| OCR Text |
Show journal of Neuw- Ophthalmology 16( 4): 264- 268, 1996. © 1996 Lippincott- Raven Publishers, Philadelphia Idiopathic Hypertrophic Cranial Pachymeningitis: Clinical- Radiological- Pathological Correlation of Bone Involvement Daniel M. Jacobson, M. D., Dennis R. Anderson, M. D., George M. Rupp, M. D., and John J. Warner, M. D. We present the clinical, radiological, and pathological findings in an elderly man who developed a progressive superior orbital fissure syndrome due to idiopathic hypertrophic cranial pachymeningitis. The unique aspect of this case concerned the increased density of the sphenoid ridge and lateral orbital wall observed by using computed tomography, and the enhancement of the marrow signal seen on magnetic resonance imaging. These neuroimaging abnormalities of bone resulted from an indirect nonspecific response of the marrow to the adjacent soft tissue and dural inflammatory process. Key Words: Pachymeningitis- Ophthalmoplegia- Optic neuropathy- Meningioma- Sphenoid. Idiopathic hypertrophic cranial pachymeningitis is an unusual chronic inflammatory disorder of unknown etiology that causes deficits by mass effect and infiltration of neurologic structures adjacent to the involved dura ( 1). We present the following case report to emphasize a unique aspect of this disorder that concerned bone involvement identified by using computed tomography ( CT) and magnetic resonance imaging ( MRI). The pathological correlate of these neuroimaging abnormalities of bone was a nonspecific reactive change of the marrow in response to the adjacent inflammatory process affecting the soft tissues and dura. To the best of our knowledge, this case represents the first pathological report demonstrating bone involvement in idiopathic hypertrophic cranial pachymeningitis. CASE REPORT Manuscript received lune 15, 1995; accepted December 18, 1995. From the Departments of Neurology ( D. M. J.), Ophthalmology ( D. M. J., D. R. A.), Pathology ( G. M. R.), and Radiology ( J. J. W.), Marshfield Clinic, Marshfield, Wisconsin, U. S. A. Address correspondence and reprint requests to Dr. D. M. Jacobson, Marshfield Clinic, 1000 North Oak Avenue, Marshfield, WI 54449, U. S. A. This work was presented at the 26th Annual Frank Walsh Meeting in Chicago, April 9- 10, 1994. A 78- year- old man was referred for evaluation of a recently discovered right- sided retro- orbital mass lesion. Ten months earlier, he began experiencing progressive vertical diplopia. A dull headache developed above and behind his right eye 3 months before referral. He then noted progressive loss of vision of his right eye. Orbital CT revealed an enhancing soft tissue mass lesion extending from the right superior orbital fissure into the orbital apex, the inferior orbital fissure and pterygopalatine fossa, the anterior cavernous sinus, and along the greater wing of the sphenoid bone ( Fig. 1). Increased density of the lateral wall of the orbit and greater wing of the sphenoid bone were also noted ( Fig- 2). 264 HYPERTROPHIC CRANIAL PACHYMENINGITIS 265 FIG. 1. Computerized tomography in the axial plane revealing a right- sided enhancing soft tissue mass extending through the superior orbital fissure into the orbital apex ( short black arrow) and into the middle cranial fossa along the greater wing of the sphenoid bone ( long white arrow). His visual acuity was 20/ 25- 2 with marked dys-chromatopsia in his right eye and 20/ 15 in his left eye. Goldmann perimetry revealed a generalized central depression and an inferior arcuate defect in FIG. 2. Computerized tomography at a level similar to that depicted in Fig. 1, with window settings optimized for bone detail, revealing increased density of the greater wing of the sphenoid bone and lateral wall of the orbit on the right side ( white arrow). Note the loss of anatomical distinction between the cortex and diploic space. his right eye. The right optic disc appeared slightly full and hyperemic. There was moderate right-sided ptosis and impairment of levator function, but without evidence of aberrant regeneration of the affected eyelid. Horizontal ductions and depression of the right eye were restricted. He had 4 mm of relative axial proptosis of his right globe. His corneal reflex and sensitivity were reduced on the right. MRI revealed that the mass was isointense relative to brain on Tl- weighted sequences ( Fig. 3), and enhanced uniformly following administration of gadolinium ( Fig. 4). The meninges of the middle cranial fossa and along the greater wing of the sphenoid bone were thick and demonstrated enhancement ( Fig. 4). The marrow signal intensity of the sphenoid