J Neurosurg 47:109-112, 1977
Sarcoid of the cauda equina Case report JAMES N . CAMPBELL,M . D . , PERRY BLACK,M . D . , C . M . , AND PETER T. OSTROW, M . D . , PH.D.
Departments of Neurological Surgery and Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland An unusual case of sarcoid involving the cauda equina, paraparesis, is presented. There was no evidence of sarcoid nervous system, and the diagnosis was established through tion. The patient was treated with steroids postoperatively, covery of neurological function. KEY WORDS 9 arachnoiditis 9 cauda equina prednisone 9 sarcoidosis 9 spinal cord
LINICALLY apparent involvement of the nervous system is estimated to occur in a b o u t 5% of all patients with sarcoid? ,s,xS,x4 W h e n sarcoid affects the nervous system, the affected tissues are usually the meninges at the base of the brain and the cranial nerves, while the spinal cord is only rarely involved. In the infrequently reported cases with cord involvement, the presumptive diagnosis has generally been based on clinical g r o u n d s or p r o v e n at a u t o p sy. 1,4-7,9-1x,~ In a few instances, however, the diagnosis was confirmed during life through exploratory laminectomy. T u m o r - l i k e masses compressing the spinal cord were seen in three cases at cervical laminectomy, 6,n,15 and in one case at thoracic laminectomy. H Histological examination in each case revealed a g r a n u l o m a t o u s lesion without necrosis, surr o u n d e d by round-cell infiltration, epithelioid cells, and giant cells. Operatively documented sarcoid of the cauda equina has been described in two
c
J. Neurosurg. / Volume 47 / July, 1977
resulting in progressive involvement outside the thoracolumbar exploraand had a moderate re-
9 paraplegia
9
cases; ~4ae in one of these cases the lesion was believed to be confined to the nervous system. Owing to the rarity of this condition and the difficulty of its preoperative diagnosis, we report here a case of sarcoid affecting the nerve roots of the cauda equina which was diagnosed through l u m b a r exploration. Case Report
This 40-year-old m a n was admitted to The Johns H o p k i n s Hospital because of progressive inability to walk, which began 18 months before admission. History. At the age of 20 years, he had h a d several episodes of vertigo and the development o f deafness over a 2-month period. At 38 years of age he developed numbness of the left foot which spread to involve the left leg. This was associated with gradually increasing weakness of both lower extremities and bladder incontinence. H e was first evaluated at another hospital where a right brachial "109
J. N. C a m p b e l l , P. B l a c k a n d P. T. O s t r o w
FIG. 1. Supine radiograph (anteroposterior view) of the thoracolumbar region from the myelogram showing possible arachnoid adhesions. The L-3 marker is lateral to the T-12 vertebral body.
arteriogram and isotope brain scan were normal. Family history revealed that his mother had died of tuberculosis when the patient was 5 years old. Examination. On admission, the general physical examination, routine laboratory studies, including x-ray films of the chest, skull, and cervical, thoracic, and lumbosacral spine, were normal. The cranial nerves were normal with the exception of bilateral total deafness, confirmed by formal audiometry. The oculovestibular reflex was bilaterally absent. Optokinetic nystagmus was normal bilaterally. Slit-lamp examination of the optic fundi revealed no abnormalities. The. patient was unable to stand because of lower extremity weakness which was greater proximally than distally. The iliopsoas, glutei, hamstring, and quadriceps 1 ]0
