Spinal angiomas The meaning of cerebrospinal
fluid lactic acid in the etiology and differential
diagnosis of transverse
myelopathy
W.H.J.P. Linssen*, K.J.B. Lamers*
A.P.H.
Jansen*,
F.J.M.
GabreCls*,
H.O.M.
Thijssen*
*,
Introduction Summary
Transverse myelopathy (TM) can be caused by a number of disorders including infectionsL‘4, multiple sclerosis (MS)3,4, auto-immune disorders as systemic lupus erythemathosus5g6, mixed connective tissue disease7*8and progressive systemic sclerosis’, traumatic spinal cord lesions, space occupying lesions and spinal vascular malformations.*O Spinal angiomas (SA) represent 3% to 15% of all spinal space occupying lesions”~‘*. The majority of SA is located in the lower thoracic or thoracolumbar region 11,13*14, though they can occur throughout the length of the spinal cord. Symptoms may arise at all ages, but most frequently occur between 30 and 70 years of age. There is a 4: 1 male predominance”. The clinical diagnosis of SA remains difficult. Usually the onset of myelopathy due to SA is gradual and shows an intermittent progressive course. Predisposing factors are exercise, posture, environmental temperature, trauma, infections, pregnancy and menstrual periods16~17. Commonly there are combined upper and lower motor neuron manifestations with non-radicular sensory deficits14,18. Accompagnying signs to SA are spine deformity, a spinal or paraspinal bruit and cutaneous angiodysplasia 14*“.The diagnosis of SA is made
Institute of Neurology*
and Department of Neuroradiology’
Address for correspondence: Nijmegen, The Netherlands.
We report five out of eleven patients with transverse myelopathy due to spinal angiomas. Transverse myelopathy can be caused by a number of disorders. The formation of lactic acid points to ischaemia within the spinal cord, due to altered haemodynamics as in spinal angiomas. The meaning of cerebrospinal fluid lactic acid is discussed with respect to etiology and differential diagnosis of transverse myelopathy. The combined finding of CSF lactic acid and protein elevation in the absence of cell count and immunological and/or serological abnormalities points to an isolated vascular cause of the transverse myelopathy. Key words: Spinal angioma, cerebrospinal fluid lactic acid, ischaemia, haemodynamics.
on clinical presentation, (CT-)myelography and confirmed by selective spinal angiography. In recent years magnetic resonance imaging (MRI) will replace (CT-)myelography in cases of large angiomas. We describe five out of eleven patients with SA in detail and discuss the meaning of lactate in the etiology and differential diagnosis of transverse myelopathy.
*, University Hospital Nijmegen, The Netherlands
F.J. M. Gabret%, Institute of Neurology,
University Hospital Nijmegen, P.O. Box 9101, SO0 HB
Accepted 29.9.89 Clin Neural Neurosurg 1990. Vol. 92-2
131
Case reports Case 1 (BF)
A 51-year-old male started complaining about sacral pains five years ago. The pain was steadily intensifying. Coughing did not aggravate his complaints. The last four years progressive difficulties in walking appeared. When walking he initially experienced no problems but after a few minutes he became stumbling. Later on the patient developed difficulties in micturition, defaecation and sexual potency. On neurological examination the muscular volume was reduced, the muscle tone was lessened and strength had decreased in both legs. Fasciculations were seen. In the arms no abnormalities were found. The sensory qualities were disturbed below L3L4 level. The arm reflexes were normal, while knee and ankle reflexes were diminished. Abdominal skin reflexes were absent. Plantar reflexes were extensor on both sides. Radicular signs were negative. Standard blood and urine analysis, including serum lactic acid were normal. CSF abnormalities are summarized in Table 1. A diagnosis of conus-cauda syndrome was made with a sensory level at L3. The thoracolumbar myelography showed an arterio-venous malformation located between Th2 and Th9. Probably the AVM was extending to the lower cervical cord. Subclavian arteriography disclosed no abnormal vasculature of the cervical spinal cord. Selective spinal angiography showed
an AVM at Th6 level, located dorsally on the cord. The AVM was fed by two intercostal arteries arising from Th6 and Th7. The greater radicular artery lied on the anterior surface of the cord and was fed by two arteries coming from Th8 and L2. At the medullary conus no abnormal vasculature was found. The patient refused operation.
