Clivus and cervical spinal osteomyelitis with epidural abscess presenting with multiple cranial neuropathies

Clinical Neurology and Neurosurgery

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Clinical Neurology and Neurosurgery 97 (1995) 239-244

Case report

Clivus and cervical spinal osteomyelitis with epidural abscess presenting with multiple cranial neuropathies S. Ausim Azizi a'*, Pierre B. Fayad a, Robert Fulbright b, Monique L. Giroux a, Stephen G. Waxman a'c "Department of Neurology, Yale School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA bDepartment of Diagnostic Imaging, Yale School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA CNeuroscience Research Center, Veterans Administration Hospital West Haven, CT 06516, USA Received 10 January 1995; accepted 23 March 1995

Abstract A 65-year-old diabetic man with a history of otitis was admitted with headache, neck and shoulder pain and cranial nerve abnormalities including sixth, seventh and twelfth nerve palsies, hearing loss and ptosis. Lumbar puncture revealed an elevated CSF protein and pleocytosis. Imal~ng procedures demonstrated osteomyelitis of the clivus that involved the epidural space and extended within the prevertebral space to the cervical spine. The patient improved after treatment with antibiotics and immobilization of the neck. This case illustrates the importance of recognizing infections of the clivus in patients with cranial nerve abnormalities.

Keywords: Clivus; Osteomyelitis; Cranial neuropathies; Epidural abscess

1. Introduction In this report we describe a case of clivus infection with spinal epidural abscess. The clivus (Latin: hill), situated between the foramen magnum and dorsum sellae, is originally a cartilaginous structure that ossifies to form the basisphenoid and the basiocciput [1]. It is contiguous with the prevertebral space, an anatomic compartment enveloped by the innermost layer of deep cervical fascia. The vertebral bodies, disc spaces and epidural space of the cervical and upper thoracic spine are located in the prevertebral space. A wide spectrum of pathological processes including neoplasms [2~5], granulomatous diseases [7-8], infections [9-11], vascular and hematopoietic [12] disorders can involve the clivus and paraclival structures. The present report illustrates a rare case of clivus inflammation, associated with cranial nerve abnormali-

*Corresponding author. At: Department of Neurology, Thomas Jefferson University, 1025 Walnut St., Suite 511, Philadelphia, PA 19107, USA. Tel.: (215) 955-0707, Fax: (215) 955-5515. 0303-8467/95/$9.50 © 1995 Elsevier Science B.V. All rights reserved S S D I 0303-8467(95)00036-.4

ties, following otitis with contiguous spread, apparently via the prevertebral space, to cervical epidural and retropharyngeal spaces as well as the neighboring vertebral bodies and discs. In this report we discuss pathogenesis, anatomical relations, radiologic and microbiologic aspects of this case.

2. Case report A 65-year-old right-handed man with a history of diabetes was admitted to the hospital because of cranial nerve abnormalities, headache and carotid stenosis. He was in his usual state of health until approximately 6 months prior to this admission, when he experienced left ear pain. He was treated with antibiotics for a month for a presumed diagnosis of otitis externa without resolution. Two months later a mastoidectomy and tympanoplasty were performed and reportedly the infection cleared. However, he continued to complain of headache, left temporal and facial pain and hearing loss. He was started on prednisone after a presumed diagnosis of

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S. Ausim Azizi et al./ Clinical Neurology and Neurosurgery 97 (1995) 239-244

Fig. 1. Axial Tl-weighted image through skull base demonstrates gadolinium enhanced soft tissue in the clival epidural space (arrows). Dark line of posterior clival cortex appears disrupted, with soft tissue of intermediate signal replacing the bright signal of marrow fat in the clivus (curved arrow).