muscles were 2/5, and the tibialis and gastrocnemius muscles were 4/5 in strength. There was moderate atrophy of the calves and thighs. The knee and ankle reflexes were absent and the plantar responses were flexor. There was hypalgesia and hypesthesia in a stocking distribution in the lower extremities to the mid-thigh level. Vibratory and position sense in both feet were moderately reduced. Nerve conduction velocities in all four extremities were normal. Electromyography revealed denervation and fasciculation potentials in both quadriceps muscles. A cystometrogram revealed a hypotonic neurogenic bladder. A pan-myelogram showed no gross abnormality; an additional volume of Pantopaque was therefore added and supine films were obtained in order to attempt to exclude such lesions as an arteriovenous malformation of the cord. In view of the patient's deafness, the Pantopaque was also carried up to the cerebellopontine cisterns which revealed no evidence of a mass lesion in this location. Detailed review of the myelogram films suggested equivocal arachnoidal adhesions ("matting") of the nerve roots of the cauda equina (Fig. 1). A pneumoencephalogram showed mild symmetrical ventricular dilatation and poor entry of air over the convexity of the brain. A cisternal isotope scan demonstrated ventricular stasis with little or no passage of isotope over the convexity after 48 hours. Several lumbar punctures revealed clear cerebrospinal fluid (CSF) with pressures always below 150 mm H,O. The protein content ranged from 224 to 330 mg%. Protein electrophoresis indicated an elevated globulin fraction of 16% (normal 4.3% to 12.3% of total CSF protein). Cell count ranged from 14 to 46 white blood cells (WBC), all mononuclear. Cytopathological study of the cells revealed no evidence of neoplasia. The CSF glucose content was normal (55 mg%). Cultures of the CSF for bacteria, fungi, and acidfast bacilli were negative. India ink preparations on fresh CSF samples were negative also. The CSF and serum venereal disease (VDRL) assays were negative. A serum protein electrophoresis was normal. Anti-nuclear antibody titers were normal, and multiple lupus erythematosus (LE) preparations were negative. The peripheral WBC count and differential were normal, and the erythrocyte J. Neurosurg. / Volume 47 / July, 1977
Sarcoid of the cauda equina sedimentation rate ranged from 31 to 41 ram/hr. In view of the progressive deterioration of the patient's neurological function, and the clinical evidence suggesting a lesion of the cauda equina, we elected to proceed with a thoracolumbar exploration. Operation. A complete laminectomy was carried out from T-10 to L-2. On opening the dura, gross examination revealed only delicate fibrous adhesions of the nerve roots of the cauda equina. When the operative microscope was used, however, irregular bluishpink nodules, 2 to 3 mm in diameter, could be seen embedded in continuity with many of the nerve roots. Samples of several of these nodules were dissected from the nerve fascicles and submitted for histological examination. Histological Examination. The nodules consisted of sparsely cellular granulomas comprising lymphocytes, histiocytes, and fibroblasts in a dense collagenous matrix (Fig. 2). No areas of necrosis were seen. SpeFIG. 2. Photomicrograph of noncaseating cial stains for acid-fast bacilli and fungi were negative. The morphology was interpreted as granuloma attached to nerve root of the cauda equina. H & E, X 330. most consistent with sarcoid. Postoperative Course. Skin testing revealed a positive Kveim reaction, negative intermediate strength purified protein derivament has been sustained. Steroid therapy will tive (PPD), and positive Candida test. Liver be reinstituted if his neurological function biopsy revealed no granulomata. The patient deteriorates. was placed on prednisone, 40 mg/day, and by the seventh postoperative day there was imDiscussion proved strength in the lower extremities. When systemic manifestations of sarSteroid therapy was stopped on the 10th postoperative day because of a staphylococcal coidosis are present, the finding of CSF pleowound infection, which cleared with drain- cytosis, and elevated CSF protein with norage and antibiotic therapy. After a course of mal glucose should ordinarily suggest central physical therapy, the patient was discharged, nervous system (CNS) sarcoidosis. In this able to walk with assistance, and with im- case, however, there was no evidence for sarproved bladder function. coid involvement outside the CNS, and the These improvements regressed over the diagnosis could be established only through ensuing 6 months while the patient was off lumbar exploration. The positive Kveim reacsteroid therapy. At this time, with the wound tion and histological findings obtained from well healed, prednisone (40 mg/day) therapy biopsy