Case 2 (HK)
A 52-year-old male experienced progressive weakness in his left leg, later on in both lower extremities, over four years. During the same period he gradually suffered sensory loss and burning feelings in both legs. Micturition, defaecation and sexual functions became disturbed. On admission the neurological examination revealed a spastic paraparesis with muscular atrophy. Exteroceptive sensory functions were disturbed below the dermatome level Ll on the right, and below L2 on the left side. Reflexes were increased bilaterally and plantar responses were extensor. The sphincters were malfunctioning. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. Myelography of the thoracic spinal cord revealed irregular filling defects located dorsally in the spinal canal, suspected for vascular malformations. Selective spinal arteriography re-
Table 1. Cerebrospinal fluid findings in 11 patients with spinal angioma Patient
Sex
Age
White cells
Total protein (mg/l)
Lactic acid (ymol/l
IgC index
Oligoclonal bands*
BF HK AP AD AS HL KH SB ws HK PK
M M M M F M M M M M M
51 52 34 50 17 58 64 15 48 45 60
82 N N N N N 176 N N 32 N
2350 670 11400 410 500 930 900 505 800 952 936
2840 2340 3160 2320 1760 2115 2180 1700 2590 2440 4200
nd N N N N N + N N N N
nd
+ _ -
Reference values (P5-P95 estimated percentiles): white cell count, O-15/3 mm3; total protein content, 245-460 mg/l; lactic acid, 1380-1900 pmoU1; IgG index, 0.36-0.62. * Gamma oligoclonal bands in protein electrophoresis on cellulose acetate. M = male, F = female, N = normal, + = increased, - = not present, nd = not determined.
132
vealed a massive arteriovenous malformation (AVM), ranging from Th6 to Th9. After embolisation of the main supplying artery, laminectomy was performed and the AVM was excised in toto. Post-operatively the patient exhibited a complete myelosection at the level ThlO, which didn’t improve. Case 3 (AP) A 34-year-old male developed sensory disturbances in his right leg and a spastic paraparesis over a ten year period. He experienced attacks of low back pain with numbness in both legs and gradually developed sphincter disturbances. Neurological examination showed a spastic gait pattern, paresis and muscular atrophy in both legs. There was hypaesthesia and hypalgesia in dermatomes Ll - L3 and the lateral side of the right leg. Reflexes were increased bilaterally with clonus in the right leg. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. Conventional myelography showed serpiginous filling defects. Selective spinal angiography confirmed the presence of an SA at the level Th12 - L2, which was supplied by the artery of Adamkiewicz. After embolisation of this SA, the hypertonic paraparesis showed a slight improvement and his sphincteric malfunction cured. Case 4 (AD) This 50-year-old male had already complaints about low back pain for years, when he experienced burning feelings and loss of discrimination in changes in temperature in his feet. He also showed urinary frequency and urge-incontinence. After six months he developed a severe hypertonic paraparesis with widebased walking. Neurological examination showed a healthy looking male with sensory disturbances below LClevel. Reflexes of the legs were brisk, showing knee and ankle clonus bilaterally. Plantar reflexes were extensor while abdominal skin, anal and cremasteric reflexes were absent. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are given in Table 1. Thoracic myelography showed irregular curling vessels, dorsally and medially located at
Th12 level. Selective spinal arteriography revealed an AVM, fed by the first lumbar artery and descending into a dorsally medullary vein (Fig. 1). After laminectomy and clipping of the SA at the Th12 level the patient made an excellent recovery. He left the hospital with a slight paraparesis but walked unaided. Micturition returned to normal.
Fig. 1. Spinal angiography. Selective injection of the 8th intercostal artery on the left side. Filling of a large radiculomedullary artery (Adamkiewics) and a spinal angioma at Th12 level.
Case 5 (AS)
A 17-year-old female experienced progressive thoracic scoliosis with low back pain for several years when she was first seen. Previously, there were two episodes with acute severe low back pain, accompanied by nuchal rigidity, headache, and paresis of her right leg. Neurological examination revealed a thoracic scoliosis and a hypertonic paraparesis. Reflexes were increased in her legs showing clonus. The right side was more affected than the left. Plantar reflexes were extensor on both sides. A sensory level could be markated at thoracic 10. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. A thoraco-lumbar myelography revealed a arteriovenous malformation with an incomplete block at ThlO level. Selective spinal arteriography showed a SA, located at Th9 - ThlO. The angioma seemed supplied by the intercostal arteries from Th8 till Th12 and by a spinal artery Ll. After laminectomy the SA was only partially removed because of intramedullary venous connections. The patient made a good recovery and only a slight spastic paresis of her right leg remained. After six years she was readmitted because of an acute onset relapse with severe low back pain, radiating in her legs, nuchal rigidity and progressive paraparesis. Again a selective spinal angiography was performed, which showed a SA at Th9 - ThlO level. After a second operation our patient developed an incomplete transsection of the spinal cord, which did not improve. Discussion Spinal angiomas (SA) or arteriovenous malformations (AVM) are circumscribed collections of blood vessels, abnormal in both structure and number. They can be located extra - as well as intradurally, and extra - as well as intramedullary “-19. They represent a developmental anomaly of the spinal vascular system with a persistence of a primitive vascular pattern with altered haemodynamicsi3. Angiographic studies have indicated that they consist essentially of a arteriovenous shunt without an intervening capillary plexus’4J0. The 134