ridge, normally bright on Tl-weighted images, was diminished on the right side ( Fig. 3) and also demonstrated enhancement ( Fig. 4). His medical evaluation included a normal chest radiograph, an erythrocyte sedimentation rate ( Westergren) of 47 mm/ h, and normal results of Venereal Disease Research Laboratory and fluorescent treponemal antibody tests, angiotensin-converting enzyme assay, antinuclear and neutrophil- cytoplasmic antibody assays, rheumatoid factor assay, and serum protein electrophoresis analysis. Skin testing revealed no reaction against FIG. 3. Precontrast T1- weighted ( TR 500 ms, TE 15 ms) magnetic resonance image in the axial plane revealing diminished signal intensity of the sphenoid bone marrow on the right side ( thin white arrow), reflecting marrow replacement. Note the normal-appearing bright marrow signal intensity on the left side ( thick black arrow). / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 266 D. M. ] ACOBSON ET AL. FIG. 4. Postcontrast T1- weighted ( TR 500 ms, TE 15 ms) magnetic resonance image at a similar level as depicted in Fig. 3, demonstrating enhancement of the marrow space on the right side ( long black arrow). Note also the dural thickening and enhancement ( long white arrow), and extension of the soft tissue enhancing lesion into the orbit and the temporal fossa ( short black arrows). purified protein derivative, but positive reactivity against two control antigens. His cerebrospinal fluid contained three white cells/ ml3, protein 59 mg/ dl ( normal, 15- 45 mg/ dl), and negative Venereal Disease Research Laboratory and fluorescent treponemal antibody tests, cytology, cryptococcal antigen assay, and stains and cultures for bacteria, fungi, and tuberculosis. He underwent a frontotemporal craniotomy and transcranial orbitotomy. The dura at the base of the skull was thick and indurated. The anterior / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 clinoid and sphenoid wing appeared abnormally dense. The orbital apex and optic canal were decompressed. Bone and dura were submitted for pathological examination. The dura was thick, fi-brotic, and revealed pachymeningitis with a lym-phoplasmacytic infiltrate, most marked on the parenchymal surface ( Fig. 5). Meningothelial hyperplasia was seen, but without features of a meningioma. The bone was irregular and the marrow spaces were replaced by fibrous tissue ( Figs. 6 and 7). No direct infiltration of the inflammatory process between the dura and bone was observed. There were no granulomas or areas of vasculitis. Special stains for spirochetes and fungi were negative. Immunostains showed that the inflammatory population was polyclonal. He was initially treated with azathioprine and intravenous pulse methylprednisolone followed by oral prednisone 60 mg daily. Azathioprine was discontinued within a few weeks due to a severe allergic reaction. He has been maintained on slowly tapering doses of oral prednisone alone for the past 24 months. His clinical findings improved to a moderate degree during the first few months of treatment and have since remained stable. MRI, repeated 6 and 18 months after the initiation of corticosteroids, demonstrated marked improvement in size and degree of enhancement of the lesion. DISCUSSION Hypertrophic cranial pachymeningitis can be associated with a variety of infectious and systemic inflammatory disorders, including syphilis ( 2), tuberculosis ( 3), rheumatoid arthritis ( 4), sarcoidosis FIG. 5. Photomicrograph revealing fibrotically thickened dura, with a chronic inflammatory infiltrate located primarily on the parenchymal aspect of the dura ( black arrow). H & E, original magnification x25. HYPERTROPHIC CRANIAL PACHYMENINGITIS 267 FIG. 6. Photomicrograph showing irregularly thickened bony trabeculae with marrow replacement by fibrous tissue. H & E, original magnification x25. ( 5), and Wegener's granulomatosis ( 6). Hypertrophic cranial pachymeningitis can also occur as an idiopathic disorder, either restricted to the intracranial compartment or as part of a systemic disorder associated with fibrosclerosis at multiple sites. The idiopathic localized presentation can develop as an extension of other sclerosing disorders affecting the skull base, including idiopathic sclerosing inflammation of the orbit ( 7,8) and some forms of the Tolosa- Hunt syndrome ( 9- 11). The systemic disorder, often referred to as multifocal fibrosclerosis, encompasses some combination of idiopathic hypertrophic cranial pachymeningitis, idiopathic sclerosing orbital inflammation, retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, and Riedel's thyroiditis ( 