of lesions in the cauda equina prowas reinstituted and continued for 6 months. vided the basis for the diagnosis of sarAt the end of this course of therapy the pa- coidosis. Basilar meningitis with multiple cranial tient could walk up and down stairs without assistance, and could void without nerve deficits is one of the most common catheterization. The CSF protein at this time forms of CNS involvement with sarcoid. In a was 79 rag% and'there were 3 mononuclear series of 26 patients with neurological deficits cells per ml. Steroid therapy was then from sarcoid reported by Wiederholt and Siegradually discontinued, and 18 months since kert, TM all had cranial nerve signs and eight the time of surgery the neurological improve- patients had decreased hearing, which was J. Neurosurg. / Volume 47 / July, 1977
11 l
J. N. Campbell, P. Black and P. T. Ostrow bilateral in all cases. That our patient could have had bilateral nerve deafness from sarcoid 20 years before any other manifestation of this disease would be unusual. This possibility is supported, however, by the finding of hydrocephalus on pneumoencephalography with little entry of air over the convexity of the brain, suggesting that the patient may well have had an episode of basilar meningitis some time in the past. The operative findings in two previously r e p o r t e d cases of c a u d a equina sarcoidosis 14,18 included increased dural vascularity, and dense adhesions of the nerve roots with grayish arachnoid tissue. In the present case, the lesions were more subtle, consisting of bluish-pink nodules in continuity with the nerve roots; the arachnoid was slightly thicker than normal. The neurological deficit presumably resulted from the inflammatory lesions within the nerve roots themselves. The operative microscope proved to be of inestimable value in this case, since the minute lesions imbedded in the nerve roots might well have been overlooked without magnification. The value of establishing a diagnosis in such cases is obvious in view of potential favorable response to steroid therapy?,2.6ao,,~,xe
5.
6. 7. 8.
9.
10. 11. 12. 13. 14.
References 1. Askanazy CL: Sarcoidosis of the central nervous system. J Neuropathoi Exp Neurol 11:392-400, 1952 2. Banerjee T, Hunt WE" Spinal cord sarcoidosis. Case report. J Neurosurg 36:490-493, 1972 3. Douglas AC, Malony AF: Sarcoidosis of the central nervous system. J Neurol Neurosurg Psychiatry 36:1024-1033, 1973 4. de Morsier G, Maurice P, Martin F: BesnierBoeck diffus des muscles et 16si0ns du syst~me nerveux central (deux observations anatomo-
] ]2
15. 16.
cliniques). Acta Neuroi Psychiatr Belg 54:34-51, 1954 Ericksen TC, Odom G, Stern K: Boeck's disease (sarcoid) of the central nervous system. Report of a case, with complete clinical and pathologic study. Arch Neurol Psychiatry 48:613-621, 1942 Jefferson M: Sarcoidosis of the nervous system. Brain 80:540-556, 1957 Kirks DR, Newton TH: Sarcoidosis: a rare cause of spinal cord widening. Radiology 102:643, 1972 Mayock RL, Bertrand P, Morrison CE, et al: Manifestations of sarcoidosis. Analysis of 145 patients, with a review of nine series selected from the literature. Am J Med 35:67-89, 1963 Moldover A: Sarcoidosis of the spinal cord. Report of a case with remission associated with cortisone therapy. Arch Intern Med 102:414-417, 1958 Pennell WH: Boeck's sarcoid involvement of the central nervous system. Arch Neurol Psychiatry 66:728-737, 1951 Semins H, Nugent GR, Chou SM: Intramedullary spinal cord sarcoidosis. J Neurosurg 37:233-236, 1972 Siltzbach LE: Effects of cortisone in sarcoidosis. A study of thirteen patients. Am J Med 12:139-160, 1952 Silverstein A, Feuer MM, Siltzbach LE: Neurologic sarcoidosis. A study of 18 cases. Arch Neurol 12:1-11, 1965 Strickland GT Jr, Moser KM: Sarcoidosis with a Landry-Guillain-Barr6 syndrome and clinical response to corticosteroids. Am J Med 43:131-135, 1967 Snyder R, Towfighi J, Gonatus NK: Sarcoidosis of the spinal cord. Case report. J Neurosurg 44:740-743, 1976 Wiederholt WC, Siekert RG: Neurological manifestations of sarcoidosis. Neurology 15:1147-1154, 1965
Address reprint requests to: James N. Campbell, M.D., Department of Neurological Surgery, The Johns Hopkins Hospital, 601 North Broadway, Baltimore, Maryland 21205.
