J Oral Maxillofac 48:9SMSl,
Surg
1990
Eosinophilia-Myalgia Syndrome Masquerading as Facial Pain LARRY D. CHESLEY,
DDS,* AND JOHN W. VAN GILDER, DDSt
Eosinophilia and severe myalgia occurring in patients taking oral preparations of L-tryptophan (LT) is a recently recognized condition. It is now termed eosinophilia-myalgia syndrome (EMS) by the Centers for Disease Control (CDC). The CDC defines the characteristic signs and symptoms of EMS as 1) an eosinophil count greater than 1,000 cells per cubic millimeter, 2) generalized myalgia at some point during the course of the illness of sufficient severity to affect a patient’s ability to pursue his or her usual daily activities, and 3) absence of any infection or neoplasm that could account for 1 or 2 above. Presenting symptoms may also include subjective weakness, fever, arthralgia, shortness of breath, rash, peripheral edema, and pneumonia.“’ L-Tryptophan is an essential amino acid found in dietary protein and is a precursor to the neurotransmitters serotonin. It is available over the counter in many formulations and has been widely prescribed for the treatment of premenstrual syndrome, insomnia, and depression.’ A nationwide recall of LT-containing products (LTCP) was intiated on November 17, 1989, by the Food and Drug Administration (FDA). The current focus of studies on LTCP by the FDA is a search for chemical or microbial contaminants. L-Tryptophan is produced in Japan for ultimate sale and consumption in the United States, and the production practices of the Japanese pharmaceutical companies are now under close scrutiny by the FDA.2 Three hundred sixty cases of suspected EMS have been reported to the CDC by November 21, 1989, including a fatal case in the state of New
been elucidated.2 In this report, we describe a patient in whom the onset of facial pain eventually led to the diagnosis of EMS. Report of a Case A 42-year-old white woman was started on generic LT, 515 mg three times a day, by her gynecologist in July 1989 for premenstrual syndrome. Four weeks later she noted muscle aching in her arms and legs. Six weeks after beginning LT, she began experiencing facial muscle aching and jaw pain. The muscle pain progressed in a cyclical manner, worsening during waking hours and with mild exertion. She sought the advice of her internist and a rheumatologist in September 1989. The past medical history was significant only for allergic rhinitis and migraine headaches. The physical examination was essentially unremarkable. Laboratory evaluation, including complete blood cell count with differential, a chemistry 16 panel, urinalysis, antinuclear antibody study, and erythrocyte sedimentation rate, were all within normal limits except for an increase in the eosinophil count to 1,144/mm3. At this time, the eosinophila was thought to be idiopathic, and the tenative diagnosis was a connective tissue disease. L-Tryptophan was discontinued and the patient was referred to her general dentist to evaluate her for dental sources of her facial pain. The patient was subsequently referred to the Orofacial Pain Center for evaluation in October 1989. Her symptoms were unchanged at the time of this evaluation except that she reported early jaw fatigue with mastication. Generalized tenderness was noted bilaterally over the masseter and medial and lateral pterygoid muscles. The diagnosis of myofascial pain-dysfunction syndrome with possible connective tissue disease was made, and treatment consisting of a bite appliance and physical therapy was initiated. The patient returned in November 1989 for follow-up after newspaper accounts by the Associated Press had described a possible link between eosinophilia, myalgia, and LTCP. The diagnosis of EMS was then suggested to the rheumatologist by the oral and maxillofacial surgeons. After further review of the case, the rhematologist retrospectively confiied the diagnosis of EMS according to the guidelines of the CDC. The case was subsequently reported to the state department of public health. At the time of this report the patient’s symptoms have remained unimproved; however, no progression has been noted. Therapy has been directed toward symptomatic relief.
