reach the distal external carotid artery. Sparing of the 11th nerve was indirect evidence for the dual vascularization of this nerve. In one of the cases reported by Devoize et al [14], similar symptoms occurred after therapeutic embolization of the APA, preceding surgery for cerebellar meningioma. Recovery was complete 1 year later. Earlier, in 1981, Lapresle and Faux [15] reported a 58-year-old patient with a right-sided cervical herpes zoster eruption complicated by severe and persisting involvement of the last four cranial nerves. The unilateral and systematic distribution of the lesion suggested a vascular disorder affecting the APA. Angiography was performed 3 weeks after the onset of the paralysis. No underlying lesion was demonstrated, but the APA could not be visualized, suggesting that occlusion of this artery was responsible for the involvement of the last four cranial nerves. In our two patients, lesions evoking arteritis of the external carotid system, as seen on angiography, especially of the APA (Figures 2 and 3), are probably consecutive to GCA in the absence of other etiologies. The effectiveness of corticoid therapy on the neurologic symptoms confirms this hypothesis and is in favor of &hernia of the lower cranial nerves being responsible for the Vernet’s syndrome. Because GCA is a segmented arteritis, the lack of trapezius and sternocleidomastoid muscle palsy in our patients was probably due to the relation of the musculospinal branch. In GCA, ischemic optic neuritis is the most common cause of blindness and results from involvement of the posterior ciliary arteries. Nerve ischemia has also been held responsible for several neurologic manifestations of GCA including facial palsy, neuro-otologic symptoms, and ophthalmoplegia. Arteritis of the vasa nervorum resulting in ischemit neuropathies involving particularly the seventh cranial nerve has been described during other forms of vasculitis, including polyarteritis nodosa, Wegener’s disease, polychondritis, and Cogan’s syndrome [16,17]. To our knowledge, this is the first description of Verne0 syndrome of ischemic origin occurring with GCA. These two cases emphasize the clinical diversity of GCA. Cranial nerve involvement in the elderly, with an associated biologic inflammatory syndrome, should raise the diagnosis of GCA, after exclusion of a local origin, especially tumoral causes [18]. Nerve ischemia is a possible mechanism for some idiopathic cranial palsies. PATRICK CHERIN, M.D. CHRISTIAN DE GENNES, M.D. OLIVIER BLETRY, M.D. L-&UN LAMAS, M.D.
352
September 1992 The American Journal of Medicine
MICHEL LAUNA Y, M.D ALAIN DUBS, M.D PIERRE GODEAU, M.D H&pita1 Pit&SalpBtriBrt Paris, France 1. Hamrin 6. Aortic arch syndrome. Acta Med Stand 1972; 533 (Suppl): 86-99 2. Ostberg G. Temporal arteritis in a large necropsy series. Ann Rheum Dis 1971 30: 224-35. 3. Bakchine S, Cote D, Massiou H, Derouesne C. Un MS de paralysie facials peripherique au tours dune maladie de Horton. Presse Med 1985; 14: 2062. 4. Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology 1988; 38: 352-9. 5. Paulley JW. Hugues JP. Giant-cell arteritis. or arteritis of the aged. BMJ 1960; 26: 1562-7. 6. Mehler MF. Rabinowich L. The clinical neuro-ophthalmologic spectrum of temporal arteritis. Am J Med 1988; 85: 839-44. 7. Delvigne JM, Piette AM, Chapman A. Maladie de Horton r&&lee par un trismus. Presse Med 1985; 4: 1151-2. 6. Gentric A, Saccino E, Mottier D. Islam S, Ctedes J. Temporal arteritis revealed by a syndrome of inappropriate secretion of antidiuretic hormone. Am J Med 1988; 85: 559-60. 9. Hunder GG, Bloch DA, Michel EA. et a/. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33: 1122-B. 10. Lapresle J, Lasjaunias P. Cranial nerve ischaemic arterial syndromes. Brain
1986; 1091207-15. 11. Lasjaunias P, Doyon D. The ascending
pharyngeal artery and the blood supply of the lower cranial nerves. J Neuroradiol 1978; 5: 287-301. 12. Patterson EL. Sources of arterial blood supply to the superior and middle cervical sympathetic ganglia and the ganglion intermediaire. J Anat 1950; 84: 329-41. 13. Lapresle J. Lasjaunias P. Thevenier D. Atteinte transitoire des IX, X et XII ainsi que du VII gauches au d&ours dune angiographie. Rev Neurol19BO; 136: 787-91. 14. Devoize JL. Rouanet J. Cellerier P, Georget AM, Tournilhac M. Paralysie benigne des quatre derniers nerfs craniens. Arguments en faveur dun mecanisme ischemique. Presse Med 1985; 14: 132B-30. 15. Lapresle J, Faux N. Atteinte unilaterale des IX, X. XI, et XII au tours d’un zona cervical. J Neurol Sci 1981; 52: 352-7. 16. Vathenen AS, Skinner DW. Shale DJ. Treatment response with bilateral mixed deafness
and facial palsy in polyarteritis
nodosa.
