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Temporal arteritis with normal erythrocyte sedimentation rates presenting as occipital neuralgia.

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A retrospective review of 46 patients with biopsyproven giant cell (temporal) arteritis revealed 8 (17%)
whose initial presentation was occipital pain. The most
likely etiology of occipital pain in these patients was
occipital artery inflammation. A Westergren erythrocyte sedimentation rate <40 mm/hour was noted at
presentation in 6 of these 8 patients. Giant cell arteritis
should be considered in the differential diagnosis of
elderly patients who present with occipital pain and
demonstrate a normal erythrocyte sedimentation rate.
Giant cell (temporal) arteritis (GCA) is often
associated with a markedly elevated erythrocyte sedimentation rate (ESR). In patients with a normal ESR,
the diagnosis may be delayed, especially in those with
atypical symptoms. Significant occipital pain was
noted in 8 (17%) of our patients with GCA. At presentation, 6 of the 8 patients with occipital pain had a
normal ESR. The ESR became elevated in only 1 other
of the 8 patients during the course of the disease. We
present the cases of 2 of these patients to illustrate this
atypical presentation of GCA.
Patient 1. Patient 1, an 87-year-old white man,
presented on January 5 , 1989 with a 9-day history of
aching pain and paresthesia in the left occipital area.
From Rheumatology and Internal Medicine, Scott & White
Clinic, Temple, and Texas A & M University, College of Medicine,
College Station, Texas.
Jeffrey W. Jundt, MD: Senior Staff Physician; David Mock,
MD: Internal Medicine Resident Physician.
Address reprint requests to Jeffrey W. Jundt, MD, Scott &
White Clinic, 2401 South 31st Street, Temple, TX 76508.
Submitted for publication July 10, 1990; accepted in revised
form October 8, 1990.
Arthritis and Rheumatism, Vol. 34, No. 2 (February 1991)
He had no history of temporal area pain, changes in
vision, jaw claudication, or proximal muscle stiffness.
His medical history included rheumatic heart disease
and chronic atrial fibrillation.
Physical examination revealed moderate, localized left occipital tenderness. The temporal arteries
were nontender, pulsatile, and had no nodules or
induration. His cardiac rhythm was irregularly irregular, and a grade III/VI systolic ejection murmur was
noted at the left sternal border. The Westergren ESR
was 10 mm/hour. The C-reactive protein level was not
measured. Results of liver function studies, including
serum glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), and alkaline phosphatase levels, were within normal limits.
The patient was diagnosed as having occipital
neuralgia. He was treated with ibuprofen 400 mg every
6 hours for 14 days, but there was no benefit. He was
then given prednisone, 20 mg twice daily, for 5 days.
The occipital pain subsided 24 hours after the initiation
of steroid therapy. Bilateral occipital pain recurred 3
days after discontinuing the prednisone, and bilateral
occipital nerve block was then performed, but there
was relief of pain only on the left side. On February 16,
1989, the patient was hospitalized after experiencing
the sudden onset of bilateral loss of vision, which had
lasted 15 minutes. A subsequent episode involving
only the left visual field occurred 5-6 hours later.
Results of visual field and funduscopic examinations
were unremarkable. Results of a computed tomography scan of the head and an echocardiogram were
within normal limits. The ESR was again measured
and was 32 mm/hour. An additional diagnosis of
vertebral-basilar transient ischemic attacks was made,
and the patient was given anticoagulant medications.
The occipital pain worsened, and on February
23, 1989, a temporal artery biopsy was performed.
Examination of the biopsy sample showed an inflammatory exudate, consisting of lymphocytes and eosinophils, which was present in the adventitia, media, and
intima of both arteries, most prominently in the left
temporal artery. A few giant cells were present along
the lamina elastica interna in the right temporal artery.
These findings were consistent with a diagnosis of
giant cell arteritis. Prednisone, 20 mg 3 timedday, was
prescribed, and there was prompt resolution of the
symptoms. The prednisone dosage was gradually tapered, and at followup, 1 year after the onset of
disease, there had been no recurrence of the occipital
pain or loss of vision. No elevation of the ESR was
noted during the course of his disease.
Patient 2. Patient 2, a 78-year-old white woman,
presented with a 3-week history of right-sided occipital
pain and tenderness. There was no history of changes
in vision, jaw claudication, or proximal muscle stiffness. Physical examination revealed tenderness limited to the right occipital and suboccipital area. The
temporal arteries were normal. The initial ESR was 23
mm/hour, and the hemoglobin level was 11.5 gm/dl.
The C-reactive protein level was not determined.
