Sudden hypotonic paraparesis caused by tophaceous gout of the lumbar spine M. Vervaeck*, J. D e Keyser*, P. Pauwels**, N. F r e c o u r t * * * * , J. D ' H a e n s * * * , and G. Ebinger*
Case report Summary The patient, a 52-year old man, had a 25 years history of gouty arthritis involving the metatarsal phalangeal joints, ankles, knees and fingers, with tophi on both hands and feet. He also complained of chronic low back pain. Previous treatment had consisted of intermittent courses of colchicine and indomethacin for acute episodes of arthritis. Allopurinol had also been prescribed for some years, but the patient took it only episodically. There was no family history of gout. His medical history further included a mild arterial hypertension, for which no treatment was given. Three days before admission the patient suddenly experienced an intense pain in the lower back when lifting a bucket. The pain did not radiate into the lower extremities. On the next day he experienced a sudden loss of strength in his legs, and he fell to the ground. He had no sensory symptoms and sphincter control remained normal. Neurologic examination disclosed bilateral paresis of the hip flexors, knee extensors and adductors of the thigh. Pain sensation, position and vibration sense were intact. The knee jerk reflexes were absent, the ankle jerks were weak. Superficial abdominal reflexes were all present. Plantar responses were flexor. Laboratory findings were as followed: hemo-
Gouty arthritis of the axial skeleton is rare, and neurological complications due to spinal cord or nerve root compression secondary to tophaceous deposits have been seldom reported. We describe a patient with chronic gouty arthritis who developed an acute paraparesis caused by tophaceous cauda equina compression. Surgical removal of the mass resulted in complete recovery. Key words: Tophaceous gout, cauda equina, paraparesis, hyperuricemia.
globin 7.6 mmol/L; white blood cell count 6.3.109/L with a normal differential cell count; hematocrit 0.36; platelets 145.109/L; glucose 4.8 mmol/L; urea nitrogen 12.1 mmol/L; creatinine 103 micromol/L; uric acid 625 micromol/L; calcium 2.22 mmol/L; sodium 144 mmol/L; potasskium 4.0 mmol/L; total protein 64 g/L; cholesterol 4.03 mmol/L; biluribin 11.9 micromol/L; erythrocyte sedimentation rate 64 mm/h. Plain radiographs of the lumbar spina showed narrowing of the intervertebral spaces at all levels, but especially at L4-L5 on the right and L5-S1 on the left side, with signs of hyperostotic spondylosis. There was no collapse or destruction of the vertebrae. A computerized tomogram of the lumbosa-
*Department of Neurology, * *Department of Surgery, * * *Department of Neurosurgery, ** * *Department of Pathological Anatomy. University Hospital, Vrije Universiteit Brussel, Brussels, Belgium. Address for correspondence and reprint requests: M. Vervaeck, M.D., Department of Neurology, A.Z. V. U.B., Laarbeeklaan 101, B-1090 Brussels, Belgium. Accepted 21-8-90 Clin Neurol Neurosurg 1991. Vol. 93-3
233
cral spine showed a narrow lumbar spinal canal at all levels and suggested a central protusion of the disc at the level of L2-L3. Marked degenerative~changes were noted with osteophytes and narrowing of the intervertebral foramina on both sides, especially at the levels L4-L5 and L5-S1. A technetium bone scan demonstrated osteoblastic foci at both hands and feet, the left knee and the lumbar spine. Lumbar myelography revealed a complete block at the level of L2-L3 (Figure 1). The cerebrospinal fluid was clear with a glucose level of 3.4 mmol/L, protein 0.55 g/L, lactate 2.1 mmol/L, 2 red blood cells and 2 white blood cells 106/L. In view of these findings we strongly suspected that the paraparesis was due to a compression of the cauda equina at the level of L2-L3 secondary to a herniated disc. A laminectomy performed at L2-L3, however, revealed a chalky material surrounding the dural sac. The ligamentum flavum was also thickened by this pasty material. Histological examination disclosed a granulomatous infiltrate consisting of multinucleated giant cells and histiocytes surrounding an amor-
Fig. 2: Histological section demonstrating amorphous deposits surrounded by a granulomatous reaction containing foreign body giant cells (HES, x 120).