Am J Med 1988; 84:
1081-2. 17. Kovarsky
J. Otorhinolaryngologic
complications
of rheumatic
min Arthritis Rheum 1984; 14: 141-7. 16.Tanaka M, lsaka KI. Morimatsu M, Hirai S. Jugular Neurology 1983; 32: 119-20. Submitted
February
5, 1992, and accepted
in revised
foramen
diseases. Sesyndrome.
form June 11, 1992
NOT EVERYTHINGTHAT GLITTERSIS LYME DISEASE In view of the protean clinical manifestations of Lyme disease, patients with a wide range of symptoms or signs may be diagnosed and treated for Lyme borreliosis. In addition, despite poorly standardized serologic testing for Borrelia burgdorferi infection [l], clinicians may nevertheless accept a positive result as diagnostic, and treat both symptomatic and asymptomatic patients. Our recent experience with three patients, in whom the diagnosis of Lyme borreliosis was plausible, illustrates the importance of first excluding other reasonable possibilities.
Volume 93
Patient 1. A 62-year-old man was bitten by a tick in rural Michigan. Several days later, he developed low-grade fever, arthralgias, and myalgias. B. burgdorferi serology by enzyme-linked immunosorbent assay for combined IgG and IgM was positive; IgM was negative. Myalgias, arthralgias, and malaise resolved with doxycycline, but low-grade fever persisted for 3 weeks after therapy was discontinued. Multiple blood cultures were then drawn, and all were positive for Streptococcus mitior. An echocardiogram showed mitral and aortic regurgitation, supporting the diagnosis of subacute bacterial endocarditis. Patient 2. A IO-year-old man was bitten twice by ticks in Westchester County, New York. Despite 3 weeks of “prophylactic” doxycycline for Lyme disease, he developed fatigue, myalgias, and arthralgias. The white blood cell count (WBC) was 2,900/mm3. Although the result of serologic testing for B. burgdorferi was negative, Lyme disease was still suspected, and he was retreated with tetracycline. Two weeks later, the WBC was 2,300/mm3, and tetracycline was stopped. One month later, promyelocytes were found on the peripheral blood smear, and bone marrow biopsy confirmed acute monoblastic leukemia. Patient 3. A 60-year-old man residing in Westchester County, New York, was treated with tetracycline for acute Lyme disease after developing a “bull’s-eye” rash following a tick bite. Over the next few months, however, he had three episodes of transient right-sided paresthesias and hemiparesis. After an extensive and unremarkable neurologic evaluation, which included normal scans, normal cerebrospinal fluid (CSF), and negative serum and CSF tests for B. burgdorferi antibody, separate courses of oral and then intravenous antibiotics were given for Lyme neuroborreliosis. Two months later, right hemiparesis recurred and treatment with ceftriaxone was restarted. However, a magnetic resonance imaging scan of the brain revealed a new left pontine infarct, a cerebral angiogram showed left vertebral artery stenosis, and the diagnosis of cerebral
infarction secondary to an atheroembolic event was made. Comments. Primarily because of exposure to ticks in an endemic area, these three patients were diagnosed as having Lyme borreliosis despite findings not described in the spectrum of this disease: persistent fever (Patient l), leukopenia (Patient 2), and episodes resembling transient ischemic attacks (Patient 3). With regard to these three particular findings, it is worth pointing out that (1) subacute bacterial endocarditis may mimic Lyme disease and be associated with positive serology [2], (2) the reported range of WBCs in patients with Lyme borreliosis is 4,600 to 13,200/mm3 [3], and (3) patients considered to have neuroborreliosis typically show either a CSF abnormality, positive CSF serology, or neuropsychologic or electrophysiologic deficits [4]. With hindsight, each of our cases displayed at least one clinical or laboratory feature not consistent with the reported spectrum of Lyme borreliosis. Since the diagnosis of this infection using currently available testing is difficult to secure (unless patients show characteristic skin lesions), our experience suggests that with inconsistent clinical or laboratory findings, it would be best to consider Lyme disease as a diagnosis of exclusion. DIEGO MIRALLES, BARRY HARTMAN, BARRY BRAUSE, LAURA FISHER, HENRY W. MURRAY,
M.D. M.D. M.D. M.D. M.D.
