Occult Epidural Chloroma Complicated by Acute Paraplegia Following ar Puncture Meng C. Wong, MRCP,” George Krol, MI),+ and Marc K.Rosenblum, MDS,
Acute paraplegia complicating lumbar puncture i n a leukemic patient with an unsuspected epidural chloroma is described, including the postmortem findings. Lumbar puncture can precipitate irreversible injury to the spinal cord in the patient w i t h a n occult lesion causing subarachnoid block. T h i s account, i n addition to documenting this phenomenon, suggests i i potential advantage to magnetic resonance imaging over myelography. Wong MC, Krol G, Rosenblurn MK. Occult epidural chloroma complicated by acute paraplegia following lumbar puncture. Ann Neurol 1992;11.110-112
Herniation syndromes have been associated with space-occupying lesions producing intracranial hypertension and pressure differentials across neural compartments { 11. T h a t acute neurological deterioration may be precipitated by lumbar puncture in this setting is well known [Z], but lesions i m p e d h g t h e flow of cerebrospinal fluid (CSF) at spinal levels may also create significant pressure differentials potentially aggravated by this procedure and complicated by spinal cord dysfunction { 3- 51. Catastrophic myelopath y, however, remains an infrequently documented p h e n o m e n o n after lumbar puncture in patients with epidural masses causing subarachnoid block [4, 5). We describe t h e clinical, radiological, and pathological features of acute paraplegia complicating lumbar puncture in a patient with unsuspected epidural spinal cord compression.
Case Report A 64-year-old man with a history of myelodysplastic syndrome and biopsy-proved cutaneous chloromas was admitted to Memorial Hospital (New York, NY) complaining of fatigue, myalgias, headache, and impaired concentration. Physi-
From the Departments of ‘Neurology, tRadiology (Neuroradiology), and iPathology (Neuropathology ), Memorial Sloan-Kettering Cancer Centcr, New York, N Y . Received Apt 18, 1931, and in revised form Jul 17. Accepted for publication Jul 21. 1991. Address correspondence to Dr Rosenblum, Departmen:: of Pathologv (Neuroparhology), Memorial Sloan-Kettering Cancer Center, 1275 York Avc, Mew York, NY 10021.
I10 Copyright
(c)
cal examination revealed pallor, multiple cutaneous papules, splenomegaly, and mild cognitive dysfunction. Significant admission laboratory studies included a complete blood count demonstrating a white blood cell (WBC) count of 27.9 K/ pl with 18% blasts, hemoglobin 6.4/dl, hematocrit 21.2%, platelets 7 1 Kiwi, and a lumbar puncture with WBC 6/mmi, red blood cell (RBC) Oimm’, glucose 73 mgidl, protein 30 mgidl, and negative cytology. Cranial magnetic resonance imaging (MRI) was unremarkable. The impression was of myelodysplastic syndrome in transition to acute myeloblastic leukemia and metabolic encephalopathy. A subsequent bone marrow biopsy was consistent with acute myeloblastic leukemia. Over the next several days, the patient was intermittently confused and complained of vague lumbar backache. Physical examination was unchanged save for slight tenderness to percussion over the lumbar spine. Despite treatment with cytosine P-D-arabinofuranoside ( Ara-C; 20 mg/m2). his WBC rose steadily to reach 136 K/kl by the seventh hospital day. Lumbar puncture was repeated at this time and approximately 3 ml of CSF obtained. The upening pressure was less than 20 mm of water, WBC 5/mmi, RBC 3/mm‘, protein 246 mgidl, glucose 88 mg/dl, and cytology again negative. Minutes after completing this procedure, the patient complained of weakness of both legs and pain radiating bilaterally from the lower back to the upper abdomen. Within 2 hours he was paraplegic with T-8 sensory level to all modalitie:?and loss of sphincter function. Myelogram at L2-3 was unsuccessful ( n o flow of CSF). A cervical myelogram at C1-2 revealed complete block with the upper level at the seventh thoracic vertebral body. MRI demonstrated pronounced compression of the spinal cord by a mass filling the posterior spinal canal from T-7 to T-9 (Fig 1). Prominence of the paraspinal soft tissues at T-8 was also noted after contrast administration. The patient was treated with high-dose steroids, spinal radiation