--- a
+++ b/processing/MACCROBAT/26336183.txt
@@ -0,0 +1,29 @@
+A 35-year-old woman was admitted with a 5-day history of increasing drowsiness, fatigue, personality changes, generalised weakness and deteriorating mobility.
+Her medical history included a 10-year history of schizo-affective disorder, lithium-induced diabetes insipidus and hypothyroidism.
+Her medications included sodium valproate 1 g twice daily, carbamazepine, quetiapine and lithium.
+On admission, the patient was drowsy, difficult to rouse (sleeping all night and in the afternoon).
+Her Glasgow Coma Scale (GCS) was 14/15, her abbreviated mini-mental test score was 6/10 and she was noted to be slow in her responses.
+Neurological examination showed reduced power in her lower limbs (3–4/5) in a pyramidal distribution, and brisk reflexes with intact sensation in all modalities.
+Cardiovascular, respiratory and gastrointestinal examinations were unremarkable and there were no demonstrable features of hepatic disease.
+Routine biochemistry was unremarkable.
+The patient's alanine transaminase was 7 u/L (1–15), alkaline phosphatase 61 u/L (30–130), γ-glutamyl transferase 17 u/L (<45) and bilirubin was 6 μmol/L (<21).
+She had normal serum B12 and folate levels, and normal thyroid function tests.
+Serum lithium levels were within the therapeutic range.
+Her serum ammonia was 47 μmol/L (11.2–35.4) and valproate concentrations were elevated 140 mg/L (50–100).
+Brain imaging including CT and MRI did not show any haemorrhage or intracranial mass.
+Lumbar puncture demonstrated clear cerebrospinal fluid (white cell count <1×106/L, protein 0.25 g/L (0.2–0.4)).
+EEG demonstrated mild variable slowing and irregularity of background activity (likely due to medication).
+The differential diagnosis included encephalitis, Guillain-Barré syndrome and acute disseminated encephalomyelitis (ADEM), which were excluded through our investigations.
+In view of the raised valproate and ammonia levels, a diagnosis of valproate hyperammonaemic encephalopathy (VHE) was made at this point
+The dose of sodium valproate was reduced by half rather than being completely withdrawn.
+This was due to the complex and challenging nature of managing the patient's schizo-affective disorder.
+Within 3 days, there was evident clinical improvement in her mental status.
+The patient was able to sit and communicate with the team, and her abbreviated mental test score improved to 10/10.The serum ammonia normalised and valproate levels decreased to the therapeutic range.
+After 4 days she was discharged home.
+Results of investigations of her urea cycle were awaited.
+Three weeks postdischarge, the patient was clinically stable in terms of mental functioning, having had no further episodes of confusion or drowsiness.
+However, she continued to have fatigue and walking difficulties (due to residual leg weakness).
+Results of her serum amino acids revealed that our patient had carnitine deficiency evident by low acyl-carnitine, low free serum carnitine (14.2 μmol/L, normal range: 23–52) and low total serum carnitine (19.9 μmol/L, normal range: 27–63).
+She was started on oral carnitine 1 g twice daily.
+Within 2 weeks, her symptoms resolved completely with normalisation of serum carnitine levels (free carnitine 25.2 μmol/L and total carnitine of 41.3 μmol/L).
+The sodium valproate was initially stopped completely, but due to a serious relapse of the patient's schizo-affective disorder, it was restarted and she is now well on valproate 1 g once daily and long-term carnitine supplements.