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Diffuse central nervous system lupus involving white matter, basal ganglia, thalami and brainstem
Brain & Development 21 (1999) 337±340
Case report
Diffuse central nervous system lupus involving white matter, basal ganglia, thalami and brainstem Machiko Shibata a, Tetsuya Kibe a, Shinji Fujimoto a, b,*, Tatsuya Ishikawa a, Mayumi Murakami c, Takashi Ichiki c, Yoshiro Wada a b
a Department of Pediatric, Nagoya City University Medical School, Kawasumi, Mizuho±cho, Mizuho±ku, Nagoya 467±8601, Japan Department of Laboratory Medicine, Nagoya City University Medical School, Kawasumi, Mizuho±cho, Mizuho±ku, Nagoya 467±8601, Japan c Department of Pediatrics, Kainan Hospital, Yatomi±cho, Ama±gun, Aichi, Japan
Received 31 August 1998; received in revised form 15 February 1999; accepted 16 March 1999
Abstract We described an 11±year±old girl with acute central nervous system lupus showing diffuse lesions. She developed generalized convulsions followed by prolonged coma, and her psychomotor ability recovered fully after 3 months of steroid therapy. Cranial magnetic resonance imaging (MRI) showed high signal intensity in the cerebral deep white matter, bilateral basal ganglia, thalami, and brainstem on T2-weighted image. These lesions resolved over 1 month with residual atrophic change in the heads of the caudate nucleus on MRI. Acute SLE leukoencephalopathy may be recognized as a subtype of CNS lupus. q 1999 Elsevier Science B.V. All rights reserved. Keywords: Systemic lupus erythematosus; Magnetic resonance imaging; Basal ganglia; Acute encephalopathy; Central nervous system lupus
1. Introduction Central nervous system (CNS) involvement has been reported in 13-60% of children with systemic lupus erythematosus (SLE) in the course of the disease [1,2]. Psychiatric disorder, seizures, headache, and cerebral infarction are common clinical manifestations of CNS lupus in children [1]. We describe here an 11-year-old girl with acute CNS lupus with diffuse lesions including on the cerebral white matter, bilateral basal ganglia, thalami and brainstem.
2. Case report An 11 year-old Japanese girl had been in good health until May 1997, when a malar erythematous rash developed. In June 1997, she developed photosensitivity, discoid rash, stomatitis, and prolonged low-grade fever. She was admitted to a local hospital for examination and treatment on July 3rd, 1997. The patient's maternal aunt had SLE, but other members of the family were all in good health. Neuro* Corresponding author. Tel.: 1 81±52±853±8246; fax: 1 81±52±842± 3449. E-mail address: [email protected]±cu.ac.jp (S. Fujimoto)
logical examination on admission showed no abnormalities. Both antinuclear antibody and anti-dsDNA IgG were elevated, and total hemolytic complement (CH50), C3, and C4 were reduced. These ®ndings led to a diagnosis of SLE. Intravenous administration of prednisolone at a dose of 1 mg/kg per day was started on July 7th. Two days later, the patient began to feel dizzy on standing up and to complain of headache. Cranial magnetic resonance imaging (MRI) and computed tomography (CT) showed no abnormality on that day. On July 14th, the patient developed sudden generalized convulsions followed by prolonged unconsciousness, and she was transferred to the University Hospital. On admission, she barely responded to pain and showed involuntary movements of the extremities and mouth with ®xed open-eyes. Blood pressure was 124/78 mmHg, and temperature 36.28C. Neurological examination showed hyper-re¯exes in the upper and lower extremities with Babinski sign. Ophthalmoscopic examination showed no papilledema. Electroencephalogram (EEG) two days after CNS manifestation showed a marked suppression of cerebral activity (Fig. 1). Auditory brainstem response (ABR) and somatosensory evoked potential (SEP) showed normal ®ndings. Laboratory ®ndings were: hemoglobin 12.0 g/dl, red blood cell count 434 £ 104 /mm 3, white blood cell count 9:2 £ 103 /mm 3, platelet count 14:8 £ 104 /mm 3, erythrocyte
0387-7604/99/$ - see front matter q 1999 Elsevier Science B.V. All rights reserved. PII: S 0387-760 4(99)00027-3
338
M. Shibata et al. / Brain & Development 21 (1999) 337±340
Fig. 1. Electroencephalogram on July 16 showed periodic synchronous discharges associated with marked low voltage basic activity.
