Age: 1.07
Sex: F
Vaccine: HBHEPB, IPV, MMR, PNC
Symptoms: Laboratory test, Lumbar puncture, Metabolic function test, Muscular weakness, Myoclonic epilepsy
Clinical narrative: 05/18/05 found seizing in bed, transported to hospital via ambulance, seizure lasted approx 45 min, until Ativan given IV, fever left side weakness, pt born on 4/15/2004. As an infant pt received routine pediatric medical attention and vaccinations. Except for colic like symptoms as a newborn and a stomach virus on her first birthday, she was a well child who achieved developmental milestones at a normal rate. Before one year of age she received the full complement of childhood vaccinations (Hepatitis B, Prevnar, DTaP, IPV and HIB). Because of her stomach virus on her first birthday, her parents delayed her one year vaccinations for a few weeks to be certain that she was fully well and recovered. On 5/9/2005, pt presented to health center for her one year immunizations. She was noted to be afebrile, active alert and V/S reviewed. Pt received a MMR immunization. Pt was seen on 5/14/2005 for her one year well baby exam. She weight 20 pounds, 4 ounces, measured 28 1/2 inches in length, her head circumference was 46 centimeters. The physicians documented that she was able to help with dressing, play pat a cake, bat 2 cubes together, had a neat pincer grasp, able to say and differentiate between Mama and Dada, pull to standing and walk alone. It was noted that she had received her twelve month vaccines at health clinic. Physicians instructed mother to return with pt at 15 months of age. Pt began to run a fever on 5/17/2005. She also seemed clumsy throughout the day, but mom thought maybe it was simply because pt had only been walking for three weeks. She was otherwise asymptomatic, and mom treated fever with infant Tylenol during the day and before bedtime. Dad worked 2nd shift that day, and arrived home around 11:30pm that night. Shortly after his arrival, pt woke up crying and dad went in to check on her. He found her easily consolable, checked her temperature and found no fever, and pt quickly laid back down and slept through the rest of the night. On 5/18/2005 at 9:55am, mom heard pt on her baby monitor in her bed making chattering noises or singing to herself. She noticed on the video monitor that pt was still lying down, not sitting up as usual when she first awoke in the morning. Upon entering pts room, mom found pt lying in her bed, seizing. Pt's eyes were open and the chattering noise was pt hyperventilating. Her movement was generalized at first, but would later become more pronounced on the left side. Mom activated EMS to transport pt to the hospital. While waiting for EMS to arrive, mom stayed at pt's side to stabilize and comfort her. She took a tympanic temperature reading at 101 degrees. Pt continued to seize for forty five minutes, not stopping until Ativan was administered in the ED. Pt vital signs in the ED were temp 102.8, pulse 190-200, respiration 22, blood pressure 97/49. Labs drawn at the time were CBC, blood culture, BMP. Urine sample was obtained for urine dip and urine culture. A CT of the head without contrast was done, and was normal. When pt woke up, she was groggy and uncoordinated, and parents noticed that she wasn't using her left arm or leg. When assessed the physician noted decreased movement and weakness of her left arm and left leg. Pt was admitted for observation. Upon arrival to the hospital room from the ED, pt vomited three times. On the third day in the hospital, neurology was consulted, and ordered an EEG, a MRI of the head, and a Lumbar Puncture. The EEG showed focal slowing over the right parietooccipital region, as well as an electrical seizure emanating from the right parietooccipital region lasting 1 minute and 15 seconds. The MRI showed focal signal abnormalities involving the right posterior parietal lobe, the right temporal lobe and the posterior right thalamus. The Lumbar Puncture for CSF studies were normal Encephalitis test were negative, as were the arbovirus, EBV, West Nile Virus. Prior to the CSF results, pt was started on IV antibiotic doses of Zithromax and Acyclovir. The neurologist stated that pt has encephalitis, as well as infarctions in the right posterior parietal lobe that caused the seizures and the hemiparesis. Pt experienced additional seizures during hospital admission, each lasting less than two minutes. Once the viral labs came back negative the IV antibiotics were discontinued. Pt was discharged from hospital 5/24/2005 on Trileptal. She was also prescribed prophylactic baby aspirin, pending the results of coagulation studies, which later came back negative. The baby aspirin was discontinued. To help regain use of her left side, pt began outpatient Occupational therapy three times per week, and outpatient Physical Therapy twice per week, which she is still attending currently. She has made gradual progress with her gross and fine motor skills. Initially, pt ignored the left side of her body, and regarded her left arm and leg as alien objects when they entered her line of vision. She now is attentive to both sided of her body. She began walking again on 11/24/2005, although her gait is still unnatural, and she swings her left leg out to the side due to the increased tone in her