feeding arterial blood vessel appears to be solitary in most cases. Located along the peripheral nerve root sleeves, the feeding vessel penetrates the spinal dural sleeve. From here on the close association with the nerve root is lost and the feeder reaches the dorsum of the cord, where it anastomoses with a collection of abnormal vessels. From the SA small perforating vessels run into the spinal cord tissuezl. The venous drainage of the intra-medullary angiomas is via the anterior and posterior intra-medullar and spinal veins. Frequently multiple dilated tortuosities are located along the course of these spinal veins’*. The clinical picture is a syndrome of progressive neurological deterioration tending towards a transsection of the cord, which may cause considerable difficulties in diagnosis especially in the early stages. The symptoms show combined upper and lower motor neuron signs with non-radicular sensory deficits 14,16 . About 50% of the patients is complaining of low back pain*l. The onset of symptoms may show insidious progressive paraparesis, episodic paraparesis, sudden impairment of motor function with rapidly developing signs of complete section of the spinal cord, or spinal subarachnoid haemorrhage14,20,23. Intraspinal haemorrhage is uncommonzl. The clinical symptoms of our eleven patients show no essential differences with the clinical findings of others (Table 2). As can be deduced from the findings in case 1, the level of myelopathy is not compatible with the angiographic localisation of the SA. This latter finding offers a clue to the etiology of the myelopathy and accompagnied complaints about low back pain. The discrepancy between the localisation of the angioma and the neurological signs at a distance from it, suggests that damage to the nerve fibers may be caused by ischaemia probably due to either an arterial steal syndrome’9 or due to raised venous pressure*‘. Grossly, about 50% of the patients is complaining of some sort of back pain, either radicular or non-radicular (Table 2). These complaints may be due to the altered haemodynamits resulting in tissue hypoxia and nerve fibre ischaemia. Logue et al. 21conclude that the nerve fibre hypoxia is caused by a prolonged increase
Table 2. Clinical features in our patients with spinal angioma compared with clinical features in patients from the literature
Pain: radicular non-radicular local back pain Leg weakness Muscular atrophy Sensory disturbances: radicular non-radicular cord level Disturbances of: micturition defaecation
Aminofr4
Tobin”
Pia”
(n=60)
(n=71)
(n=97)
48% 17% 55% 95% 68% 90% 3% 87%
17% 10% 23% 75% 11%
44% 44% 58% 60% 27%
13% 54% 55%
11%
93% 65%
52% 24%
of venous pressure or to compression within the spinal cord due to intrinsic dilated venules. In our opinion a full examination of the cerebrospinal fluid will support .the above mentioned view, since the neurological signs and symptoms in SA refer to a certain level of myelopathy, without giving any clues to the etiological differential diagnosis. The CSF is abnormal in 75% of cases of SA. Most authors found a normal cell count together with an increased protein content14,“. Except for an increased protein content and normal cell count, we also found a moderate increased CSF lactic acid in more than 80% of our patients, confirming neuronal tissue ischaemia’l. The differential diagnosis of a progressive myelopathy accompagnied with a raised CSF lactic acid, includes disorders such as specific meningitis, meningitis carcinomatosa, sarcomatoss, and leucaemica, multiple sclerosis, systemic lupus erythemathosus and infective tranverse myelitis. An increase of the CSF lactic acid content is also found in patients suffering mitochondriopathies and in patients with space occupying lesions of the CNSz4,*‘. A full CSF examination, including white cell count, total protein, lactic acid and search for humoral immunological abnormalities enables further differentiation. After exclusion of meningitis (white cell count and differentiation, abnormal cells, lactic acid and protein), MS, SLE, or infective myelitis (immunological and serological tests), an increased CSF protein and lactic acid content in the absence of a clear increase of the white cell number, and of immunological and/or serological abnormalities,
64%
54%
Our patients (n=ll) 40% 10% 60% 100% 60% 100% 10% 90% 70% 100% 30%
should raise suspicion of a spinal angioma or space occupying lesion. Myelography and/or MRI will further differentiate. Although MRI will replace’ myelography in cases of large spinal angiomas, the advanced radiological methods of myelography and selective spinal angiography are invasive, technically demanding and take considerable amounts of time and radiation26. A more precise indication for these advanced neuroradiological investigations may be achieved after a full CSF examination. If the amount of CSF lactic acid is elevated it indicates amongst others ischaemia within the central nervous system and one should consider SA as a possible cause of transverse myelopathy. However, if the CSF lactate is within the normal range, this finding does not exclude SA with certainty and further neuroradiological investigations will be necessary. The value of a raised CSF lactic acid content remains debatable but it certainly offers clues to the differential and etiological diagnosis of transverse myelopathy caused by spinal angiomas. Acknowledgement We wish to thank O.R. Van Eikema Hommes, MD, PhD,
for critically reading the manuscript.
