caa a22 4500 445356383 CHVBK 20180317142901.0 cr unu---uuuuu 170323e20110501xx s 000 0 eng 10.1007/s10072-011-0518-3 doi (NATIONALLICENCE)springer-10.1007/s10072-011-0518-3 Headache and neuropsychic disorders in the puerperium: a case report with suspected deficiency of urea cycle enzymes [Elektronische Daten] [Maria Tonini, V. Bignamini, M. Mattioli] An enzymatic abnormality of the urea cycle is a metabolic disorder occasionally seen in adults, but particularly in the puerperium. The main risk is acute hyperammoniemic encephalopathy, leading to psychosis, coma and even death if not diagnosed promptly and treated appropriately. Headache is frequent in the puerperium normally manifesting between 3 and 6days after delivery. We describe here a 39-year-old woman, who 3days after delivery presented diffuse tension-type headache and depression, followed by behavioral disorders, psychomotor agitation, epileptic seizures, and finally coma 2days later. Pregnancy and normal delivery: routine blood chemistry findings, CT scan, MR imaging, angio-MR of the brain, and lumbar puncture were normal. EEG when seizures started, it showed diffuse slowing, as in the case of metabolic encephalopathy. This led us to assay blood ammonia, which was high at >400mmol. Liver function and abdominal US were normal; hence, we suspected a urea cycle enzymatic abnormality, and requested for genetic tests. These confirmed a congenital primary metabolic deficiency of arginine succinate synthetase, with high citrullinemia (type II, adult form). Dialysis was started promptly, with initially iv arginine, then orally, plus medical therapy for the hyperammoniemia and a low protein diet; plasma ammonia dropped swiftly to normal, and her state of consciousness gradually improved until all the clinical symptoms had resolved. Ammonia assay should always be considered in the first few days of the puerperium in women with headache and behavioral disorders, to exclude an inborn deficiency of the urea cycle, which may have gone unnoticed until then. Springer-Verlag, 2011 Puerperium nationallicence Headache nationallicence Hyperammoniemia nationallicence Urea cycle disease nationallicence Neuropsychic disorder nationallicence Tonini Maria Headache Center, Neurology Department, "G. Salvini” Hospital, AO "G. Salvini”, Viale Forlanini, 121, 20200, Garbagnate Milanese (MI), Italy aut Bignamini V. Neurology Department, Treviglio Hospital (BG), Treviglio, Italy aut Mattioli M. Headache Center, Neurology Department, "G. Salvini” Hospital, AO "G. Salvini”, Viale Forlanini, 121, 20200, Garbagnate Milanese (MI), Italy aut Neurological Sciences Springer Milan 32(2011-05-01), 157-159 1590-1874 32<157 2011 32 10072 https://doi.org/10.1007/s10072-011-0518-3 text/html Onlinezugriff via DOI 1 brief-communication jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10072-011-0518-3 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Tonini Maria Headache Center, Neurology Department, "G. Salvini” Hospital, AO "G. Salvini”, Viale Forlanini, 121, 20200, Garbagnate Milanese (MI), Italy aut NATIONALLICENCE 700 1- Bignamini V. Neurology Department, Treviglio Hospital (BG), Treviglio, Italy aut NATIONALLICENCE 700 1- Mattioli M. Headache Center, Neurology Department, "G. Salvini” Hospital, AO "G. Salvini”, Viale Forlanini, 121, 20200, Garbagnate Milanese (MI), Italy aut NATIONALLICENCE 773 0- Neurological Sciences Springer Milan 32(2011-05-01), 157-159 1590-1874 32<157 2011 32 10072 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer