J Neurosurg 48:1015-1018, 1978
Subarachnoid hemorrhage and papilledema due to a cervical neurilemmoma Case report
LINDA M. LUXON, B.Sc., M.B., M.R.C.P., AND MICHAEL J. G. HARRISON, D . M . , F . R . C . P .
The Department of Neurological Studies, The Middlesex Hospital, 'Mortimer Street, London, England ~" An unusual case of a cervical neurilemmoma is presented. A 67-year-old man developed papilledema on two occasions in association with the symptoms and signs of a subarachnoid hemorrhage (SAH). He proved to have an otherwise asymptomatic cervical neurilemmoma. It is suggested that both acute and subclinical bleeding from such tumors may sometimes underlie the development of raised intracranial pressure occasionally encountered. The clinical clue to the spinal origin of the SAH was an acute onset with root pain. This case is discussed with reference to similar cases in the literature. 9 cervical neurilemmoma 9 spinal cord tumor subarachnoid hemorrhage 9 increased intracranial pressure
KEY W O R D S
I
NCREASED intracranial pressure ( I C P ) is on rare occasions caused by a spinal tumor. In most reported instances of spinal t u m o r there have been signs and s y m p t o m s of the lesion although investigations have often initially been directed toward an intracranial cause for the elevated | C P and papilledema. It has usually been argued that the raised I C P develops from the effects of an elevated cerebrospinal fluid (CSF) protein. Spinal subarachnoid h e m o r r h a g e ( S A H ) is most c o m m o n l y due to an angioma, but S A H from other spinal tumors is well documented. There are often few clinical clues to the spinal origin of subarachnoid bleeding in such cases, however.
J. Neurosurg. / Volume 48 / June, 1978
9
In a recently studied case, raised I C P developed on two separate occasions in a patient with an otherwise a s y m p t o m a t i c cervical tumor. The circumstances suggest that subarachnoid bleeding f r o m the neurilemm o m a was the cause of the elevated I C P . Case Report
This 67-year-old m a n was taken ill in September, 1976, with the sudden onset of pain in the tip of his left shoulder radiating to the neck and occipital region. As he had recently washed the floors throughout his house, a mechanical strain was assumed, and the pain improved over the following week. 1015
L. M. Luxon and M. J. G. Harrison
FIG. 1. Myodil myelogram showing rounded intradural extramedullary mass on the left side in the mid-cervical region.
FIG. 2. Operative specimen showing hemorrhagic areas. 1016
He then travelled to London where he suddenly developed severe occipital headache, nausea, and vomiting. This persisted throughout the night and during the following day when he returned home. He then sought medical attention and was found to have mild neck stiffness and bilateral papilledema. He was admitted to a regional center for neurological investigation. A computerized tomographic (CT) scan showed no hydrocephalus, and bilateral carotid angiography revealed no abnormality. A lumbar puncture was performed, with an opening pressure of 150 mm H20; the CSF was bloodstained, with 11,000 red blood cells (RBC)/cu mm in the second bottle, and 14,000 R B C / c u mm in the third. It was thought that he had had an SAH, although the CSF showed no xanthochromia. He was treated conservatively and given dexamethasone. His headache resolved as did the papilledema. He remained well until February 4, 1977, when he again developed pain in the left shoulder tip, accompanied on this occasion by severe bifrontal and temporal headache with nausea and vomiting. On this occasion he also felt rotational vertigo. He was admitted locally and again found to have mild neck stiffness. He had again developed bilateral papilledema. He was transferred to the Middlesex Hospital where the neurological examination revealed no further findings. Spinal fluid was obtained on three occasions: February 18 and 21, and March 3, 1977. The CSF pressure was initially elevated at 220 and 240 mm H~O. The first sample was xanthochromic with 15,000 RBC/cu mm, the second contained 172 RBC/cu mm, and the third was clear and colorless, with 13 RBC/cu mm. The CSF protein was 46 mg%. Routine