J Neurol (1984) 231:281-282 Journal of

Neurology © Springer-Verlag 1984

Lymphocytic meningoradiculitis of Bannwarth and erythema migrans disease

Klaus Weber

Rosenstrasse 6, D-8000 Miinchen 2, Federal Republic of Germany

Dear Sir--Neurological manifestations associated with ery- thema migrans have been known in central and northern Europe for several decades. In 1922, Garin and Bujadoux published the case of a patient who developed a migrating ery- thema and meningoradiculitis after a tick bite [8]. However, the migrating erythema described was not recognised as ery- thema migrans, which had been known in the dermatological literature since 1910 [2]. In the early 1940s, Bannwarth gave a careful description of a series of patients with lymphocytic meningoradiculitis [3, 4]. Unfortunately, he missed the rela- tion of the described syndrome to tick bite and erythema migrans, although at least two of his patients seemed to have had that skin condition.

In the Scandinavian literature, however, the relationship of neurological symptoms to tick bite and/or erythema migrans was recognised in several case reports, first by Heller- strrm in 1930 (cited in [12, 24]). In addition, Hollstrrm re- ported on the beneficial effect of penicillin in patients with erythema migrans and one patient with meningitis [11]. Hel- lerstrrm believed that an infectious agent with allergizing properties caused erythema migrans with or without menin- gitis. Because of the work of Lennhoff who claimed to have found spirochetes in a variety of skin disorders including ery- thema migrans, Hellerstrrm even mentioned spirochetes as a possible cause [10].

Despite these observations the general opinion favoured a viral origin of lymphocytic meningoradiculitis [14]. In 1974, I published the case of a patient whose erythema migrans dis- appeared after treatment with oral penicillin but the patient still developed meningitis. High doses of parenteral penicillin induced a dramatic beneficial response in this patient. I thought that erythema migrans and the accompanying menin- gitis were caused by one and the same unknown bacterium. Borrelia was one of the possibilities discussed [22]. Later, I postulated the persistence of the causative agent and suggest- ed appropriate antibiotic therapy for later manifestations of erythema migrans and Lyme disease [23].

Lymphocytic meningoradiculitis Bannwarth has been given much attention in the neurological literature, but there are other neurological manifestations associated with ery- thema migrans in Europe [24]. The patient of Hellerstrrm had intermittent "hallucinations and psychic disorientation" [9]. Meningitis without peripheral neuropathy or encephalitis has been noted in a few instances [24].

The term 'erythema chronicum migrans meningitis' was suggested in 1974 [22]. The description of a series of prospec-

tively observed patients seen in the United States followed soon afterwards, first under the name of Lyme arthritis, later of Lyme disease [18, 19]. A predominantly prospective study then demonstrated that Lyme disease and erythema migrans disease (EMD) closely resemble each other [24].

A variety of symptoms is associated with EMD [24, 25]. Erythema migrans, the hallmark of the disease, has been men- tioned already. Erythema migrans may persist for months as erythema chronicum migrans. It can disappear spontaneously within 4 weeks after onset, or it may be absent [24]. Erythema migrans can be confused with nonspecific erythema due to tick or insect bite and with erysipelas. Several nonspecific symp- toms such as fever, fatigue, and headaches may occur [24]. Of our 49 patients with EMD, 35% had joint involvement [25]. Elbow, knee and finger joints were most frequently affected. All patients had pain on motion and some had also swelling of the joints. Most patients had only one attack of joint symp- toms. These findings are similar to those described by Steere et al. in Lyme arthritis [18]. Cardiac involvement was estab- lished as manifestation of Lyme disease by Steere et al. [19]. Bannwarth had observed tachycardia previously [3]. Of our patients 14% showed clinical signs of cardiac involvement, especially episodes of palpitations [25]. 18% of our patients had clinical signs of peripheral neuropathy, severe headaches, and/or symptoms possibly consistent with mild encephalitis [25]. Combinations of symptoms are common. For instance, one of our patients had lymphocytic meningoradiculitis, poly- arthritis in 22 joints and bradycardia [24].

EMD can be transmitted by ticks but many patients do not observe a tick bite [24]. This can mean that the tick bite went unnoticed or that the causative agent was acquired in another way, possibly by a flying insect.

In June 1982, Burgdorfer et al. reported on the discovery of spirochetes in ixodic ticks, cultivation of these spirochetes, elicitation of animal lesions, and positive serologic reactions in patients with Lyme disease [6]. Spirochetes were then re- covered from blood, skin and cerebrospinal fluid of patients with Lyme disease in March 1983 [5, 20] and from blood and cerebrospinal fluid of patients with EMD early in 1984 [1, 15]. These spirochetes are similar to each other if not identical. They are now classified as Borreliae. The suggested name is Borrelia burgdorferi [13].

In this journal, Pfister et al. present more details on the patients they have studied. They compare lymphocytic menin- goradiculitis with Lyme disease, and one of their conclusions is that "Bannwarth's syndrome may be a spirochetal infection limited to the nervous system.. ." [14]. I take another view.

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Firstly, in my opinion, lymphocytic meningoradiculit is is a neurological manifestat ion of E M D and not a disease entity per se. Thus, it seems to be more appropriate to compare neu- rological manifestations of E M D and Lyme disease. The dif- ference may not be as pronounced as recently indicated by Reik et al. [16]. An interesting point seems to be the fact that contrary to Lyme disease the peripheral neuropathy of patients seen in Europe almost always starts in the area affect- ed by tick bite and/or e ry thema migrans [12]. Fur thermore , Bannwarth ' s syndrome has recently been regarded as manifes- tation of Lyme disease in another report [7].