Fig. 1: Anteroposterior lumbar myelogram showing a complete interruption of the flow of contrast material at the level of L2-L3.
234
phous material, consistent with tophaceous gout (Figur e 2). Postoperatively, the patient regained normal muscle strength of the lower extremities within the following ten days. Discussion
Monosodium urate crystal deposits in joints and adjacent soft tissue complicate frequently longstanding, poorly controlled hyperuricemia. However, axial tophi are rare, and neurological complications are even more uncommon. There exist only 10 reports in the literature of
spinal cord or r o o t compression by tophi and they are listed in Table 11"10. Eight of the previously reported patients were males, with ages ranging from 33 to 76 years. Seven patients had a long history, of severe polyarticular tophaceous gout. The remainder did not show peripheral tophi and had no history of painful gouty arthritis except for arthritis of the first metatarsal phalangeal joint. Hyperuricemia was present in alle cases. The spinal column can be affected over its entire length, and neurologi symptoms are related to the level of the tophaceous deposits. The
Table 1. neurologic
level tophi
uric acid
Sex; Age; duration of gout (years)
Clinical description of gout
symptoms
1
M;44;12
severe polyart. ; tophi;
backpain Progressive paraparesis; urinary retention
sensory level Tll; paraplegia hyperactive reflexes
T9-T11
642
2
M;73 ;25
severe; polyart.; no tophi
progressive paraparesis
sensation intact; paraparesis; absent reflexes legs
L3-L5
297-446
3
M;74;10
not available
backpain; radiculopathy; progressive paraparesis
sensory impairment legs; distal paraparesis; absent ankle jerks
L4-L5
422-630
4
F;50;29
diagnosis of RA
backpain; proges, paraparesis; radiculopathy; sphincter dysfunction
sensory level T6-T8; paraparesis; absent reflexes legs; radiculopathy C6 right arm
C1-C2 T2-T9
666
5
M;61 ;"long history"
severe; polyart. ; tophi;
quadriparesis; urinary incontinence
spastic quadriparesis; sensory level T3
C3-C4
559
6
M;67,14
severe; polyart.; tophi
radiculopathy; neck pain
C5-C7
535
7
M;33 ;5
severe; polyart. ; tophi
backpain; progr, paraparesis; paresthesias; urinary retention
radiculopathy fight arm; absent biceps reflex sensory level T7; paraplegia; hyperact, reflexes
T7-T11
535
8
F;76;nnknown to havegout
mild; no tophi
back pain; radiculopathy fight leg; footdrop
distal paraparesis; radiculopathy; absent ankle jerk right
L4-S1
512
9
M;69;unknown
none; no tophi
progressive paraparesis; no backpain
spastic paraparesis; hyperact, reflexes; sensation impaired
occipitocervical
535
10
M;53;4
severe; polyartic; tophi
neck pain progressive quadriparesis
spastic quadriparesis; sensation impaired
C1-C2 type I1 odontoid fracture
1106
11
M;52;25
severe; polyartic; tophi
back pain; acute paraparesis
L2-L3 paraparesis; absent reflexes legs; no sensory impairment
Patient reference
examination
(micromol/L)
625
235
back or neck pain is also mostly noted at the corresponding affected level. Two patients suffered from quadriparesis, six had a paraparesis and two had a radiculopathy with paresis and paresthesias. All of them had deficits of a slowly progressive nature (up to several years). In contrast, our patient suffered from a paraparesis of acute onset, probably due to a sudden expulsion of tophaceous material into the extradural space. In contrast to the peripheral joints, wheregouty erosions may be seen, radiographs of the spine do not contribute to the diagnosis. Laminectomy reveals a characteristic mass of chalky or pasty material. Histologically, a granulomatous infiltrate of multinucleated giant cells and histiocytes is seen surrounding an amorphous, eosinophilic material. Because of dissolution of urate in aqueous solutions during fixation, only occasionally a 'shadow' of the crystals can be seen. The clinical characteristics of symptomatic tophi around the spinal cord may mimic a tumor or, as in our case, a protruded disc. We emphasize that, although rare, spinal cord or root compression secondary to tophaceous gout should be considered in any gout
236
patient, especially in those with severe longstanding tophaceous gout. References 1
2
3
KOSKOFF YD, MORRIS LE, LUBIC LG.