therapy, and chemotherapy ( Ara-C 1 gm/mL and daunomycin 102 mg), but his neurological status did not improve. Repeat MRI on the sixteenth hospital day demonstrated the mass, reduced in size but still occupying 50(%of the diameter of the spinal canal. He became pancytopenic, developed sepsis and disseminated intravascular coagulation, and died 22 days after admission. The systemic autopsy revealed disseminated canclidiasis, hemorrhage into multiple organs, and necrotic leukemic infiltrates in the pericardium, pancreas, peripancreatic soft rissues, and bone marrow, which was virtually acellular. Foci of cerebral subarachnoid hemorrhage were noted and the thecal sac was distended by clotted blood. As these lesions show’ed no histological evidence of organization, they were judged agonal phenomena. At the midrhoracic level, a necrotic m~iss adhered to the epidural aspect of the spinal cord, extending over 3 cm of its length. The adjacent cord was softened and discolored, its gross architecture obliterated (Fig 2). Histological examination confirmed the mass was composed of leukemic cells and revealed complete transverse coagulative necrosis of the cord at this level. A solitary thrombosed intr;imedullary vein was identified here, the blood vessels otherwise remarkable solely for congestion. There was no leukemic or fungal involvement of the cord proper. The necrotic zone tapered caudally to a hemorrhagic pencil-shaped softening involving the basal posterior columns and left posterior
1992 by the American Neurological Association
Fig 2. These horizontal sections of the spinal cord demonstrate transerve necrosis at thoracic levels associated with remnants of necrotic chloroma adherent t o the epidural aspect of the meninges (arrow). Rostval levels exhibit central hemorrhagic necrosis and a circumscribed zone of necrosis is also evident at the base of the posterior columns in caudal sections. The cauda equina are embedded in clotted blood.
A
horn. The rostra1 portion of the cord evidenced central hemorrhagic necrosis.
Discussion
B Fig 1. (A,Bi TI -weighted sagittal (A) and axial (B) magnetic resonance imaging scans showing a posterior epidural soft tissue mass at the level of T-7 to T-9, obliterating the subarachnoid Jpace and compressing the spinal cord (arrows). The normal marrow signal has been replaced with low signal consistent with difluse leukemic znfiltration.
Neurological deterioration is a recognized complication of lumbar puncture in the setting of complete spinal subarachnoid block [3-51. This phenomenon, originally described some 50 years ago by Eaton and Craig 13] and subsequently referred to as “spinal coning” [GI, may be more common than is generally appreciated. Hollis and colleagues [4] noted a significant decline in motor function after lumbar myelography in 7 of 50 consecutively studied patients found to have complete block, 3 of whom also developed sphincter dysfunction. In 6 instances, the offending lesion was an epidural mass, including 4 metastatic carcinomas and single examples of osteoblastoma and abscess. Only their Patient 2, however, seems to have suffered a precipitous and catastrophic course similar to that we describe. This patient, a 67-year-old man with epidural spinal cord compression and T-4 block secondary to metastatic prostatic adenocarcinoma, progressed from relatively mild weakness to paraplegia within 30 minutes of undergoing lumbar puncture. The remaining patients deteriorated over a 1 to 4 day interval after myelography. The brief report of Blatt and Goldhammer [5] is the only other published account of acute paraplegia associated with lumbar puncture in the setting of epidural spinal cord compression. Their patient, a 68-year-old woman with low thoracic back pain, weakness of both legs, and a thoracic sensory level,
Brief Communication: Wong et al: Paraplegia after Lumbar Puncture
11 1
