BRIEF CLINICAL OBSERVATIONS
DIAGNOSISOF GIANT CELL ARTERITISBY OCCIPITALARTERYBIOPSY Seventeen percent of cases of giant cell arteritis may present with occipital pain [l], and involvement of the cervical branches of the external carotid artery, including the occipital artery, has been reported in 35% to 60% of autopsied cases [2]. Despite recognition of the diffuse nature of this disorder, superficial temporal artery biopsy remains the standard for its diagnosis, probably related to the frequent involvement and accessibility of the artery, the established safety of the procedure, and surgeons’ experience. However, biopsy of a clinically abnormal site has been associated with a higher diagnostic yield than blind biopsy [3]. In this report, two cases of giant cell arteritis are described that presented with occipital and cervical symptoms in which the diagnosis was readily and safely established by occipital artery biopsy. Patient 1. A 55-year-old woman was referred for the evaluation of low-grade fever, malaise, night sweats, weight loss, and headache in the left occipital area for approximately 1 month. She had no temporal pain or visual difficulty. She complained of “jaw cramping,” but it was not clearly associated with mastication. Physical examination showed a temperature of 37.3OC and notable point tenderness at the junction of the left occiput and the cervi-
Figure 1. stain;
original
Occipital artery magnification
biopsy X200,
specimens showing reduced by 20%.)
giant
cal spine. There was no temporal tenderness, and the temporal arteries were not palpable. The hemoglobin level was 1.4 mmol/L (9.0 g/dL), the hematocrit was 30.9%, and the Westergren erythrocyte sedimentation rate was 125 mm/h. Antinuclear antibody (ANA) was positive at 1:160 with a speckled pattern. Rheumatoid factor was negative. Radiographs of the cervical spine showed mild degenerative changes. Because the patient localized her symptoms to the left occipital area, the left occipital artery was selected for biopsy. A 3.5~cm skin incision was made below the nuchal line over the palpable artery in the occipital region. After the occipital nerve was identified, dissection was carried down to the occipital artery, which appeared to have a patent lumen; there was a whitish discoloration of its external surface, and it was abnormally large in diameter. A 2cm section was removed. The specimen (Figure 1, left) showed focal disruption of the arterial internal elastic lamina by multiple giant cells as well as intimal thickening and mild disruption of the media by chronic inflammatory infiltrate consistent with giant cell arteritis. Prednisone, 60 mg/d, brought about rapid resolution of the patient’s symptoms. There were no complications from the biopsy. Patient 2. A 59-year-old woman was seen for the evaluation of fever, stiff neck, and headache. She had been admitted for the suspicion of acute men-
cell arteritis.
August
Patient
1992
1, left;
The American
Patient
Journal
2, right.
(Hematoxylin
of Medicine
Volume
and eosin
93
231
BRIEF CLINICAL OBSERVATIONS
ingitis, but the cerebrospinal fluid was normal. There was a 5-day history of low-grade fever, myalgias, and malaise. Migraine headache had been treated in the remote past with methysergide. She stated the current pain was diffuse but maximal posteriorly and was distinct from her previous migraine headache pain. On examination, the temperature was 38.3OC and the patient appeared to be in significant discomfort. She had extreme pain on palpation of the posterior cervical area and neck stiffness. There was also tenderness over the frontal and temporal regions with a suggestion of thickening