Seven-year-old girl who attended the emergency department in springtime for an episode of complex generalised seizure with disconnection from the environment and falling to the ground while walking, together with hypertonia, sialorrhoea and movements of the corner of the mouth lasting about three minutes, with spontaneous recovery. In the previous days the child had presented with myalgia, headache and fever up to 39°C, but had been afebrile for 24 hours.
She had no relevant physiological history: pregnancy, childbirth and perinatal period were of no interest, nor did she have any pathological history of interest, and her only family history was her mother's treatment with lithium for bipolar disorder that had been going on for years. On arrival at the ED, the patient presented a post-critical state with a Glasgow Glasgow score of 10, but the rest of the examination, both neurological and by systems, was normal. The blood tests were outstanding in the biochemistry and haemogram:
- Biochemistry:
- Glutamic-oxalacetic transaminase (GOT): 93 UI/l.
- Glutamic-pyruvic transaminase (GPT): 44 IU/l.
- C-reactive protein (CRP): 2 mg/l.
- Haemogram:
- Leukocytes activated aspect: 6000 (N: 30% L: 66% M: 4%).
- Platelets 125 000.
- Normal red blood count.
In view of the afebrile seizure, it was decided to admit him to complete the study. Two hours after admission, he had a new seizure that ceased with the administration of intravenous midazolam at 0.15 mg/kg, but he suffered respiratory arrest requiring T-piece ventilation and was admitted to intensive care for close monitoring and surveillance.
On arrival at the intensive care unit, he presented post-critical state, with a Glasgow Glasgow of 7 and all other vital signs normal except for an axillary temperature of 38°C in this case. Neurological examination revealed marked hyperreflexia of the lower limbs, with negative meningeal signs. Urgent cranial computed tomography (CT), lumbar puncture, urine toxicity and lithemia (due to maternal history) were performed, with normal results. An urgent electroencephalogram (EEG) was performed, reporting global slowing of activity compatible with meningoencephalitis. Cerebrospinal fluid (CSF) serology was obtained for enterovirus and herpes virus, both negative. Serology was also obtained for multiple agents such as toxoplasma, rubella, lupus, herpes simplex 1 and 2, varicella-zoster virus, Mycoplasma, adenovirus, mumps, parvovirus, measles, Coxsackie, ECHO virus and Borrelia, also negative.
In the following hours after admission, the patient presented episodes of agitation with somnolence and again presented two generalised convulsive episodes, so maintenance levetiracetam was started at 40 mg/kg/day, with a previous bolus of 20 mg/kg, together with prophylactic acyclovir at 60 mg/kg/day, despite the negative serology. Magnetic resonance imaging (MRI) of the brain was requested urgently, with normal results and no evidence of bleeding or hyperdensity lesions.
Given the history of myalgia and fever in the previous days and the poor recovery of the neurological symptoms, it was decided to determine influenza A antigens in the nasal exudate, which was positive, so treatment was started with oseltamivir 5 mg/kg/day for a total of five days, and acyclovir was discontinued. The patient progressively improved, but suffered a new convulsive episode and it was decided to extend serology for cytomegalovirus (CMV), Ebstein-Barr virus (EBV), herpes and influenza A negative, and a repeat MRI of the brain was performed, which remained normal. CSF oligoclonal bands were requested, which were negative, and the autoimmunity study with anti-DNA, anti-Ro, anti-La, anti-histone, anti-Sm, anti-RNP, anti-SCL70, anti-Jo, and anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Valproic acid was administered at a dose of 40 mg/kg/day, with complete cessation of seizures.
The patient had a total of five seizure episodes and on discharge the EEG improved, although not completely normal. Treatment was continued with valproic acid 40 mg/kg/day and levetiracepam 60 mg/kg/day.
Currently, the patient continues to be monitored at Neuropaediatrics consultations, now without anti-comedic medication, and remains asymptomatic, with normal EEG at the last visit.