Abstract
Lyme disease is most prevalent in the northeast and upper Midwest regions of the United States. While early symptoms may be mild (eg, rash, flu-like symptoms, joint pain), late or persistent infection can cause chronic neurologic impairments. Because of this range of symptoms, physicians can have difficulty diagnosing Lyme disease, especially in the absence of erythema chronicum migrans. We report a case of a woman who initially presented with severe vertigo and vomiting and later with fever, headache, and facial droop. After more than 3 weeks of misdiagnosis, the patient tested positive for Lyme disease and was diagnosed as having neuroborreliosis presenting as Bell palsy and meningitis. The authors review the history, diagnosis, and management of Lyme disease.
Lyme disease is the most common vector-borne illness in the United States. In 2009, the Centers for Disease Control and Prevention reported almost 29,000 cases and more than 6000 probable cases, yielding a national average of 9.4 cases per 100,000 people.
1,2 Though Lyme disease is frequently associated with erythema chronicum migrans, many patients with the disease do not present with this pathognomonic finding.
The current report documents the case of a 63-year-old woman who presented to our emergency department three times before the correct diagnosis was made. This case emphasizes the challenges in diagnosing Lyme disease of the nervous system, discusses its pathogenesis and clinical manifestations, and helps clarify the current treatment guidelines.
In September 2008, 3.5 weeks earlier, the patient presented to our emergency department complaining of severe vertigo and vomiting. She was treated with meclizine hydrochloride after a head computerized tomography scan yielded normal results. The patient followed up with her primary care physician 2 days later, at which time her symptoms were nearly resolved.
Three days after seeing her primary care physician, the patient presented to our emergency department again with a fever. She was diagnosed as having a urinary tract infection and was prescribed a 10-day course of orally-administered ciprofloxacin. At a 2-day follow-up with her primary care physician, the patient described severe headaches that felt like “bombs going off in her head.” The physician told the patient that she did not have a urinary tract infection and diagnosed temporal arteritis and prescribed prednisone. The physician did not confirm this diagnosis with a biopsy.
Two weeks after being diagnosed with temporal arteritis, the patient returned to her primary care physician and requested a Lyme disease titer blood test. The physician agreed with her request, and the following day, the titer test results were positive for Lyme disease. The patient immediately began doxycycline therapy, administered orally.
Two days after beginning doxycycline therapy, the patient awoke and noticed that the right side of her face was drooping, with her right eyebrow approximately 0.5 inches lower than her left eyebrow. The patient had difficulty closing her right eye, and saliva drooled from the right corner of her mouth. The patient also described severe pains shooting down her neck and blurred vision in her right eye.
Later that day, the patient visited an ophthalmologist, who suggested that she had Bell palsy. The ophthalmologist sent the patient to our emergency department for further evaluation.
At our emergency department, we performed a lumbar puncture and consulted an infectious disease specialist. Results of cerebrospinal fluid testing showed lymphocytosis (>85% lymphocytes) and an elevated protein level (166 mg/dL). In light of these findings and the positive Lyme titer blood test results, the patient was admitted to our hospital with a diagnosis of neuroborreliosis presenting as Bell palsy and meningitis.
During hospitalization, the patient received a peripherally inserted central catheter (PICC) line and was administered ceftriaxone sodium intravenously at a dosage of 2 g/d and a tapering course of prednisone. Findings from magnetic resonance imaging and magnetic resonance angiography scans of the patient's brain were normal.
The patient was discharged from our hospital 6 days later and followed up with an infectious disease specialist. Her right-sided facial droop had improved and her meningeal symptoms resolved.
Four days after being discharged, however, the patient developed new symptoms. That evening, the patient had difficulty moving her lips, and the next morning she noticed that she also had a left-sided facial droop. Due to these new symptoms, that patient was concerned that she might have had a stroke and she presented again to our emergency department.
At presentation, the patient was weak on both sides of her face and had difficulty swallowing liquids. The patient also complained of occasional headaches and difficulty speaking. She denied any vertigo, nausea, diplopia, or blurred vision and was able to chew without much difficulty despite some numbness to her tongue. The patient's hearing was normal, and she did not describe arthralgia, rashes, or joint swelling. Her neck was supple, and she did not have any meningeal signs on examination.
Initial laboratory and diagnostic evaluations at this time included an electrocardiogram, complete blood cell count, bone morphogenetic protein, and plain chest radiograph, all of which revealed normal findings. The patient continued ceftriaxone sodium therapy via the PICC line and began a tapering course of prednisone starting at 40 mg/d.
During this second hospitalization, a neurologist was consulted. Other than the patient's previously identified symptoms (ie, facial droop), results of the patient's neurologic examination were normal. While the neurologist thought the patient's facial diplegia was secondary to neuroborreliosis, blood tests were ordered to rule out other causes such as sarcoidosis, vasculitis, myasthenia gravis, and idiopathic polyneuritis. Erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, lupus anticoagulant, angiotensin converting enzyme, rapid plasma reagin, acetylcholine receptor antibody, vitamin B12, and folic acid level test results were normal.
The patient was also examined by a speech therapist during her second hospitalization. The patient had no intrinsic problem swallowing—just difficulty with facial muscle paralysis, which improved greatly during this second admission. The patient continued to have a mild right-sided facial droop, however.
The patient was discharged to home 2 days after second hospital admission. She was prescribed the same tapering course of prednisone and continued ceftriaxone sodium therapy via a PICC line.
After discharge, the patient continued to follow-up with the infectious disease specialist. By January 2009, she had achieved near complete resolution of her symptoms except for mild right facial palsy and droop with some weakness when she attempted to pucker her lips. She was instructed to follow up with her primary care physician and the infectious disease specialist as needed.
The patient, an avid gardener, was advised to wear long sleeves and long pants when outside the home and to use DEET in the summer. She was also given information on how to carefully examine herself and remove ticks when going indoors.