progressed to irreversible paraplegia within 2 hours of undergoing a lumbar myelogram that demonstrated an epidural mass causing complete subarachnoid block at T-11. Subsequent evaluation disclosed “.disseminated malignancy” that was not further characterized. The close temporal association of lumbar puncture with neurological decline in these patients [4, 51 would appear to implicate this procedure as the cause of socalled spinal coning. The withdrawal of CSF (and, in some instances, continued leakage from the puncture site) may precipitate impaction of the spinal cord in such patients by aggravating pressure differentials across the compressed segment and, further, by depriving the cord of a critical volume of fluid buffering it from the neighboring mass 171. The patient we describe demonstrates that this process may rapidly evolve to irreversihle injury in the patient with little or no evidencc: of preexistent myelopathy. The neuropathology of epidural spinal cord compression is subject to great individual variation, but is most often characterized by circumscribed foci of axonal degeneration and demyelination in the lateral and posterior columns with relative preservation of the anterior columnns and gray matter 18-10]. Complete transverse necrosis, central hemorrhagic necrosis, and pencil-shaped softening, although well documented in this setting [lo, 111, are less frequent finding:; and considered by some authors more characteristic of acute mechanical trauma [lo]. In addition, central hemorrhagic necrosis is a particularly prominent feature of some experimental models of acute impacr injury to the spinal cord E12). The pathogenesis of these varied patterns of spinal cord damage remains to be elucidated, but the time course of the compressive insult, typically subacute or chronic in the case of epidural neoplasia, may be a determinant of the final histopathological picture. We speculate that lumbar puncture precipitated acute and severe spinal cord impaction in our patient, resulting in a pattern of tissue injury commonly observed in the cord subjected to sudden traumatic insult. His preterminal bleeding diathesis may well have contributed to the hemorrhagic narure of the spinal cord lesions encountered at autopsy. Hollis and colleagues 141 proposed (31-2 puncture as a safe and effective alternative to lumbar myelogra-
I12
Annals of Neurology
Vol 31
No 1 January 1992
phy in the study of patients with suspected subarachnoid block. None of their 50 patients so studied exhibited neurological decline after this procedure. Upward spinal coning, however, documented by Jooma and Hayward [ 6 )after ventricular drainage for hydrocephalus in patients with primary intracranial neoplasms and “drop” metastases at spinal levels, remains a theoretical complication of this approach. The present account suggests that MRI be considered as the initial diagnostic procedure in the patient with known neoplastic disease and any complaint referable to the spine or spinad neuraxis. We thank D r Jerome B. Posner for critical review of this manuscript.
References 1. Plum F, Posner JB. The diagnosis of stupor and coma. Philadelphia: FA Davis, 1980 2. Duffy GP. Lumbar puncture in the presence of raised inrracrariial pressure. Br Med J 1969;1:407-409 3. Eaton LM, Craig WM. Tumor of the spinal cord: sudden paralysis following lumbar puncture. Proc Staff Meet Mayo Clin
1940;15:170-172
4. Hollis PH, Malis LI, Zappulla RA. Neurological deterioration after lumbar puncture below complete spinal subarachnoid block. J Neurosurg 1986;64:253-256 5. Blatt I, Goldhammer Y. Deterioration after lumbar puncture below spinal block. J Neurosurg 1088;69:313-3 14 6. Jooma R, Hayward RD. Upward spinal coning: impaction of occult spinal tumours following relief of hydrocephalus. J Neuro Neurosurg Psychiatry 1984;47:386- 390 7. Elsberg CA. Surgical diseases of the spinal cord, membran’cs and nerve roots. London: H K Lewis, 1942:598 8. McAlhany HJ, Nersky MG. Compression of the spinal cord by extramedullary neoplasms. J Neuropathol Exp Nenrol 1955, 141276-287 9. Barron KD, Hirano A, Araki S, Terry RD. Experiences with metastatic neoplasms involving the spinai cord Neurology 1959;9:91-106 10. Kakulas BA, Harper CG, Shibasaki K, Bedbrook GM. Vertebral metastases and spinal cord comprebsion. Clin Exp Neurol 1978;15:98-113 11. Hashizumc Y , lljima S, Kishimoto H, tlirano A. Pencil shapi.4 softening of the spinal cord. Acra Neuroparhol (Rerl) IV3;(5l: 219-224 12. Drucker TB. Experimental injury of the spinal cord. I r i - Vink’en PJ, Bruyn GW, eds. Handbook of clinical neurolology, vol 2 5 : injuries of the spine and spinal cord. Amsterdam. NnrrhHolland, 19760-26