temporal arteritis was made. Two months later he was admitted to a local hospital because of gastrointestinal bleeding. During this hospitalization he reportedly developed a left abducens palsy and right tongue deviation. He was referred to Yale-New Haven hospital for further evaluation and management. The patient complained of severe pain at the base of his neck, face and shoulders. He had a history of increased blood pressure and mild obesity with an obscure history of collagen vascular disease. He also suffered from diabetes mellitus type II and peripheral vascular disease. He had an aorto-femoral bypass 2 years prior to admission. The patient's temperature was 38.2°C, pulse was 92, and blood pressure was 170/90. On physical examination, there was no scalp tenderness. His cheeks were indurated without rash. The neck was rigid and tender upon movement. Pain radiated to shoulders. The carotid pulses were ++ bilaterally, with mild bruits. No rash, petechiae or splinter hemorrhages were found. Chest and heart examination were unremarkable except for a grade 3 holosystolic murmur. On neurological examination the patient was awake, alert, and oriented. He had mild slurred speech with profound hearing loss (left worse than right). He had right ptosis, left sixth nerve palsy and left facial weakness. He also had right tongue deviation and a decreased gag reflex. Other cranial nerve functions were preserved. Upper extremities had normal tone and bulk. Strength

was grossly symmetric and normal, although testing was hampered by severe neck and shoulder pain. Lower extremities had normal tone and bulk, and strength was normal. Limited examination of cerebellar function was unremarkable. Reflexes were decreased but symmetrical. Plantar reflexes were flexor. Sensory examination was unremarkable. Laboratory studies, including hematologic and chemistry profiles, were within normal limits. Erythrocyte sedimentation rate was 132. Serological tests for syphilis, antinuclear antibodies, Lyme and HIV were negative. Multiple blood cultures were negative. A lumbar puncture revealed elevated CSF protein (153 mg/dl) and white blood cell count of 156/¢tl with 56% neutrophils. CSF glucose was 85 mg/dl. CSF cultures were negative. MR scan of the brain revealed enhancing soft tissue in the epidural space adjacent to the clivus (Fig. 1). Ceftazidime was started. Although the patient's fever resolved, there was no significant improvement of symptoms or neurological signs. Two weeks after treatment, repeat lumbar puncture revealed CSF protein of 88 mg/dl, and white cell count of 6/~tl with a lymphocytic predominance. Repeat CSF cultures were negative. MR imaging showed enhancing soft tissue in the epidural space behind the clivus, and in the prevertebral and retropharyngeal spaces anterior to the clivus (Figs. 2A-C). The enhancing tissue extended inferiorly within the prevertebral space to involve the vertebral bodies, disc space, and epidural space (Figs. 2 and 3). Irregular contour of the posterior clivus was seen on CT (Fig. 4). A bone scan demonstrated increased uptake in the midcervical area and base of the skull. A trans-nasopharyngeal needle biopsy was performed and revealed a whitish exudate; cultures were negative. A halo neck stabilizer was installed and antibiotics were continued. The patient received a total of 6 weeks of Ceftazidime and was discharged without pain on ciprofloxacine. On discharge his ptosis and facial weakness had improved. His arms were mobile and without pain. Oral ciprottoxacine was continued for six weeks. Upon return for a follow-up evaluation in the neurosurgical clinic he had a residual left sixth nerve palsy, but other cranial neuropathies had improved. A gallium scan revealed no white blood cells infiltration in the neck or base of the skull region. He underwent a cervical spinal fusion. Repeat biopsy and microbiological studies were unrevealing. Per recommendation of the infectious disease service he was continued on long term oral ciprofloxacine. Repeat MRI scan showed no new lesions. He had no apparent further clinical problems. Since then he has moved from this area.

3. Discussion

The patient presented above illustrates an unusual

S. Ausim Azizi et al./ Clinical Neurology and Neurosurgery 97 (1995) 239-244

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S. Ausim Azizi et al. / Clinical Neurology and Neurosurgery 97 (1995) 239-244

Fig. 3. Axial Tl-weighted image of the cervical spine showsenhancing tissue in the epidural space (arrow).