sedimentation rate (1 h) 20 mm, AST 371 U/l (standard: below 28 U/l), ALT 348 U/l (below 25 U/l), CK 586 U/l (10±180 U/l), LDH 1220 U/l (96±220 U/l). Coagulation tests including prothrombin time, activated partial thromboplastin time, and ®brinogen were within normal range. Immunological studies showed: CRP , 0:3 mg/dl, CH50 11.4 U/ml, C3 26 mg/dl, C4 6 mg/dl, anti-dsDNA antibody 58 IU/ml (below 12 IU/ml), anti-ssDNA antibody 120 IU/ml (below 25 IU/ml), anti-RNP antibody 23 IU/ml (below 7 IU/ ml), anti Sm antibody 16 IU/ml (below 5 IU/ml), anti-SSA antibody 58 IU/ml (below 7 IU/ml), interleukin 6 (IL-6) 102 pg/ml (below 4 pg/ml). No anti-phospholipid antibodies including lupus anticoagulant or anti cardiolipin-b 2-glycoprotein I antibody were found. Urinalysis was not remarkable. A lumbar puncture showed one cell/mm 3 under normal pressure, with a raised protein concentration of 96 mg/dl and IgG level of 12 mg/dl. An oligoclonal IgG band of cerebrospinal ¯uid (CSF) was negative. Based on the diagnosis of CNS lupus, intravenous highdose (1000 mg/day for 3 days) methylprednisolone was started from July 14, and thereafter intravenous and oral administrations of prednisolone at a dose of 1 mg/kg per day were continued. The patient responded to a stimulus of pain on July 21st and voluntary movements appeared on July 23rd. She responded to voices on July 27th and could talk and walk on July 31st, 16 days after CNS lupus onset.
Ophthalmoscopic examination on August 6th showed a central retinal artery occlusion in the left eye in addition to bilateral SLE retinopathies. Because of progressive retinal lesions and a skin rash, intravenous (500 mg/day for 3 days) methylprednisolone was started from August 28th. She made a full recovery in motor ability within one month and in mental ability over 3 months. Immunological studies including CH50, C3, C4, IL-6, and anti-dsDNA, anti-ssDNA, anti-RNP, and anti-Sm antibodies returned to normal range by 2 months. The EEG showed 4±6 Hz theta waves in background activity on July 22nd and almost normal ®nding on September 2nd. Serial neuroradiological studies were performed. Magnetic resonance imaging on July 15th showed high signal intensity in the bilateral basal ganglia and thalami on T2-weighted image (Fig. 2A). MRI showed high signal intensity in the deep white matter and pons in addition to the bilateral caudate heads, putamens, and thalami on T2-weighted image on July 24th (Fig. 2B) and August 8th (Fig. 2C). Slight low signal intensity areas were noted in the same areas on T1-weighted image (Fig. 2D). Cranial CT on August 18th demonstrated diffuse low density areas in the cerebral white matter. MRI on August 28th showed the same ®ndings on T1- and T2weighted images, and disclosed diffuse white matter lesions on ¯uid-attenuated inversion recovery (FLAIR) images (Fig. 2E). Follow-up MRI 6 months later showed a
M. Shibata et al. / Brain & Development 21 (1999) 337±340
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Fig. 2. Serial MRI ®ndings. (A) July 15. High signal intensity in the bilateral heads of caudate nucleus, putamens and thalami on T2-weighted image (TR 4,027/TE 112). (B) July 24. High signal intensity in the pons and deep white matter on T2-weighted image (TR 4,027/TE112). (C) August 8. High signal intensity in the bilateral heads of caudate nucleus, putamens, thalami and deep white matter on T2 weighted image (TR 4,000/TE 112). (D) August 8. Slight low signal intensity in the above-mentioned areas on T1-weighted image (TR 539/TE 15). (E) August 28. Abnormal signal intensity areas in bilateral heads of caudate nucleus, putamens and diffuse white matter on ¯uid-attenuated inversion recovery (FLAIR) images (TR6,000/TE 90). (F) Six months later. A decreased abnormal signal intensity areas, and atrophy of bilateral caudate heads on T2 weighted image (TR 4,000/TE 112).