Cornell University Medical College New York, New York 1. Luger SW, Krauss E. Serologic tests for Lyme disease: interlaboratory variability. Arch Intern Med 1990; 150: 761-3. 2. Kaell AT, Volkman DJ, Gorevic PD, Dattwyler RJ. Positive Lyme serology in subacute bacterial endocarditis. JAMA 1990; 264: 2916-8. 3. Steere AC, Malawista SE, Hardin JA, et al. Erythema chronicum migrans and Lyme arthritis: the enlarging clinical spectrum. Ann Intern Med 1977; 86: 68%-98. 4. Logigian EL, Kaplan RF, Steere AC. Chronic neurologic manifestations of Lyme disease N Engl J Med 1990; 323: 1438-44. Submitted
April 21, 1992, and accepted
(NOTE: This work was supported
in revised form
by the Horace Goldsmith
September 1992 The American Journal of Medicine
May 8, 1992
Foundation.)
Volume 93
393
352
September 1992 The American Journal of Medicine
MICHEL LAUNA Y, M.D ALAIN DUBS, M.D PIERRE GODEAU, M.D H&pita1 Pit&SalpBtriBrt Paris, France 1. Hamrin 6. Aortic arch syndrome. Acta Med Stand 1972; 533 (Suppl): 86-99 2. Ostberg G. Temporal arteritis in a large necropsy series. Ann Rheum Dis 1971 30: 224-35. 3. Bakchine S, Cote D, Massiou H, Derouesne C. Un MS de paralysie facials peripherique au tours dune maladie de Horton. Presse Med 1985; 14: 2062. 4. Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology 1988; 38: 352-9. 5. Paulley JW. Hugues JP. Giant-cell arteritis. or arteritis of the aged. BMJ 1960; 26: 1562-7. 6. Mehler MF. Rabinowich L. The clinical neuro-ophthalmologic spectrum of temporal arteritis. Am J Med 1988; 85: 839-44. 7. Delvigne JM, Piette AM, Chapman A. Maladie de Horton r&&lee par un trismus. Presse Med 1985; 4: 1151-2. 6. Gentric A, Saccino E, Mottier D. Islam S, Ctedes J. Temporal arteritis revealed by a syndrome of inappropriate secretion of antidiuretic hormone. Am J Med 1988; 85: 559-60. 9. Hunder GG, Bloch DA, Michel EA. et a/. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33: 1122-B. 10. Lapresle J, Lasjaunias P. Cranial nerve ischaemic arterial syndromes. Brain
1986; 1091207-15. 11. Lasjaunias P, Doyon D. The ascending
pharyngeal artery and the blood supply of the lower cranial nerves. J Neuroradiol 1978; 5: 287-301. 12. Patterson EL. Sources of arterial blood supply to the superior and middle cervical sympathetic ganglia and the ganglion intermediaire. J Anat 1950; 84: 329-41. 13. Lapresle J. Lasjaunias P. Thevenier D. Atteinte transitoire des IX, X et XII ainsi que du VII gauches au d&ours dune angiographie. Rev Neurol19BO; 136: 787-91. 14. Devoize JL. Rouanet J. Cellerier P, Georget AM, Tournilhac M. Paralysie benigne des quatre derniers nerfs craniens. Arguments en faveur dun mecanisme ischemique. Presse Med 1985; 14: 132B-30. 15. Lapresle J, Faux N. Atteinte unilaterale des IX, X. XI, et XII au tours d’un zona cervical. J Neurol Sci 1981; 52: 352-7. 16. Vathenen AS, Skinner DW. Shale DJ. Treatment response with bilateral mixed deafness
and facial palsy in polyarteritis
nodosa.
Am J Med 1988; 84:
1081-2. 17. Kovarsky
J. Otorhinolaryngologic
complications
of rheumatic
min Arthritis Rheum 1984; 14: 141-7. 16.Tanaka M, lsaka KI. Morimatsu M, Hirai S. Jugular Neurology 1983; 32: 119-20. Submitted
February
5, 1992, and accepted
in revised
foramen
diseases. Sesyndrome.
form June 11, 1992
NOT EVERYTHINGTHAT GLITTERSIS LYME DISEASE In view of the protean clinical manifestations of Lyme disease, patients with a wide range of symptoms or signs may be diagnosed and treated for Lyme borreliosis. In addition, despite poorly standardized serologic testing for Borrelia burgdorferi infection [l], clinicians may nevertheless accept a positive result as diagnostic, and treat both symptomatic and asymptomatic patients. Our recent experience with three patients, in whom the diagnosis of Lyme borreliosis was plausible, illustrates the importance of first excluding other reasonable possibilities.