case of clivus osteomyelitis that extended from the clival epidural space into the prevertebral space of the cervical spine. Symptoms and signs included headache, neck and shoulder pain, left sixth nerve palsy, left facial weakness, right tongue deviation and right ptosis. These lesions encompass the Gradenigo's syndrome [13]. This syndrome, described in 1904 by Gradenigo, consists of sixth nerve palsy and ipsilateral facial sensory loss. Classically, it results from petrositis secondary to middle ear infection; however, other processes including neoplasms [14] may present with this syndrome, presumably due to the involvement of soft tissues investing the abducens and facial nerves. Abducens palsy has been reported as a common complication of clivus and/or paraclival pathology [15]. In the present case, epidural infection of the skull base (Fig. 1) involved the foramina through which cranial nerves travel, most likely accounting for the cranial neuropathies. Our search of the literature did not reveal any reported cases of spinal epidural abscess associated with otitis and paraclival infection. We believe that the pathological involvement of the present case included, in sequence, otitis, petrositis and contiguous spread of inflammation/infection to the clivus, preclivus, prevertebral space and subsequently to the cervical spine and spinal epidural space. This process was likely aided by the compromised status of the patient (steroid therapy and diabetes mellitus) and was probably facilitated by the anatomical relations of the deep fascial layers in the head and neck region. Three layers of deep cervical fascia - superficial, middle and deep - divide the head and neck regions into various spaces [16]. The prevertebral space is a corridor linking the skull base

with the posterior mediastinum and extends from the clival epidural space to the fourth thoracic vertebra. The innermost layer of the deep cervical fascia forms the boundaries of the prevertebral space, as described in the recent imaging literature [16-17]. The fascia is attached to the transverse processes of the cervical vertebrae, and posteriorly to the nuchal ligaments [17]. The transverse processes form the boundary between the anterior and posterior compartments of the prevertebral space. The retropharyngeal space, located anterior to the prevertebral space, is bordered anteriorly by the middle layer of the deep cervical facia, and laterally and posteriorly by the deep layer of the cervical fascia [17-18]. Infection can spread within the prevertebral and retropharyngeal spaces and, as seen in this case, can break through fascial boundaries to involve both spaces simultaneously. Imaging procedures for the clivus have been well described [1,19-21]. Differential diagnosis on the basis of the imaging studies alone included lymphoma, en plaque meningioma or an inflammatory/infectious lesion. Although chordomas constitute approximately 35% of clival lesions [1], in this case they are an unlikely candidate. Chordomas are neoplasms that arise from the remnants of the notochord with proclivity for either end of the spine [4-5]. Meningiomas usually present as mass lesions abutting the brain and causing focal neurological deficits [6]. Lymphomas appear as isointense masses [2-3] and/or infiltrates involving the brain and the extradural spaces. They generally cause increased intracranial pressure and mental status changes, although focal deficits and cranial nerve abnormalities have been reported [22].

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References

Fig. 4. CT with bone windows show irregularity of the posterior clival margin (arrows), consistent with osteomyelitis.

The infectious agent(s)was not identified, presumably because of the prolonged antibiotic treatment. The predominant organism causing skull base infections, associated with otitis, is Pseudomonas aeruginosa [11,23]. This may have been the infectious agent in the present case. There are a few reports o17fungal infections in sphenoid sinuses involving the clivus [9,24], usually afflicting immune compromised hosts. Case review studies of spinal epidural abscess [25-26] indicate that the leading bacterial etiologic agent is Staphylococcus aureus. According to one study 81% of spinal epidural abscess cases had an underlying predisposing condition such as diabetes, previous infection, alcoholism or trauma [26], although cases of spontaneous pyosenic spinal osteomyelitis without predisposing factors have been reported [27]. Greater efficacy and good long te~aa outcome with early surgical debridement and fusion in patients with vertebral osteomyelitis has been recently reported [28]. However, in the current case initial conservative therapy and long term antibiotic treatment resulted in a satisfactory and apparently stable outcome. Although this patient exhibited signs that are often seen in Gradenigo's syndrome, the pathology extended beyond the petrous bone to the clivus and cervical spine. This case illustrates the importance of recognizing infections of the clivus in patients with cranial nerve abnormalities.

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