56
Intradural manifestation ofrheumatoid arthritis causing spinal cord compression E. Kraus . H. Klinge. M. Rautenberg
Summary Hheumatoid arthritis as a ca use of rnedu lla ry compression du e to subluxation of rh eum atically diseas ed joints is very cornmon. However, spin al cord com press ion by rheumatoid nodu les is seen rarely, usu ally by extra dura l lesions. We describe two cases of intr adural rheumatoid nodules causing spinal cord compression . Intradu ra le rheuma toide Arthritis a ls Ursac he einer Rückenmarkskompression Die rh eum atoide Arth ritis als Ursac he einer Hückenm arkskompression auf dem Boden einer Subluxation rh eumatisch verä ndert er Wirbelgelenke ist ein sehr geläufiges Krankheitsbild. Selten hin gegen ist eine Komp ression des Rückenmarks durch Rheum atoidknoten . die in der Regel extradural gelegen sind. Wir berichten über zwei Fälle eine r Rückenm a rkskomp ression du rch rein intradural gelegene Rhe umat oidknot en .
rh eumatoid nod ules. At the first sta y a t our hospit al. she was admi t· ted with a spasti c parap aresis with se nory deficit below th e level of T12. Xcrays of the spine showed osteo porosis . Myelography revealed incomplete obstruction at the level of T6 . We found an obstructive-type fluid containing 7/ 3 cells and protein content of 250mg%. Operat ion: Lam inectomy was performed of T6 and T7. Opening the dura revealed a solid tumou r adhere nt to the du ra at the left slde at the level of T6. The tumour was removed totally. J1istological examinatlon report ed a gra nulomato us nodule with lymphocytic. plasmacell and gra nulocyt ic inflamm ation and epitheloid cells. Postoperative course. The se nsory and motor funcuon improved and the patient was a ble to walk . Thr ee yea rs later she was admitted to hospital agatn with pa rae sthesia of both ban ds and progressive wea knes s of th e right arm and leg. Neurological cxamination revealed accentuated reflexes on th e right side and a sensory loss in th e right C4 der matome as weil as a weak ness of the right arm a nd leg. X-ray exami nation of the cervical spine showed marked decalcification an d degenerative bony rea ction s. Myelography showed a block at the level of C3 a nd a n obstructive-type fluid (2/3 cells, 300 mg% protein). Operati on: After laminectomy of C3 and ( 4 again an ad herent intrad ural rheu matoid nod ule was found (Abb. 1). The tumou r which had compressed th e sp ina l cord cwas removed completely. Histological examination again revealed a granulomatous rhe uma toid nodule.
Key-Wor ds Rheum at oid arthritis - Spin al cord compress ion
Spinal manifestati on of rhe uma toid a rthritis is well known commonly causing clinical signs by cornpression of peripheral nerves and by medullary compression due to subluxa tion of rh eumati cally diseased vertebral joints. Spina l cord compression due to extra dura l rh eumat oid nodules is very rare. \Ve report two cas es of direct intradural compression oft he spinal cord by rheumatic tissue. Case l A 67 -year-old female with a history of 15 yea rs of progressive rheumati c pa in was operated on twice within thr ee years because of sp ina l cord compression ca used by intradu ral
Neurochirurgia 33 (1990) 56- 57 (Supplement I)
Abb. 1 Cervical intraduralrheumatoidnodulein
© Gcorg Thieme Verlag Stuttgart · New York
a Zü-year-cld female [CaseI)
Downloaded by: Universite Laval. Copyrighted material.
Neurochiru rgisc he Universitätsklinik Kiell Direktor : Prof. Dr. H.-P. Jensenl
Nel1rochirurgia 33 (1990)
Intradural manifestation ofrheumatoid arthritis
Refer ences
Case 11
Discussion The two morphological features of rheuma toid disease, the Gran uloma of Aschoff and the necrosis, can be found in joints and many other organ s. Rheumatoid disease can affect hea rt muscle, pleura , peritoneum, spleen, lung, skin and per ipheral ner ves. The centra l nervaus system is rarely involved except in patients with medullary compression due to vertebral sub luxation. Rheumatoid nodules have been found in the cranial dura mater and the spinal subarachnoid space (Steiner and Gelblootn [5J, Maher [3]). Friedrnan (2) in 1970 reported a case with cornpression ofthe cauda equina and the nerve roots L4to 52 by a rheumatoid nodu le extending from the extradural to the extra verteb ra l space. Lindquist and Me Donnell (4) in 1970 described a large rheumatoid cyst which extended from the extradural spa ce to the sub cutaneous fat and was causing compression ofthe cauda equina (roots L3 to L5). Fairburn (1) in 1975 published a case with spinal cord compression in T4 by a rhe umatoi d nodule which was purely extradurally localized. The few cases reported show that rheumatoid lesions - although clearly defined pathologically - are very variable in size, localisation and consistency. Neurological signs can also be very variab le. In our two cases intradural rheu mato id nodu les caus ed spinal cord compr ession - in Ihe first case at the level of T6 and thre e years later at the level of C3, in our second case the tum our was localized at the level ofT4 . As the lesions in our cases had been clearly within the intradural spac e we should like to suggest that rheuma toid inflammation could have invaded the dura whilst penetrating from its supposed origin in the vertebral joints .