of the right temporal artery. The white blood cell count was 12,000/mm3, the hemoglobin level was 1.94 mmol/L (12.5 g/dL), and the Westergren erythrocyte sedimentation rate was 43 mm/h. Two days later, the patient continued to have severe head and neck pain and an elevated temperature. A subsequent sedimentation rate was 82 mm/h; when repeated 3 days later, it had further increased to greater than 150 mm/h. C-reactive protein was 1.26 mg/L, ANA was positive at 1:160 with a weak homogeneous pattern, and y-glutamyl transferase and alkaline phosphatase were elevated at 188 U/L and 363 U/L, respectively. Although the evolution of the patient’s illness was atypically acute, the clinical picture was consistent with giant cell arteritis. Because of her posterior headache and neck pain, the right occipital artery was selected for biopsy. An s-shaped incision was made between the mastoid and the inion. The occipital artery was identified beneath the subcutaneous tissue and fascia, and a 3.5-cm segment was submitted. The specimen (Figure 1, right) was consistent with giant cell arteritis. Prednisone 40 mg/d brought rapid resolution of her symptoms. She experienced no complications from the biopsy. Comments. Although standard references acknowledge involvement of the occipital artery by giant cell arteritis, to my knowledge only a single published case, reported in the Spanish literature, has described the diagnosis of giant celI arteritis by occipital artery biopsy [4]. Occipital arteritis has been presumptively diagnosed in cases that presented with occipital pain in which a temporal artery biopsy showed giant cell arteritis [ 1,5]. Because neither of our patients underwent temporal artery biopsy, it is unknown whether their diagnoses could have been made by that procedure. A study comparing the yield and complications of occipital artery biopsy with ipsilateral temporal artery biopsy in patients presenting with occipital or cervical pain would be needed to determine the procedure of choice in this situation. Although there is some belief that biopsy of the occipital artery is technically
232
August 1992 The American Journal of Medicine
more difficult than biopsy of the temporal artery, in both of our cases the procedure was straightforward, diagnostic specimens were easily obtained, and no complications occurred. In summary, these two cases emphasize that giant cell arteritis may present predominantly with occipital or cervical symptoms, and that, in such patients, occipital artery biopsy can be a safe and effective diagnostic procedure. J. JOHN WEEMS,Jr., M.D., F.A.C.P. Greenville Hospital System Greenville, South Carolina 1. Jundt JW. Mock D. Temporal arteritis with normal erythrocyte sedimentation rates presenting as occipital neuralgia. Arthritis Rheum 1991; 34: 217-9. 2. Wilkinson IMS, Russell WR. Arteries of the head and neck in giant cell arteritis. Arch Neural 1972; 27: 378-91. 3. Hall S. tie JT, Kurland LT, Persellin S. O’Brien PC, Hunder GG. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2: 1217-20. 4. Aranda EA, Garcia CP. Petri EM, Urbiola YE. Arteritis de celulas gigantes de comienzo occipital. Rev Clin Esp 1985; 176: 371-2. 5. Blaiss MS, Waxman J, Lange RK. Occipital headache as a manifestation of giant cell arteritis. South Med J 1982; 75: 887-8. Submitted
April 1. 1992, and accepted
April 13, 1992
ACKNOWLEDGMENT: I thank Drs. Morris Ray and Jerry Engleberg. SemmesMurphey Clinic, Memphis, Tennessee, who performed the biopsies, Ms. Lynn Morrell for secretarial support, and Ms. Nancy Taylor for editorial assistance. Photomicrographs courtesy of J. Nicholson, Medical University of South Carelina.