Acute paraplegia complicating lumbar puncture i n a leukemic patient with an unsuspected epidural chloroma is described, including the postmortem findings. Lumbar puncture can precipitate irreversible injury to the spinal cord in the patient w i t h a n occult lesion causing subarachnoid block. T h i s account, i n addition to documenting this phenomenon, suggests i i potential advantage to magnetic resonance imaging over myelography. Wong MC, Krol G, Rosenblurn MK. Occult epidural chloroma complicated by acute paraplegia following lumbar puncture. Ann Neurol 1992;11.110-112
Herniation syndromes have been associated with space-occupying lesions producing intracranial hypertension and pressure differentials across neural compartments { 11. T h a t acute neurological deterioration may be precipitated by lumbar puncture in this setting is well known [Z], but lesions i m p e d h g t h e flow of cerebrospinal fluid (CSF) at spinal levels may also create significant pressure differentials potentially aggravated by this procedure and complicated by spinal cord dysfunction { 3- 51. Catastrophic myelopath y, however, remains an infrequently documented p h e n o m e n o n after lumbar puncture in patients with epidural masses causing subarachnoid block [4, 5). We describe t h e clinical, radiological, and pathological features of acute paraplegia complicating lumbar puncture in a patient with unsuspected epidural spinal cord compression.
Case Report A 64-year-old man with a history of myelodysplastic syndrome and biopsy-proved cutaneous chloromas was admitted to Memorial Hospital (New York, NY) complaining of fatigue, myalgias, headache, and impaired concentration. Physi-
From the Departments of ‘Neurology, tRadiology (Neuroradiology), and iPathology (Neuropathology ), Memorial Sloan-Kettering Cancer Centcr, New York, N Y . Received Apt 18, 1931, and in revised form Jul 17. Accepted for publication Jul 21. 1991. Address correspondence to Dr Rosenblum, Departmen:: of Pathologv (Neuroparhology), Memorial Sloan-Kettering Cancer Center, 1275 York Avc, Mew York, NY 10021.
I10 Copyright
(c)
cal examination revealed pallor, multiple cutaneous papules, splenomegaly, and mild cognitive dysfunction. Significant admission laboratory studies included a complete blood count demonstrating a white blood cell (WBC) count of 27.9 K/ pl with 18% blasts, hemoglobin 6.4/dl, hematocrit 21.2%, platelets 7 1 Kiwi, and a lumbar puncture with WBC 6/mmi, red blood cell (RBC) Oimm’, glucose 73 mgidl, protein 30 mgidl, and negative cytology. Cranial magnetic resonance imaging (MRI) was unremarkable. The impression was of myelodysplastic syndrome in transition to acute myeloblastic leukemia and metabolic encephalopathy. A subsequent bone marrow biopsy was consistent with acute myeloblastic leukemia. Over the next several days, the patient was intermittently confused and complained of vague lumbar backache. Physical examination was unchanged save for slight tenderness to percussion over the lumbar spine. Despite treatment with cytosine P-D-arabinofuranoside ( Ara-C; 20 mg/m2). his WBC rose steadily to reach 136 K/kl by the seventh hospital day. Lumbar puncture was repeated at this time and approximately 3 ml of CSF obtained. The upening pressure was less than 20 mm of water, WBC 5/mmi, RBC 3/mm‘, protein 246 mgidl, glucose 88 mg/dl, and cytology again negative. Minutes after completing this procedure, the patient complained of weakness of both legs and pain radiating bilaterally from the lower back to the upper abdomen. Within 2 hours he was paraplegic with T-8 sensory level to all modalitie:?and loss of sphincter function. Myelogram at L2-3 was unsuccessful ( n o flow of CSF). A cervical myelogram at C1-2 revealed complete block with the upper level at the seventh thoracic vertebral body. MRI demonstrated pronounced compression of the spinal cord by a mass filling the posterior spinal canal from T-7 to T-9 (Fig 1). Prominence of the paraspinal soft tissues at T-8 was also noted after contrast administration. The patient was treated with high-dose steroids, spinal radiation therapy, and chemotherapy ( Ara-C 1 gm/mL and daunomycin 102 mg), but his