References 1
2 3
WM, ZONISJ,MINNICH LL. Epstein-Barrvirusassociated myelopathy in an adult. Arch Neural 1984; 41:454-5. MONTER MD. Querschnittmyelitis bei infektiliser Mononukleose. Med Klin 1969; 84:1752-5. PLUM F, OLSON ME. Myelitis and myelopathy. In: BAKER FEINBERG
135
4
’ 6
AB, BAKER LH, eds. Clinical Neurology, Vol. 3. Hagerstown: Harper and Row, 1980. ROPPERAH,POSKANZERDC.T~~ prognosisofacute and subacute transverse myelopathy based on early signs and symptoms. Ann Nemo1 1978; 451-9. PENN AS, ROWAN AJ. Myelopathy in systemic lupus erythemathosus. Arch Neurol 1968; 18:337-9. LINSSEN WHJP, FISELIER THJW, GABREfLS
FJM, WEVERS
Acute transverse myelopathy as the initial manifestation of probable systemic lupus erythemathosus in a child. Neuropediatrics 1988; 19:212-5. RA,CUPPENMPMJ,ROT-TEVEELIJ.
7
WEISSTD,NELSONJS,WOOLSEYRM,ZUCKNERJ,BALDAS-
SARE AR. Transverse myelitis in mixed connective tissue disease. Arthritis Rheum 1978; 21:982-6. 8 PEDERSENC,BONEN H,BOESENF. Transversemyelitisin mixed connective tissue disease. Clin Rheumatol 1987; 6:290-2. 9 BROWN JJ,MURPHY MJ. Transverse myelopathy in progressive systemic sclerosis. Ann Neural 1985; 17:615-7. ” ALTROCCHI PH. Acute transverse myelopathy. Arch Neurol 1963; 9:21-9. I1 DJINDJLAN R. Angiography in angiomas of the spinal cord. In PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:98-136. 12 PADOVANIR,GAISTG,PIAZZAG,PAZATTIE,FAGIOLOIL,
Arteriovenous malformations of the spinal cord: Surgical treatment of seven cases. J Neurosurg Sci 1981; 25:2126. JELLINGER K. Pathology of spinal VRSCUlIIImalformations and vascular tumors. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:18-44. AMINOFF MJ, LOGUE v. Clinical features of spinal vascular malformations. Brain 1974; 97:197-200. AMINOFFMJ,BARNARDRO,LOGUEV.T~~ pathofysiology of spinal vascular malformations. J Neural Sci 1974; 23:255-63. KENDALL BE, LOGUE v. Spinal epidural angiomatous malformations draining into intrathecal veins. NeuroraLAGHI D.
I3
I4 I5
I6
136
diol 1977; 13:181-9. PrAHw. Symptomatology of spinal angiomas. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:48-74. TOBIN WD, LAYTON DD. The diagnosis and natural history of spinal cord arteriovenous malformations. Mayo Clin Proc 1976; 51:637-47. DJINDJIANM,DJINDJlANR,HURTHM,HOUDARTR,REYA.
Steal phenomena in spinal arteriovenous malformations. .I Neuroradiol 1978; 5:187-201. DJINDJIAN M. Clinical symptomatology and natural history of arteriovenous malformations of the spinal cord. In: PIA NW, DJINDJIAN R. eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:75-83. LOGUE v. Angiomas of the spinal cord: Review of the pathogenesis, clinical features, and results of surgery. J Neurol Neurosurg Psychiatry 1979; 42:1-11. DJINDJIAN R. Angiography in vascular malformations of the spinalcord.In: DJINDJIAN R,HURTH M,HOUDARTR, eds. Angiography of the spinal cord. Paris: Masson et Cie, 1970:107-296. RICHE MC,MODENESI-FREITASJ,DJINDJIAN
M,MERLAND
JJ.Arteriovenous
malformations (AVM) of the spinal cord in children. J Neuroradiol 1982; 22:171-80. DUINKERKE JCN,
SJ,GABREI%SFJM,BOERBOOMSAMTH,
KOK
Can determination of lactic acid and pyruvic acid in cerebrospinal fluid help in diagnosing central nervous system involvement in systemic lupus erythemathosus? Clin Neural Neurosurg 1983; 85:22530. TOURTELLOITE ww. Cerebrospinal fluid in multiple sclerosis. In: VINKEN PI,BRUYN GW, eds. Handbook of Clinical Neurology. Vol. 9. Amsterdam: Elsevier-North Holland, 1972:326-S. AGN~LIAL,LAUNA,PIAHW,V~GEL.SANGH. Radiologic findings in spinal angiomas: Plain X-rays, myelography and spinal phlebography. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:84-97. RENIER wo.
fluid lactic acid in the etiology and differential
diagnosis of transverse
myelopathy
W.H.J.P. Linssen*, K.J.B. Lamers*
A.P.H.