12,13). The common denominator relating all of these presentations is a similar constellation of histopathologi-cal abnormalities, including desmoplasia and lym-phoplasmacytic infiltration, as well as a similar im-munohistochemical profile ( 14). Many recently reported cases of idiopathic hypertrophic cranial pachymeningitis have emphasized visual loss and ophthalmoplegia due to predominant mass involvement of the parasellar and superior orbital fissure regions, similar to the case described herein ( 9- 11,15,16). However, during our review of the medical literature, assisted by a computerized database ( Medline, National Library of Medicine), we encountered only one report of FIG. 7. Higher- magnification photomicrograph demonstrating fibrous tissue between irregularly thickened bony trabeculae. H & E, original magnification x100. / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 268 D. M. JACOBSON ET AL. possible bone involvement in this disorder ( 17). A 50- year- old man with chronic renal failure undergoing hemodialysis developed unexplained progressive visual loss, other cranial nerve deficits, and then a fatal intraparenchymal hematoma. Postmortem examination revealed diffusely thickened dura at the base of the skull consisting of granulomas, fibrosis, and chronic inflammatory changes. Gross erosion of the petrous portion of the temporal bone was noted, but without histo-pathological explanation ( 17). In the present case, the increased bone density seen on CT, and bone marrow signal changes observed using MRI, were the indirect result of a nonspecific bone reaction induced by the adjacent dural inflammatory process. Bony destruction or direct lymphoplasmacytic infiltration was not observed. REFERENCES 1. Masson C, Henin D, Hauw JJ, Rey A, Raverdy P, Masson M. Cranial pachymeningitis of unknown origin: a study of seven cases. Neurology 1993; 43: 1329- 34. 2. Michel D, Girard PF, Tommasi M, Masson R, Trillet M, Piccinali JP. Les pachymeningitis granulomateuses intra-craniennes a symptomatologie pseudo- tumorale: a propos de 4 observations. / Med Lyon 1969; 50: 545- 78. 3. Callebaut J, Dormont D, Dubois B, Chiras J, Bories J. Contrast- enhanced MR imaging of tuberculous pachymeningitis cranialis hypertrophica: case report. AJNR 1990; 11: 821- 2. 4. Yuh WTC, Drew JM, Rizzo M, Ryals TJ, Sato Y, Bell WE. Evaluation of pachymeningitis by contrast- enhanced MR imaging in a patient with rheumatoid disease. AJNR 1990; 11: 1247- 8. 5. Osenbach RK, Blumenkopf B, Ramirez H, Gutierrez J. Meningeal neurosarcoidosis mimicking convexity en-plaque meningioma. Surg Neurol 1986; 26: 387- 90. 6. Newman NJ, Slamovits TL, Friedland S, Wilson WB. Neuro- ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener's granulomatosis. Am J Ophthalmol 1995; 120: 613- 21. 7. Frohman LP, Kupersmith MJ, Lang J, et al. Intracranial extension and bone destruction in orbital pseudotumor. Arch Ophthalmol 1986; 104: 380- 4. 8. Noble SC, Chandler WF, Lloyd RV. Intracranial extension of orbital pseudotumor: a case report. Neurosurgery 1986; 18: 798- 801. 9. Hamilton SR, Smith CH, Lessell S. Idiopathic hypertrophic cranial pachymeningitis. / Clin Neuro Ophthalmol 1993; 13: 127- 34. 10. Mamelak AN, Kelly WM, Davis RL, Rosenblum ML. Idiopathic hypertrophic cranial pachymeningitis: report of three cases. / Neurosurg 1993; 79: 270- 6. 11. Olmos PR, Falko JM, Rea GL, Boesel CP, Chakeres DW, McGhee DB. Fibrosing pseudotumor of the sella and para-sellar area producing hypopituitarism and multiple cranial nerve palsies. Neurosurgery 1993; 32: 1015- 21. 12. Berger JR, Snodgrass S, Glaser J, Post MJD, Norenberg M, Benedetto P. Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis. Neurology 1989; 39: 1345- 9. 13. Levine MR, Kaye L, Mair S, Bates J. Multifocal fibrosclerosis: report of a case of bilateral idiopathic sclerosing pseudotumor and retroperitoneal fibrosis. Arch Ophthalmol 1993; 111: 841- 3. 14. McCarthy JM, White VA, Harris G, Simons KB, Kennerdell J, Rootman J. Idiopathic sclerosing inflammation of the orbit: immunohistologic analysis and comparison with retroperitoneal fibrosis. Mod Pathol 1993; 6: 581- 7. 15. Lam BL, Barrett DA, Glaser JS, Schatz NJ, Brown HH. Visual loss from idiopathic intracranial pachymeningitis. Neurology 1994; 44: 694- 8. 16. Willing SJ, Broghamer W. Internal carotid artery occlusion due to idiopathic cranial pachymeningitis. AJNR 1992; 13: 1594- 6. 17. Feringa ER, Weatherbee L. Hypertrophic granulomatous cranial pachymeningitis causing progressive blindness in a chronic dialysis patient. / Neurol Neurosurg Psychiatry 1975; 38: 1170- 6. / Neuro- Ophthnlmol, Vol. 16, No. 4, 1996 |