York. Case-control studies were initiated in Minnesota and New Mexico that yielded a statistically significant association between the use of LTCP and EMS. The mechanism of the disease has not * Former fellow in Oral and Maxillofacial Surgery, St Mary’s Health Center, St Louis, MO; currently in private practice, Amarillo. TX. t 1; private practice, Orofacial Pain Center, St Louis, MO. Address correspondence to Dr Chesley: 1900 Coulter, Suite J, Amarillo, TX 79106-1784. 0 1990 American geons
Association
of Oral and Maxillofacial
Discussion
Sur-
EMS is now gaining wide public attention. The diagnosis of EMS must exclude other known causes
0278-2391/90/4909-0012$3.00/0
980
981
MARMARY ET AL
of eosinophilia, eg, parasitic or fungal infection, end-stage renal disease, leukemia, allergic disorder, and drug reaction. The number of reported cases of EMS is rising exponentially as health care workers become alerted to this new diagnostic entity.’ The pathophysiology of EMS remains elusive. However, contamination of LTCP is strongly suspected. Also unknown and currently under investigation are 1) the existence of a possible doseresponse effect, 2) the incubation period between exposure and onset of the disease, 3) epidemic considerations, 4) determination of the full spectrum of clinical manifestations, 5) elucidation of pathogenetic mechanisms, and 6) determination of prognosis with appropriate therapies. Current therapy in-
J Oral Maxillofac
eludes discontinuance of LTCP, symptomatic relief, and use of corticosteroids. Research and epidemiologic studies by the CDC, FDA, and other groups are ongoing.* Oral and maxillofacial surgeons must remember to include EMS in their differential diagnosis of facial pain in patients taking LTCP. New cases should be reported to the state public health department. References 1. Centers for Disease Control: Eosinophilia-myalgia syndrome-New Mexico. MMWR 38:765, 1989. 2. Centers for Disease Control: Eosinophilia-myalgia and Ltryptophan-containing products-New Mexico, Minnesota, Oregon, and New York. MMWR 38:785, 1989
Surg
49:981-994.1990
Lymphoepithelial Parotid Cysts as Presenting Symptom of lmmunodeficiency Virus Infection: Clinical, Sialographic, and Magnetic Resonance Imaging Findings Y. MARMARY,
DMD, MScD,* J.M. GOMORI, MD,t AND D.W. NITZAN, DMD$.
Swellings of the parotid salivary glands that enlarge at a slow rate may be due to tumors, cysts, Sjogren syndrome, malnutrition, sarcoidosis, granulomatous infections, liver cirrhosis, and, in rare cases, drug intoxication.’ In the last few years, with the increased spread of the acquired immunodeficiency syndrome (AIDS), reports have appeared linking AIDS and parotid gland swellings. The AIDS-related parotid swellings have been diagnosed as lymphadenopathy,*-“ lymphomas,‘,4-6 KaReceived from The Hebrew University Hadassah Medical Center, Jerusalem, Israel. * Head, Unit of Oral Radiology. t Head, Unit of Magnetic Resonance Imaging. $ Senior lecturer, Department of Oral and Maxillofacial Sur-
gery. Address correspondence and reprint requests to Dr Marmary: Unit of Oral Radiology, Hadassah Medical Center, PO Box 12000, il-91 120, Jerusalem, Israel. 0 1990 American geons.
