PMC4408387_01
Electroencephalogram (EEG). Triphasic waves indicate an acute non-convulsive status epilepticus triggered by hyperammonemic encephalopathy.
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The electroencephalogram (EEG) examination showed triphasic waves reflecting a non-convulsive status epilepticus (Figure 1), which was successfully treated by intravenous administration of phenytoin.
Serum levels of ammonia over time. Ammonia serum level high above upper reference point at the first admission to the emergency department. Administration of lactulose, paromomycin and L-carnithin only temporarily induces a significant decrease. After modification of the antiepileptic treatment (replacement of valproic acid by levetiracetam), ammonia levels declined to normal values in concordance with clinical symptoms. Three weeks later, a recurrent episode of elevated ammonia serum levels with clinical symptoms under ongoing medical treatment. Decrease of the elevated serum level 48 hours after bilateral insertion of percutaneous nephrostomies. De novo increase of ammonia with neurological symptoms after clamp of nephrostomies. Normal serum levels of ammonia over long-term follow-up. The upper reference point of serum ammonia (<55 micromol/l) indicated by the dashed line.
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Extended laboratory examinations were subsequently performed and revealed a hyperammonemic hyperchloremic metabolic acidosis (max. ammonia 305 micromol/l, chloride 125 mmol/l; Figure 2). After replacing valproic acid with levetiracetam (3000 mg daily) for antiepileptic treatment, the ammonia levels declined to standard values (Figure 2).
Antegrade pyelography. Contrast study after insertion of nephrostomies testifies absence of impaired upper tract drainage and retention of urine in the sigmoid colon.
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To verify this hypothesis, bilateral percutaneous nephrostomies were inserted (Figure 3) and 48 hours later, the elevated serum levels of ammonia and chloride indeed decreased to the normal range.