Jansen*,
F.J.M.
GabreCls*,
H.O.M.
Thijssen*
*,
Introduction Summary
Transverse myelopathy (TM) can be caused by a number of disorders including infectionsL‘4, multiple sclerosis (MS)3,4, auto-immune disorders as systemic lupus erythemathosus5g6, mixed connective tissue disease7*8and progressive systemic sclerosis’, traumatic spinal cord lesions, space occupying lesions and spinal vascular malformations.*O Spinal angiomas (SA) represent 3% to 15% of all spinal space occupying lesions”~‘*. The majority of SA is located in the lower thoracic or thoracolumbar region 11,13*14, though they can occur throughout the length of the spinal cord. Symptoms may arise at all ages, but most frequently occur between 30 and 70 years of age. There is a 4: 1 male predominance”. The clinical diagnosis of SA remains difficult. Usually the onset of myelopathy due to SA is gradual and shows an intermittent progressive course. Predisposing factors are exercise, posture, environmental temperature, trauma, infections, pregnancy and menstrual periods16~17. Commonly there are combined upper and lower motor neuron manifestations with non-radicular sensory deficits14,18. Accompagnying signs to SA are spine deformity, a spinal or paraspinal bruit and cutaneous angiodysplasia 14*“.The diagnosis of SA is made
Institute of Neurology*
and Department of Neuroradiology’
Address for correspondence: Nijmegen, The Netherlands.
We report five out of eleven patients with transverse myelopathy due to spinal angiomas. Transverse myelopathy can be caused by a number of disorders. The formation of lactic acid points to ischaemia within the spinal cord, due to altered haemodynamics as in spinal angiomas. The meaning of cerebrospinal fluid lactic acid is discussed with respect to etiology and differential diagnosis of transverse myelopathy. The combined finding of CSF lactic acid and protein elevation in the absence of cell count and immunological and/or serological abnormalities points to an isolated vascular cause of the transverse myelopathy. Key words: Spinal angioma, cerebrospinal fluid lactic acid, ischaemia, haemodynamics.
on clinical presentation, (CT-)myelography and confirmed by selective spinal angiography. In recent years magnetic resonance imaging (MRI) will replace (CT-)myelography in cases of large angiomas. We describe five out of eleven patients with SA in detail and discuss the meaning of lactate in the etiology and differential diagnosis of transverse myelopathy.
*, University Hospital Nijmegen, The Netherlands
F.J. M. Gabret%, Institute of Neurology,
University Hospital Nijmegen, P.O. Box 9101, SO0 HB
Accepted 29.9.89 Clin Neural Neurosurg 1990. Vol. 92-2
131
Case reports Case 1 (BF)
A 51-year-old male started complaining about sacral pains five years ago. The pain was steadily intensifying. Coughing did not aggravate his complaints. The last four years progressive difficulties in walking appeared. When walking he initially experienced no problems but after a few minutes he became stumbling. Later on the patient developed difficulties in micturition, defaecation and sexual potency. On neurological examination the muscular volume was reduced, the muscle tone was lessened and strength had decreased in both legs. Fasciculations were seen. In the arms no abnormalities were found. The sensory qualities were disturbed below L3L4 level. The arm reflexes were normal, while knee and ankle reflexes were diminished. Abdominal skin reflexes were absent. Plantar reflexes were extensor on both sides. Radicular signs were negative. Standard blood and urine analysis, including serum lactic acid were normal. CSF abnormalities are summarized in Table 1. A diagnosis of conus-cauda syndrome was made with a sensory level at L3. The thoracolumbar myelography showed an arterio-venous malformation located between Th2 and Th9. Probably the AVM was extending to the lower cervical cord. Subclavian arteriography disclosed no abnormal vasculature of the cervical spinal cord. Selective spinal angiography showed
an AVM at Th6 level, located dorsally on the cord. The AVM was fed by two intercostal arteries arising from Th6 and Th7. The greater radicular artery lied on the anterior surface of the cord and was fed by two arteries coming from Th8 and L2. At the medullary conus no abnormal vasculature was found. The patient refused operation.