decreased T2-signal abnormality, and atrophy of the bilateral caudate heads (Fig. 2F). Single-photon emission CT (SPECT) using 123I-IMP on July 24 disclosed a low perfusion area in the pons, and a second SPECT using 99mTc-ECD on August 14th showed multiple low perfusion areas in the medial sites of the bilateral frontal and temporal lobes, bilateral caudate heads and left thalamus as well. These ®ndings improved on October 13th. Neurological examination 1 year after the onset of CNS lupus showed no abnormality except for poor vision in the left eye. Her intelligence quotient was 90 by the Wechsler intelligence scale for children-revised.
3. Discussion The ®ndings of MRI, CT and clinical symptoms in our patient would suggest acute leukoencephalopathy. Only four patients with acute leukoencephalopathy in SLE, aged 16±47, have been reported [3±6] (Table 1). This is the ®rst case report of a child with acute SLE leukoencephalopathy. Although patient 4 in Table 1 was similar to our patient in clinical and CT ®ndings, MRI was not performed and long-term prognosis was unavailable [6]. Patient 4 clinically recovered gradually after methylprednisolone pulse therapy, and low-density lesions on CT disappeared 1 month later.
340
M. Shibata et al. / Brain & Development 21 (1999) 337±340
Table 1 Clinical and neuroradiological ®ndings in patients with acute SLE leukoencephalopathy a Patient Age/Sex CT ®ndings
MRI ®ndings
Neurological symptoms
Recovery after s teroid therapy
Reference
1
35/F
ND
Headache
Immediately
3
2
47/F
Headache
Immediately
4
3
43/F
5
16/?
Consciousness disturbance Coma tetraplegia convulsion
Gradually
4
diffuse high intensity in cerebral white matter on T2-WI Diffuse high intensity in cerebral white matter on T2-WI ND
Gradually
6
5
11/F
Gradually
Present report
a
Diffuse low density lesions in cerebral white matter ND Diffuse low density in cerebral white matter on T2-WI Diffuse atrophy and multiple low density matter, bilateral putamens external capsulae, claustrums and right thalamus Diffuse low density lesions in cerebral white matter
High intensity in the cerebral white Coma matter bilateral basal ganglia, tetraplegia thalami, and brainstem on T2-WI convulsion
F, female; ND, not done; WI, weighted images.
Neuroradiological ®ndings of our patient revealed two features. First, our patient showed multiple symmetrical lesions of basal ganglia, thalami and brainstem in addition to diffuse cerebral white matter. The brainstem lesion is particularly important, because it has not yet reported in acute SLE leukoencaphalopathy, and may be associated with coma and tetraplegia in our patient. Furthermore, our patient and patient 4 would suggest that young patients tend to have more extensive lesions. Second, repeated MRI in our patient demonstrated the combination of large reversible lesions and small irreversible ones. Pathological examination of CNS lupus has shown vasculopathy, vasculitis, infarcts, and hemorrhages [7,8]. The ®ndings of MRI and CT in our patient apparently differ from those of infarcts and hemorrhages. Furthermore, SPECT demonstrated multiple areas of decreased cerebral blood ¯ow. These ®ndings would suggest that the diffuse lesions consisted largely of focal edema together with a few irreversible disturbances of cerebral circulation, probably due to diffuse microangiopathy [5,8]. Despite extensive white matter lesions on MRI and marked suppression of EEG, the ABR and SEP in our patient showed normal ®ndings. This would indicate that at least some parts of white matter were preserved, but previous reports have not mentioned evoked potential. Benign intracranial hypertension (BIH) or pseudotumor cerebri secondary to SLE is also known to show abnormality in the diffuse white matter on CT and MRI, and is thought to be caused by the decrease of CSF absorption [9]. Because BIH is de®ned as elevated CSF pressure without spaceoccupying lesion or cerebrovascular disease, our patient was not diagnosed as having BIH. It is noteworthy that the patient under study showed full clinical recovery after steroid therapy, despite the child's severe clinical condition, marked suppression of EEG, and
extensive cerebral lesions. Other reports of acute SLE leukoencephalopathy also indicated a relatively good prognosis. Taken together, acute SLE leukoencephalopathy may be recognized as a relatively benign subtype of CNS lupus.
