3-year-old girl was admitted for the first time to our hospital because of status epilepticus. The girl was born to healthy, unrelated parents, who had an unremarkable family history, and her birth parameters were within the normal range. The girl had started to walk and had used single words at around 12 months. At 6 months, she had a right hemiclonic seizure 48 hours after being given pentavalent (pertussis, tetanus, diphtheria, inactivated polio, and haemophilus) vaccination. An electroencephalogram was normal. Between 7 and 10 months, she had nine prolonged (20–30 min) clonic or hemiclonic seizures, involving alternating sides, which occurred during febrile illness. An electroencephalogram at 13 months showed generalised spike waves, slowed background activity, and photosensitivity. Despite therapy with phenobarbital, the convulsive seizures reoccurred during fever, and she had atypical absences and myoclonus at rest from 20 months old. Delayed psychomotor development, which involved motor and linguistic activities, was also evident. At 2 years and 9 months, the girl had five prolonged febrile or afebrile convulsive seizures. Valproate was added to her regimen, and seizure control was almost complete, apart from weekly atypical absences. However, owing to a mild increase in serum amylase concentrations (112 U/L [normal range 15–110]), her general practitioner replaced valproate with lamotrigine. The girl had intense myoclonus in the arms, neck, and lower limbs 6 weeks after starting lamotrigine (150 mg/day). The dose was lowered to 100 mg/day, and the myoclonus was dramatically reduced; 4 weeks later, the dose was increased to 200 mg/day, and the myoclonic jerks reoccurred, culminating within 5 days in a myoclonic state. On admission to our department, the patient had intense, continuous, erratic myoclonus that affected different parts of the body. An electroencephalogram showed slowed background activity and multifocal spikes. The patient was hypothermic (35·0°C) and had signs of respiratory insufficiency. Blood and CSF analysis showed severe metabolic acidosis and high concentrations of serum transaminases (alanine transaminase 259 U/L, aspartate transaminase 214 U/L [normal range ≤40]) and creatine kinase (897 U/L [normal range 30–180]). Brain MRI was unremarkable. The myoclonic status did not respond to oral clonazepam and intravenous lorazepam, and the patient was transferred to the intensive care unit where, after intubation and ventilation, thiopental was given

Refractory, life-threatening status epilepticus in a 3-year-old girl / Striano, Pasquale; Striano, Salvatore; Minetti, C; Zara, F.. - In: LANCET NEUROLOGY. - ISSN 1474-4422. - ELETTRONICO. - 7:3(2008), pp. 278-284. [10.1016/S1474-4422(08)70043-7]

Refractory, life-threatening status epilepticus in a 3-year-old girl.

STRIANO, PASQUALE;STRIANO, SALVATORE;
2008

Abstract

3-year-old girl was admitted for the first time to our hospital because of status epilepticus. The girl was born to healthy, unrelated parents, who had an unremarkable family history, and her birth parameters were within the normal range. The girl had started to walk and had used single words at around 12 months. At 6 months, she had a right hemiclonic seizure 48 hours after being given pentavalent (pertussis, tetanus, diphtheria, inactivated polio, and haemophilus) vaccination. An electroencephalogram was normal. Between 7 and 10 months, she had nine prolonged (20–30 min) clonic or hemiclonic seizures, involving alternating sides, which occurred during febrile illness. An electroencephalogram at 13 months showed generalised spike waves, slowed background activity, and photosensitivity. Despite therapy with phenobarbital, the convulsive seizures reoccurred during fever, and she had atypical absences and myoclonus at rest from 20 months old. Delayed psychomotor development, which involved motor and linguistic activities, was also evident. At 2 years and 9 months, the girl had five prolonged febrile or afebrile convulsive seizures. Valproate was added to her regimen, and seizure control was almost complete, apart from weekly atypical absences. However, owing to a mild increase in serum amylase concentrations (112 U/L [normal range 15–110]), her general practitioner replaced valproate with lamotrigine. The girl had intense myoclonus in the arms, neck, and lower limbs 6 weeks after starting lamotrigine (150 mg/day). The dose was lowered to 100 mg/day, and the myoclonus was dramatically reduced; 4 weeks later, the dose was increased to 200 mg/day, and the myoclonic jerks reoccurred, culminating within 5 days in a myoclonic state. On admission to our department, the patient had intense, continuous, erratic myoclonus that affected different parts of the body. An electroencephalogram showed slowed background activity and multifocal spikes. The patient was hypothermic (35·0°C) and had signs of respiratory insufficiency. Blood and CSF analysis showed severe metabolic acidosis and high concentrations of serum transaminases (alanine transaminase 259 U/L, aspartate transaminase 214 U/L [normal range ≤40]) and creatine kinase (897 U/L [normal range 30–180]). Brain MRI was unremarkable. The myoclonic status did not respond to oral clonazepam and intravenous lorazepam, and the patient was transferred to the intensive care unit where, after intubation and ventilation, thiopental was given
2008
Refractory, life-threatening status epilepticus in a 3-year-old girl / Striano, Pasquale; Striano, Salvatore; Minetti, C; Zara, F.. - In: LANCET NEUROLOGY. - ISSN 1474-4422. - ELETTRONICO. - 7:3(2008), pp. 278-284. [10.1016/S1474-4422(08)70043-7]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11588/373607
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