Case 2 (HK)
A 52-year-old male experienced progressive weakness in his left leg, later on in both lower extremities, over four years. During the same period he gradually suffered sensory loss and burning feelings in both legs. Micturition, defaecation and sexual functions became disturbed. On admission the neurological examination revealed a spastic paraparesis with muscular atrophy. Exteroceptive sensory functions were disturbed below the dermatome level Ll on the right, and below L2 on the left side. Reflexes were increased bilaterally and plantar responses were extensor. The sphincters were malfunctioning. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. Myelography of the thoracic spinal cord revealed irregular filling defects located dorsally in the spinal canal, suspected for vascular malformations. Selective spinal arteriography re-
Table 1. Cerebrospinal fluid findings in 11 patients with spinal angioma Patient
Sex
Age
White cells
Total protein (mg/l)
Lactic acid (ymol/l
IgC index
Oligoclonal bands*
BF HK AP AD AS HL KH SB ws HK PK
M M M M F M M M M M M
51 52 34 50 17 58 64 15 48 45 60
82 N N N N N 176 N N 32 N
2350 670 11400 410 500 930 900 505 800 952 936
2840 2340 3160 2320 1760 2115 2180 1700 2590 2440 4200
nd N N N N N + N N N N
nd
+ _ -
Reference values (P5-P95 estimated percentiles): white cell count, O-15/3 mm3; total protein content, 245-460 mg/l; lactic acid, 1380-1900 pmoU1; IgG index, 0.36-0.62. * Gamma oligoclonal bands in protein electrophoresis on cellulose acetate. M = male, F = female, N = normal, + = increased, - = not present, nd = not determined.
132
vealed a massive arteriovenous malformation (AVM), ranging from Th6 to Th9. After embolisation of the main supplying artery, laminectomy was performed and the AVM was excised in toto. Post-operatively the patient exhibited a complete myelosection at the level ThlO, which didn’t improve. Case 3 (AP) A 34-year-old male developed sensory disturbances in his right leg and a spastic paraparesis over a ten year period. He experienced attacks of low back pain with numbness in both legs and gradually developed sphincter disturbances. Neurological examination showed a spastic gait pattern, paresis and muscular atrophy in both legs. There was hypaesthesia and hypalgesia in dermatomes Ll - L3 and the lateral side of the right leg. Reflexes were increased bilaterally with clonus in the right leg. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. Conventional myelography showed serpiginous filling defects. Selective spinal angiography confirmed the presence of an SA at the level Th12 - L2, which was supplied by the artery of Adamkiewicz. After embolisation of this SA, the hypertonic paraparesis showed a slight improvement and his sphincteric malfunction cured. Case 4 (AD) This 50-year-old male had already complaints about low back pain for years, when he experienced burning feelings and loss of discrimination in changes in temperature in his feet. He also showed urinary frequency and urge-incontinence. After six months he developed a severe hypertonic paraparesis with widebased walking. Neurological examination showed a healthy looking male with sensory disturbances below LClevel. Reflexes of the legs were brisk, showing knee and ankle clonus bilaterally. Plantar reflexes were extensor while abdominal skin, anal and cremasteric reflexes were absent. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are given in Table 1. Thoracic myelography showed irregular curling vessels, dorsally and medially located at
Th12 level. Selective spinal arteriography revealed an AVM, fed by the first lumbar artery and descending into a dorsally medullary vein (Fig. 1). After laminectomy and clipping of the SA at the Th12 level the patient made an excellent recovery. He left the hospital with a slight paraparesis but walked unaided. Micturition returned to normal.
Fig. 1. Spinal angiography. Selective injection of the 8th intercostal artery on the left side. Filling of a large radiculomedullary artery (Adamkiewics) and a spinal angioma at Th12 level.
Case 5 (AS)
A 17-year-old female experienced progressive thoracic scoliosis with low back pain for several years when she was first seen. Previously, there were two episodes with acute severe low back pain, accompanied by nuchal rigidity, headache, and paresis of her right leg. Neurological examination revealed a thoracic scoliosis and a hypertonic paraparesis. Reflexes were increased in her legs showing clonus. The right side was more affected than the left. Plantar reflexes were extensor on both sides. A sensory level could be markated at thoracic 10. Standard blood and urine analysis, including serum lactic acid were normal. CSF findings are presented in Table 1. A thoraco-lumbar myelography revealed a arteriovenous malformation with an incomplete block at ThlO level. Selective spinal arteriography showed a SA, located at Th9 - ThlO. The angioma seemed supplied by the intercostal arteries from Th8 till Th12 and by a spinal artery Ll. After laminectomy the SA was only partially removed because of intramedullary venous connections. The patient made a good recovery and only a slight spastic paresis of her right leg remained. After six years she was readmitted because of an acute onset relapse with severe low back pain, radiating in her legs, nuchal rigidity and progressive paraparesis. Again a selective spinal angiography was performed, which showed a SA at Th9 - ThlO level. After a second operation our patient developed an incomplete transsection of the spinal cord, which did not improve. Discussion Spinal angiomas (SA) or arteriovenous malformations (AVM) are circumscribed collections of blood vessels, abnormal in both structure and number. They can be located extra - as well as intradurally, and extra - as well as intramedullary “-19. They represent a developmental anomaly of the spinal vascular system with a persistence of a primitive vascular pattern with altered haemodynamicsi3. Angiographic studies have indicated that they consist essentially of a arteriovenous shunt without an intervening capillary plexus’4J0. The 134
