Steroid-Responsive Encephalitis Lethargica Syndrome with Malignant Catatonia

□ CASE REPORT □ Steroid-Responsive Encephalitis Lethargica Syndrome with Malignant Catatonia Yoichi Ono 1, Yasuhiro Manabe 1, Yoshiyuki Hamakawa 1, Nobuhiko Omori 1 and Koji Abe 2 Abstract We report a 47-year-old man who is considered to have sporadic encephalitis lethargica (EL). He presented with hyperpyrexia, lethargy, akinetic mutism, and posture of decorticate rigidity following coma and respiratory failure. Intravenous methylprednisolone pulse therapy improved his condition rapidly and remarkably. Electroencephalography (EEG) showed severe diffuse slow waves of bilateral frontal dominancy, and paralleled the clinical course. Our patient fulfilled the diagnostic criteria for malignant catatonia, so we diagnosed secondary malignant catatonia due to EL syndrome. The effect of corticosteroid treatment remains controversial in encephalitis; however, some EL syndrome patients exhibit an excellent response to corticosteroid treatment. Therefore, EL syndrome may be secondary to autoimmunity against deep grey matter. It is important to distinguish secondary catatonia due to general medical conditions from psychiatric catatonia and to choose a treatment suitable for the medical condition. Key words: encephalitis lethargica, catatonia, steroid therapy (DOI: 10.2169/internalmedicine.46.6179) Introduction Case Report Encephalitis lethargica (EL), named by von Economo, is A 47-year-old man, who had a past history of delusional severe encephalitis which appeared in epidemic form in disorder for about 2 years, developed a pyrexia, vomiting Europe and elsewhere towards the end of World War I and and diarrhea. He was brought to our hospital because his lasted into the 1920’s (1, 2). Since then, occasional cases condition had deteriorated. On admission, his examination that resembled EL syndrome have been described in the showed a body temperature of 39℃ and systolic blood pres- medical literature. Case descriptions reported during this sure of 70 mmHg. Other physical findings, including heart time have striking similarities to the modern definitions of rate, were normal. His eyes were open, and he had a fixed catatonia (3). Catatonia, a nonspecific neuropsychiatric syn- stare without other focal findings of neurological examina- drome is characterized by motor, affective and behavioral tion. On the night of admission his level of consciousness symptoms (4). It is found in not only psychiatric but also deteriorated. He responded to voices, but he was mute and general medical conditions (5). unable to follow verbal commands. He continued to lie in We describe a case of EL syndrome with malignant cata- bed, refused to eat and drink, and urinated in bed. Down- tonia which showed definite improvement after intravenous ward gaze restriction and impaired swallowing were ob- corticosteroid administration. Electroencephalography EEG served. He had parkinsonism including decorticate rigidity showed diffuse slow waves of bilateral frontal dominancy in all four limbs, more marked in the arms, and severe aki- and paralleled the course of the disease. nesia. Tendon reflexes were normal without pathological reflexes. Meningeal signs were difficult to evaluate due to nuchal rigidity. His psychiatric symptoms were mutism and negativism. Neuropsychiatric symptoms including staring, １ Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama and ２ Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, Okayama Received for publication August 11, 2006; Accepted for publication October 24, 2006 Correspondence to Dr. Yasuhiro Manabe, [email protected] 307 DOI: 10.2169/internalmedicine.46.6179 Figure 1. Axial fluid attenuated inversion recovering (FLAIR) image shown as normal. mutism, decorticate rigidity posture and akinesia suggested mia, parkinsonism, opthalmoplegia and respiratory failure, manifestations of catatonia. Laboratory tests showed a white we made a diagnosis of EL syndrome with catatonic symp- cell count (WBC) of 11,000/μl, C-reactive protein of 13.78 toms, and initiated the administration of intravenous methyl- mg/dl and creatine phosphokinase (CK) of 1,363 IU/l. The prednisolone (mPSL) pulse therapy (1,000 mg/day for 3 blood urea nitrogen (BUN) level of 36 mg/dl and creatine days). His level of consciousness and respiratory failure im- level of 4.77 mg/dl suggested renal dysfunction. Viral sero- proved dramatically, and he became almost alert within 24 logic tests in blood were negative including herpes simplex hours, accompanied by weaning from ventilatory support on virus, cytomegalovirus, influenza virus and Japanese en- the 2nd day of mPSL pulse therapy. On the 3rd day, his cephalitis virus. Blood tests for vasculitis, immunological level of consciousness and respiration