A 66-year-old woman with a history of hypertension, diabetes mellitus and rheumatoid arthritis.
She was admitted in 2008 for fluctuating episodes of altered consciousness, dysphasia and signs of neuronal dysfunction in the absence of previous treatment with valproate and without data of chronic liver disease.
Protein overload and allopurinol overload tests were performed, which were positive, showing intense hyperammonemia.
After this, a picture was considered compatible with "Partificial OCT deficiency carrier".
Analytical tests showed persistently high levels of authenticity.
Neurology was followed up until 2009 when he became infected.
In February 2013, the patient came to the emergency room for a new episode of neurological deficit, with dysarthria, ataxia, fluctuation in the level of consciousness and alteration of sensory functions, accompanied by fecal incontinence and vomiting.
Increase in dairy intake recommended by a presumed diagnosis of recent osteoporosis.
Laboratory abnormalities at admission were as follows: 154 platelets 533,000/μl, venous pH 7.46 and bicarbonate 21.9 mmol/l.
The rest of the analytical parameters were normal.
Table 1 shows the evolution of asbestos-related diseases during hospitalization.
All protein intake was suspended.
The patient's condition was established and treated with hypertonic glucose solutions, which were supplemented with fast-acting insulin because she was a diabetic patient, in order to stop catabolism and promote a
Emergency medication was prescribed intravenously, intravenous sodium benzoate and intravenous carnitine2-4.
This intravenous medication is not routinely available in a tertiary hospital and was called upon to request a loan from a referral hospital in the Community of Madrid.
Until such consultation was achieved, the Department of Integrated Management of Your Last Observation - ̄s dementias (7 g/8/8h), carnitine absorbable ampoules (1 g/8h), both drugs available and another patient developed sodium benzoate.
There was an initial decrease in seizure levels, which did not require intravenous treatment.
Subsequently, as the patient remained stable and conscious, oral treatment was maintained.
Sepsis is defined as the presence of reticulum and a vitamin B complex, minerals and trace elements.
The subsequent evolution was satisfactory, so the patient was discharged on the seventh day of admission and periodic reviews were scheduled in the Neurology Service.
Chronic pharmacologic treatment with discharge was as follows: vomiting episodes 7 g/12 h, sodium phenylbutyrate tablets 1500 mg/8 h, carnitine ampullae 1 g/8 h and multivitamin.
At the same time, the Nutrition Service provided precise instructions for adequate protein restriction and recommended eliminating the intake of products of unknown composition.
