Age: 1.0
Sex: F
Vaccine: HEPA, MMR, PNC13, VARCEL
Symptoms: CSF white blood cell count negative, Cerebral ischaemia, Cytogenetic analysis, Dyskinesia, Echocardiogram normal
Clinical narrative: Patient is a 12 m.o. female who was previously healthy until being admitted to the hospital in status epilepticus. She was in her usual state of health when she began having rhinorrhea, then congestion and fevers up to 103-104 F. During this time she did not have any other symptoms and was acting herself and tolerating meals and fluids. There was no rash, no vomiting/diarrhea, no mental status changes, no ataxia. Fever was intermittent and well controlled on Tylenol and Ibuprofen. On about day 5 of illness (4/18) she began to have a slight decrease in oral intake. That evening she slipped and fell on the kitchen floor and bonked her head with no loss of consciousness, no vomiting, no mental status changes, no cranial changes. This was about an hour before bed. Prior to bedtime she was afebrile and had last received Motrin at 3 pm that day, therefore no further antipyretic was given. The next morning at 6:45 am dad found her in her crib unresponsive with rhythmic movement in her right arm and leg and she was blankly staring. EMS gave midazolam en route which did not stop the seizure. On arrival to the ED seizures stopped with 1 mg of ativan. She also received one dose of rocephin for right acute otitis media. CBC and CMP were within normal limits and flu swab was negative. On admission to the floor it was thought that she had a complex febrile seizure and it was estimated that she had been seizing for 1 to 1.5 hours. On the floor she never returned to baseline, was minimally responsive to parents and staff, had decreased right sided movement, and looked "encephalopathic." EEG showed L posterior spikes and an MRI was ordered which showed restricted diffusion in the left temporal stem and post parietal areas, consistent with ischemia likely from prolonged seizure activity. She required an additional dose of Ativan, fosphenytoin, keppra, and was placed on continuous EEG. CSF studies showed 1 WBC, normal protein and glucose, and culture thus far has been negative. On day 2 of admission she continued to look encephalopathic and had worsening left hemi paresis. EEG showed 4 seizure events that morning and keppra was increased and was given a dose of lacosamide.  Vancomycin and acyclovir were given. HSV CSF PCR resulted as negative, therefore acyclovir was not continued. HHV-6 was added to the CSF studies which is still pending. Due to her worsening status, she was sent for MRA to assess for stroke or other vasculitides and then transferred to the ICU. MRA showed normal vascular structures. Echo was ordered to assess for PFO, which was normal. Film array was negative. She continued to have subclinical seizures requiring induction of pentobarbital coma with intubation, soon afterward burst suppression was achieved. She has not had any seizure activity since the evening of  4/21. She was also started on methylprednisone daily on 4/22 to possibly help with any autoimmune or inflammatory etiologies. Metabolic workup has also been initiated, for which most labs have been collected and are pending. Primary team is also pursuing genetic etiologies and have sent a micro array epilepsy panel and are consulting genetics. At this point, the neurology team is thinking this could be Rasmussen's encephalitis and needs to completely exclude an infectious etiology in the setting of continued steroid administration. They are planning on repeating the LP. Prior to this event she was previously healthy and has been developmentally appropriate (says "ma-ma" "da-da", crawls, pulls to stand, cruises, has good pincer grasp). The family did take a trip from 3/28-4/6 during which they spent time at a resort and at grandparents home. They went through a drive-through safari and had some contact with a giraffe, but otherwise there were no known animal contacts. There is a cat at grandparents home she had minimal interaction with and no known bites or scratches. Her baby sitter has a dog that she has been around her whole life. She has not consumed unpasteurized milk, has not had any mosquito or known tick bites. Family has not been camping or been though thick woods or tall grass. On 4/9 she did receive her hepatitis A, MMR, PREVNAR, and varicella vaccines. She is up-to-date with vaccinations.