leg. She can grasp light objects in her left hand, and can rake up very small items with a claw like movement. She still hasn't regained per left pincer grasp, and has difficulty holding objects of very much weight in her left hand. Her clap isn't midline. Pt seemed to remain seizure free after discharge on 5/24/2005, but parents began to notice pt having episodes of jerking movements, lasting only a split second each, a few times per day. Pt's neurologist stated that the movements sounded like myoclonic seizures, and advised the parents to increase pt's Topamax. On 10/16/2005 at around 3:00pm, father was with pt and noticed that she started to star off into space, and ignored him when he said her name. She then began to make a smacking sound with her lips. Dad took pt into the bedroom to mom who was lying down before working that evening stating, somethings wrong. Pt went into a generalized tonic clonic seizure which lasted approx 45 minutes. EMS was activated for transport to hospital. Pt was given Ativan rectally in the ambulance, with no improvement. Another dose of Ativan IV in the Ed finally stopped the seizure. Pt was admitted with the diagnosis of status epilepticus. She has frequent myoclonic seizures during this admission, some of which were recorded during a video EEG (results were in favor of myoclonic seizures). She was started on Keppra in addition to the Trileptal at discharge and also given a prescription for Diastat rectal gel for seizures lasting longer than three minutes and Klonopin ODT as needed for clusters of myoclonic seizures. She was discharged home on 10/18, with her new prescriptions and orders to resume PT on OT. Pt had a second MRI on 8/4/2005 which showed subtle prominence of the cerebral sulci in the right frontoparietal region consistent with volume loss and chronic infarction. Decreased white matter myelination is noted in the right cerebral hemisphere compared to the left. A third MRT on 11/23/2005 revealed subtle volume loss in the posterior right frontal lobe and right parietal lobe consistent with the patients known prior infarction. T2 signal abnormality in the right cerebral hemispheric white matter mildly decreased compared to the prior examination. This may be gliosis or under myelinated white mater related to the prior infarction. Pt continues with outpatient PT and OT twice a week, along with in home PT/OT visits once per week. She was weaned off the Trileptal after no change in the frequency of myoclonic seizures, and started on Topamax. She is still experiencing 5-7 myoclonic seizures daily, as her parents and physicians struggle to find the correct dose of her current medications, on the right medicine to switch to. The myoclonic seizures create a constant safety battle. She has been taken to the dentist for x rays after falling and hitting her mouth on the tile floor due to a myoclonic, and her right upper front tooth is now darkened from the damage. She has fallen face first in a bathtub of water. A visit to the chiropractor revealed that she was extremely out of alignment on the left side, presumably from either the whiplash like force of some of her myoclonic seizures or from the position she held herself in to hop around after the stroke or the uneven gait she now has. Due to her young age, it will be some time before we know whether there are any speech problems, or any problems with learning. 06/13/2006-3 page DC summary and 44 page medical record received from Hospital. DC summary documented DC DX 1. Complex febrile seizure, 2. postinfectious cerebritis, presumed. ER report Temp 102.2 with seizure in progress observed with harder jerking on left side. RX with Ativan 1mg IV in ER, after seizure patient awoke became appropriate and was drinking, noted to have left sided hemiparesis on exam. RX with Acyclovir and Zithromax. Prior to admission 3 day HX of fever, prior medical HX indicated: no HX of seizures. Previous medication included Rhinotan, last dose on 5/11/05. FMH includes questionable HX of possible febrile seizure in patient's father and maternal half brother. Vomiting x3 after her seizure episode on 5/18/05, on and off erythematous pinpoint rash on abdomen Six day Hospital course   Neuro consult on 5/20/05 documented assessment of complex febrile seizure versus seizure provoked by fever of right hemispheric onset. Mother reported patient fussy and clumsy on 5/16/05 prior to seizure on 5/17/05. Mother ill 3 days prior to 5/16/05 with 24 hour bout of vomiting without fever.  RX in hospital with Dilantin on day 3 during which time she was observed to have short focal seizures on the left, a 45 second seizure on day 4. At time of discharge the patient had not had any clinical seizures for over 24 hours, she had regained most of the use of her left lower and upper extremity. One day prior to DC patient started on Trileptal and Dilantin discontinued on day of discharge. Follow-up repeat BMP in 4 weeks, occupational and physical therapy, DC medications ASA, Trileptal. 60 Day Follow-up Information 20-JUN-2006: Patient still has seizure activity, is on medications, and remains in occupational and physical therapy for left-sided hemiplegia. Due to her young age, it is unknown whether she will have any learning disability or whether her speech will be affected.