1 2 3
Fair bum. 8.: Spinal cord compression by a rheumatoid nodule. J.
Neurol. Neurosurg. Psychiatry 38 (1975) 1056-1058 Friedman, 11.: Intraspinal rheumatoid nodule causing nerve root eompression. J. Neurosurg. 32 (1970) 689 -69 1 M atter , J. A. : Dural nodules in rheumatoid arthritis. Archs. Path. 58 119541354 - 359
~ Lindc utst. P. H.. D. E. M cDonnell:
Rheumatoid eyst eausing extradural eompression. Journal of Bone and Joint Surgery 52A
5
1197011235 - 1240 S t einer, J. W.. A. J. Gelb/oom: Intracranial manifestations in two
cases of systemic rheumatoid disease. Arthritis Hheum. 2 (1959) 537- 545
DA Dr. H. Kraus Neurochirurgische Universitätsklinik Weimarer Str. 8 0 -2300 Kiel Downloaded by: Universite Laval. Copyrighted material.
A 77-year-old ma le had sufTere d from r heu matoid art hritis sinee 16 yea rs. For six months there had been a pr ogressive weakness of the legs . He was ad mitted to hos pital beca use of pain in the mid-th or acic s pine .l\'euro logical examination revealed a distally and left-accentua ted spastic par a paresis a nd a sensory deficit below the level of T7. There was ur ina ry incont inence. Plain X-ray of th e thoracic s pine was norm al. Myelography revealed obstruc tion of contrast medium at the level of T617. The CSF pr otein level wa s 57 1 mg%. Nuclea r Magnetic Resonance showed a n int ras pinal tumo ur at the lcvel of T617. Operat ion: After lam inectomy a ven tr ally locat ed intradural so lid tumo ur which had caused marked spinal eompressi on could be rem oved totally. Histological cxaminat ion showed fibro tic granulom at ous tissu e wh ich was conflrmed as a rh cumatoid nodu le by different tech niques Including filament and immunohistochemical techn iques for leucocytie mar kers. Postop era tive course : Th e patient was pain -free and sens ory and motor function improved.
57
Intradural manifestation ofrheumatoid arthritis causing spinal cord compression E. Kraus . H. Klinge. M. Rautenberg
Summary Hheumatoid arthritis as a ca use of rnedu lla ry compression du e to subluxation of rh eum atically diseas ed joints is very cornmon. However, spin al cord com press ion by rheumatoid nodu les is seen rarely, usu ally by extra dura l lesions. We describe two cases of intr adural rheumatoid nodules causing spinal cord compression . Intradu ra le rheuma toide Arthritis a ls Ursac he einer Rückenmarkskompression Die rh eum atoide Arth ritis als Ursac he einer Hückenm arkskompression auf dem Boden einer Subluxation rh eumatisch verä ndert er Wirbelgelenke ist ein sehr geläufiges Krankheitsbild. Selten hin gegen ist eine Komp ression des Rückenmarks durch Rheum atoidknoten . die in der Regel extradural gelegen sind. Wir berichten über zwei Fälle eine r Rückenm a rkskomp ression du rch rein intradural gelegene Rhe umat oidknot en .
rh eumatoid nod ules. At the first sta y a t our hospit al. she was admi t· ted with a spasti c parap aresis with se nory deficit below th e level of T12. Xcrays of the spine showed osteo porosis . Myelography revealed incomplete obstruction at the level of T6 . We found an obstructive-type fluid containing 7/ 3 cells and protein content of 250mg%. Operat ion: Lam inectomy was performed of T6 and T7. Opening the dura revealed a solid tumou r adhere nt to the du ra at the left slde at the level of T6. The tumour was removed totally. J1istological examinatlon report ed a gra nulomato us nodule with lymphocytic. plasmacell and gra nulocyt ic inflamm ation and epitheloid cells. Postoperative course. The se nsory and motor funcuon improved and the patient was a ble to walk . Thr ee yea rs later she was admitted to hospital agatn with pa rae sthesia of both ban ds and progressive wea knes s of th e right arm and leg. Neurological cxamination revealed accentuated reflexes on th e right side and a sensory loss in th e right C4 der matome as weil as a weak ness of the right arm a nd leg. X-ray exami nation of the cervical spine showed marked decalcification an d degenerative bony rea ction s. Myelography showed a block at the level of C3 a nd a n obstructive-type fluid (2/3 cells, 300 mg% protein). Operati on: After laminectomy of C3 and ( 4 again an ad herent intrad ural rheu matoid nod ule was found (Abb. 1). The tumou r which had compressed th e sp ina l cord cwas removed completely. Histological examination again revealed a granulomatous rhe uma toid nodule.
