Neurologice manifestations in Lyme borreliosis

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Neurologic manifestations may occur in early disseminated or late chronic Lyme borreliosis. Signs and symptoms of peripheral neuropathy had already been reported in patients with acrodermatitis chronica atrophicans (ACA), the principal dermatologic manifestation of late chronic Lyme borreliosis, at the turn of this century 1,2 (see review in Ref 3). The first case report of a patient with Bannwarth's syndrome, a painful radiculoneuritis that follows tick bite and erythema migrans (EM), was published in 1922 by Garin and Bujadoux. 4 Unfortunately, the authors were not aware that the enlarging erythema they observed was virtually identical to the EM described in 1909 by Afzelius 5 and in 1913 by Lipschütz. 6 A case report on EM followed by meningitis was first published in 1930 by Hellerström. 7 In the 1960s, Schaltenbrand 8 and other European neurologists 9–13 studied large cohorts of patients, all of whom developed painful radiculoneuritis, cranial neuritis, or lymphocytic meningoencephalitis as a sequela of tick bite and EM. Although the pathogenesis was unknown, it seemed clear that these patients suffered from a clinically characteristic disease, which was named meningopolyneuritis Garin-Bujadoux-Bannwarth 14 or Bannwarth's syndrome. 13 Despite reports that showed a beneficial effect of penicillin on EM-meningitis, 15,16 most European neurologists thought an arbovirus was the cause of Bannwarth's syndrome. 8–10,12,13 This concept was drawn from the knowledge of the viral etiology of central European encephalitis, another tick-transmitted neurologic disease observed in Europe. Yet dermatologists, familiar with the long-known therapeutic effect of antibiotics in EM and ACA, continued to postulate that bacterial agents caused Bannwarth's syndrome. 16 In the 1960s, Hopf found signs and symptoms of peripheral neuropathy in 40% of 92 patients with ACA. 3,17 This particular feature is known as ACA-associated neuropathy and represents a characteristic neurologic manifestation of late chronic Lyme borreliosis. 18–20 Neurologic abnormalities in Lyme disease were also observed in the United States. Eleven percent of patients with EM or Lyme arthritis were found to suffer from radiculoneuritis similar to Bannwarth's syndrome or from encephalitis. 21,22 Shortly after Borrelia burgdorferi was identified to be the causative agent of Lyme disease, 23,24 the pathogenesis of Bannwarth's syndrome 25,26 as well as of ACA and ACA-associated neuropathy 20,27 could also be disclosed. Subsequently, neurologic syndromes such as a chronic debilitating encephalomyelitis, 19,28,29 cerebral vasculitis, 30–33 and focal myositis 34–36 could reliably be linked to borrelial infection by several investigators. On the other hand, the frequent and “defensive” use of serodiagnostic tests to “rule out” Lyme borreliosis in patients with unclear neurologic phenomena has led to an increasing amount of anecdotal reports, which ascribe a large variety of neurologic conditions to Lyme borreliosis. Many of these reports lack evidence of borrelial infection except for the demonstration of serum antibodies to B. burgdorferi. It must be kept in mind that in endemic areas, as much as 10 to 30% of the clinical normal population have been found to be seropositive. 37–40 The increasing number of patients who undergo serodiagnostic testing for uncharacteristic symptoms of presumed Lyme borreliosis will subsequently lead to an increasing number of false-positive results. The overabundance of such “atypical” — in many instances, probably “falsepositive” — cases in the literature has led to a misconception of the clinical picture of neuroborreliosis. This has also led to potentially dangerous or useless extended therapeutic trials. 41–43 If the diagnosis is based on reliable criteria 44–46 neuroborreliosis will generally present with characteristic manifestations. A carefully performed prospective study has proven that atypical neurologic cases play no role in a large cohort of patients with neuroborrelfiosis. 47