SELECTIVELOSSOF OPTIC NERVE,&TUBULIN IN VINCRISTINE-INDUCEDBLINDNESS Vincristine is a widely used chemotherapeutic agent of the antitubulin class. It binds specifically to dimeric tubulin and interferes with microtubular assembly. An antimitotic effect is achieved together with reversible and dose-dependent neurologic dysfunction in the majority of patients. Neurotoxicity usually consists of peripheral neuropathy, and less frequently ptosis, ophthalmoplegia, or other cranial nerve palsies. These neurologic side effects are related to impairment of axonal transport due to depolymerization of neurotubules and neurofibrillary degeneration [l]. Optic atrophy is potentially the most severe complication of vincristine therapy. It has been reported in nine patients (reviewed in [2]). We report the case of a 25-year-old woman from Zaire with acquired immunodeficiency syndrome diagnosed 2 years earlier (described in detail elsewhere [2]). Nine months before her death, she was treated for disseminated Kaposi’s sarcoma with vincristine, Adriamycin (doxorubicin), and bleomytin. At that time, her visual acuity was normal and the result of ophthalmologic examination was unre-
Volume 93
DIAGNOSISOF GIANT CELL ARTERITISBY OCCIPITALARTERYBIOPSY Seventeen percent of cases of giant cell arteritis may present with occipital pain [l], and involvement of the cervical branches of the external carotid artery, including the occipital artery, has been reported in 35% to 60% of autopsied cases [2]. Despite recognition of the diffuse nature of this disorder, superficial temporal artery biopsy remains the standard for its diagnosis, probably related to the frequent involvement and accessibility of the artery, the established safety of the procedure, and surgeons’ experience. However, biopsy of a clinically abnormal site has been associated with a higher diagnostic yield than blind biopsy [3]. In this report, two cases of giant cell arteritis are described that presented with occipital and cervical symptoms in which the diagnosis was readily and safely established by occipital artery biopsy. Patient 1. A 55-year-old woman was referred for the evaluation of low-grade fever, malaise, night sweats, weight loss, and headache in the left occipital area for approximately 1 month. She had no temporal pain or visual difficulty. She complained of “jaw cramping,” but it was not clearly associated with mastication. Physical examination showed a temperature of 37.3OC and notable point tenderness at the junction of the left occiput and the cervi-
Figure 1. stain;
original
Occipital artery magnification
biopsy X200,
specimens showing reduced by 20%.)
giant
cal spine. There was no temporal tenderness, and the temporal arteries were not palpable. The hemoglobin level was 1.4 mmol/L (9.0 g/dL), the hematocrit was 30.9%, and the Westergren erythrocyte sedimentation rate was 125 mm/h. Antinuclear antibody (ANA) was positive at 1:160 with a speckled pattern. Rheumatoid factor was negative. Radiographs of the cervical spine showed mild degenerative changes. Because the patient localized her symptoms to the left occipital area, the left occipital artery was selected for biopsy. A 3.5~cm skin incision was made below the nuchal line over the palpable artery in the occipital region. After the occipital nerve was identified, dissection was carried down to the occipital artery, which appeared to have a patent lumen; there was a whitish discoloration of its external surface, and it was abnormally large in diameter. A 2cm section was removed. The specimen (Figure 1, left) showed focal disruption of the arterial internal elastic lamina by multiple giant cells as well as intimal thickening and mild disruption of the media by chronic inflammatory infiltrate consistent with giant cell arteritis. Prednisone, 60 mg/d, brought about rapid resolution of the patient’s symptoms. There were no complications from the biopsy. Patient 2. A 59-year-old woman was seen for the evaluation of fever, stiff neck, and headache. She had been admitted for the suspicion of acute men-
cell arteritis.
August
Patient
1992
1, left;
The American
Patient
Journal
2, right.
(Hematoxylin
of Medicine
Volume
and eosin
93
231
BRIEF CLINICAL OBSERVATIONS