Key-Wor ds Rheum at oid arthritis - Spin al cord compress ion
Spinal manifestati on of rhe uma toid a rthritis is well known commonly causing clinical signs by cornpression of peripheral nerves and by medullary compression due to subluxa tion of rh eumati cally diseased vertebral joints. Spina l cord compression due to extra dura l rh eumat oid nodules is very rare. \Ve report two cas es of direct intradural compression oft he spinal cord by rheumatic tissue. Case l A 67 -year-old female with a history of 15 yea rs of progressive rheumati c pa in was operated on twice within thr ee years because of sp ina l cord compression ca used by intradu ral
Neurochirurgia 33 (1990) 56- 57 (Supplement I)
Abb. 1 Cervical intraduralrheumatoidnodulein
© Gcorg Thieme Verlag Stuttgart · New York
a Zü-year-cld female [CaseI)
Downloaded by: Universite Laval. Copyrighted material.
Neurochiru rgisc he Universitätsklinik Kiell Direktor : Prof. Dr. H.-P. Jensenl
Nel1rochirurgia 33 (1990)
Intradural manifestation ofrheumatoid arthritis
Refer ences
Case 11
Discussion The two morphological features of rheuma toid disease, the Gran uloma of Aschoff and the necrosis, can be found in joints and many other organ s. Rheumatoid disease can affect hea rt muscle, pleura , peritoneum, spleen, lung, skin and per ipheral ner ves. The centra l nervaus system is rarely involved except in patients with medullary compression due to vertebral sub luxation. Rheumatoid nodules have been found in the cranial dura mater and the spinal subarachnoid space (Steiner and Gelblootn [5J, Maher [3]). Friedrnan (2) in 1970 reported a case with cornpression ofthe cauda equina and the nerve roots L4to 52 by a rheumatoid nodu le extending from the extradural to the extra verteb ra l space. Lindquist and Me Donnell (4) in 1970 described a large rheumatoid cyst which extended from the extradural spa ce to the sub cutaneous fat and was causing compression ofthe cauda equina (roots L3 to L5). Fairburn (1) in 1975 published a case with spinal cord compression in T4 by a rhe umatoi d nodule which was purely extradurally localized. The few cases reported show that rheumatoid lesions - although clearly defined pathologically - are very variable in size, localisation and consistency. Neurological signs can also be very variab le. In our two cases intradural rheu mato id nodu les caus ed spinal cord compr ession - in Ihe first case at the level of T6 and thre e years later at the level of C3, in our second case the tum our was localized at the level ofT4 . As the lesions in our cases had been clearly within the intradural spac e we should like to suggest that rheuma toid inflammation could have invaded the dura whilst penetrating from its supposed origin in the vertebral joints .
1 2 3
Fair bum. 8.: Spinal cord compression by a rheumatoid nodule. J.
Neurol. Neurosurg. Psychiatry 38 (1975) 1056-1058 Friedman, 11.: Intraspinal rheumatoid nodule causing nerve root eompression. J. Neurosurg. 32 (1970) 689 -69 1 M atter , J. A. : Dural nodules in rheumatoid arthritis. Archs. Path. 58 119541354 - 359
~ Lindc utst. P. H.. D. E. M cDonnell:
Rheumatoid eyst eausing extradural eompression. Journal of Bone and Joint Surgery 52A
5
1197011235 - 1240 S t einer, J. W.. A. J. Gelb/oom: Intracranial manifestations in two
cases of systemic rheumatoid disease. Arthritis Hheum. 2 (1959) 537- 545
DA Dr. H. Kraus Neurochirurgische Universitätsklinik Weimarer Str. 8 0 -2300 Kiel Downloaded by: Universite Laval. Copyrighted material.
A 77-year-old ma le had sufTere d from r heu matoid art hritis sinee 16 yea rs. For six months there had been a pr ogressive weakness of the legs . He was ad mitted to hos pital beca use of pain in the mid-th or acic s pine .l\'euro logical examination revealed a distally and left-accentua ted spastic par a paresis a nd a sensory deficit below the level of T7. There was ur ina ry incont inence. Plain X-ray of th e thoracic s pine was norm al. Myelography revealed obstruc tion of contrast medium at the level of T617. The CSF pr otein level wa s 57 1 mg%. Nuclea r Magnetic Resonance showed a n int ras pinal tumo ur at the lcvel of T617. Operat ion: After lam inectomy a ven tr ally locat ed intradural so lid tumo ur which had caused marked spinal eompressi on could be rem oved totally. Histological cxaminat ion showed fibro tic granulom at ous tissu e wh ich was conflrmed as a rh cumatoid nodu le by different tech niques Including filament and immunohistochemical techn iques for leucocytie mar kers. Postop era tive course : Th e patient was pain -free and sens ory and motor function improved.
57