neurological status did not improve. Repeat MRI on the sixteenth hospital day demonstrated the mass, reduced in size but still occupying 50(%of the diameter of the spinal canal. He became pancytopenic, developed sepsis and disseminated intravascular coagulation, and died 22 days after admission. The systemic autopsy revealed disseminated canclidiasis, hemorrhage into multiple organs, and necrotic leukemic infiltrates in the pericardium, pancreas, peripancreatic soft rissues, and bone marrow, which was virtually acellular. Foci of cerebral subarachnoid hemorrhage were noted and the thecal sac was distended by clotted blood. As these lesions show’ed no histological evidence of organization, they were judged agonal phenomena. At the midrhoracic level, a necrotic m~iss adhered to the epidural aspect of the spinal cord, extending over 3 cm of its length. The adjacent cord was softened and discolored, its gross architecture obliterated (Fig 2). Histological examination confirmed the mass was composed of leukemic cells and revealed complete transverse coagulative necrosis of the cord at this level. A solitary thrombosed intr;imedullary vein was identified here, the blood vessels otherwise remarkable solely for congestion. There was no leukemic or fungal involvement of the cord proper. The necrotic zone tapered caudally to a hemorrhagic pencil-shaped softening involving the basal posterior columns and left posterior
1992 by the American Neurological Association
Fig 2. These horizontal sections of the spinal cord demonstrate transerve necrosis at thoracic levels associated with remnants of necrotic chloroma adherent t o the epidural aspect of the meninges (arrow). Rostval levels exhibit central hemorrhagic necrosis and a circumscribed zone of necrosis is also evident at the base of the posterior columns in caudal sections. The cauda equina are embedded in clotted blood.
A
horn. The rostra1 portion of the cord evidenced central hemorrhagic necrosis.
Discussion
B Fig 1. (A,Bi TI -weighted sagittal (A) and axial (B) magnetic resonance imaging scans showing a posterior epidural soft tissue mass at the level of T-7 to T-9, obliterating the subarachnoid Jpace and compressing the spinal cord (arrows). The normal marrow signal has been replaced with low signal consistent with difluse leukemic znfiltration.
Neurological deterioration is a recognized complication of lumbar puncture in the setting of complete spinal subarachnoid block [3-51. This phenomenon, originally described some 50 years ago by Eaton and Craig 13] and subsequently referred to as “spinal coning” [GI, may be more common than is generally appreciated. Hollis and colleagues [4] noted a significant decline in motor function after lumbar myelography in 7 of 50 consecutively studied patients found to have complete block, 3 of whom also developed sphincter dysfunction. In 6 instances, the offending lesion was an epidural mass, including 4 metastatic carcinomas and single examples of osteoblastoma and abscess. Only their Patient 2, however, seems to have suffered a precipitous and catastrophic course similar to that we describe. This patient, a 67-year-old man with epidural spinal cord compression and T-4 block secondary to metastatic prostatic adenocarcinoma, progressed from relatively mild weakness to paraplegia within 30 minutes of undergoing lumbar puncture. The remaining patients deteriorated over a 1 to 4 day interval after myelography. The brief report of Blatt and Goldhammer [5] is the only other published account of acute paraplegia associated with lumbar puncture in the setting of epidural spinal cord compression. Their patient, a 68-year-old woman with low thoracic back pain, weakness of both legs, and a thoracic sensory level,
Brief Communication: Wong et al: Paraplegia after Lumbar Puncture
11 1