ingitis, but the cerebrospinal fluid was normal. There was a 5-day history of low-grade fever, myalgias, and malaise. Migraine headache had been treated in the remote past with methysergide. She stated the current pain was diffuse but maximal posteriorly and was distinct from her previous migraine headache pain. On examination, the temperature was 38.3OC and the patient appeared to be in significant discomfort. She had extreme pain on palpation of the posterior cervical area and neck stiffness. There was also tenderness over the frontal and temporal regions with a suggestion of thickening of the right temporal artery. The white blood cell count was 12,000/mm3, the hemoglobin level was 1.94 mmol/L (12.5 g/dL), and the Westergren erythrocyte sedimentation rate was 43 mm/h. Two days later, the patient continued to have severe head and neck pain and an elevated temperature. A subsequent sedimentation rate was 82 mm/h; when repeated 3 days later, it had further increased to greater than 150 mm/h. C-reactive protein was 1.26 mg/L, ANA was positive at 1:160 with a weak homogeneous pattern, and y-glutamyl transferase and alkaline phosphatase were elevated at 188 U/L and 363 U/L, respectively. Although the evolution of the patient’s illness was atypically acute, the clinical picture was consistent with giant cell arteritis. Because of her posterior headache and neck pain, the right occipital artery was selected for biopsy. An s-shaped incision was made between the mastoid and the inion. The occipital artery was identified beneath the subcutaneous tissue and fascia, and a 3.5-cm segment was submitted. The specimen (Figure 1, right) was consistent with giant cell arteritis. Prednisone 40 mg/d brought rapid resolution of her symptoms. She experienced no complications from the biopsy. Comments. Although standard references acknowledge involvement of the occipital artery by giant cell arteritis, to my knowledge only a single published case, reported in the Spanish literature, has described the diagnosis of giant celI arteritis by occipital artery biopsy [4]. Occipital arteritis has been presumptively diagnosed in cases that presented with occipital pain in which a temporal artery biopsy showed giant cell arteritis [ 1,5]. Because neither of our patients underwent temporal artery biopsy, it is unknown whether their diagnoses could have been made by that procedure. A study comparing the yield and complications of occipital artery biopsy with ipsilateral temporal artery biopsy in patients presenting with occipital or cervical pain would be needed to determine the procedure of choice in this situation. Although there is some belief that biopsy of the occipital artery is technically
232
August 1992 The American Journal of Medicine
more difficult than biopsy of the temporal artery, in both of our cases the procedure was straightforward, diagnostic specimens were easily obtained, and no complications occurred. In summary, these two cases emphasize that giant cell arteritis may present predominantly with occipital or cervical symptoms, and that, in such patients, occipital artery biopsy can be a safe and effective diagnostic procedure. J. JOHN WEEMS,Jr., M.D., F.A.C.P. Greenville Hospital System Greenville, South Carolina 1. Jundt JW. Mock D. Temporal arteritis with normal erythrocyte sedimentation rates presenting as occipital neuralgia. Arthritis Rheum 1991; 34: 217-9. 2. Wilkinson IMS, Russell WR. Arteries of the head and neck in giant cell arteritis. Arch Neural 1972; 27: 378-91. 3. Hall S. tie JT, Kurland LT, Persellin S. O’Brien PC, Hunder GG. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2: 1217-20. 4. Aranda EA, Garcia CP. Petri EM, Urbiola YE. Arteritis de celulas gigantes de comienzo occipital. Rev Clin Esp 1985; 176: 371-2. 5. Blaiss MS, Waxman J, Lange RK. Occipital headache as a manifestation of giant cell arteritis. South Med J 1982; 75: 887-8. Submitted
April 1. 1992, and accepted
April 13, 1992
ACKNOWLEDGMENT: I thank Drs. Morris Ray and Jerry Engleberg. SemmesMurphey Clinic, Memphis, Tennessee, who performed the biopsies, Ms. Lynn Morrell for secretarial support, and Ms. Nancy Taylor for editorial assistance. Photomicrographs courtesy of J. Nicholson, Medical University of South Carelina.
SELECTIVELOSSOF OPTIC NERVE,&TUBULIN IN VINCRISTINE-INDUCEDBLINDNESS Vincristine is a widely used chemotherapeutic agent of the antitubulin class. It binds specifically to dimeric tubulin and interferes with microtubular assembly. An antimitotic effect is achieved together with reversible and dose-dependent neurologic dysfunction in the majority of patients. Neurotoxicity usually consists of peripheral neuropathy, and less frequently ptosis, ophthalmoplegia, or other cranial nerve palsies. These neurologic side effects are related to impairment of axonal transport due to depolymerization of neurotubules and neurofibrillary degeneration [l]. Optic atrophy is potentially the most severe complication of vincristine therapy. It has been reported in nine patients (reviewed in [2]). We report the case of a 25-year-old woman from Zaire with acquired immunodeficiency syndrome diagnosed 2 years earlier (described in detail elsewhere [2]). Nine months before her death, she was treated for disseminated Kaposi’s sarcoma with vincristine, Adriamycin (doxorubicin), and bleomytin. At that time, her visual acuity was normal and the result of ophthalmologic examination was unre-
Volume 93