References [1] Steinlin MI, Blaser SI, Gilday DL, Eddy AA, Logan WJ, Laxer RM, et al. Neurologic manifestations of pediatric systemic lupus erythematosus. Pediatr. Neurol. 1995;13:191±197. [2] Reiff A, Miller J, Shaham B, Bernstein B, Szer IS. Childhood central nervous system lupus; longitudinal assessment using single photon emission computed tomography. J. Rheumatol. 1997;24:2461±2465. [3] Boulter M, Brink A, Mathias C, Peart S, Stevens J, Stewart G, et al. Unusual cranial and abdominal computed tomographic (CT) scan appearances in a case of systemic lupus erythematosus (SLE). Ann. Rheum. Dis. 1987;46:162±165. [4] Shintani S, Ono K, Hiroshita H, Shiigai T, Tsuruoka S. Unusual neuroradiological ®ndings on systemic lupus erythematosus. Eur. Neurol. 1993;33:13±16. [5] Isshi K, Hirohata S, Hashimoto T, Miyashita H. Systemic lupus erythematosus presenting with diffuse low density lesions in the cerebral white matter on computed axial tomography scans: its implication in the pathogenesis of diffuse central nervous system lupus. J. Rheumatol. 1994;21:1758±1762. [6] Kondoh H, Inuzuka T, Miyatake T, Satoh M, Arakawa M. Transient multiple low density area demonstrated by CT in systemic lupus erythematosus with central nervous system involvement (in Japanese). Shinkei Naika (Tokyo) 1987;27:357±360. [7] Johnson RT, Richardson EP. The neurological manifestations of systemic lupus erythematosus: a clinico-pathological study of 24 cases and review of the literature. Medicine 1968;47:337±369. [8] Van Dam AP. Diagnosis and pathogenesis of CNS lupus. Rheumatol. Int. 1991;11:1±11. [9] Ogura N, Atsumi T, Sagawa A, Jodo S, Amasaki Y, Nakabayashi T, et al. Systemic lupus erythematosus associated with benign intracranial hypertension: a case report (in Japanese). Ryumachi (Tokyo) 1992;32:66±72.
Case report
Diffuse central nervous system lupus involving white matter, basal ganglia, thalami and brainstem Machiko Shibata a, Tetsuya Kibe a, Shinji Fujimoto a, b,*, Tatsuya Ishikawa a, Mayumi Murakami c, Takashi Ichiki c, Yoshiro Wada a b
a Department of Pediatric, Nagoya City University Medical School, Kawasumi, Mizuho±cho, Mizuho±ku, Nagoya 467±8601, Japan Department of Laboratory Medicine, Nagoya City University Medical School, Kawasumi, Mizuho±cho, Mizuho±ku, Nagoya 467±8601, Japan c Department of Pediatrics, Kainan Hospital, Yatomi±cho, Ama±gun, Aichi, Japan
Received 31 August 1998; received in revised form 15 February 1999; accepted 16 March 1999
Abstract We described an 11±year±old girl with acute central nervous system lupus showing diffuse lesions. She developed generalized convulsions followed by prolonged coma, and her psychomotor ability recovered fully after 3 months of steroid therapy. Cranial magnetic resonance imaging (MRI) showed high signal intensity in the cerebral deep white matter, bilateral basal ganglia, thalami, and brainstem on T2-weighted image. These lesions resolved over 1 month with residual atrophic change in the heads of the caudate nucleus on MRI. Acute SLE leukoencephalopathy may be recognized as a subtype of CNS lupus. q 1999 Elsevier Science B.V. All rights reserved. Keywords: Systemic lupus erythematosus; Magnetic resonance imaging; Basal ganglia; Acute encephalopathy; Central nervous system lupus
1. Introduction Central nervous system (CNS) involvement has been reported in 13-60% of children with systemic lupus erythematosus (SLE) in the course of the disease [1,2]. Psychiatric disorder, seizures, headache, and cerebral infarction are common clinical manifestations of CNS lupus in children [1]. We describe here an 11-year-old girl with acute CNS lupus with diffuse lesions including on the cerebral white matter, bilateral basal ganglia, thalami and brainstem.