Association
0270-2391/90/4809-0013$3.00/0
of Oral and Maxillofacial
Sur-
posi’s sarcoma,‘,’ as well as lymphoepithelial cysts. 4,9-1’ We present a case of chronic parotid swelling demonstrated by sialography and magnetic resonance imaging (MRI) to be cystic in nature that led to the unsuspected diagnosis of AIDS. Report of a Case A 34-year-old man was seen at the Oral and Maxillofacial Surgery clinic of Hadassah Medical Center complaining of a painless swelling below his right ear. The slowly enlarging swelling was first noted 6 months before the visit. The past medical history was uneventful. Physical examination showed a marked swelling measuring .3 x 4 cm, extending posteriorly to the ascending ramus of the mandible. The movable mass, doughlike in texture, was located deep in the anterior neck triangle. It was not tender, and the overlying skin was normal. A diagnosis of developmental cyst or tumor was proposed. Sialography of the right parotid gland showed gross changes. The posteroinferior part of the gland was totally missing and the remainder was markedly affected. Only a few secondary ducts were opacifed, and no acinar tissue was dem-
Surg
1990
Eosinophilia-Myalgia Syndrome Masquerading as Facial Pain LARRY D. CHESLEY,
DDS,* AND JOHN W. VAN GILDER, DDSt
Eosinophilia and severe myalgia occurring in patients taking oral preparations of L-tryptophan (LT) is a recently recognized condition. It is now termed eosinophilia-myalgia syndrome (EMS) by the Centers for Disease Control (CDC). The CDC defines the characteristic signs and symptoms of EMS as 1) an eosinophil count greater than 1,000 cells per cubic millimeter, 2) generalized myalgia at some point during the course of the illness of sufficient severity to affect a patient’s ability to pursue his or her usual daily activities, and 3) absence of any infection or neoplasm that could account for 1 or 2 above. Presenting symptoms may also include subjective weakness, fever, arthralgia, shortness of breath, rash, peripheral edema, and pneumonia.“’ L-Tryptophan is an essential amino acid found in dietary protein and is a precursor to the neurotransmitters serotonin. It is available over the counter in many formulations and has been widely prescribed for the treatment of premenstrual syndrome, insomnia, and depression.’ A nationwide recall of LT-containing products (LTCP) was intiated on November 17, 1989, by the Food and Drug Administration (FDA). The current focus of studies on LTCP by the FDA is a search for chemical or microbial contaminants. L-Tryptophan is produced in Japan for ultimate sale and consumption in the United States, and the production practices of the Japanese pharmaceutical companies are now under close scrutiny by the FDA.2 Three hundred sixty cases of suspected EMS have been reported to the CDC by November 21, 1989, including a fatal case in the state of New
been elucidated.2 In this report, we describe a patient in whom the onset of facial pain eventually led to the diagnosis of EMS. Report of a Case A 42-year-old white woman was started on generic LT, 515 mg three times a day, by her gynecologist in July 1989 for premenstrual syndrome. Four weeks later she noted muscle aching in her arms and legs. Six weeks after beginning LT, she began experiencing facial muscle aching and jaw pain. The muscle pain progressed in a cyclical manner, worsening during waking hours and with mild exertion. She sought the advice of her internist and a rheumatologist in September 1989. The past medical history was significant only for allergic rhinitis and migraine headaches. The physical examination was essentially unremarkable. Laboratory evaluation, including complete blood cell count with differential, a chemistry 16 panel, urinalysis, antinuclear antibody study, and erythrocyte sedimentation rate, were all within normal limits except for an increase in the eosinophil count to 1,144/mm3. At this time, the eosinophila was thought to be idiopathic, and the tenative diagnosis was a connective tissue disease. L-Tryptophan was discontinued and the patient was referred to her general dentist to evaluate her for dental sources of her facial pain. The patient was subsequently referred to the Orofacial Pain Center for evaluation in October 1989. Her symptoms were unchanged at the time of this evaluation except that she reported early jaw fatigue with mastication. Generalized tenderness was noted bilaterally over the masseter and medial and lateral pterygoid muscles. The diagnosis of myofascial pain-dysfunction syndrome with possible connective tissue disease was made, and treatment consisting of a bite appliance and physical therapy was initiated. The patient returned in November 1989 for follow-up after newspaper accounts by the Associated Press had described a possible link between eosinophilia, myalgia, and LTCP. The diagnosis of EMS was then suggested to the rheumatologist by the oral and maxillofacial surgeons. After further review of the case, the rhematologist retrospectively confiied the diagnosis of EMS according to the guidelines of the CDC. The case was subsequently reported to the state department of public health. At the time of this report the patient’s symptoms have remained unimproved; however, no progression has been noted. Therapy has been directed toward symptomatic relief.