normalized with ame- and metabolic disorders were all negative. Serum IgM and lioration of hyperthermia and catatonia (Fig. 3). The follow- IgG antibodies to GM2, GM1, GD1a, GalNAc-GD1a, ing EEG paralleled the clinical course, revealing the worst GD1b, GT1b and GQ1b were all negative. Cerebrospinal findings of diffuse slow waves on day 7 and gradually dem- fluid (CSF) examination showed a normal cell count and onstrated background activity of α waves as the clinical CSF protein (50 mg/dl) was slightly elevated (normal: <45 condition improved (Fig. 2B). Oral corticosteroid treatment mg/dl). Oligoclonal bands were not detected. CSF cultures was not substituted for one course of intravenous corti- were negative. Urinalysis, chest X-ray, and electrocardio- costeroid treatment. He was discharged without any motor, gram were all normal. Two cranial magnetic resonance affective or behavioral problems. imaging (MRI) scans performed during the first week of admission were normal (Fig. 1). Electroencephalography EEG Discussion recorded on day 2 showed diffuse slow waves of bilateral frontal dominancy without epileptiform discharge (Fig. 2A). EL is a rare central nervous system (CNS) disorder pre- We concluded that his condition was catatonia rather than senting with pharyngitis followed by sleep disorder, basal neuroleptic malignant syndrome (NMS) because his family ganglia signs (particularly parkinsonism) and neuropsychiat- said that he had never taken anti-psychotic drugs for more ric sequelae (6). The EL epidemic occurred during the same than one month. On day 2, his level of consciousness fell period as the influenza pandemic, therefore, in the medical into lethargy and he had difficulty cleariing his airway. In- literature the two outbreaks have been suspected to be travenous liquids and antibiotics resolved hypotension, diar- linked in (6); however, a recent examination of archived EL rhea and laboratory results including CK and renal function. syndrome brain material failed to demonstrate influenza With persistent pyrexia up to 39℃, catatonia and respiratory RNA (6). There have been no further EL epidemics since failure worsened, and his level of consciousness was altered the 1920’s, although sporadic cases have continued to be re- from lethargy to coma. Repeat CSF examinations did not ported (1, 3, 6-11). Dale et al proposed that this recent phe- show elevated protein levels or an oligoclonal band. On day notype, which they called EL syndrome, could be a postin- 7, arterial blood gas analysis under 5 l/min O2 inhalation fectious autoimmune CNS disorder (6). 3- showed pH 7.33, PaO2 155 Torr, PaCO2 62 Torr and HCO Howard and Lees (7) proposed seven major diagnostic 32.6 mmol/l. Because of respiratory insufficiency and hyper- criteria for EL, defining it as an acute or subacute encepha- capnia, endotracheal intubation and respiratory support were litic illness with at least three of the following criteria: 1) required. Based on the clinical features including hyperther- signs of basal ganglia involvement; 2) oculogyric crises; 3) 308 DOI: 10.2169/internalmedicine.46.6179 Figure 3. Clinical course. CK, creatine phosphokinase; mPSL, methylprednisolone. ported successful treatment with intravenous mPSL pulse therapy of EL syndrome patients with diffuse slow activity of the EEG (10), but the subsequent EEG changes were not described. To our knowledge, no EL syndrome patient has been reported in which EEG was useful in evaluating the effect of corticosteroid treatment. The present patient responded rapidly and dramatically to corticosteroid pulse therapy (administered on day 7 after onset). The EEG of our patient showed diffuse slow waves of bilateral frontal dominancy, and paralleled the clinical course. The successful effect of corticosteroid treatment for EL syndrome was reported in only one previous report (10); however, the effect of corticosteroid treatment remains controversial. Raghav et al reported that 2 of 3 EL syndrome patients treated with in- Figure 2. Electroencephalography (EEG) findings on days travenous mPSL therapy showed no effect, but the other pa- 7 and 64. A, The patient was the worst on day 7. His level of tient improved gradually (8). One reason that the present EL consciousness was coma with high-grade pyrexia, catatonia syndrome patient responded to corticosteroid treatment more and respiratory failure. EEG revealed diffuse 8 waves of bi- rapidly and more dramatically than the previous steroid- lateral frontal dominancy. B, On day 64, after a course of cor- responsive EL syndrome patients may be the administration ticosteroid pulse therapy, the patient improved rapidly and of corticosteroid treatment at an earlier stage of EL syn- dramatically. He was discharged

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