Paraplegiaa s
a com-
of gout. J A M A 1953; 152:37-38. LITVAK J, BRINEY W. E x t r a d u r a l spinal depositions o f urates producing paraplegia. J Neurosurg 1973; 39:656638. plication
REYNOLDS AF, WYLER AR, NORRIS HT.
Paraparesis s e c -
to sodium urate deposits in the ligamentum flavum. Arch Neurol 1976; 33:795. 4 MAGID SK, GRAY G E , ANAND A. Spinal cord compression by tophi in a patient with chronic polyarthritis: case report and literature review. Arthritis Rheum 1981; 24:1431-1434. 5 SEQUEIRA W, BOUFFARD A, SALGIA K, SKOSEY J. Quadriparesis in tophaceous gout. Arthritis Rheum 1981; 24:1428-1430. 6 MILLER JDR, PERCY JS. Tophaceousgout i n t h e c e r v i c a l spine. J Rheumatol 1984; 11:862-865. 7 LEANLY B J, CALVERT JM. Tophaceous gout producing spinal cord compression. J Neurosurg 1983; 58:580-582. 8 VARGA Jr GIAMPAOLO C, GOLDENBERG DL. Tophaeceous gout of the spine in a patient with no peripheral tophi: case report and review of the literature. Arthritis Rheum 1985; 28:1312-1315. ondary
9
VAN DE LAAR MAFJ~ VAN SOESBERGEN RM~ MATRICALI B.
10
Tophaceous gout of the cervical spine without peripheral tophi. Arthritis Rheum 1987; 30:237-238. JACOBS SR, EDEIKEN, J, RUBIN B, DEHORATIUS RJ. Medically reversible quadriparesis in tophaceous gout. Arch Phys Med Rehabil 1985; 66:188-190.
Case report Summary The patient, a 52-year old man, had a 25 years history of gouty arthritis involving the metatarsal phalangeal joints, ankles, knees and fingers, with tophi on both hands and feet. He also complained of chronic low back pain. Previous treatment had consisted of intermittent courses of colchicine and indomethacin for acute episodes of arthritis. Allopurinol had also been prescribed for some years, but the patient took it only episodically. There was no family history of gout. His medical history further included a mild arterial hypertension, for which no treatment was given. Three days before admission the patient suddenly experienced an intense pain in the lower back when lifting a bucket. The pain did not radiate into the lower extremities. On the next day he experienced a sudden loss of strength in his legs, and he fell to the ground. He had no sensory symptoms and sphincter control remained normal. Neurologic examination disclosed bilateral paresis of the hip flexors, knee extensors and adductors of the thigh. Pain sensation, position and vibration sense were intact. The knee jerk reflexes were absent, the ankle jerks were weak. Superficial abdominal reflexes were all present. Plantar responses were flexor. Laboratory findings were as followed: hemo-
Gouty arthritis of the axial skeleton is rare, and neurological complications due to spinal cord or nerve root compression secondary to tophaceous deposits have been seldom reported. We describe a patient with chronic gouty arthritis who developed an acute paraparesis caused by tophaceous cauda equina compression. Surgical removal of the mass resulted in complete recovery. Key words: Tophaceous gout, cauda equina, paraparesis, hyperuricemia.