J. Neurosurg. / Volume 47 / July, 1977
Sarcoid of the cauda equina Case report JAMES N . CAMPBELL,M . D . , PERRY BLACK,M . D . , C . M . , AND PETER T. OSTROW, M . D . , PH.D.
Departments of Neurological Surgery and Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland An unusual case of sarcoid involving the cauda equina, paraparesis, is presented. There was no evidence of sarcoid nervous system, and the diagnosis was established through tion. The patient was treated with steroids postoperatively, covery of neurological function. KEY WORDS 9 arachnoiditis 9 cauda equina prednisone 9 sarcoidosis 9 spinal cord
LINICALLY apparent involvement of the nervous system is estimated to occur in a b o u t 5% of all patients with sarcoid? ,s,xS,x4 W h e n sarcoid affects the nervous system, the affected tissues are usually the meninges at the base of the brain and the cranial nerves, while the spinal cord is only rarely involved. In the infrequently reported cases with cord involvement, the presumptive diagnosis has generally been based on clinical g r o u n d s or p r o v e n at a u t o p sy. 1,4-7,9-1x,~ In a few instances, however, the diagnosis was confirmed during life through exploratory laminectomy. T u m o r - l i k e masses compressing the spinal cord were seen in three cases at cervical laminectomy, 6,n,15 and in one case at thoracic laminectomy. H Histological examination in each case revealed a g r a n u l o m a t o u s lesion without necrosis, surr o u n d e d by round-cell infiltration, epithelioid cells, and giant cells. Operatively documented sarcoid of the cauda equina has been described in two
c
J. Neurosurg. / Volume 47 / July, 1977
resulting in progressive involvement outside the thoracolumbar exploraand had a moderate re-
9 paraplegia
9
cases; ~4ae in one of these cases the lesion was believed to be confined to the nervous system. Owing to the rarity of this condition and the difficulty of its preoperative diagnosis, we report here a case of sarcoid affecting the nerve roots of the cauda equina which was diagnosed through l u m b a r exploration. Case Report
This 40-year-old m a n was admitted to The Johns H o p k i n s Hospital because of progressive inability to walk, which began 18 months before admission. History. At the age of 20 years, he had h a d several episodes of vertigo and the development o f deafness over a 2-month period. At 38 years of age he developed numbness of the left foot which spread to involve the left leg. This was associated with gradually increasing weakness of both lower extremities and bladder incontinence. H e was first evaluated at another hospital where a right brachial "109
J. N. C a m p b e l l , P. B l a c k a n d P. T. O s t r o w
FIG. 1. Supine radiograph (anteroposterior view) of the thoracolumbar region from the myelogram showing possible arachnoid adhesions. The L-3 marker is lateral to the T-12 vertebral body.
arteriogram and isotope brain scan were normal. Family history revealed that his mother had died of tuberculosis when the patient was 5 years old. Examination. On admission, the general physical examination, routine laboratory studies, including x-ray films of the chest, skull, and cervical, thoracic, and lumbosacral spine, were normal. The cranial nerves were normal with the exception of bilateral total deafness, confirmed by formal audiometry. The oculovestibular reflex was bilaterally absent. Optokinetic nystagmus was normal bilaterally. Slit-lamp examination of the optic fundi revealed no abnormalities. The. patient was unable to stand because of lower extremity weakness which was greater proximally than distally. The iliopsoas, glutei, hamstring, and quadriceps 1 ]0
