A 41-year-old woman visited the emergency room at our hospital, complaining of muscle weakness in the limbs, hypoesthesia in the lower extremity, and abdominal distention caused by acute urinary retention. At the age of 26, the patient had a renal biopsy due to generalized edema, multiple arthralgia and anemia, and was diagnosed with type-IV lupus nephritis. Since then she had been followed up with medication for fifteen years. The patient was catheterized for acute urinary retention soon after visiting the emergency room, where she passed 2,300 mL of urine. She was then hospitalized in the Department of Neurology for assessment and treatments. High signal intensity was observed around the 5th and 6th cervical spinal cord (
Fig. 1A) on T2-weighted spinal magnetic resonance imaging (MRI), performed on the first day of hospitalization; and one month later, they were extended from the 3rd to 6th cervical spinal cord (
Fig. 1B), which manifested cervical myelopathy. In addition high signal intensity on diffusion weighted brain MRI (
Fig. 2A) and low signal intensity on the apparent diffusion coefficient map (
Fig. 2B) were observed in the left internal capsule, which manifested acute cerebral infarction. On fluid attenuated inversion recovery imaging, high signal intensity was observed around the medulla oblongata and bilateral cerebellum (
Fig. 2C), which manifested multiple cerebral vasculitis. Abnormalities were not observed on the cerebrospinal fluid examination, and also the oligoclonal band was not observed. The immunological test showed an increase in double-stranded DNA (dsDNA) antibodies (152 IU/mL; reference value, 0-7 IU/mL) and an increase in lupus anticoagulant (one of antiphospholipid antibodies; 71.8 seconds; reference value, 31-44 seconds); and the anticardiolipin antibody test was negative. The manual muscle test (MMT) was conducted on the basis of the Medical Research Council (MRC) grading, whereof results were as follows. Voluntary muscle contraction was not observed in the right upper and lower extremities. In the left upper extremity, muscle strength was graded 2/5 at the level from the 5th cervical spinal cord to the 1st thoracic spinal cord. In the left lower extremity, it was graded 1/5 at the level from the 2nd lumbar spinal cord to the 1st sacral spinal cord. With respect to hypoesthesia, electromyography was performed in its early stage. On the sensory nerve conduction study, it showed low amplitudes in the right median and ulnar nerves stimulation and no response in the bilateral sural nerves stimulation (
Table 1). On the motor nerve conduction study, it showed low amplitudes in the right median and ulnar nerves stimulation and no response in the bilateral tibial and peroneal nerves stimulation. With respect to the right median and ulnar nerves, F-waves were within the normal range, which manifested peripheral neuropathy (
Table 2). Such findings show a case of NPSLE defined by the American College of Rheumatology; specifically, a concurrence of myelopathy, cerebrovascular disease and peripheral neuropathy [
2]. After being treated with steroid pulse therapy, prednisolone, immunosuppressants, and plasma exchange for five months, the patient was transferred to the Department of Neurology at the hospital. Following two months of medication, seven months after the occurrence of symptoms, she was retransferred to the Department of Rehabilitation Medicine. On the physical examination, conducted on the first day of transfer, was shown mild dysarthria, declines in gag and cough reflex and left-sided tongue deviation. In the right upper and lower extremities, deep tendon reflex was hyperreflexive, but spasticity was not observed. Both sides tested positive on the Babinski and Hoffman test, but ankle clonus was not observed. Light touch and pinprick sensation was impaired below the level of the 4th cervical spinal cord, on both sides. Also hypoesthesia occurred around the anus. Voluntary anal contraction was possible, but anal sphincter showed hypotonous. The bulbocavernosus reflex was absent. MMT was conducted again seven months after the onset of disease. In the right part, muscular strengths were graded: 4/5 from the 5th cervical spinal cord to the 7th one; 3/5 from the 8th cervical spinal cord to the 1st thoracic spinal cord; 1/5 from the 2nd lumbar spinal cord to the 5th one; and 2/5 at the 1st sacral spinal cord, respectively. In the left part, they were graded: 3/5 at the level from the 5th cervical spinal cord to the 1st thoracic spinal cord; 2/5 from the 2nd lumbar spinal cord to the 3rd one; 1/5 from the 4th lumbar spinal cord to the 5th one; and 2/5 at the 1st sacral spinal cord, respectively. Altogether muscle weakness was manifested in the four extremities. The patient could roll side to side by herself, but had a poor sense of dynamic sitting balance. Also the patient was able to urinate by herself with residual urine of 100 to 200 mL, but had poor control of sphincter. Independent defecation was observed. The Korean-version Modified Barthel Index, evaluated seven months and nine months after the onset of disease, increased from 15 to 27, out of 100. The spinal cord independence measure increased from 15 to 32, out of 100. At nine months after the onset of disease, neurologic changes were not observed except for the spasticity being graded 1 on the four extremities (the Modified Ashworth Scale) and bulbocavernosus reflex being present.