SGOT and SGPT levels were within normal limits. The
alkaline phosphatase level was elevated, at 186 IU/liter
(normal 30-1 15).
Occipital neuralgia was diagnosed, and the patient underwent 2 consecutive occipital nerve block
procedures 24 hours apart. The pain persisted. A
repeat ESR measured 24 hours later was 61 mm/hour.
The right temporal artery was biopsied, and
revealed marked inflammation, primarily in the zone
of the internal and external elastic lamella. Multinucleated giant cells, as well as lymphocytes, neutro-
Anterlor Branch
Posterior Branch
Superficial Temporal
Figure 1. Anatomic relationship between the occipital artery,
cipital nerve, and temporal artery.
phils, and histiocytes, were present. There was rather
prominent thickening of the intima in areas of inflammation, with considerable narrowing of the lumen.
Prednisone, 20 mg 3 timedday , was prescribed,
the dosage was gradually tapered, and after 4 years,
the prednisone was discontinued. No recurrence or
other complications of GCA were noted during the
course of the disease.
We reviewed the records of all patients with
biopsy-proven giant cell (temporal) arteritis who at-
Table 1. Clinical features of 8 patients with biopsy-proven giant cell (temporal) arteritis and occipital pain*
ESR (mmlhour)
Location of
occipital pain
517 1/M
Right unilateral
Left unilateral
Left unilateral
Associated symptoms
Transient bilateral loss of
Jaw claudication
Frontal headache
Decreased hearing
Temporal artery
Transient loss of vision
Polymyalgia rheumatica
Time between
ESR measurements (days)
* All patients were Caucasian. The erythrocyte sedimentation rates (ESR) were measured in followup prior to initiation of prednisone therapy.
tended our clinic from 1981 to 1989. Of the 46 patients,
there had been significant occipital pain at presentation in 8 (17%). To the best of our knowledge, occipital
artery involvement in biopsy-proven GCA has been
alluded to, but has not specifically been evaluated in
any large series (1,2).
Occipital neuralgia is described as a paroxysmal
or continuous aching or burning occipital pain that
may radiate to the frontal region. Localized tenderness
of the nerve over the superior nuchal ridge is noted in
the majority of cases (3,4). The pain is thought to arise
from irritation or injury to the occipital nerve somewhere along its course from the C2 dorsal root, where
it originates (5-7). In close proximity to the occipital
nerve lies the occipital artery (Figure 1). The occipital
pain in the patients reviewed here was most likely
secondary to inflammation of the occipital artery. The
paresthesia in patient 1 may have arisen from diminished blood supply to the occipital nerve.
The occipital artery arises from the posterior
portion of the external carotid, opposite the origin of
the facial artery. It runs posterolaterally and pierces
the superficial fascia of the scalp in the inferior portion
of the occipital region. Tortuous branches arise, and
some of these form an anastomosis with the posterior
branch of the ipsilateral superficial temporal artery (8)
(Figure 1). It is apparent that similar pathologic processes may affect both arteries, and if symptomatic or
tender, the occipital artery could be considered for
A Westergren ESR of <40 mm/hour was found
in 15% (7 of 46) of these cases of biopsy-proven GCA,
which included patients with and without occipital
pain. GCA associated with a normal ESR has been
reported in 10-30% of patients (9-11). The frequency
may vary depending upon the ESR test used and the
definition of “normal” when interpreting the results.
Normal values for elderly patients may range from
<20 mm/hour to 40 mm/hour (12,13). Unexplainably, 5
of our 8 patients with occipital pain never had an
elevation of their ESR beyond 40 mm/hour (Table 1).
A larger number of patients would be needed to assess
any possible association between a normal ESR and
occipital artery involvement in GCA. A Swedish series
involving 95 patients with biopsy-proven GCA noted 3
patients with a history of occipital pain, 2 of whom had
occipital artery tenderness upon physical examination
(14). The ESR results for these patients were not
available (14).
In this retrospective study, 17% (8 of 46) of our
patients with GCA had significant occipital pain as the
predominant symptom at presentation. The initial ESR
was normal in 6 of the 8 patients, with subsequent
documented elevation in only 1 additional patient. The
significance of this association is unclear. A normal
ESR may occur with both typical and atypical presentations of GCA (15). We have described 2 of these
patients who presented with a normal ESR and symptoms consistent with occipital neuralgia. The differential diagnosis of occipital pain in the elderly should
include giant cell arteritis, even when typical symptoms of temporal arteritis and an elevated ESR are
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occipital, neuralgia, sedimentation, rate, arteritis, erythrocytes, presenting, norman, temporal
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