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Connecticut Girl, Seven Years Old VIII International Conference on Lyme Borreliosis and other Emerging Tick-borne Diseases, Munich, Germany June 25,1999


Liegner KB & Jones CR. Private Practices, Armonk, NY & Hamden, CT, USA.

An engorged deer tick was removed from the right aspect of the neck of a 6 year old Fairfield County, Connecticut girl March 1995.

Parental request for prophylactic antibiotic treatment was refused by the child's physician. No eruption occurred at the tick bite site.

Summer 1995 flu-like symptoms and conjunctivitis developed and October 1995, headache, stiff neck, and sleep disturbance. November 1995 right supraclavicular lymphadenitis, fever, lethargy and hypersomnolence developed. Admitted to a local hospital, focal seizures ensued. Phenytoin was administered. Lumbar puncture showed 3 white blood cells and normal glucose and protein. Phenytoin, ceftriaxone, ampicillin, and acycolvir were administered. Tests for rabies and Lyme disease were negative. MRI of brain was normal. Transfer was made to a tertiary care facility where high dose pentobarbital coma was required to control status epilepticus. Feeding gastrostomy and Boviac catheter were required for nutrition and medications. Adenovirus serology, arbovirus serology and CSF serology and culture and CSF serology, culture, and PCR for HSV-1, HSV-2, ANCA, ANA, ASO, Bartonella, cold agglutinins, febrile agglutinins, influenza, para-influenza, CSF india ink prep, malaria screen, measles, mycoplasma, Q fever, rabies, RMSF, RSV, rotovirus, rubella, toxoplasmosis, typhus, varicella, Lyme disease serologies and VDRL were negative. EBV antibodies were present. HSV IFA was positive and rose following administration of IVIG. IgG for ehrlichia was positive at 1:256 by the Centers for Disease Control. Intravenous immunoglobulins were given for putative Rasmussen's Syndrome, steroids for "vasculitis", and intravenous acyclovir for the possiblity of herpes encephalitis. Intravenous nafcillin was given for coagulase negative staphylococcal bacteremia. CT scans and MRIs of the brain, initially normal, demonstrated evolution of cerebral atrophy and periventricular white matter disease. June 1996 the patient demonstrated arthritis involving hands, wrists, ankles, knees, and hips, was experiencing frequent seizures and was unable to walk, speak, respond to verbal commands, or feed herself. Paired Lyme ELISAs in CSF and serum 7/96 were negative, but Lyme IgG immunoblot in serum disclosed the presence of 30, 41, 66, & 93 kiloDalton bands as well as 60 kDa band. CSF cell count, glucose protein, and IgG were normal. CSF, blood, and urine Lyme PCRs were negative as was culture for borrelia in BSK-H . Myelin basic protein and oligoclonal bands were absent. Osp A antigen capture assay in CSF and Lyme-specific immune complexes in CSF and serum were negative. Treatment with intravenous ceftriaxone initially resulted in worsened seizure activity and treatment was changed to cefotaxime. Arthritis resolved within one month of starting antibiotics. A short course of doxycycline was given to cover the possibility of co-infection with ehrlichia. During six months of treatment with intravenous cephalosporins seizures, which had remained poorly tractable despite intensive oral anticonvulsant therapy, diminished and became readily controllable with lower dosage of anticonvulsants. The patient became able to walk, vocalize in simple sentences, feed herself, and use a swing set but remained severely neurologically impaired with significant brain injury evident on brain MRI and CT scan. Antibiotic therapy was stopped 12/3/96. Seizures reoccurred within one week of cessation of antibiotics and became increasingly difficult to manage despite continuation of anticonvulsant therapy. While in a tertiary care hospital her condition deteriorated and she died 1/30/97.

An autopsy was performed.