|Year : 2006 | Volume
| Issue : 4 | Page : 236-239
Magnetic resonance imaging features of neuropsychiatric lupus
Suyash Mohan, Ashish Verma, Sunil Kumar
Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareily Road, Lucknow - 226 014, UP, India
Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences/Raebareily Road, Lucknow - 226 014, UP
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Central nervous system (CNS) involvement in patients of systemic lupus erythematosus (SLE) spans a wide spectrum of neurological and psychiatric features. In the absence of a diagnostic gold standard for neuropsychiatric lupus a range of investigations are employed to support the clinical diagnosis. New imaging techniques are available to assist the diagnosis and monitoring of the disease course. MRI findings correlate well with CNS manifestations in SLE and when there is a high suspicion of CNS involvement, MRI may be useful in predicting CNS manifestations.
Keywords: Neuropsychiatric lupus, magnetic resonance imaging, systemic lupus erythematosus, central nervous system involvement
|How to cite this article:|
Mohan S, Verma A, Kumar S. Magnetic resonance imaging features of neuropsychiatric lupus. Ann Indian Acad Neurol 2006;9:236-9
A 29-year-old female with normal psychomotor development presented with severe headache and two episodes of convulsions on the preceding two days. Following seizures, she had loss of consciousness for about two to three minutes. She was also running low to moderate grade fever and was passing reddish urine for the past two days, with no history of dysuria. She was diagnosed as a case of systemic lupus erythematosus (SLE) one week ago on the basis of clinical findings and immunological investigations. Her laboratory results showed low hemoglobin (6 g/dl), lymphocytosis, eosinophilia and a raised ESR. Renal functions were impaired (serum creatinine of 6 mg/dl and blood urea nitrogen of 62 mg/dl). Urine analysis indicated presence of hemoglobinuria and albuminuria. Renal histopathology and immunofluorescence confirmed the presence of lupus nephritis. Serology for HIV and HBsAg was negative. Magnetic resonance imaging (MRI) of the brain was done using 1.5 T scanner (GE Signa, Milwaukee, Wis, USA) using T1W, T2W, fluid attenuated inversion recovery (FLAIR) and diffusion weighted (DWI) sequences. Multiple confluent patchy hyper intensities of different sizes were seen on T2W [Figure - 1] and FLAIR [Figure - 2]a, b sequences involving bilateral cerebral and cerebellar hemispheres, basal ganglia and brain stem. The hemispheric lesions were predominantly located in the white matter involving both the sub-cortical and deep regions with the involvement of overlying cortex at a few sites. No cerebral atrophy was noted. The lesions were isointense on DWI [Figure - 3], with mildly increased apparent diffusion coefficient (ADC) on ADC maps. No contrast enhancement [Figure - 4] was noted on injection of intravenous Gadoliniun compound (Omniscan, Amersham health AS, Oslo, Norway). 3D Time of flight (TOF) angiography of intracranial vessels was within normal limits [Figure - 5]. On the basis of clinical and radiological findings a diagnosis of neuropsychiatric lupus (NPSLE), was considered.
Nervous system disease in patients of systemic lupus erythematosus (SLE) spans a wide spectrum of neurologic (N) and psychiatric (P) manifestations. These may be attributed to a primary feature of SLE, complications of the disease or its therapy or a coincidental disease process. Involvement of the central nervous system (CNS) is one of the most important complications accounting for significant morbidity. Neuropsychiatric events are seen in 14-75% of patients with SLE with a wide range of clinical features described under the term NPSLE., MRI is an excellent modality to document the presence and course of cerebral damage in SLE and represents a valid way to assess disease progression or reversal in NPSLE. We describe the clinical and MRI features of a young female with SLE. Atypical in the present case were the early and florid CNS involvement, affecting the posterior fossa and brain stem as well.