progressed to irreversible paraplegia within 2 hours of undergoing a lumbar myelogram that demonstrated an epidural mass causing complete subarachnoid block at T-11. Subsequent evaluation disclosed “.disseminated malignancy” that was not further characterized. The close temporal association of lumbar puncture with neurological decline in these patients [4, 51 would appear to implicate this procedure as the cause of socalled spinal coning. The withdrawal of CSF (and, in some instances, continued leakage from the puncture site) may precipitate impaction of the spinal cord in such patients by aggravating pressure differentials across the compressed segment and, further, by depriving the cord of a critical volume of fluid buffering it from the neighboring mass 171. The patient we describe demonstrates that this process may rapidly evolve to irreversihle injury in the patient with little or no evidencc: of preexistent myelopathy. The neuropathology of epidural spinal cord compression is subject to great individual variation, but is most often characterized by circumscribed foci of axonal degeneration and demyelination in the lateral and posterior columns with relative preservation of the anterior columnns and gray matter 18-10]. Complete transverse necrosis, central hemorrhagic necrosis, and pencil-shaped softening, although well documented in this setting [lo, 111, are less frequent finding:; and considered by some authors more characteristic of acute mechanical trauma [lo]. In addition, central hemorrhagic necrosis is a particularly prominent feature of some experimental models of acute impacr injury to the spinal cord E12). The pathogenesis of these varied patterns of spinal cord damage remains to be elucidated, but the time course of the compressive insult, typically subacute or chronic in the case of epidural neoplasia, may be a determinant of the final histopathological picture. We speculate that lumbar puncture precipitated acute and severe spinal cord impaction in our patient, resulting in a pattern of tissue injury commonly observed in the cord subjected to sudden traumatic insult. His preterminal bleeding diathesis may well have contributed to the hemorrhagic narure of the spinal cord lesions encountered at autopsy. Hollis and colleagues 141 proposed (31-2 puncture as a safe and effective alternative to lumbar myelogra-
I12
Annals of Neurology
Vol 31
No 1 January 1992
phy in the study of patients with suspected subarachnoid block. None of their 50 patients so studied exhibited neurological decline after this procedure. Upward spinal coning, however, documented by Jooma and Hayward [ 6 )after ventricular drainage for hydrocephalus in patients with primary intracranial neoplasms and “drop” metastases at spinal levels, remains a theoretical complication of this approach. The present account suggests that MRI be considered as the initial diagnostic procedure in the patient with known neoplastic disease and any complaint referable to the spine or spinad neuraxis. We thank D r Jerome B. Posner for critical review of this manuscript.
References 1. Plum F, Posner JB. The diagnosis of stupor and coma. Philadelphia: FA Davis, 1980 2. Duffy GP. Lumbar puncture in the presence of raised inrracrariial pressure. Br Med J 1969;1:407-409 3. Eaton LM, Craig WM. Tumor of the spinal cord: sudden paralysis following lumbar puncture. Proc Staff Meet Mayo Clin
1940;15:170-172
4. Hollis PH, Malis LI, Zappulla RA. Neurological deterioration after lumbar puncture below complete spinal subarachnoid block. J Neurosurg 1986;64:253-256 5. Blatt I, Goldhammer Y. Deterioration after lumbar puncture below spinal block. J Neurosurg 1088;69:313-3 14 6. Jooma R, Hayward RD. Upward spinal coning: impaction of occult spinal tumours following relief of hydrocephalus. J Neuro Neurosurg Psychiatry 1984;47:386- 390 7. Elsberg CA. Surgical diseases of the spinal cord, membran’cs and nerve roots. London: H K Lewis, 1942:598 8. McAlhany HJ, Nersky MG. Compression of the spinal cord by extramedullary neoplasms. J Neuropathol Exp Nenrol 1955, 141276-287 9. Barron KD, Hirano A, Araki S, Terry RD. Experiences with metastatic neoplasms involving the spinai cord Neurology 1959;9:91-106 10. Kakulas BA, Harper CG, Shibasaki K, Bedbrook GM. Vertebral metastases and spinal cord comprebsion. Clin Exp Neurol 1978;15:98-113 11. Hashizumc Y , lljima S, Kishimoto H, tlirano A. Pencil shapi.4 softening of the spinal cord. Acra Neuroparhol (Rerl) IV3;(5l: 219-224 12. Drucker TB. Experimental injury of the spinal cord. I r i - Vink’en PJ, Bruyn GW, eds. Handbook of clinical neurolology, vol 2 5 : injuries of the spine and spinal cord. Amsterdam. NnrrhHolland, 19760-26
