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Steroid-Responsive Limbic Encephalitis Yasuhiro WATANABE*' **, Yasutaka Sfflmlzu*, Shinji Ooi***, Keiko TANAKA****, Takashi INUZUKA***** and Kenji NAKASHIMA**

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Steroid-Responsive Limbic Encephalitis Yasuhiro WATANABE*' **, Yasutaka Sfflmlzu*, Shinji Ooi***, Keiko TANAKA****, Takashi INUZUKA***** and Kenji NAKASHIMA** CASE REPORT Steroid-Responsive Limbic Encephalitis Yasuhiro WATANABE*' **, Yasutaka SfflMlZU*, Shinji Ooi***, Keiko TANAKA****, Takashi INUZUKA***** and Kenji NAKASHIMA** Abstract pre-senile dementia that has been successfully treated with steroids, i.e. so called steroid-responsive dementia (1) or A 71-year-old man presented with gradually progress- steroid-sensitive dementia (2). ing cognitive decline following acute febrile exanthe- Weencountered such a case with cognitive impairment, matous disorder. The MRIshowed an abnormality in the one whoresponded excellently to both intravenous and oral bilateral limbic systems. An elevation of cerebrospinal steroid administrations, and whofurthermore showedlimbic fluid (GSF) protein with lymphocyte pleocytosis was abnormalities on MRLCerebrospinal fluid (CSF) analyses noted. Immunoblot of the CSFrevealed the presence of revealed the presence of autoantibodies that selectively rec- anti-white matter antibodies that mainly recognized ognized central nervous system (CNS) components, espe- astrocytes. Intravenous steroid followed by oral steroid cially astrocytes. Weconsidered that the patient had limbic reduced the symptomsto a remarkable degree. The pa- encephalitis. There was, however, no evidence of any malig- tient has now been successfully sustained with steroid for nant neoplasm. Furthermore, antoantibodies found in this more than two years. Weconsidered that this case is clas- case differed from the ones found in the patients with sified as non-paraneoplastic limbic encephalitis, and ac- paraneoplastic limbic encephalitis (PLE) (3). This case quired autoimmunity played a major role in the should be meaningful regarding encephalitis associated with pathogenesis of this case. limbic abnormalities, steroid sensitivity and autoantibodies (Internal Medicine 42: 428-432, 2003) in limbic encephalitis. Wetherefore report the details of this case, as well as a literature review of similar cases. Key words: encephalitis, dementia, MRI, limbic system, white matter, autoimmunity Case Report A 71-year-old man experienced low-grade fever and fine tremors in both hands at the end of December 1996. One Introduction weekafter the onset, five or six red spotty eruptions were no- ticed which looked as if he had been stuck by a needle in the Amongpatients with encephalitis or encephalopathy of left femoral cutis. The eruptions disappeared after several unknownetiology, there exist those who predominantly days without any treatment. Five days later, he became show cognitive decline with a sub-acute to chronic clinical aware of an itching pain in the bilateral femoral regions, and course and whoshow an excellent response to steroid with- he complained that this was unbearable even when his own out obvious autoimmune disorders or steroid-dependent clothes rubbed against the femoral cutis. The next day, the endocrinopathy. Consideration of the possibility of various same pain spread to the left temporal skin of his scalp. On types of encephalitis/encepalopathy as well as steroid- January 12, he was admitted to another hospital with a 38°C dependent cognitive impairment is easier when these condi- fever, a shaking chill and loss of appetite. The patient had tions occur in a younger population than in older patients. lost 7 kg in weight since the onset. Several days after admis- This is because cognitive impairment in elderly patients sion, he could not remember how, why and with whomhe might be diagnosed as being merely senile or presenile de- had been admitted to the hospital. The unbearable pain in the mentia. Actually, there have been several reports of senile or head and femoral cutis disappeared after use of a non- From Department of ^Neurology and **internal Medicine, Matsue Red Cross Hospital, Matsue, **Division of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, ****Department of Neurology, Brain Research Institute, Niigata University, Niigata and *****Department of Geriatrics and Neurology, School of Medicine, Gifu University, Gifu Received for publication March 29, 2002; Accepted for publication February 13, 2003 Reprint requests should be addressed to Dr. Yasuhiro Watanabe, Division of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 36-1 Nishimachi, Yonago 683-8504 428 Internal Medicine Vol. 42, No. 5 (May 2003) Steroid-Responsive Limbic Encephalitis steroidal anti-inflammatory agent. He was discharged from disappeared. This fact might indicate that intracranial hyper- the hospital on January 26, although a minor fever still per- tension existed