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Pseudotumor Cerebri: Early Manifestation of Adult Lyme Disease

2008; Elsevier BV; Volume: 121; Issue: 7 Linguagem: Inglês

10.1016/j.amjmed.2008.02.032

1555-7162

John Castaldo, E. F. Griffith, Daniel Monkowski,

Viral Infections and Vectors

Neuroborreliosis is an infection of the nervous system by Borrelia burgdorferi. Symptoms of central nervous system (CNS) Lyme disease typically include headache, nuchal rigidity, fatigue, nausea, vomiting, cranial neuropathy, and radiculoneuritis. In a minority of patients with Lyme disease, encephalitis can result.1Pachner A.R. Steiner I. Lyme neuroborreliosis: infection, immunity, and inflammation.Lancet Neurol. 2007; 6: 544-552Abstract Full Text Full Text PDF PubMed Scopus (135) Google Scholar, 2Steere A.C. Lyme disease.N Engl J Med. 2001; 345: 115-125Crossref PubMed Scopus (1030) Google Scholar We describe a patient who initially presented with symptoms of increased intracranial pressure, papilledema, and serum testing positive for Lyme disease. Pseudotumor cerebri has been reported as a complication in pediatric patients with Lyme disease.3Hartel C. Schilling S. Neppert B. et al.Intracranial hypertension in neuroborreliosis.Dev Med Child Neurol. 2002; 44: 641-642Crossref PubMed Google Scholar, 4Kan L. Sood S.K. Maytal J. Pseudotumor Cerebri in Lyme disease: a case report and literature review.Pediatr Neurol. 1998; 18: 439-441Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar, 5Raucher H.S. Kaufman D.M. Goldfarb J. et al.Pseudotumor cerebri and Lyme disease: a new association.J Pediatr. 1985; 107: 931-933Abstract Full Text PDF PubMed Scopus (57) Google Scholar In addition, a recent report described a case of pseudotumor cerebri as a late complication of Lyme disease in an adult.6Nord J.A. Karter D. Lyme disease complicated with pseudotumor cerebri.Clin Infect Dis. 2003; 37: e25-e26Crossref PubMed Scopus (13) Google Scholar We report the first case of pseudotumor cerebri documented as the initial presentation of CNS Lyme disease in an adult. A 34-year-old man presented with a 6-day history of unrelenting headache described as frontal in location, throbbing, and moderately painful. He had experienced mild low back pain, nausea, and blurred vision during the previous 24 hours and had seen his optometrist. A vision examination demonstrated normal acuity with bilateral papilledema (Figure 1), at which time he was referred to neurology. The patient had an unremarkable medical and social history. On neurologic examination, the patient was lethargic but cooperative with normal mentation, language, and affect. The funduscopic examination showed papilledema with an absence of venous pulsations. Visual fields were intact and normal with enlarged blind spots to confrontation bilaterally. The rest of his cranial nerve testing results were normal, including extraocular movement without nystagmus. Motor examination showed normal bulk, power, and tone on all 4 extremities. Multimodality sensory testing results of the extremities were normal, as were coordination testing, deep tendon reflexes, gait, and station. The physical examination results were otherwise normal with no nuchal rigidity. The complete blood count, metabolic profile, sedimentation rate, and tests for leptospira ehrlichiosis and Babesia all showed normal results. Brain computed tomography angiogram and computed tomography venogram were negative, as were magnetic resonance angiography, magnetic resonance venography, and magnetic resonance imaging with gadolinium contrast. An initial lumbar puncture found clear cerebrospinal fluid (CSF) with an elevated opening pressure of 260 mm/H2O, 1 red blood cell/cumm, 6 white blood cells/cumm (neutrophils 4%, lymphs 74%, monocytes 22%), glucose level of 84 mg/dL, and protein elevated at 100 mg/dL. Serum/CSF Lyme ratio was not elevated by immunoglobulin (Ig)M or IgG, but serum Lyme IgM antibodies were positive and serum IgG was not. A diagnosis of CNS Lyme disease was made,7Roos K.L. Berger J.R. Is the presence of antibodies in cerebrospinal fluid sufficient to make a definitive diagnosis of Lyme disease?.Neurology. 2007; 69: 949-950Crossref PubMed Scopus (10) Google Scholar, 8Balcer L.J. Winterkorn J.M. Galetta S.L. Neuro-ophthalmic manifestations of Lyme disease.J Neuroophthalmol. 1997; 17: 108-121PubMed Google Scholar and the patient was treated with ceftriaxone 2 g administered intravenously every 24 hours for 4 weeks.9Halperin J.J. Shapiro E.D. Logigian E. et al.Practice parameter: treatment of nervous system Lyme disease (an evidence based review).Neurology. 2007; 69: 91-102Crossref PubMed Scopus (263) Google Scholar The patient made a complete recovery, with resolution of the papilledema and headache. A repeat lumbar puncture 15 days later showed a normal opening pressure, a CSF polymerase chain reaction negative for Lyme, 5 red blood cells/cumm, 46 white blood cells/cumm (neutrophils 0%, lymphs 89%, monocytes 11%), glucose level of 55 mg/dL, and protein elevated at 107 mg/dL. (Sample handling precluded a repeat serum/CSF Lyme ratio.) We report a rare presentation of CNS Lyme disease, the first of its kind reported in an adult. The most reliable laboratory testing methods to diagnose Lyme disease are indirect and confoundable (antibody testing, including enzyme-linked immunosorbent assay and western blot) or are useful only in specific settings with unclear cutoffs for positive and negative (polymerase chain reaction). The remainder of the diagnosis of Lyme is clinical and subject to misinterpretation, especially given the type of symptoms. This patient showed evidence of recent Lyme infection, a clinical syndrome suggestive of CNS manifestation, and a response to therapy. These findings strongly suggest B. burgdorferi as the cause. Until more reliable testing methods are found, the diagnosis and management of Lyme disease will continue to be troublesome. This case demonstrates that the clinical presentation may be more varied than previously thought.