feeding arterial blood vessel appears to be solitary in most cases. Located along the peripheral nerve root sleeves, the feeding vessel penetrates the spinal dural sleeve. From here on the close association with the nerve root is lost and the feeder reaches the dorsum of the cord, where it anastomoses with a collection of abnormal vessels. From the SA small perforating vessels run into the spinal cord tissuezl. The venous drainage of the intra-medullary angiomas is via the anterior and posterior intra-medullar and spinal veins. Frequently multiple dilated tortuosities are located along the course of these spinal veins’*. The clinical picture is a syndrome of progressive neurological deterioration tending towards a transsection of the cord, which may cause considerable difficulties in diagnosis especially in the early stages. The symptoms show combined upper and lower motor neuron signs with non-radicular sensory deficits 14,16 . About 50% of the patients is complaining of low back pain*l. The onset of symptoms may show insidious progressive paraparesis, episodic paraparesis, sudden impairment of motor function with rapidly developing signs of complete section of the spinal cord, or spinal subarachnoid haemorrhage14,20,23. Intraspinal haemorrhage is uncommonzl. The clinical symptoms of our eleven patients show no essential differences with the clinical findings of others (Table 2). As can be deduced from the findings in case 1, the level of myelopathy is not compatible with the angiographic localisation of the SA. This latter finding offers a clue to the etiology of the myelopathy and accompagnied complaints about low back pain. The discrepancy between the localisation of the angioma and the neurological signs at a distance from it, suggests that damage to the nerve fibers may be caused by ischaemia probably due to either an arterial steal syndrome’9 or due to raised venous pressure*‘. Grossly, about 50% of the patients is complaining of some sort of back pain, either radicular or non-radicular (Table 2). These complaints may be due to the altered haemodynamits resulting in tissue hypoxia and nerve fibre ischaemia. Logue et al. 21conclude that the nerve fibre hypoxia is caused by a prolonged increase
Table 2. Clinical features in our patients with spinal angioma compared with clinical features in patients from the literature
Pain: radicular non-radicular local back pain Leg weakness Muscular atrophy Sensory disturbances: radicular non-radicular cord level Disturbances of: micturition defaecation
Aminofr4
Tobin”
Pia”
(n=60)
(n=71)
(n=97)
48% 17% 55% 95% 68% 90% 3% 87%
17% 10% 23% 75% 11%
44% 44% 58% 60% 27%
13% 54% 55%
11%
93% 65%
52% 24%
of venous pressure or to compression within the spinal cord due to intrinsic dilated venules. In our opinion a full examination of the cerebrospinal fluid will support .the above mentioned view, since the neurological signs and symptoms in SA refer to a certain level of myelopathy, without giving any clues to the etiological differential diagnosis. The CSF is abnormal in 75% of cases of SA. Most authors found a normal cell count together with an increased protein content14,“. Except for an increased protein content and normal cell count, we also found a moderate increased CSF lactic acid in more than 80% of our patients, confirming neuronal tissue ischaemia’l. The differential diagnosis of a progressive myelopathy accompagnied with a raised CSF lactic acid, includes disorders such as specific meningitis, meningitis carcinomatosa, sarcomatoss, and leucaemica, multiple sclerosis, systemic lupus erythemathosus and infective tranverse myelitis. An increase of the CSF lactic acid content is also found in patients suffering mitochondriopathies and in patients with space occupying lesions of the CNSz4,*‘. A full CSF examination, including white cell count, total protein, lactic acid and search for humoral immunological abnormalities enables further differentiation. After exclusion of meningitis (white cell count and differentiation, abnormal cells, lactic acid and protein), MS, SLE, or infective myelitis (immunological and serological tests), an increased CSF protein and lactic acid content in the absence of a clear increase of the white cell number, and of immunological and/or serological abnormalities,
64%
54%
Our patients (n=ll) 40% 10% 60% 100% 60% 100% 10% 90% 70% 100% 30%
should raise suspicion of a spinal angioma or space occupying lesion. Myelography and/or MRI will further differentiate. Although MRI will replace’ myelography in cases of large spinal angiomas, the advanced radiological methods of myelography and selective spinal angiography are invasive, technically demanding and take considerable amounts of time and radiation26. A more precise indication for these advanced neuroradiological investigations may be achieved after a full CSF examination. If the amount of CSF lactic acid is elevated it indicates amongst others ischaemia within the central nervous system and one should consider SA as a possible cause of transverse myelopathy. However, if the CSF lactate is within the normal range, this finding does not exclude SA with certainty and further neuroradiological investigations will be necessary. The value of a raised CSF lactic acid content remains debatable but it certainly offers clues to the differential and etiological diagnosis of transverse myelopathy caused by spinal angiomas. Acknowledgement We wish to thank O.R. Van Eikema Hommes, MD, PhD,
for critically reading the manuscript.