skull films showed no abnormality, and cervical spine films revealed only degenerative osteoarthritic changes. Carotid and vertebral angiograms revealed no source for subarachnoid bleeding, and the lateral ventricles appeared of a normal size. Myodil myelography revealed a rounded intradural extramedullary mobile mass, 12 • 18 mm, on the left side in the cervical region (Fig. 1). On March 10, 1977, a laminectomy was carried out (Mr. J. Jakubowski) at C3-5, revealing a typical neurilemmoma in the subarachnoid space between the bodies of C-3 and C-4. It appeared to arise from a posterior root and have an anterior root stretched over J. Neurosurg. / Volume 48 / June, 1978
Cervical neurilemmoma with SAH and papilledema it. The tumor was removed in toto. Its external surface was patchily bloodstained (Fig. 2). The histological appearance (Dr. H. C. Grant) was of a typical neurilemmoma, with many large vascular channels with thick walls and aggregates o f hemosiderin (Fig. 3). The patient is currently well and asymptomatic with no papilledema. Discussion Spinal S A H is rare. Walton, 21 reporting in 1953 on 312 cases of S A H of unusual etiology, identified only two as being of spinal origin. Intradural arteriovenous malformations, although rare among spinal tumors, are the most c o m m o n cause of spontaneous hemorrhage, 4,12,16a~ but they rarely present in this way? 1 Intradural tumors are the second most c o m m o n cause of spontaneous spinal S A H ? ,2,s,~~ The tumors that behave in this way are usually ependymomas of the conus, cauda equina, or ilium terminale, but include neurofibromata and astrocytomas.~ 8,11,15 Spontaneous hemorrhage from a cervical tumor appears to be rare. Three previous reports relate to an astrocytoma, ~ a sarcoma, TM and an angioma, 9 all notably in children. The present patient is therefore unusual in having apparently had two SAH's late in life from a cervical neurilemmoma. The diagnosis of the spinal origin of SAH is recognized to be difficult. There are frequently no signs referable to the spinal cord. The diagnosis is worth pursuing, however, as many of the responsible lesions are benign and operable. In 1928, Michon, 13 in an account of the clinical features of spontaneous spinal SAH, stressed the intensity of pain at the site of bleeding ("le coup de poignard rachidien"), and the prominence of this symptom together with root pain at the same level has been recognized by subsequent authors, e,8,9`15,1sThe frequent relationship of the onset of a hemorrhage to a period of exertion has also been documented. 9,xs,18 It is of interest that our patient twice developed severe pain in the shoulder tip, once after exercising the upper limbs. It is possible that movement of the head and neck caused stretching of the mass, a mechanism proposed by Nassar and CorrelP 4 as an explanation for the bleeding of cauda equina tumors. Raised ICP and papilledema have also been described with spinal lesions including J. Neurosurg. / Volume 48 / June, 1978
FIG. 3. Histological appearance of cervical neurilemmoma showing darkly stained hemosiderin aggregates. Perls' Prussian blue reaction for iron, • 80. neurilemmomas. The affected patients have mostly been young adults, and the female sex has predominated. The explanation for the production of raised ICP has usually been that CSF absorptive mechanisms have been affected in the spinal theca by block and intracranially by the elevated protein content. The myelographic evidence of block is often incomplete, however, and isolated cases have had normal or near normal C S F protein. Arseni and Maretsis s noted in their review of cases in the literature that red cells were often to be found in the xanthochromic C S F of patients with spinal t u m o r s showing papilledema. In the present case it seems likely that subarachnoid bleeding from the cervical n e u r i l e m m o m a caused two episodes of elevated I C P with papilledema. Both episodes had clinical features of S A H with headache, neck stiffness, and vomiting preceded by acute severe root pain in the shoulder tip. In one episode the C S F contained red cells, in 1017
