEG was consistent with complex partial status epilepticus but there was no response to benzodiazepines. Nasogastric feeding and sodium valproate were given and shortly afterwards she lapsed into a deep coma. Blood ammonia and urinary orotate were raised, and genetic testing confirmed that she was a carrier of a mutation in exon 3 of the ornithine transcarbamylase gene (C to T at position 92). Treatment with protein restriction, carnitine, and sodium phenylbutyrate led to a full recovery over a period of 3 months. To our knowledge this is the oldest age of onset yet described in a manifesting carrier. She is the fifth patient with heterozygous ornithine transcarbamylase deficiency reported to have had a severe reaction to sodium valproate. Hyperammonaemic encephalopathy should be considered in patients of any age who experience fluctuating confusion. (J Neurol Neurosurg Psychiatry 2000;69:813-815)
We believe that forensic medicine should play a significant role in the COVID-19 pandemic. Forensic pathologists should ask and answer various questions through autopsy cases during the COVID-19 period, thus providing a significant contribution to science. Some of the potential roles of forensic medicine in this issue include: determining the exact cause of death among the deceased who were SARS-CoV-2 positive, contribution to the accuracy of mortality statistics, understanding pathological mechanisms of COVID-19, tracking the presence of the virus over time, survival of the virus after death as well as dealing with medicolegal issues. A detailed multidisciplinary analysis of autopsy samples would undoubtedly help understand this new illness and its clinical management. Therefore, autopsies during the COVID-19 pandemic should not be an exception, but certainly a rule.
There is an extensive literature on epilepsy and violence, but no study has addressed aggression (i.e. apparently intentional violence) in a residential-care population. We performed a retrospective study at the Chalfont Centre for Epilepsy (a residential-care facility in rural Buckinghamshire) in order to determine the frequency and character of episodes of aggression. This allowed us to identify a group of aggressive subjects who were compared with age- and sex-matched control subjects drawn from the remaining residents. We found the prevalence of aggression to be 27.2% in 1 year amongst long-term residents. The overall frequency was estimated at between 121 and 207 incidents per 100 persons per year. A few incidents (0.7%) were related to an acute psychosis but they were more likely to result in significant injury. Offenders were younger than non-aggressive residents. Gender, age of onset of epilepsy, history of psychosis, mobility, abnormality on MRI scan, learning disability and seizure frequency were not associated with aggressive conduct.
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