References 1
2 3
WM, ZONISJ,MINNICH LL. Epstein-Barrvirusassociated myelopathy in an adult. Arch Neural 1984; 41:454-5. MONTER MD. Querschnittmyelitis bei infektiliser Mononukleose. Med Klin 1969; 84:1752-5. PLUM F, OLSON ME. Myelitis and myelopathy. In: BAKER FEINBERG
135
4
’ 6
AB, BAKER LH, eds. Clinical Neurology, Vol. 3. Hagerstown: Harper and Row, 1980. ROPPERAH,POSKANZERDC.T~~ prognosisofacute and subacute transverse myelopathy based on early signs and symptoms. Ann Nemo1 1978; 451-9. PENN AS, ROWAN AJ. Myelopathy in systemic lupus erythemathosus. Arch Neurol 1968; 18:337-9. LINSSEN WHJP, FISELIER THJW, GABREfLS
FJM, WEVERS
Acute transverse myelopathy as the initial manifestation of probable systemic lupus erythemathosus in a child. Neuropediatrics 1988; 19:212-5. RA,CUPPENMPMJ,ROT-TEVEELIJ.
7
WEISSTD,NELSONJS,WOOLSEYRM,ZUCKNERJ,BALDAS-
SARE AR. Transverse myelitis in mixed connective tissue disease. Arthritis Rheum 1978; 21:982-6. 8 PEDERSENC,BONEN H,BOESENF. Transversemyelitisin mixed connective tissue disease. Clin Rheumatol 1987; 6:290-2. 9 BROWN JJ,MURPHY MJ. Transverse myelopathy in progressive systemic sclerosis. Ann Neural 1985; 17:615-7. ” ALTROCCHI PH. Acute transverse myelopathy. Arch Neurol 1963; 9:21-9. I1 DJINDJLAN R. Angiography in angiomas of the spinal cord. In PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:98-136. 12 PADOVANIR,GAISTG,PIAZZAG,PAZATTIE,FAGIOLOIL,
Arteriovenous malformations of the spinal cord: Surgical treatment of seven cases. J Neurosurg Sci 1981; 25:2126. JELLINGER K. Pathology of spinal VRSCUlIIImalformations and vascular tumors. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:18-44. AMINOFF MJ, LOGUE v. Clinical features of spinal vascular malformations. Brain 1974; 97:197-200. AMINOFFMJ,BARNARDRO,LOGUEV.T~~ pathofysiology of spinal vascular malformations. J Neural Sci 1974; 23:255-63. KENDALL BE, LOGUE v. Spinal epidural angiomatous malformations draining into intrathecal veins. NeuroraLAGHI D.
I3
I4 I5
I6
136
diol 1977; 13:181-9. PrAHw. Symptomatology of spinal angiomas. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:48-74. TOBIN WD, LAYTON DD. The diagnosis and natural history of spinal cord arteriovenous malformations. Mayo Clin Proc 1976; 51:637-47. DJINDJIANM,DJINDJlANR,HURTHM,HOUDARTR,REYA.
Steal phenomena in spinal arteriovenous malformations. .I Neuroradiol 1978; 5:187-201. DJINDJIAN M. Clinical symptomatology and natural history of arteriovenous malformations of the spinal cord. In: PIA NW, DJINDJIAN R. eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:75-83. LOGUE v. Angiomas of the spinal cord: Review of the pathogenesis, clinical features, and results of surgery. J Neurol Neurosurg Psychiatry 1979; 42:1-11. DJINDJIAN R. Angiography in vascular malformations of the spinalcord.In: DJINDJIAN R,HURTH M,HOUDARTR, eds. Angiography of the spinal cord. Paris: Masson et Cie, 1970:107-296. RICHE MC,MODENESI-FREITASJ,DJINDJIAN
M,MERLAND
JJ.Arteriovenous
malformations (AVM) of the spinal cord in children. J Neuroradiol 1982; 22:171-80. DUINKERKE JCN,
SJ,GABREI%SFJM,BOERBOOMSAMTH,
KOK
Can determination of lactic acid and pyruvic acid in cerebrospinal fluid help in diagnosing central nervous system involvement in systemic lupus erythemathosus? Clin Neural Neurosurg 1983; 85:22530. TOURTELLOITE ww. Cerebrospinal fluid in multiple sclerosis. In: VINKEN PI,BRUYN GW, eds. Handbook of Clinical Neurology. Vol. 9. Amsterdam: Elsevier-North Holland, 1972:326-S. AGN~LIAL,LAUNA,PIAHW,V~GEL.SANGH. Radiologic findings in spinal angiomas: Plain X-rays, myelography and spinal phlebography. In: PIA HW, DJINDJIAN R, eds. Advances in diagnosis and therapy. Berlin, Heidelberg, New York: Springer Verlag, 1978:84-97. RENIER wo.