L. M. Luxon and M. J. G. Harrison the other the fluid was xanthochromic and under raised pressure. The papilledema resolved in each case, once with dexamethasone, and once spontaneously. It seems reasonable to suppose that chronically elevated I C P might have developed had recurrent bleeding continued. The evidence of bloodstained fluid in m a n y of the cases discussed by Arseni and Maretsis s suggests that bleeding can occur without the clinical features of an S A H . Histological examination of neurilemm o m a s often shows a b n o r m a l vascular channels and hemosiderin aggregates as in our case. M i c r o s c o p i c bleeding in such tumors thus appears common. Evidence of microscopic bleeding before surgery was found in 21 of 40 surgical specimens studied in this hospital. Runnels and Hanbery TM reported a case in which they attributed spinal S A H to hemorrhage and infarction within a neurilemmoma. There was no evidence of infarction in the present case. It is suggested that bleeding into the subarachnoid space from spinal tumors may on occasion be the cause of the elevated CSF pressure and papilledema in such cases. In some instances the clinical picture may suggest an acute S A H , in others the C S F is unexpectedly bloodstained. The protein may or may not be concomitantly elevated. References 1. Abbott KH: Subarachnoid hemorrhage from an ependymoma arising in the ilium terminale. Report of a case. Bull Los Angeles Neurol Soc 4:127-132, 1939 2. Andr6-Thomas, Ferrand, Schaeffer H, et al: Syndrome d'h6morragie m6ning6e r6alis6 par une tumeur de la queue de cheval. Paris Med 77:292-296, 1930 3. Arseni C, Maretsis M: Tumors of the lower spinal cord associated with increased intracranial pressure and papilledema. J Neurosurg 27:105-110, 1967 4. Baick CAJA: A case of angioma of the spinal cord, with recurrent baemorrhages. Br Med J 2:1707-1708, 1900 5. Bhandari YS: Subarachnoid hemorrhage due to cervical cord tumor in a child. Case report. J Neurosurg 30:749-751, 1969 6. Fincher EF: Spontaneous subarachnoid hemorrhage in intradural tumors of the lumbar sac. A clinical syndrome. J Neurosurg 8:576-584, 1951 7. Fortuna A, La Torre E: Neurinoma della cauda son emorrhagia subarachnoidea circo1018
8.
9. 10.
11.
12.
13.
14. 15.
16. 17.
18.
19. 20. 21. 22.
scritta. Lab Neuropsichiatr 43:1157-1164, 1968 Halpern L, Feldman S, Peyser E: Subarachnold hemorrhage with papdledema due to a spinal neurofibroma. Arch Neurol Psychiatr 79:138-141, 1958 Henson RA, Croft PB: Spontaneous spinal subarachnoid hemorrhage. Q J Meal 25:53-66, 1956 Krayenbahl H: Spontane spinal Subarachnoidalblutung und akute Rackenmarkskompression bei intraduralem Neurinom. Schweiz Med Wocbenschr 77:692-694, 1947 Krayenbahl H, Yasargil MG, McCiintock HG: Treatment of spinal cord vascular malformations by surgical excision. J Neurosurg 30:427-435, 1969 Lorenz O (1901): Cited in Cobb S: Haemangioma of the spinal cord associated with skin naevi of the same metamere. Ann Surg 62:641-649, 1915 Michon P: Le coup de poignard rachidien: Sympt6me initial de certaines h6morragies sous-arachnoidiennes. Essai sur les h6morragies m6ning6es spinales. Presse Med 36:964-966, 1928 Nassar SI, Correll JW: Subarachnoid hemorrhage due to spinal cord tumors. Neurology 18:87-94, 1968 Prieto A Jr, Cantu RC: Spontaneous subarachnoid hemorrhage associated with neurofibroma of the cauda equina. Case report. J Neurosurg 27:63-69, 1967 Robertson EG: A case of arterial angioma of the spinal cord. Med J Aust 2:384-385, 1938 Roger H, Paillas JE, Duplay J: H6morragie m6ning6e spino-c6rebrale r6v61atrice d'une tumeur de la queue de cheval chez deux jeunes sujets. Bull Mere Soc Med Hop Paris 65:37-40, 1949 Runnels JB, Hanbery JW: Spontaneous subarachnoid hemorrhage associated with spinal cord tumours. Case report. J Neurosurg 40:252-254, 1974 Tarlov IM, Keener EB: Subarachnoid hemorrhage and tumor implants from spinal sarcoma in an infant. Neurology 3:384-390, 1953 Trupp M, Sachs E: Vascular tumors of the brain and spinal cord and their treatment. J Neurosurg 5:354-371, 1948 Walton JN: Subarachnoid hemorrhage of unusual etiology. Neurology 3:517-543, 1953 Wyburn-Mason R: The Vascular Abnormalities and Tumours of the Spinal Cord and Its Membranes. London: Henry Kimpton, 1943, 196 pp 9
Y
.
.