muscles were 2/5, and the tibialis and gastrocnemius muscles were 4/5 in strength. There was moderate atrophy of the calves and thighs. The knee and ankle reflexes were absent and the plantar responses were flexor. There was hypalgesia and hypesthesia in a stocking distribution in the lower extremities to the mid-thigh level. Vibratory and position sense in both feet were moderately reduced. Nerve conduction velocities in all four extremities were normal. Electromyography revealed denervation and fasciculation potentials in both quadriceps muscles. A cystometrogram revealed a hypotonic neurogenic bladder. A pan-myelogram showed no gross abnormality; an additional volume of Pantopaque was therefore added and supine films were obtained in order to attempt to exclude such lesions as an arteriovenous malformation of the cord. In view of the patient's deafness, the Pantopaque was also carried up to the cerebellopontine cisterns which revealed no evidence of a mass lesion in this location. Detailed review of the myelogram films suggested equivocal arachnoidal adhesions ("matting") of the nerve roots of the cauda equina (Fig. 1). A pneumoencephalogram showed mild symmetrical ventricular dilatation and poor entry of air over the convexity of the brain. A cisternal isotope scan demonstrated ventricular stasis with little or no passage of isotope over the convexity after 48 hours. Several lumbar punctures revealed clear cerebrospinal fluid (CSF) with pressures always below 150 mm H,O. The protein content ranged from 224 to 330 mg%. Protein electrophoresis indicated an elevated globulin fraction of 16% (normal 4.3% to 12.3% of total CSF protein). Cell count ranged from 14 to 46 white blood cells (WBC), all mononuclear. Cytopathological study of the cells revealed no evidence of neoplasia. The CSF glucose content was normal (55 mg%). Cultures of the CSF for bacteria, fungi, and acidfast bacilli were negative. India ink preparations on fresh CSF samples were negative also. The CSF and serum venereal disease (VDRL) assays were negative. A serum protein electrophoresis was normal. Anti-nuclear antibody titers were normal, and multiple lupus erythematosus (LE) preparations were negative. The peripheral WBC count and differential were normal, and the erythrocyte J. Neurosurg. / Volume 47 / July, 1977
Sarcoid of the cauda equina sedimentation rate ranged from 31 to 41 ram/hr. In view of the progressive deterioration of the patient's neurological function, and the clinical evidence suggesting a lesion of the cauda equina, we elected to proceed with a thoracolumbar exploration. Operation. A complete laminectomy was carried out from T-10 to L-2. On opening the dura, gross examination revealed only delicate fibrous adhesions of the nerve roots of the cauda equina. When the operative microscope was used, however, irregular bluishpink nodules, 2 to 3 mm in diameter, could be seen embedded in continuity with many of the nerve roots. Samples of several of these nodules were dissected from the nerve fascicles and submitted for histological examination. Histological Examination. The nodules consisted of sparsely cellular granulomas comprising lymphocytes, histiocytes, and fibroblasts in a dense collagenous matrix (Fig. 2). No areas of necrosis were seen. SpeFIG. 2. Photomicrograph of noncaseating cial stains for acid-fast bacilli and fungi were negative. The morphology was interpreted as granuloma attached to nerve root of the cauda equina. H & E, X 330. most consistent with sarcoid. Postoperative Course. Skin testing revealed a positive Kveim reaction, negative intermediate strength purified protein derivament has been sustained. Steroid therapy will tive (PPD), and positive Candida test. Liver be reinstituted if his neurological function biopsy revealed no granulomata. The patient deteriorates. was placed on prednisone, 40 mg/day, and by the seventh postoperative day there was imDiscussion proved strength in the lower extremities. When systemic manifestations of sarSteroid therapy was stopped on the 10th postoperative day because of a staphylococcal coidosis are present, the finding of CSF pleowound infection, which cleared with drain- cytosis, and elevated CSF protein with norage and antibiotic therapy. After a course of mal glucose should ordinarily suggest central physical therapy, the patient was discharged, nervous system (CNS) sarcoidosis. In this able to walk with assistance, and with im- case, however, there was no evidence for sarproved bladder function. coid involvement outside the CNS, and the These improvements regressed over the diagnosis could be established only through ensuing 6 months while the patient was off lumbar exploration. The positive Kveim reacsteroid therapy. At this time, with the wound tion and histological findings obtained from well healed, prednisone (40 mg/day) therapy biopsy of lesions in the cauda