Secondly, it is an interesting, long-held view that B. burg- dorferi may spread along the nerves into the central nervous system. We have strong evidence that it spreads within the skin because it could be isolated from the migrating edge of an e ry thema migrans lesion [20]. However , it can also be found in the blood and may be carried from there into the central nervous system. In addition, we do not know where else B. burgdorferi can hide. It might be present in the joints, the heart or elsewhere without producing symptoms at the time of the lymphocytic meningoradiculit is. It is then conceivable that Borrel iae present outside the nervous system are capable o f inducing symptoms once the lymphocytic meningoradiculit is has cleared spontaneously.

The increase of specific antibody titers in patients with Bannwarth ' s syndrome is yet another example of what can happen if B. burgdorferi or a closely related Borrelia invades the body. Significantly elevated antibody titers have also been found in patients with Lyme disease [20], acrodermatit is chronica atrophicans and spirochetal lymphocytoma [25]. The latter two disorders are related to E M D [25].

Neurological manifestations of E M D can be treated suc- cessfully with antibiotics. High doses of parenteral penicillin have been found to be effective in recent investigations [17, 21,22, 24].

Dr. Klaus Weber

References

1. Ackermann R, Kabatzki J, Boisten HP, Steere AC, Grodzicki RL, Hartung S, Runne U (1984) Spirochfiten-Ji~tiologie der Erythema-chronicum-migrans-Krankheit. Dtsch Med Wochen- schr 109 : 92-97

2. Afzelius A (1910) Arch Dermatol Syph 101:404 3. Bannwarth A (1941) Chronische lymphocytfire Meningitis, ent-

zfindliche Polyneuritis und ,,Rhematismus". Arch Psychiatr Ner- venkr 113 : 284-376

4. Bannwarth A (1944) Zur Klinik und Pathogenese der ,,chroni- schen lymphocytfiren Meningitis". Arch Psychiatr Nervenkr 117: 161-185

5. Benach JL, Bosler EM, Hanrahan JP, Coleman JL, Habicht GS, Bast TF, Cameron D J, Ziegler JL, Barbour AG, Burgdorfer W, Edelman R, Kaslow RA (1983) Spirochetes isolated from the blood of two patients with Lyme disease. N Engl J Med 308 : 740- 742

6. Burgdorfer W, Barbour AG, Hayes SF, Benach JL, Grunwatdt E, Davis JP (1982) Lyme disease--a tick-borne spirochetosis? Science 216:1317-1319

7. Case record of the Massachusetts General Hospital (1984) N Engl J Med 311 : 172-181

8. Garin C, Bujadoux (1922) Paralysie par les tiques. J Med Lyon 71 : 765-767

9. Hellerstr6m S (1930) Erythema chronicum migrans Afzelii. Acta Derm Venereol (Stockh) 11:315-321

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11. Hollstr0m E (1951) Successful treatment of erythema migrans Afzelius. Acta Derm Venereol (Stockh) 31 : 235-243

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14. Pfister H-W, Einhfiupl K, Preac-Mursic V (1984) The spirochetal etiology of lymphocytic meningoradiculitis Bannwarth (Bann- warth's syndrome). J Neurol 231:141-144

15. Preac-Mursic V, Schierz G, Pfister H-W, Einh/iupl K, Wilske B, Weber K (1984) Isolierung einer Spiroch~ite aus Liquor cerebro- spinalis bei Meningoradikulitis Bannwarth. Mfinch Med Wochenschr 126: 275-276

16. Reik L, Steere AC, Bartenhagen NH, Shope RE, Malawista SE (1979) Neurologic abnormalities of Lyme disease. Medicine (Bal- timore) 58 : 281-294

17. Sk61denberg B, Stiernstedt G, Garde A, Kolmodin G, Carlstr6m A, Nord CE (1983) Chronic meningitis caused by a penicillin-sen- sitive microorganism? Lancet II : 75-78

18. Steere AC, Malawista SE, Hardin JA, Ruddy S, Askenase PW, Anoliman WA (1977) Erythema chronicum migrans and Lyme arthritis: the enlarging clinical spectrum. Ann Intern Med 86: 685- 698

19. Steere AC, Batsford WP, Weinberg M, Alexander J, Berger HJ, Wolfson S, Malawista SE (1980) Lyme carditis: cardiac abnormal- ities of Lyme disease. Ann Intern Med 93 : 8-16

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21. Steere AC, Pachner AR, Malawista SE (1983) Neurologic abnor- malities of Lyme disease: successful treatment with high-dose intravenous penicillin. Ann Intern Med 99 : 767-772

22. Weber K (1974) Erythema-chronicum-migrans-Meningitis - eine bakterielle Infektionskrankheit? Mfinch Med Wochenschr 116: 1993-1998

23. Weber K (1981) Treatment of Lyme disease. Ann Intern Med 94 : 137 (letter)

24. Weber K, Puzik A, Becker T (1983) Erythema-migrans-Krank- heit: Beitrag zur Klinik und Beziehung zur Lyme-Krankheit. Dtsch Med Wochenschr 108:1182-1190

25. Weber K, Schierz G, Wilske B, Preac-Mursic V (1984) European erythema migrans disease and related disorders. Yale J Biol Med

Received August 16, 1984 / Accepted August 20, 1984