Address reprint requests to: Linda M. Luxon, M.B., The National Hospital, Queen Square, London WC1N 3BG, England. J. Neurosurg. / Volume 48 / June, 1978
Subarachnoid hemorrhage and papilledema due to a cervical neurilemmoma Case report
LINDA M. LUXON, B.Sc., M.B., M.R.C.P., AND MICHAEL J. G. HARRISON, D . M . , F . R . C . P .
The Department of Neurological Studies, The Middlesex Hospital, 'Mortimer Street, London, England ~" An unusual case of a cervical neurilemmoma is presented. A 67-year-old man developed papilledema on two occasions in association with the symptoms and signs of a subarachnoid hemorrhage (SAH). He proved to have an otherwise asymptomatic cervical neurilemmoma. It is suggested that both acute and subclinical bleeding from such tumors may sometimes underlie the development of raised intracranial pressure occasionally encountered. The clinical clue to the spinal origin of the SAH was an acute onset with root pain. This case is discussed with reference to similar cases in the literature. 9 cervical neurilemmoma 9 spinal cord tumor subarachnoid hemorrhage 9 increased intracranial pressure
KEY W O R D S
I
NCREASED intracranial pressure ( I C P ) is on rare occasions caused by a spinal tumor. In most reported instances of spinal t u m o r there have been signs and s y m p t o m s of the lesion although investigations have often initially been directed toward an intracranial cause for the elevated | C P and papilledema. It has usually been argued that the raised I C P develops from the effects of an elevated cerebrospinal fluid (CSF) protein. Spinal subarachnoid h e m o r r h a g e ( S A H ) is most c o m m o n l y due to an angioma, but S A H from other spinal tumors is well documented. There are often few clinical clues to the spinal origin of subarachnoid bleeding in such cases, however.
J. Neurosurg. / Volume 48 / June, 1978
9
In a recently studied case, raised I C P developed on two separate occasions in a patient with an otherwise a s y m p t o m a t i c cervical tumor. The circumstances suggest that subarachnoid bleeding f r o m the neurilemm o m a was the cause of the elevated I C P . Case Report
This 67-year-old m a n was taken ill in September, 1976, with the sudden onset of pain in the tip of his left shoulder radiating to the neck and occipital region. As he had recently washed the floors throughout his house, a mechanical strain was assumed, and the pain improved over the following week. 1015
L. M. Luxon and M. J. G. Harrison
FIG. 1. Myodil myelogram showing rounded intradural extramedullary mass on the left side in the mid-cervical region.
FIG. 2. Operative specimen showing hemorrhagic areas. 1016
He then travelled to London where he suddenly developed severe occipital headache, nausea, and vomiting. This persisted throughout the night and during the following day when he returned home. He then sought medical attention and was found to have mild neck stiffness and bilateral papilledema. He was admitted to a regional center for neurological investigation. A computerized tomographic (CT) scan showed no hydrocephalus, and bilateral carotid angiography revealed no abnormality. A lumbar puncture was performed, with an opening pressure of 150 mm H20; the CSF was bloodstained, with 11,000 red blood cells (RBC)/cu mm in the second bottle, and 14,000 R B C / c u mm in the third. It was thought that he had had an SAH, although the CSF showed no xanthochromia. He was treated conservatively and given dexamethasone. His headache resolved as did the papilledema. He remained well until February 4, 1977, when he again developed pain in the left shoulder tip, accompanied on this occasion by severe bifrontal and temporal headache with nausea and vomiting. On this occasion he also felt rotational vertigo. He was admitted locally and again found to have mild neck stiffness. He had again developed bilateral papilledema. He was transferred to the Middlesex Hospital where the neurological examination revealed no further findings. Spinal fluid was obtained on three occasions: February 18 and 21, and March 3, 1977. The CSF pressure was initially elevated at 220 and 240 mm H~O. The first sample was xanthochromic with 15,000 RBC/cu mm, the second contained 172 RBC/cu mm, and the third was clear and colorless, with 13 RBC/cu mm. The CSF