2. Case report An 11 year-old Japanese girl had been in good health until May 1997, when a malar erythematous rash developed. In June 1997, she developed photosensitivity, discoid rash, stomatitis, and prolonged low-grade fever. She was admitted to a local hospital for examination and treatment on July 3rd, 1997. The patient's maternal aunt had SLE, but other members of the family were all in good health. Neuro* Corresponding author. Tel.: 1 81±52±853±8246; fax: 1 81±52±842± 3449. E-mail address: [email protected]±cu.ac.jp (S. Fujimoto)
logical examination on admission showed no abnormalities. Both antinuclear antibody and anti-dsDNA IgG were elevated, and total hemolytic complement (CH50), C3, and C4 were reduced. These ®ndings led to a diagnosis of SLE. Intravenous administration of prednisolone at a dose of 1 mg/kg per day was started on July 7th. Two days later, the patient began to feel dizzy on standing up and to complain of headache. Cranial magnetic resonance imaging (MRI) and computed tomography (CT) showed no abnormality on that day. On July 14th, the patient developed sudden generalized convulsions followed by prolonged unconsciousness, and she was transferred to the University Hospital. On admission, she barely responded to pain and showed involuntary movements of the extremities and mouth with ®xed open-eyes. Blood pressure was 124/78 mmHg, and temperature 36.28C. Neurological examination showed hyper-re¯exes in the upper and lower extremities with Babinski sign. Ophthalmoscopic examination showed no papilledema. Electroencephalogram (EEG) two days after CNS manifestation showed a marked suppression of cerebral activity (Fig. 1). Auditory brainstem response (ABR) and somatosensory evoked potential (SEP) showed normal ®ndings. Laboratory ®ndings were: hemoglobin 12.0 g/dl, red blood cell count 434 £ 104 /mm 3, white blood cell count 9:2 £ 103 /mm 3, platelet count 14:8 £ 104 /mm 3, erythrocyte
0387-7604/99/$ - see front matter q 1999 Elsevier Science B.V. All rights reserved. PII: S 0387-760 4(99)00027-3
338
M. Shibata et al. / Brain & Development 21 (1999) 337±340
Fig. 1. Electroencephalogram on July 16 showed periodic synchronous discharges associated with marked low voltage basic activity.
sedimentation rate (1 h) 20 mm, AST 371 U/l (standard: below 28 U/l), ALT 348 U/l (below 25 U/l), CK 586 U/l (10±180 U/l), LDH 1220 U/l (96±220 U/l). Coagulation tests including prothrombin time, activated partial thromboplastin time, and ®brinogen were within normal range. Immunological studies showed: CRP , 0:3 mg/dl, CH50 11.4 U/ml, C3 26 mg/dl, C4 6 mg/dl, anti-dsDNA antibody 58 IU/ml (below 12 IU/ml), anti-ssDNA antibody 120 IU/ml (below 25 IU/ml), anti-RNP antibody 23 IU/ml (below 7 IU/ ml), anti Sm antibody 16 IU/ml (below 5 IU/ml), anti-SSA antibody 58 IU/ml (below 7 IU/ml), interleukin 6 (IL-6) 102 pg/ml (below 4 pg/ml). No anti-phospholipid antibodies including lupus anticoagulant or anti cardiolipin-b 2-glycoprotein I antibody were found. Urinalysis was not remarkable. A lumbar puncture showed one cell/mm 3 under normal pressure, with a raised protein concentration of 96 mg/dl and IgG level of 12 mg/dl. An oligoclonal IgG band of cerebrospinal ¯uid (CSF) was negative. Based on the diagnosis of CNS lupus, intravenous highdose (1000 mg/day for 3 days) methylprednisolone was started from July 14, and thereafter intravenous and oral administrations of prednisolone at a dose of 1 mg/kg per day were continued. The patient responded to a stimulus of pain on July 21st and voluntary movements appeared on July 23rd. She responded to voices on July 27th and could talk and walk on July 31st, 16 days after CNS lupus onset.