Volume 93
Patient 1. A 62-year-old man was bitten by a tick in rural Michigan. Several days later, he developed low-grade fever, arthralgias, and myalgias. B. burgdorferi serology by enzyme-linked immunosorbent assay for combined IgG and IgM was positive; IgM was negative. Myalgias, arthralgias, and malaise resolved with doxycycline, but low-grade fever persisted for 3 weeks after therapy was discontinued. Multiple blood cultures were then drawn, and all were positive for Streptococcus mitior. An echocardiogram showed mitral and aortic regurgitation, supporting the diagnosis of subacute bacterial endocarditis. Patient 2. A IO-year-old man was bitten twice by ticks in Westchester County, New York. Despite 3 weeks of “prophylactic” doxycycline for Lyme disease, he developed fatigue, myalgias, and arthralgias. The white blood cell count (WBC) was 2,900/mm3. Although the result of serologic testing for B. burgdorferi was negative, Lyme disease was still suspected, and he was retreated with tetracycline. Two weeks later, the WBC was 2,300/mm3, and tetracycline was stopped. One month later, promyelocytes were found on the peripheral blood smear, and bone marrow biopsy confirmed acute monoblastic leukemia. Patient 3. A 60-year-old man residing in Westchester County, New York, was treated with tetracycline for acute Lyme disease after developing a “bull’s-eye” rash following a tick bite. Over the next few months, however, he had three episodes of transient right-sided paresthesias and hemiparesis. After an extensive and unremarkable neurologic evaluation, which included normal scans, normal cerebrospinal fluid (CSF), and negative serum and CSF tests for B. burgdorferi antibody, separate courses of oral and then intravenous antibiotics were given for Lyme neuroborreliosis. Two months later, right hemiparesis recurred and treatment with ceftriaxone was restarted. However, a magnetic resonance imaging scan of the brain revealed a new left pontine infarct, a cerebral angiogram showed left vertebral artery stenosis, and the diagnosis of cerebral
infarction secondary to an atheroembolic event was made. Comments. Primarily because of exposure to ticks in an endemic area, these three patients were diagnosed as having Lyme borreliosis despite findings not described in the spectrum of this disease: persistent fever (Patient l), leukopenia (Patient 2), and episodes resembling transient ischemic attacks (Patient 3). With regard to these three particular findings, it is worth pointing out that (1) subacute bacterial endocarditis may mimic Lyme disease and be associated with positive serology [2], (2) the reported range of WBCs in patients with Lyme borreliosis is 4,600 to 13,200/mm3 [3], and (3) patients considered to have neuroborreliosis typically show either a CSF abnormality, positive CSF serology, or neuropsychologic or electrophysiologic deficits [4]. With hindsight, each of our cases displayed at least one clinical or laboratory feature not consistent with the reported spectrum of Lyme borreliosis. Since the diagnosis of this infection using currently available testing is difficult to secure (unless patients show characteristic skin lesions), our experience suggests that with inconsistent clinical or laboratory findings, it would be best to consider Lyme disease as a diagnosis of exclusion. DIEGO MIRALLES, BARRY HARTMAN, BARRY BRAUSE, LAURA FISHER, HENRY W. MURRAY,
M.D. M.D. M.D. M.D. M.D.
Cornell University Medical College New York, New York 1. Luger SW, Krauss E. Serologic tests for Lyme disease: interlaboratory variability. Arch Intern Med 1990; 150: 761-3. 2. Kaell AT, Volkman DJ, Gorevic PD, Dattwyler RJ. Positive Lyme serology in subacute bacterial endocarditis. JAMA 1990; 264: 2916-8. 3. Steere AC, Malawista SE, Hardin JA, et al. Erythema chronicum migrans and Lyme arthritis: the enlarging clinical spectrum. Ann Intern Med 1977; 86: 68%-98. 4. Logigian EL, Kaplan RF, Steere AC. Chronic neurologic manifestations of Lyme disease N Engl J Med 1990; 323: 1438-44. Submitted
April 21, 1992, and accepted
(NOTE: This work was supported
in revised form
by the Horace Goldsmith
September 1992 The American Journal of Medicine
May 8, 1992
Foundation.)
Volume 93
393