protein was 46 mg%. Routine skull films showed no abnormality, and cervical spine films revealed only degenerative osteoarthritic changes. Carotid and vertebral angiograms revealed no source for subarachnoid bleeding, and the lateral ventricles appeared of a normal size. Myodil myelography revealed a rounded intradural extramedullary mobile mass, 12 • 18 mm, on the left side in the cervical region (Fig. 1). On March 10, 1977, a laminectomy was carried out (Mr. J. Jakubowski) at C3-5, revealing a typical neurilemmoma in the subarachnoid space between the bodies of C-3 and C-4. It appeared to arise from a posterior root and have an anterior root stretched over J. Neurosurg. / Volume 48 / June, 1978
Cervical neurilemmoma with SAH and papilledema it. The tumor was removed in toto. Its external surface was patchily bloodstained (Fig. 2). The histological appearance (Dr. H. C. Grant) was of a typical neurilemmoma, with many large vascular channels with thick walls and aggregates o f hemosiderin (Fig. 3). The patient is currently well and asymptomatic with no papilledema. Discussion Spinal S A H is rare. Walton, 21 reporting in 1953 on 312 cases of S A H of unusual etiology, identified only two as being of spinal origin. Intradural arteriovenous malformations, although rare among spinal tumors, are the most c o m m o n cause of spontaneous hemorrhage, 4,12,16a~ but they rarely present in this way? 1 Intradural tumors are the second most c o m m o n cause of spontaneous spinal S A H ? ,2,s,~~ The tumors that behave in this way are usually ependymomas of the conus, cauda equina, or ilium terminale, but include neurofibromata and astrocytomas.~ 8,11,15 Spontaneous hemorrhage from a cervical tumor appears to be rare. Three previous reports relate to an astrocytoma, ~ a sarcoma, TM and an angioma, 9 all notably in children. The present patient is therefore unusual in having apparently had two SAH's late in life from a cervical neurilemmoma. The diagnosis of the spinal origin of SAH is recognized to be difficult. There are frequently no signs referable to the spinal cord. The diagnosis is worth pursuing, however, as many of the responsible lesions are benign and operable. In 1928, Michon, 13 in an account of the clinical features of spontaneous spinal SAH, stressed the intensity of pain at the site of bleeding ("le coup de poignard rachidien"), and the prominence of this symptom together with root pain at the same level has been recognized by subsequent authors, e,8,9`15,1sThe frequent relationship of the onset of a hemorrhage to a period of exertion has also been documented. 9,xs,18 It is of interest that our patient twice developed severe pain in the shoulder tip, once after exercising the upper limbs. It is possible that movement of the head and neck caused stretching of the mass, a mechanism proposed by Nassar and CorrelP 4 as an explanation for the bleeding of cauda equina tumors. Raised ICP and papilledema have also been described with spinal lesions including J. Neurosurg. / Volume 48 / June, 1978
FIG. 3. Histological appearance of cervical neurilemmoma showing darkly stained hemosiderin aggregates. Perls' Prussian blue reaction for iron, • 80. neurilemmomas. The affected patients have mostly been young adults, and the female sex has predominated. The explanation for the production of raised ICP has usually been that CSF absorptive mechanisms have been affected in the spinal theca by block and intracranially by the elevated protein content. The myelographic evidence of block is often incomplete, however, and isolated cases have had normal or near normal C S F protein. Arseni and Maretsis s noted in their review of cases in the literature that red cells were often to be found in the xanthochromic C S F of patients with spinal t u m o r s showing papilledema. In the present case it seems likely that subarachnoid bleeding from the cervical n e u r i l e m m o m a caused two episodes of elevated I C P with papilledema. Both episodes had clinical features of S A H with headache, neck stiffness, and vomiting preceded by acute severe root pain in the shoulder tip. In one episode the C S F contained red cells, in 1017