equina prowas reinstituted and continued for 6 months. vided the basis for the diagnosis of sarAt the end of this course of therapy the pa- coidosis. Basilar meningitis with multiple cranial tient could walk up and down stairs without assistance, and could void without nerve deficits is one of the most common catheterization. The CSF protein at this time forms of CNS involvement with sarcoid. In a was 79 rag% and'there were 3 mononuclear series of 26 patients with neurological deficits cells per ml. Steroid therapy was then from sarcoid reported by Wiederholt and Siegradually discontinued, and 18 months since kert, TM all had cranial nerve signs and eight the time of surgery the neurological improve- patients had decreased hearing, which was J. Neurosurg. / Volume 47 / July, 1977
11 l
J. N. Campbell, P. Black and P. T. Ostrow bilateral in all cases. That our patient could have had bilateral nerve deafness from sarcoid 20 years before any other manifestation of this disease would be unusual. This possibility is supported, however, by the finding of hydrocephalus on pneumoencephalography with little entry of air over the convexity of the brain, suggesting that the patient may well have had an episode of basilar meningitis some time in the past. The operative findings in two previously r e p o r t e d cases of c a u d a equina sarcoidosis 14,18 included increased dural vascularity, and dense adhesions of the nerve roots with grayish arachnoid tissue. In the present case, the lesions were more subtle, consisting of bluish-pink nodules in continuity with the nerve roots; the arachnoid was slightly thicker than normal. The neurological deficit presumably resulted from the inflammatory lesions within the nerve roots themselves. The operative microscope proved to be of inestimable value in this case, since the minute lesions imbedded in the nerve roots might well have been overlooked without magnification. The value of establishing a diagnosis in such cases is obvious in view of potential favorable response to steroid therapy?,2.6ao,,~,xe
5.
6. 7. 8.
9.
10. 11. 12. 13. 14.
References 1. Askanazy CL: Sarcoidosis of the central nervous system. J Neuropathoi Exp Neurol 11:392-400, 1952 2. Banerjee T, Hunt WE" Spinal cord sarcoidosis. Case report. J Neurosurg 36:490-493, 1972 3. Douglas AC, Malony AF: Sarcoidosis of the central nervous system. J Neurol Neurosurg Psychiatry 36:1024-1033, 1973 4. de Morsier G, Maurice P, Martin F: BesnierBoeck diffus des muscles et 16si0ns du syst~me nerveux central (deux observations anatomo-
] ]2
15. 16.
cliniques). Acta Neuroi Psychiatr Belg 54:34-51, 1954 Ericksen TC, Odom G, Stern K: Boeck's disease (sarcoid) of the central nervous system. Report of a case, with complete clinical and pathologic study. Arch Neurol Psychiatry 48:613-621, 1942 Jefferson M: Sarcoidosis of the nervous system. Brain 80:540-556, 1957 Kirks DR, Newton TH: Sarcoidosis: a rare cause of spinal cord widening. Radiology 102:643, 1972 Mayock RL, Bertrand P, Morrison CE, et al: Manifestations of sarcoidosis. Analysis of 145 patients, with a review of nine series selected from the literature. Am J Med 35:67-89, 1963 Moldover A: Sarcoidosis of the spinal cord. Report of a case with remission associated with cortisone therapy. Arch Intern Med 102:414-417, 1958 Pennell WH: Boeck's sarcoid involvement of the central nervous system. Arch Neurol Psychiatry 66:728-737, 1951 Semins H, Nugent GR, Chou SM: Intramedullary spinal cord sarcoidosis. J Neurosurg 37:233-236, 1972 Siltzbach LE: Effects of cortisone in sarcoidosis. A study of thirteen patients. Am J Med 12:139-160, 1952 Silverstein A, Feuer MM, Siltzbach LE: Neurologic sarcoidosis. A study of 18 cases. Arch Neurol 12:1-11, 1965 Strickland GT Jr, Moser KM: Sarcoidosis with a Landry-Guillain-Barr6 syndrome and clinical response to corticosteroids. Am J Med 43:131-135, 1967 Snyder R, Towfighi J, Gonatus NK: Sarcoidosis of the spinal cord. Case report. J Neurosurg 44:740-743, 1976 Wiederholt WC, Siekert RG: Neurological manifestations of sarcoidosis. Neurology 15:1147-1154, 1965
Address reprint requests to: James N. Campbell, M.D., Department of Neurological Surgery, The Johns Hopkins Hospital, 601 North Broadway, Baltimore, Maryland 21205.
J. Neurosurg. / Volume 47 / July, 1977