for a certain period of time. HDS-Rincreased sisted. OnFebruary 14, about six weeksafter onset of the to 19 on May 21. MRI was followed up on May 29, 1997 initial symptoms, he was admitted on foot with his family's (Fig. IE and F). Abnormal signal intensities in the limbic support to our hospital' s department of internal medicine. On systems showed improvement, while cerebral atrophy and admission, he displayed no abnormality on physical exami- white matter hyperintensity along the lateral ventricles be- nation except for oral moniliasis. Routine admission studies, came obvious. He was discharged on June 1, 1997. On out- including urinalysis, hemogram, blood chemistries, erythro- patient examination several months later, a markedimprove- cyte sedimentation rate, chest X-ray and electrocardiogram, ment was noted in both HDS-R (28 point) and CSF analysis were all normal and the blood examination at this time was (protein: 60 mg/dl, lymphocytes: 5/jul and PMNcells: O/jul). the first since the onset. Physical and serological examina- The prednisolone doses were gradually decreased and sus- tion ruled out collagen diseases, infectious endocarditis, pended altogether from late October 1997. The memorydefi- sarcoidosis, Behc,et disease and tuberculosis. During hospi- cits subsequently reappeared, however. HDS-Rdecreased to talization his memoryloss and disorientation were markedly 10 points. CSF analysis showed protein 91 mg/dl, lympho- worsened. He often mistook his hospital room and could not cytes 18/jul, and PMNcells O/jul. Prednisolone 10 mg was rememberthe names of inquirers. After further consultancy consequently restarted on December 1, 1997. Subsequently he was transferred to the neurology division. HDS-R recovered to 22 points on December 22 and 24 At the first neurological examination, the patient's con- points on May 31 1998. The patient now visits our hospital sciousness was alert but severe disorientation was noticed. once a monthand shows no neurological deficits with a daily He could not say what day of the week it was, nor tell us his 7.5 mg intake of prednisolone and the patient and his family ownage or birthday. Revised Hasegawa's dementia scale have no complaints. His wife says that previously he was (HDS-R) indicated 10 (full score is 30 and anything under 20 very shy and reticent before the onset of the disease but that is suspected as dementia). Neither aphasia, apraxia, nor subsequently he has become a talkative and jolly person. We agnosia were present. Cranial nerves were intact except for consider that the patient's mental alteration is a consequence the bilateral papilloedema. There was a mild degree of of the disease rather than any adverse effect of steroid ther- Barre's sign in his right arm but no laterality in his deep ten- apy. Wecould not rule out the possibility that this patient don reflexes. Planter responses were flexor. Mild rigidity was suffering from PLE, because paraneoplastic symptoms was noticed bilaterally in his arms. There was no abnormal- sometimesprecede the appearance of any evident neoplasm ity in the sensory or cerebellar systems. An electroencepha- (3). Using immunoblotting we subsequently analyzed the lograph (EEG) showed slow, diffuse activity with paroxys- CSFderived from the patient to confirm whether or not there mal slow wave bursts. Somatosensory evoked potentials, were antibodies that recognized the CNScomponents as in a brainstem auditory evoked potentials and visually evoked previous report (4) (Fig. 2A). As a consequence we found potentials indicated no abnormalities. Lyme disease anti- antibodies that mainly react with humanwhite matter extract body, Tsutsugamushi disease antibody and Weil-Felix reac- at 38 to 42 kDa fractions. There were several indistinct bands tion were all negative. CSFanalyses revealed initial pressure in the white matter at 38 to 50 kDa and in the gray matter at 80 mmH20, total protein 92 mg/dl, sugar 57 mg/dl, lympho- 42 kDa. These antibodies did not react with non-CNS tissues cytes 33/jul, polymorph nuclear (PMN) cells 6/jul, and red orcells. blood cells O/jul. Viral antibodies in CSF (HTLV-1, HIV, Immunohistological procedures (4) involving staining ac- eccho, coxsackie, mumps, measles, rubella, varicella zoster, cording to the avidin-biotin peroxydase complex (ABC) herpes simplex, Epstein-Barr, Japanese encephalitis, and method showedthat the autoantibodies reacted with a cyto- cytomegalo virus) were all within normal limits. Myelin plasm of astrocytes in an acetone-fixed specimen derived basic protein was 4.9 ng/ml (normal<4.0 ng/ml). Anti- from frozen rat cerebrum (subpial, cortico-medullary junc- neuronal-cell antibodies seen in paraneoplastic syndrome, in- tion and other whole cerebral regions) (Fig. 2B). These re- cluding anti-Hu antibodies were absent in the CSF. Cranial sults seemedto correspond to the white matter abnormalities MRI revealed bilateral limbic system
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