L. M. Luxon and M. J. G. Harrison the other the fluid was xanthochromic and under raised pressure. The papilledema resolved in each case, once with dexamethasone, and once spontaneously. It seems reasonable to suppose that chronically elevated I C P might have developed had recurrent bleeding continued. The evidence of bloodstained fluid in m a n y of the cases discussed by Arseni and Maretsis s suggests that bleeding can occur without the clinical features of an S A H . Histological examination of neurilemm o m a s often shows a b n o r m a l vascular channels and hemosiderin aggregates as in our case. M i c r o s c o p i c bleeding in such tumors thus appears common. Evidence of microscopic bleeding before surgery was found in 21 of 40 surgical specimens studied in this hospital. Runnels and Hanbery TM reported a case in which they attributed spinal S A H to hemorrhage and infarction within a neurilemmoma. There was no evidence of infarction in the present case. It is suggested that bleeding into the subarachnoid space from spinal tumors may on occasion be the cause of the elevated CSF pressure and papilledema in such cases. In some instances the clinical picture may suggest an acute S A H , in others the C S F is unexpectedly bloodstained. The protein may or may not be concomitantly elevated. References 1. Abbott KH: Subarachnoid hemorrhage from an ependymoma arising in the ilium terminale. Report of a case. Bull Los Angeles Neurol Soc 4:127-132, 1939 2. Andr6-Thomas, Ferrand, Schaeffer H, et al: Syndrome d'h6morragie m6ning6e r6alis6 par une tumeur de la queue de cheval. Paris Med 77:292-296, 1930 3. Arseni C, Maretsis M: Tumors of the lower spinal cord associated with increased intracranial pressure and papilledema. J Neurosurg 27:105-110, 1967 4. Baick CAJA: A case of angioma of the spinal cord, with recurrent baemorrhages. Br Med J 2:1707-1708, 1900 5. Bhandari YS: Subarachnoid hemorrhage due to cervical cord tumor in a child. Case report. J Neurosurg 30:749-751, 1969 6. Fincher EF: Spontaneous subarachnoid hemorrhage in intradural tumors of the lumbar sac. A clinical syndrome. J Neurosurg 8:576-584, 1951 7. Fortuna A, La Torre E: Neurinoma della cauda son emorrhagia subarachnoidea circo1018
8.
9. 10.
11.
12.
13.
14. 15.
16. 17.
18.
19. 20. 21. 22.
scritta. Lab Neuropsichiatr 43:1157-1164, 1968 Halpern L, Feldman S, Peyser E: Subarachnold hemorrhage with papdledema due to a spinal neurofibroma. Arch Neurol Psychiatr 79:138-141, 1958 Henson RA, Croft PB: Spontaneous spinal subarachnoid hemorrhage. Q J Meal 25:53-66, 1956 Krayenbahl H: Spontane spinal Subarachnoidalblutung und akute Rackenmarkskompression bei intraduralem Neurinom. Schweiz Med Wocbenschr 77:692-694, 1947 Krayenbahl H, Yasargil MG, McCiintock HG: Treatment of spinal cord vascular malformations by surgical excision. J Neurosurg 30:427-435, 1969 Lorenz O (1901): Cited in Cobb S: Haemangioma of the spinal cord associated with skin naevi of the same metamere. Ann Surg 62:641-649, 1915 Michon P: Le coup de poignard rachidien: Sympt6me initial de certaines h6morragies sous-arachnoidiennes. Essai sur les h6morragies m6ning6es spinales. Presse Med 36:964-966, 1928 Nassar SI, Correll JW: Subarachnoid hemorrhage due to spinal cord tumors. Neurology 18:87-94, 1968 Prieto A Jr, Cantu RC: Spontaneous subarachnoid hemorrhage associated with neurofibroma of the cauda equina. Case report. J Neurosurg 27:63-69, 1967 Robertson EG: A case of arterial angioma of the spinal cord. Med J Aust 2:384-385, 1938 Roger H, Paillas JE, Duplay J: H6morragie m6ning6e spino-c6rebrale r6v61atrice d'une tumeur de la queue de cheval chez deux jeunes sujets. Bull Mere Soc Med Hop Paris 65:37-40, 1949 Runnels JB, Hanbery JW: Spontaneous subarachnoid hemorrhage associated with spinal cord tumours. Case report. J Neurosurg 40:252-254, 1974 Tarlov IM, Keener EB: Subarachnoid hemorrhage and tumor implants from spinal sarcoma in an infant. Neurology 3:384-390, 1953 Trupp M, Sachs E: Vascular tumors of the brain and spinal cord and their treatment. J Neurosurg 5:354-371, 1948 Walton JN: Subarachnoid hemorrhage of unusual etiology. Neurology 3:517-543, 1953 Wyburn-Mason R: The Vascular Abnormalities and Tumours of the Spinal Cord and Its Membranes. London: Henry Kimpton, 1943, 196 pp 9
Y
.
.
Address reprint requests to: Linda M. Luxon, M.B., The National Hospital, Queen Square, London WC1N 3BG, England. J. Neurosurg. / Volume 48 / June, 1978