Ophthalmoscopic examination on August 6th showed a central retinal artery occlusion in the left eye in addition to bilateral SLE retinopathies. Because of progressive retinal lesions and a skin rash, intravenous (500 mg/day for 3 days) methylprednisolone was started from August 28th. She made a full recovery in motor ability within one month and in mental ability over 3 months. Immunological studies including CH50, C3, C4, IL-6, and anti-dsDNA, anti-ssDNA, anti-RNP, and anti-Sm antibodies returned to normal range by 2 months. The EEG showed 4±6 Hz theta waves in background activity on July 22nd and almost normal ®nding on September 2nd. Serial neuroradiological studies were performed. Magnetic resonance imaging on July 15th showed high signal intensity in the bilateral basal ganglia and thalami on T2-weighted image (Fig. 2A). MRI showed high signal intensity in the deep white matter and pons in addition to the bilateral caudate heads, putamens, and thalami on T2-weighted image on July 24th (Fig. 2B) and August 8th (Fig. 2C). Slight low signal intensity areas were noted in the same areas on T1-weighted image (Fig. 2D). Cranial CT on August 18th demonstrated diffuse low density areas in the cerebral white matter. MRI on August 28th showed the same ®ndings on T1- and T2weighted images, and disclosed diffuse white matter lesions on ¯uid-attenuated inversion recovery (FLAIR) images (Fig. 2E). Follow-up MRI 6 months later showed a
M. Shibata et al. / Brain & Development 21 (1999) 337±340
339
Fig. 2. Serial MRI ®ndings. (A) July 15. High signal intensity in the bilateral heads of caudate nucleus, putamens and thalami on T2-weighted image (TR 4,027/TE 112). (B) July 24. High signal intensity in the pons and deep white matter on T2-weighted image (TR 4,027/TE112). (C) August 8. High signal intensity in the bilateral heads of caudate nucleus, putamens, thalami and deep white matter on T2 weighted image (TR 4,000/TE 112). (D) August 8. Slight low signal intensity in the above-mentioned areas on T1-weighted image (TR 539/TE 15). (E) August 28. Abnormal signal intensity areas in bilateral heads of caudate nucleus, putamens and diffuse white matter on ¯uid-attenuated inversion recovery (FLAIR) images (TR6,000/TE 90). (F) Six months later. A decreased abnormal signal intensity areas, and atrophy of bilateral caudate heads on T2 weighted image (TR 4,000/TE 112).
decreased T2-signal abnormality, and atrophy of the bilateral caudate heads (Fig. 2F). Single-photon emission CT (SPECT) using 123I-IMP on July 24 disclosed a low perfusion area in the pons, and a second SPECT using 99mTc-ECD on August 14th showed multiple low perfusion areas in the medial sites of the bilateral frontal and temporal lobes, bilateral caudate heads and left thalamus as well. These ®ndings improved on October 13th. Neurological examination 1 year after the onset of CNS lupus showed no abnormality except for poor vision in the left eye. Her intelligence quotient was 90 by the Wechsler intelligence scale for children-revised.
3. Discussion The ®ndings of MRI, CT and clinical symptoms in our patient would suggest acute leukoencephalopathy. Only four patients with acute leukoencephalopathy in SLE, aged 16±47, have been reported [3±6] (Table 1). This is the ®rst case report of a child with acute SLE leukoencephalopathy. Although patient 4 in Table 1 was similar to our patient in clinical and CT ®ndings, MRI was not performed and long-term prognosis was unavailable [6]. Patient 4 clinically recovered gradually after methylprednisolone pulse therapy, and low-density lesions on CT disappeared 1 month later.
340
M. Shibata et al. / Brain & Development 21 (1999) 337±340
Table 1 Clinical and neuroradiological ®ndings in patients with acute SLE leukoencephalopathy a Patient Age/Sex CT ®ndings
MRI ®ndings
Neurological symptoms
Recovery after s teroid therapy
Reference
1
35/F
ND
Headache
Immediately
3
2
47/F
Headache
Immediately
4
3
43/F
5
16/?
Consciousness disturbance Coma tetraplegia convulsion
Gradually
4
diffuse high intensity in cerebral white matter on T2-WI Diffuse high intensity in cerebral white matter on T2-WI ND
Gradually
6
5
11/F
Gradually
Present report
a
Diffuse low density lesions in cerebral white matter ND Diffuse low density in cerebral white matter on T2-WI Diffuse atrophy and multiple low density matter, bilateral putamens external capsulae, claustrums and right thalamus Diffuse low density lesions in cerebral white matter
High intensity in the cerebral white Coma matter bilateral basal ganglia, tetraplegia thalami, and brainstem on T2-WI convulsion
F, female; ND, not done; WI, weighted images.
