Neuropediatrics 2014; 45(02): 123-128
DOI: 10.1055/s-0033-1360479
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Epilepsia Partialis Continua and Generalized Nonconvulsive Status Epilepticus during the Course of Argininemia: A Report on Two Cases

D. Grioni
1   Child Neuropsychiatric Clinic, San Gerardo Hospital University of Milan Bicocca, Monza, Italy
,
F. Furlan
2   Center for Metabolic Diseases MBBM Foundation, San Gerardo Hospital, Monza, Italy
,
F. Canonico
3   Neuroimaging Unit, San Gerardo Hospital University of Milan Bicocca, Monza, Italy
,
R. Parini
2   Center for Metabolic Diseases MBBM Foundation, San Gerardo Hospital, Monza, Italy
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Publikationsverlauf

17. Januar 2013

23. August 2013

Publikationsdatum:
20. November 2013 (online)

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Abstract

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.