globin 7.6 mmol/L; white blood cell count 6.3.109/L with a normal differential cell count; hematocrit 0.36; platelets 145.109/L; glucose 4.8 mmol/L; urea nitrogen 12.1 mmol/L; creatinine 103 micromol/L; uric acid 625 micromol/L; calcium 2.22 mmol/L; sodium 144 mmol/L; potasskium 4.0 mmol/L; total protein 64 g/L; cholesterol 4.03 mmol/L; biluribin 11.9 micromol/L; erythrocyte sedimentation rate 64 mm/h. Plain radiographs of the lumbar spina showed narrowing of the intervertebral spaces at all levels, but especially at L4-L5 on the right and L5-S1 on the left side, with signs of hyperostotic spondylosis. There was no collapse or destruction of the vertebrae. A computerized tomogram of the lumbosa-
*Department of Neurology, * *Department of Surgery, * * *Department of Neurosurgery, ** * *Department of Pathological Anatomy. University Hospital, Vrije Universiteit Brussel, Brussels, Belgium. Address for correspondence and reprint requests: M. Vervaeck, M.D., Department of Neurology, A.Z. V. U.B., Laarbeeklaan 101, B-1090 Brussels, Belgium. Accepted 21-8-90 Clin Neurol Neurosurg 1991. Vol. 93-3
233
cral spine showed a narrow lumbar spinal canal at all levels and suggested a central protusion of the disc at the level of L2-L3. Marked degenerative~changes were noted with osteophytes and narrowing of the intervertebral foramina on both sides, especially at the levels L4-L5 and L5-S1. A technetium bone scan demonstrated osteoblastic foci at both hands and feet, the left knee and the lumbar spine. Lumbar myelography revealed a complete block at the level of L2-L3 (Figure 1). The cerebrospinal fluid was clear with a glucose level of 3.4 mmol/L, protein 0.55 g/L, lactate 2.1 mmol/L, 2 red blood cells and 2 white blood cells 106/L. In view of these findings we strongly suspected that the paraparesis was due to a compression of the cauda equina at the level of L2-L3 secondary to a herniated disc. A laminectomy performed at L2-L3, however, revealed a chalky material surrounding the dural sac. The ligamentum flavum was also thickened by this pasty material. Histological examination disclosed a granulomatous infiltrate consisting of multinucleated giant cells and histiocytes surrounding an amor-
Fig. 2: Histological section demonstrating amorphous deposits surrounded by a granulomatous reaction containing foreign body giant cells (HES, x 120).
Fig. 1: Anteroposterior lumbar myelogram showing a complete interruption of the flow of contrast material at the level of L2-L3.
234
phous material, consistent with tophaceous gout (Figur e 2). Postoperatively, the patient regained normal muscle strength of the lower extremities within the following ten days. Discussion
Monosodium urate crystal deposits in joints and adjacent soft tissue complicate frequently longstanding, poorly controlled hyperuricemia. However, axial tophi are rare, and neurological complications are even more uncommon. There exist only 10 reports in the literature of
spinal cord or r o o t compression by tophi and they are listed in Table 11"10. Eight of the previously reported patients were males, with ages ranging from 33 to 76 years. Seven patients had a long history, of severe polyarticular tophaceous gout. The remainder did not show peripheral tophi and had no history of painful gouty arthritis except for arthritis of the first metatarsal phalangeal joint. Hyperuricemia was present in alle cases. The spinal column can be affected over its entire length, and neurologi symptoms are related to the level of the tophaceous deposits. The
Table 1. neurologic
level tophi
uric acid
Sex; Age; duration of gout (years)
Clinical description of gout
symptoms
1
M;44;12
severe polyart. ; tophi;
backpain Progressive paraparesis; urinary retention
sensory level Tll; paraplegia hyperactive reflexes
T9-T11
642
2
M;73 ;25
severe; polyart.; no tophi
progressive paraparesis
sensation intact; paraparesis; absent reflexes legs
L3-L5
297-446
3
M;74;10
not available
