333 years after onset. Her spinal fluid was never examined. Anterior horn cell loss and cortical spinal tract degeneration were demonstrated at necropsy. Conventional stains for central nervous system (CNS) spirochaetes were negative. Case 2.-A 42-year-old woman who had been on holiday in northern Wisconsin had arm weakness followed by dysarthria and weakness in all four limbs. There was no history of rash or arthritis. Atrophy and fasciculation were noted on examination. B burgdorferi antibody titres ranged from 64 to 4096 in this patient. Her CSF was normal. In view of the rise in titre while she was getting progressively worse intravenous ceftriaxonewas administered. Her disease seems to have stabilised. Case 3.-A 33-year-old man had left median prolonged motor and sensory distal latencies in September, 1985. He had previously been on holiday in northern Wisconsin. These findings progressed into hand weakness, generalised muscle fasciculation, and weakness characteristic of ALS. A first test for B burgdorferi was negative but a repeat test revealed a titre of 256. Case 4.-A 61-year-old man from Chicago had developed bilateral arm weakness. There was no history of rash or arthritis. Wasting, fasciculations, and depressed reflexes were present in the arms. The serum anti-B burgdorferi titre was 512. He died of respiratory failure. No necropsy was done. The finding of cases of ALS with high titres of B burgdorferi antibodies should be viewed in the context of other CNS diseases as diverse as dementia and multiple sclerosis for which similar antibodies have been reported. This could mean that the crossreactivity potential of B burgdorferi antigen is high—or that this spirochaete causes a wider diversity of common CNS syndromes than is generally recognised. Since there is no treatment for ALS and there is for chronic Lyme disease clinicians will ask if patients with ALS who have high-titre anti-Borrelia antibodies should be treated empirically with ceftriaxone, one of the antibiotics of choice for chronic B burgdorferi infection.6,7 At the least, it seems reasonable to find out if a patient with ALS does have B burgdorferi antibodies. Clinical Cell Biology Laboratory-Milwaukee, Milwaukee, Wisconsin 53211, USA
BURTON A. WAISBREN
Department of Neurology, Montreal Neurologic Institute and Hospital, Montreal, Quebec, Canada
State Laboratory of Hygiene and Department of Medical Microbiology, University of Wisconsin-Madison
RONALD F. SCHELL
Department of Microbiology, University of Minnesota Medical School
1 Reik L, Steere AC, Bartenhagen NH, et al. Neurologic abnormalities of
Lyme disease. Ann Intern Med 1980; 93: 8-16. 2. Pachner AR, Steere AC. The triad of neurologic manifestations of Lyme disease Neurology 1985; 35: 47-53. 3. Reik L, Burgdorfer W, Donaldson JA. Neurologic abnormalities in Lyme disease without erythema chronicum migrans. Am J Med 1986; 81: 73-77. 4. Steere AR. Spirochaetal disease of the CNS. Neurol Clin 1986; 4: 207-22. 5 Russell H, Sampson JS, Schmid GP, Wilkinson HW, Plikaytes B. Enzyme linked immunosolvent assay and indirect immunofluorescent assay for Lyme disease. J Inf Dis 1984; 149: 465-70. 6. Dattyler RJ, Halperm JJ, Pash H, Luft BJ. Ceftriaxone-effective therapy in refractory Lyme disease. J Inf Dis (in press). 7. Johnson RC, Codner C, Russell M. In vitro and in vivo susceptibility of Lyme disease spirochetes, Borrelia burgdorfert to 4 antimicrobials. Antimicrob Ag Chemother (in
non-Gardner’s FPC. CHRPE should now be investigated in other FPC kindreds as well as in hereditary non-polyposis colorectal cancer families.4,5 This research could aid in clarification of the nosology of hereditary colon cancer, which is reported to be
heterogeneous.6-10 Creighton University School of Medicine, Omaha, NE 6817, USA
HENRY T. LYNCH IRA PRILUCK MARY LEE FITZSIMMONS
1. Traboulsi 2.
3. 4. 5.
6. 7. 8. 9. 10.
EI, Krush AJ, Gardner EJ, et al. Prevalence and importance of pigmented ocular fundus lesions in Gardner’s sydrome. N Engl J Med 1987; 316: 661-67. Blair NP, Trempe CL. Hypertrophy of the retinal pigment epithelium associated with Gardner’s syndrome. Am J Ophthalmol 1980; 90: 661-67. Lewis RA, Crowder WE, Elerman LA, et al. The Gardner syndrome: Significance of ocular features. Ophthalmology 1984; 91: 916-25. Lynch HT, Kimberling WJ, Schuelke GS, et al. Hereditary nonpolyposis colorectal cancer I. Cancer 1986; 56: 934-38. Lynch HT, Kimberling WJ, Schuelke GS, et al. Hereditary nonpolyposis colorectal cancer II. Cancer 1986; 56: 939-51. Bulow S. Colorectal polyposis syndromes. Scan J Gastroenterol 1984; 19: 289-93. Bulow S, Sondergaard JO, Witt I, et al Mandibular osteomas in familial polyposis coli. Dis Colon Rectum 1984; 27: 105. Sondergaard JO, Svendsen LB, Witt IN, et al. Mandibular osteomas in colorectal cancer. Scan J Gastroenterol 1985; 209: 759-61 Sondergaard JO, Svendsen LB, Witt IN, et al. Mandibular osteomas in the cancer family syndrome. Br J Cancer 1986, 52: 941-43. Lynch PM, Lynch HT, eds. Colon cancer genetics. New York van Nostrand Reinhold Co, 1985.
FIBRIN DEGRADATION PRODUCTS ARE NOT SPECIFIC MARKERS FOR THROMBOLYSIS IN MYOCARDIAL INFARCTION
SIR,—It has been suggested that fibrin degradation products (FbDPs) are specific markers for lysis of intracoronary thrombi in myocardial infarction patients who receive thrombolytic therapy.’ However, the proper controls-ie, healthy subjects-have not been studied. This was our aim. Recombinant tissue-type plasminogen activator (rt-PA; Genentech) was infused over an hour in 18 healthy volunteers (mean age 35, SD 6), who were divided into three groups of 6. No volunteer had a history of bleeding disorders, thrombotic events, aneurysms, hypertension, renal disease, or atherosclerotic disease. Group I received 0-25 mg/kg rt-PA, group II 0-50 mg/kg, and group III a placebo. Venous blood samples were taken from the arm other than that infused. Two samples were collected on ice, one in citrate (final concentration 0-01 mol/1) and one in citrate plus aprotinin (150 x 103 IU/ml). After centrifugation and storage at -20°C, FbDPs were determined by ELISA with two monoclonal antibodies.2-4 Infusion of rt-PA in healthy volunteers was well tolerated without side-effects. Plasma FbDP levels in groups I and II
CONGENITAL HYPERTROPHY OF RETINAL PIGMENT EPITHELIUM IN NON-GARDNER’S POLYPOSIS KINDREDS
SIR,—Multiple pigmented patches of congenital hypertrophy of retinal pigment epithelium (CHRPE) are an important phenotypic marker for assessment of genotypic risk status in certain patients from families with Gardner’s syndrome.1-3 We wondered whether CHRPE might occur in patients who manifest hereditary polyposis of the colon (FPC) in the absence of Gardner’s stigmata. We have therefore done ophthalmological examinations in two FPC families without Gardner’s osseous or cutaneous stigmata. All four of the patients from these two families with FPC showed the CHRPE phenotype. This is the first account of CHRPE in patients with
FbDPs (µg/ml) in plasma collected in citrate after infusion of rt-PA in healthy volunteers.