Neuroradiological ®ndings of our patient revealed two features. First, our patient showed multiple symmetrical lesions of basal ganglia, thalami and brainstem in addition to diffuse cerebral white matter. The brainstem lesion is particularly important, because it has not yet reported in acute SLE leukoencaphalopathy, and may be associated with coma and tetraplegia in our patient. Furthermore, our patient and patient 4 would suggest that young patients tend to have more extensive lesions. Second, repeated MRI in our patient demonstrated the combination of large reversible lesions and small irreversible ones. Pathological examination of CNS lupus has shown vasculopathy, vasculitis, infarcts, and hemorrhages [7,8]. The ®ndings of MRI and CT in our patient apparently differ from those of infarcts and hemorrhages. Furthermore, SPECT demonstrated multiple areas of decreased cerebral blood ¯ow. These ®ndings would suggest that the diffuse lesions consisted largely of focal edema together with a few irreversible disturbances of cerebral circulation, probably due to diffuse microangiopathy [5,8]. Despite extensive white matter lesions on MRI and marked suppression of EEG, the ABR and SEP in our patient showed normal ®ndings. This would indicate that at least some parts of white matter were preserved, but previous reports have not mentioned evoked potential. Benign intracranial hypertension (BIH) or pseudotumor cerebri secondary to SLE is also known to show abnormality in the diffuse white matter on CT and MRI, and is thought to be caused by the decrease of CSF absorption [9]. Because BIH is de®ned as elevated CSF pressure without spaceoccupying lesion or cerebrovascular disease, our patient was not diagnosed as having BIH. It is noteworthy that the patient under study showed full clinical recovery after steroid therapy, despite the child's severe clinical condition, marked suppression of EEG, and
extensive cerebral lesions. Other reports of acute SLE leukoencephalopathy also indicated a relatively good prognosis. Taken together, acute SLE leukoencephalopathy may be recognized as a relatively benign subtype of CNS lupus.
References [1] Steinlin MI, Blaser SI, Gilday DL, Eddy AA, Logan WJ, Laxer RM, et al. Neurologic manifestations of pediatric systemic lupus erythematosus. Pediatr. Neurol. 1995;13:191±197. [2] Reiff A, Miller J, Shaham B, Bernstein B, Szer IS. Childhood central nervous system lupus; longitudinal assessment using single photon emission computed tomography. J. Rheumatol. 1997;24:2461±2465. [3] Boulter M, Brink A, Mathias C, Peart S, Stevens J, Stewart G, et al. Unusual cranial and abdominal computed tomographic (CT) scan appearances in a case of systemic lupus erythematosus (SLE). Ann. Rheum. Dis. 1987;46:162±165. [4] Shintani S, Ono K, Hiroshita H, Shiigai T, Tsuruoka S. Unusual neuroradiological ®ndings on systemic lupus erythematosus. Eur. Neurol. 1993;33:13±16. [5] Isshi K, Hirohata S, Hashimoto T, Miyashita H. Systemic lupus erythematosus presenting with diffuse low density lesions in the cerebral white matter on computed axial tomography scans: its implication in the pathogenesis of diffuse central nervous system lupus. J. Rheumatol. 1994;21:1758±1762. [6] Kondoh H, Inuzuka T, Miyatake T, Satoh M, Arakawa M. Transient multiple low density area demonstrated by CT in systemic lupus erythematosus with central nervous system involvement (in Japanese). Shinkei Naika (Tokyo) 1987;27:357±360. [7] Johnson RT, Richardson EP. The neurological manifestations of systemic lupus erythematosus: a clinico-pathological study of 24 cases and review of the literature. Medicine 1968;47:337±369. [8] Van Dam AP. Diagnosis and pathogenesis of CNS lupus. Rheumatol. Int. 1991;11:1±11. [9] Ogura N, Atsumi T, Sagawa A, Jodo S, Amasaki Y, Nakabayashi T, et al. Systemic lupus erythematosus associated with benign intracranial hypertension: a case report (in Japanese). Ryumachi (Tokyo) 1992;32:66±72.