backpain; radiculopathy; progressive paraparesis
sensory impairment legs; distal paraparesis; absent ankle jerks
L4-L5
422-630
4
F;50;29
diagnosis of RA
backpain; proges, paraparesis; radiculopathy; sphincter dysfunction
sensory level T6-T8; paraparesis; absent reflexes legs; radiculopathy C6 right arm
C1-C2 T2-T9
666
5
M;61 ;"long history"
severe; polyart. ; tophi;
quadriparesis; urinary incontinence
spastic quadriparesis; sensory level T3
C3-C4
559
6
M;67,14
severe; polyart.; tophi
radiculopathy; neck pain
C5-C7
535
7
M;33 ;5
severe; polyart. ; tophi
backpain; progr, paraparesis; paresthesias; urinary retention
radiculopathy fight arm; absent biceps reflex sensory level T7; paraplegia; hyperact, reflexes
T7-T11
535
8
F;76;nnknown to havegout
mild; no tophi
back pain; radiculopathy fight leg; footdrop
distal paraparesis; radiculopathy; absent ankle jerk right
L4-S1
512
9
M;69;unknown
none; no tophi
progressive paraparesis; no backpain
spastic paraparesis; hyperact, reflexes; sensation impaired
occipitocervical
535
10
M;53;4
severe; polyartic; tophi
neck pain progressive quadriparesis
spastic quadriparesis; sensation impaired
C1-C2 type I1 odontoid fracture
1106
11
M;52;25
severe; polyartic; tophi
back pain; acute paraparesis
L2-L3 paraparesis; absent reflexes legs; no sensory impairment
Patient reference
examination
(micromol/L)
625
235
back or neck pain is also mostly noted at the corresponding affected level. Two patients suffered from quadriparesis, six had a paraparesis and two had a radiculopathy with paresis and paresthesias. All of them had deficits of a slowly progressive nature (up to several years). In contrast, our patient suffered from a paraparesis of acute onset, probably due to a sudden expulsion of tophaceous material into the extradural space. In contrast to the peripheral joints, wheregouty erosions may be seen, radiographs of the spine do not contribute to the diagnosis. Laminectomy reveals a characteristic mass of chalky or pasty material. Histologically, a granulomatous infiltrate of multinucleated giant cells and histiocytes is seen surrounding an amorphous, eosinophilic material. Because of dissolution of urate in aqueous solutions during fixation, only occasionally a 'shadow' of the crystals can be seen. The clinical characteristics of symptomatic tophi around the spinal cord may mimic a tumor or, as in our case, a protruded disc. We emphasize that, although rare, spinal cord or root compression secondary to tophaceous gout should be considered in any gout
236
patient, especially in those with severe longstanding tophaceous gout. References 1
2
3
KOSKOFF YD, MORRIS LE, LUBIC LG.
Paraplegiaa s
a com-
of gout. J A M A 1953; 152:37-38. LITVAK J, BRINEY W. E x t r a d u r a l spinal depositions o f urates producing paraplegia. J Neurosurg 1973; 39:656638. plication
REYNOLDS AF, WYLER AR, NORRIS HT.
Paraparesis s e c -
to sodium urate deposits in the ligamentum flavum. Arch Neurol 1976; 33:795. 4 MAGID SK, GRAY G E , ANAND A. Spinal cord compression by tophi in a patient with chronic polyarthritis: case report and literature review. Arthritis Rheum 1981; 24:1431-1434. 5 SEQUEIRA W, BOUFFARD A, SALGIA K, SKOSEY J. Quadriparesis in tophaceous gout. Arthritis Rheum 1981; 24:1428-1430. 6 MILLER JDR, PERCY JS. Tophaceousgout i n t h e c e r v i c a l spine. J Rheumatol 1984; 11:862-865. 7 LEANLY B J, CALVERT JM. Tophaceous gout producing spinal cord compression. J Neurosurg 1983; 58:580-582. 8 VARGA Jr GIAMPAOLO C, GOLDENBERG DL. Tophaeceous gout of the spine in a patient with no peripheral tophi: case report and review of the literature. Arthritis Rheum 1985; 28:1312-1315. ondary
9
VAN DE LAAR MAFJ~ VAN SOESBERGEN RM~ MATRICALI B.
10
Tophaceous gout of the cervical spine without peripheral tophi. Arthritis Rheum 1987; 30:237-238. JACOBS SR, EDEIKEN, J, RUBIN B, DEHORATIUS RJ. Medically reversible quadriparesis in tophaceous gout. Arch Phys Med Rehabil 1985; 66:188-190.
