Age: 11.0
Sex: F
Vaccine: HPV4, MNQ
Symptoms: Abasia, Angiotensin converting enzyme increased, Antibody test normal, Antineutrophil cytoplasmic antibody negative, Antineutrophil cytoplasmic antibody positive
Clinical narrative: In December of 2007 and January of 2008, patient became withdrawn and concerned that she did not lose weight despite diet and exercise, and she started having opsoclonus.  She had no illnesses around the time of onset of those symptoms.  Brother had been quite ill (thought initially to be meningitis, but turned out to be unknown) and is now better.  She had a meningitis vaccine and HPV vaccine at the time that she started to be withdrawn.  Over the course of 1-2 weeks she became ataxic, and had very "jumpy arms".  This myoclonus spread to her legs such that she could not walk.  She denied headaches, nausea, and or vomiting.  No muscle aches or leg pain.  No tinnitus or hearing loss.  At the time of a February 11, 2008 admission she had severe opsoclonus, myoclonus, dysmetria, and perhaps a wide amplitude tremor.  An extended evaluation was started looking for a neuroblatoma and a ganglioneuromal, mitochondrial disease, inflammatory diseases, and other diseases.  There were a few abnormal lab results, which normalized later.  The lactate normalized only after starting mitochondrial vitamins.  No evidence of a ganglioneuroma or neuroblastoma was found.  Blood mitochondrial mutations were sent and later returned as normal.  Because the lactate was high, we added Thiamine 25 mg twice daily and riboflavin 50mg twice daily, CoQ, and Keppra was started later at 1mg/kg/day, and advanced as tolerated, then lowered when she started feeling nauseated.  She received steroid x 1 week courses, with marked hyperglycemia, but no improvement.  She received IVIG, then sent to hospital.  She was discharged from hospital with improved opsoclonus, but still unable to walk or reach easily.  She returned to hospital for a second course of IVIG.  After that, the mitochondrial meds were stopped, she remained unable to walk, and had variable but usually less opsoclonus.  Physical therapists noted some weakness and atrophy, and she had one episode when she would fall to the left side when the tuning fork was put near her left ear.  She had had no convulsions and had a normal EEGs, including a normal 24 hour EEG; no emesis; no headache; no deterioration of affect or bizarre behavior.  She has had no trouble eating and she can use a fork, but has some difficulty using her knife to cut.  She was seen by doctor, who, mother says, gave a cursory exam, stated that patient's strength was adequate to walk, and that Mother should stand her up, make her walk, and she will be walking in several months.  12/1/08 MR received for 2 admissions: DOS 2/11-29/2008 with D/C DX:  Post-infectious myoclonus and opsoclonus with lower extremity weakness.  Depression. DOS: 4/17-19/2008 with D/C DX:  Upper extremity weakness.  Opsoclonus-myoclonus.  Two months prior to admission pt noticed new onset of mood changes-depression and anger. One month prior pt noticed abnormal eye movements which resulted in vertigo. Pt presented with worsening eye movements with dizziness and shakiness. Unable to walk unassisted, feeling unwell with sore throat and stomach ache. Some arm tremors. PE (+) for abnormal eye movements, 4/5 strength in lower extremities, ataxia, UE and knee DTRs brisk, difficult to elicit at ankles. Ophth, Rheum and Psych consults. Started on anti-depressant. D/C to inpt rehab. Developed new myoclonus in legs and R arm despite steroids and IVIG. Returned for admission 4/17/08 with new complaint of upper extremity weakness. Now using a w/c and difficult to transition from w/c to bed. New truncal weakness and difficulty with fine motor skills. Unable to stand due to lack of sensation in feet. Again tx with IVIG without resolution of sx.