As with other organ involvement in SLE, nervous system disease may occur at any time in the disease course. Nevertheless, it is of interest that NPSLE frequently presents early, either before or following the diagnosis of lupus. NP events in patients who have active multi-organ disease due to lupus are well recognized and when they do occur, provide support for the notion that lupus is the most likely cause of the NP manifestation. The current American College of Rheumatology (ACR) classification of NPSLE divides it into syndromes relating to central (aseptic meningitis, cerebrovascular disease, demyelinating syndrome, headache, movement disorder myelopathy, seizure disorder, acute confusion state, anxiety disorder, cognitive dysfunction, mood disorder and psychosis) and peripheral systems (Guillain-Barrι syndrome, autonomic neuropathy, mononeuropathy, myasthenia gravis, cranial neuropathy, plexopathy and polyneuropathy). The presenting complaint of our patient was seizure. Generalized and focal seizures are reported in 6-51% of patients and may occur in the setting of active generalized multi-system lupus or as an isolated neurologic event. The presence of anti phospholipids antibodies in patients of SLE having NP events is associated with microangiopathy, arterial thrombosis and subsequent cerebral infarction. In the present patient, however, no intracranial vascular abnormality was seen on 3D TOF MRA, also there was no restriction of diffusion. These findings suggest that the hyper intensities seen on T2W and FLAIR sequences were due to interstitial edema and not because of cellular swelling. Headache was another prominent complaint in this case. The association between SLE and headache is controversial with a reported prevalence of 24-72 %. Although headache may be a component of active SLE, particularly in patients who have active systemic disease, it is more likely that most headaches in SLE patients are unrelated to SLE.
When considering neuroimaging in NPSLE it is helpful to incorporate an assessment of brain structure and function. MRI is the preferred neuroimaging technique for detection of structural CNS abnormalities. Most objective functional neuroimaging study is positron emission tomography scanning but practical considerations limit its applicability. Abnormalities on MRI may be found in 19-70% of SLE patients. T2 weighted images (T2WI) identify edema and are more sensitive than T1 weighted images in patients of NPSLE. Lesions on FLAIR are glaring and less likely to be confused with non-lesional structures and have been reported to increase the diagnostic sensitivity by about 5% as compared to T2W images. No objective criterion has been mentioned to definitively establish the cause and effect relationship between the symptomatology and imaging. Magnetic resonance spectroscopy (MRS) and magnetization transfer imaging (MTI), however, hold promise in detection and quantification of brain damage in patients of NPSLE. MRS enables the detection of reduction in N-acetyl (NA) compounds. On MTI, the magnetization ratio is lower than normal healthy controls.(10) Both these sequences detect and quantify brain injury not apparent on other sequences. There sequences however could not be done in our case due to patient related factors. The chief imaging feature described in cases of NPSLE is the presence of nonspecific peri-ventricular and sub-cortical white matter hyper intensities usually in the major arterial territories. Such lesions are more commonly attributed to hypertension, disease duration and age related small vessel disease. Our patient was a non- hypertensive young female in whom the diagnosis of SLE was made only one week ago. The presence of lesions in posterior fossa and brain stem were atypical in this case. Multiple sclerosis was a close imaging differential diagnosis but was ruled out, as the lesions were T1 isointense, involving both the gray and white matter with sparing of corpus callosum. Infective pathology was ruled out by absence of any contrast enhancement. Correlating with the presence of both focal (seizure) and diffuse (headache) NP syndromes, both gray and white matter lesions, respectively, were seen on MRI. A combination of focal and diffuse manifestation of disease, both in the symptomatology and imaging is unique to this case. Also unusual is the presence of lesions in brain stem and cerebellum.
MRI is a useful tool not only in the primary diagnosis of NPSLE but also in long term follow up of these patients. It is a useful adjunct in the monitoring of the steroid therapy and contributes significantly in the better management of NPSLE.
The presence of florid CNS lesions on MRI so early in the disease course, with relative paucity of clinical manifestations, was unique in the present case. Also notable was the presence of both focal and diffuse NP syndromes and imaging features in a single patient. To the best of our knowledge involvement of cerebellum and brain stem has not been described previously.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]