PMID- 24258525
OWN - NLM
STAT- MEDLINE
DCOM- 20150106
LR  - 20140325
IS  - 1439-1899 (Electronic)
IS  - 0174-304X (Linking)
VI  - 45
IP  - 2
DP  - 2014 Apr
TI  - Epilepsia partialis continua and generalized nonconvulsive status epilepticus 
      during the course of argininemia: a report on two cases.
PG  - 123-8
LID - 10.1055/s-0033-1360479 [doi]
AB  - Argininemia is a rare inherited disorder of the urea cycle because of a 
      deficiency of the enzyme arginase I causing an increase of arginine and guanidino 
      compounds in the blood, urine, and cerebrospinal fluid. The clinical picture is 
      characterized by a mild cognitive dysfunction, progressive asymmetrical 
      paraparesis, and seizures. Here, we describe two cases of argininemia where 
      either epilepsia partialis continua (EPC) or nonconvulsive status epilepticus 
      (NCSE) were the presenting manifestations of epilepsy. This is the first report 
      of EPC in an urea cycle disorder. In both the cases, status epilepticus resolved 
      with anticonvulsive drugs. EPC was successfully treated with levetiracetam, and 
      NCSE with valproic acid. No side effects were observed. Because hyperammonemia 
      and NCSE may have the same features of stupor, a neurophysiological approach 
      might prove useful in differentiating these two conditions. Overall, our results 
      strongly indicate that a correct NCSE diagnosis is mandatory to prevent further 
      deterioration in these patients.
CI  - Georg Thieme Verlag KG Stuttgart . New York.
FAU - Grioni, D
AU  - Grioni D
AD  - Child Neuropsychiatric Clinic, San Gerardo Hospital University of Milan Bicocca, 
      Monza, Italy.
FAU - Furlan, F
AU  - Furlan F
AD  - Center for Metabolic Diseases MBBM Foundation, San Gerardo Hospital, Monza, 
      Italy.
FAU - Canonico, F
AU  - Canonico F
AD  - Neuroimaging Unit, San Gerardo Hospital University of Milan Bicocca, Monza, 
      Italy.
FAU - Parini, R
AU  - Parini R
AD  - Center for Metabolic Diseases MBBM Foundation, San Gerardo Hospital, Monza, 
      Italy.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20131120
PL  - Germany
TA  - Neuropediatrics
JT  - Neuropediatrics
JID - 8101187
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Epilepsia Partialis Continua/complications/*diagnosis
MH  - Epilepsy, Generalized/complications/*diagnosis
MH  - Humans
MH  - Hyperargininemia/complications/*diagnosis
MH  - Male
EDAT- 2013/11/22 06:00
MHDA- 2015/01/07 06:00
CRDT- 2013/11/22 06:00
PHST- 2013/11/22 06:00 [entrez]
PHST- 2013/11/22 06:00 [pubmed]
PHST- 2015/01/07 06:00 [medline]
AID - 10.1055/s-0033-1360479 [doi]
PST - ppublish
SO  - Neuropediatrics. 2014 Apr;45(2):123-8. doi: 10.1055/s-0033-1360479. Epub 2013 Nov 
      20.