York. Case-control studies were initiated in Minnesota and New Mexico that yielded a statistically significant association between the use of LTCP and EMS. The mechanism of the disease has not * Former fellow in Oral and Maxillofacial Surgery, St Mary’s Health Center, St Louis, MO; currently in private practice, Amarillo. TX. t 1; private practice, Orofacial Pain Center, St Louis, MO. Address correspondence to Dr Chesley: 1900 Coulter, Suite J, Amarillo, TX 79106-1784. 0 1990 American geons
Association
of Oral and Maxillofacial
Discussion
Sur-
EMS is now gaining wide public attention. The diagnosis of EMS must exclude other known causes
0278-2391/90/4909-0012$3.00/0
980
981
MARMARY ET AL
of eosinophilia, eg, parasitic or fungal infection, end-stage renal disease, leukemia, allergic disorder, and drug reaction. The number of reported cases of EMS is rising exponentially as health care workers become alerted to this new diagnostic entity.’ The pathophysiology of EMS remains elusive. However, contamination of LTCP is strongly suspected. Also unknown and currently under investigation are 1) the existence of a possible doseresponse effect, 2) the incubation period between exposure and onset of the disease, 3) epidemic considerations, 4) determination of the full spectrum of clinical manifestations, 5) elucidation of pathogenetic mechanisms, and 6) determination of prognosis with appropriate therapies. Current therapy in-
J Oral Maxillofac
eludes discontinuance of LTCP, symptomatic relief, and use of corticosteroids. Research and epidemiologic studies by the CDC, FDA, and other groups are ongoing.* Oral and maxillofacial surgeons must remember to include EMS in their differential diagnosis of facial pain in patients taking LTCP. New cases should be reported to the state public health department. References 1. Centers for Disease Control: Eosinophilia-myalgia syndrome-New Mexico. MMWR 38:765, 1989. 2. Centers for Disease Control: Eosinophilia-myalgia and Ltryptophan-containing products-New Mexico, Minnesota, Oregon, and New York. MMWR 38:785, 1989
Surg
49:981-994.1990
Lymphoepithelial Parotid Cysts as Presenting Symptom of lmmunodeficiency Virus Infection: Clinical, Sialographic, and Magnetic Resonance Imaging Findings Y. MARMARY,
DMD, MScD,* J.M. GOMORI, MD,t AND D.W. NITZAN, DMD$.
Swellings of the parotid salivary glands that enlarge at a slow rate may be due to tumors, cysts, Sjogren syndrome, malnutrition, sarcoidosis, granulomatous infections, liver cirrhosis, and, in rare cases, drug intoxication.’ In the last few years, with the increased spread of the acquired immunodeficiency syndrome (AIDS), reports have appeared linking AIDS and parotid gland swellings. The AIDS-related parotid swellings have been diagnosed as lymphadenopathy,*-“ lymphomas,‘,4-6 KaReceived from The Hebrew University Hadassah Medical Center, Jerusalem, Israel. * Head, Unit of Oral Radiology. t Head, Unit of Magnetic Resonance Imaging. $ Senior lecturer, Department of Oral and Maxillofacial Sur-
gery. Address correspondence and reprint requests to Dr Marmary: Unit of Oral Radiology, Hadassah Medical Center, PO Box 12000, il-91 120, Jerusalem, Israel. 0 1990 American geons.
Association
0270-2391/90/4809-0013$3.00/0
of Oral and Maxillofacial
Sur-
posi’s sarcoma,‘,’ as well as lymphoepithelial cysts. 4,9-1’ We present a case of chronic parotid swelling demonstrated by sialography and magnetic resonance imaging (MRI) to be cystic in nature that led to the unsuspected diagnosis of AIDS. Report of a Case A 34-year-old man was seen at the Oral and Maxillofacial Surgery clinic of Hadassah Medical Center complaining of a painless swelling below his right ear. The slowly enlarging swelling was first noted 6 months before the visit. The past medical history was uneventful. Physical examination showed a marked swelling measuring .3 x 4 cm, extending posteriorly to the ascending ramus of the mandible. The movable mass, doughlike in texture, was located deep in the anterior neck triangle. It was not tender, and the overlying skin was normal. A diagnosis of developmental cyst or tumor was proposed. Sialography of the right parotid gland showed gross changes. The posteroinferior part of the gland was totally missing and the remainder was markedly affected. Only a few secondary ducts were opacifed, and no acinar tissue was dem-
